Can supl tU eBCra - thalassaemia.org.cy · taLasesmIubNþalmkBI {eGm:UkøÚb‘ÍnxusFmμta} b¤ eGm:UkøÚb‘ÍnEdlmanrcnasm

esovePAenH epþateTAelItMrUvkarrbs;GñkCMgWtaLasesmIu. ral;kic©xitxMBiBN’naGMBICMgW nig

lkçN³rbs;va RtUv)aneFVItamrebobgay² ehIy)anbBa©úÚlbEnßmnUvkareCOnelOnEpñkKøInik nig

viTüasaRsþfμI² Edl)anrkeXIjkñúgb:unμanqñaMcugeRkayenH kñúgkarBüa)al nig

karBarCMgWtaLasesmIu. karpþl;nUvBt’manfμI² nig RtwmRtUvdl;GñkCMgWtaLasesmIuCaTUeTA

kñúgsklelak CakmμvtßúmYykñúgcMeNamkmμvtßúcMbg² nig CaehtuplnaMeGaybegáItCa

shB½n§GnþrCatiCMgWtaLasesmIu enHeLIg.

esovePAenHRtUv)anERbsMrYlCaexmrPasa nig e)aHBum<pSayeday

mnÞIreBTükumarCati

100/ mhavifI shB½n§rusSIú/ xN½ÐTYleKak/ PñMeBj/ km<úCa

GnuBaØatisiT§ibkERbBIÉksaredIm nig pþl;mUlniFieday

shB½n§GnþrCatiCMgWtaLasesmIu ¬s>G>t¦

Thalassaemia International Federation (TIF)

P.O. Box 28807, 2083 Nicosia, 31 Ifigenias Str., 2007 Nicosia, Cyprus

Website: http://www.thalassaemia.org.cy

ERbsMrYlCaexmrPasa

evC¢bNÐit Can supl

RKUeBTüÉkeTseraKkumar nig elahitsaRsþ

mnÞIeBTükumarCati PñMeBj

evC¢bNÐit tU eBCra

naykrgTTYlbnÞúkbec©keTs

mnÞIreBTübEg¥kkMBg;RtEbk extþéRBEvg

ISBN : 9963-623-40-9 ERbsMrYlCaexmrPasa nig e)aHBum<enAPñMeBj km<úCa qñaM 2008

Cover text.qxp 1/12/2008 9:48 AM Page 1

shB½n§GnþrCatiCMgWtaLasesmIu ¬s>G>t¦ sUmEføgGMNrKuNy:ag

RCaleRCAcMeBaHkarpþl;viPaKTand¾sb,úrsBIshB½n§ Cooley’s anemia

énshrdæGaemrik sMrab;eKalbMNgénkare)aHBum<pSayenH.


ISBN: 9963-623-40-9

© 2003 Team up Creations Ltd

14 Othonos str, 1016 Nicosia - Cyprus

rkSasiT§iRKb;y:ag

KμanEpñkNamYyénesovePAenHGacRtUvplitCafμI rkSaTukkñúgRbB½n§Gacrk)an b¤ bBa¢ÚneTAkñúgTMrg;NamYyb¤ edaybMNgNamYy dUcCaeGLicRtUnik emkanik ftcMlg mIRkUhVIlm_ b¤tamEbbepSg²edayKμankarGnuBaØat Calayl½kçN_GkSrBIshB½n§GnþrCatiCMgWtaLasesmIu nigGñkniBn§.

e)aHBum<enAnIkUsSa/ sIuRBUs (Printed in Nicosia, Cyprus)

i

GMBIGñkniBn§

evC¢> Gg;RDULa eGElhVehrIuyU (Dr. Androulla Eleftheriou) )anTTYl

sBaØab½Rt]tþmsikSa nig eRkay]tþmsikSaBIsaklviTüal½yLúgdugkñúgvis½y

GtisuxumCIvsaRsþ (Microbiology) nig visaNUsaRsþ (Virology). elakRsI

)anTTYlrgVan;CaGaharUbkrN_mYycMnYn BIGgÁkarsuxPaBBiPBelak nigBI

KN³kmμkarhVÚlR)ay (Fulbright Commission)/ ehIyelakRsI)ankan;

tYnaTICaGñkRsavRCavkitþiys enAsaklviTüal½yevC©saRsþ cRkPBGgÁeKøs.

GaharUbkrN_fñak;bNiÐtrbs;elakRsIRtUv)anbMeBjenAmCÄmNÐlRtYtRta

CMgW (Centres of Disease Control) naTIRkugGatøg;ta shrdæGaemrik.

evC¢> eGElhVehrIuyU )ankan;tYnaTICaRbFanmCÄmNÐl visaNUbEg¥k

(Virus Reference Centre) énRksYgsuxaPi)al RbeTssIuRBUs (Cyprus)

cab;taMgBIqñaM 1990 ehIyCaGñkrYmcMENkcMbg kñúgkarbegáItmCÄmNÐlenH

pgEdr. elakRsI)anerobcM nig)ancUlrYmy:agskmμkñúgsikçasala snñisiT

nigKMeragTaMgfñak;Cati nigGnþrCatiCaeRcIn. bc©úb,nμelakRsIbMerIkarCaTIRbwkSa

GgÁkarsuxPaBBiPBelak elIvis½yCMnajrbs;Kat;.

karcab;GarmμN_sMxan; nigkargarsikSaRsavRCavrbs; evC¢> eGElhVehrIuyU

KWkñúgvis½yénkarbgáeraKedayvisaNU (Viral infections) kñúgCMgWtaLasesmIu

kMritF¶n;. tamry³karsikSaRsavRCavrbs;Kat; elakRsI)anTMnak;TMngCamYy

RKUeBTü nig GñkviTüasaRsþxagkarRsavRCavCaeRcInEdleFVIkarkñúgvis½yenH

RBmTaMgCamYyGñkCMgWtaLasesmIuenAbNþaRbeTsnanakñúgBiPBelak.

cab;BIqñaM 1993 evC¢> eGElhVehrIuyU )aneFIVshkarCaGñksμ½RKcitþCamYy

shB½n§GnþrCatiCMgWtaLasesmIu ¬s>G>t¦.

tamry³kare)aHBum<pSayrbs;xøÜn s>G>t bMeBjkargareFVIeGayR)akdfaral;

Bt’manevC¢saRsþGMBICMgWtaLasesmIugaynwgrk)anedayRsYl.

sarsMxan;CagenH s>G>t pþl;»kasdl;buKÁlikeBTüeGaysikSabnþGMBI

CMgWtaLasesmIutamry³sikçasilaGb;rM.

kñúgqñaM 1997 tMENgfμImYyCaGñksMrbsMrYlviTüasaRsþrbs; s>G>t RtUv)an

begáIteLIgedIm,IemIlxusRtUvkic©karxagelIenH. evC¢> eGElhVehrIuyU

RtUveKEtgtaMgeLIgedayÉkcän© - elakRsI)anTTYlyktYnaTIenHeday

hrT½yrIkrayGs;BIdYgcitþ. cab;taMgBIeBlenaHmk evC¢> eGElhVehrIuyU

)ankan;tYnaTICaGñksMrbsMrYlviTüasaRsþenHedaybMeBjkargarrbs;elakRsI

edayELkBItYnaTIpøÚvkarkñúgrdækar.

tamry³kargarrbs;elakRsICamYy s>G>t/ evC¢> eGElhVehrIuyU

)anGnuvtþKMeragCaeRcIn TaMgkñúgRsuk nig GnþrCati kñúgshRbtibtþikareday

CitsñiT§CamYybNþaRKUeBTü nigsmaKmCMgW taLasesmIunanaBaseBj

BiPBelak. elakRsI)anniBn§ nig e)aHBum<pSayesovePAmYycMnYnkñúgnam

s>G>t RBmTaMgmYycMnYneTot edayshRbtibtþikarCamYyGgÁkarsuxPaB

BiPBelak nig GgÁkardéT²eTot GMBIRbFanbTCaeRcInEpñkviTüasaRsþ ehIy

elakRsIk¾CaniBn§naykTsSnavdþIRbcaMRtImasrbs; s>G>t.

ii

iii

esckþIEføgGMNrKuN

xJúMsUmEføgGMNrKuNcMeBaHkarpþl;viPaKTanBI evC¢> mIxaEGl Gg;háasÞIejaTIs

TIRbwkSaevC¢saRsþrbs; shB½n§GnþrCatiCMgWtaLasesmIu ¬s>G>t¦

Edl)anBinitüesovePAenH ehIy)anpþl;karENnaMCMnaj nig Gnusasn_

RBmTaMgkarTUnμand¾mantMél.

xJúMsUmEføgGMNrKuNcMeBaHkarpþl;viPaKTanBIGñkCMnajkarevC¢saRsþxageRkam

EdlCasmaCik smaCIka énRkumRbwkSaPi)alrbs; s>G>t Edl)anBinitüEpñk

nanatamÉkeTsCMnajrbs;BYkeK ehIy)anpþl;Gnusasn_ RBmTaMgkarTUnμan

d¾mantMél.

saRsþacarü cn B½rT½r evC¢> b:Ul EtlhV½r

GaKarEpñkelahitsaRsþ GñkÉkeTselahitsaRsþ

sklviTüal½yLúgdug mCÄmNÐltaLasesmIu

98 Chenies Mews London WC1E 6HX RksYgsuxaPi)al

nIkUsüa/ sIuRBUs (Nicosia, Cyprus)

evC¢> m:alkUl vkX½r evC¢> nIkUs sá½rDIs

GñkÉkeTsebHdUgsaRsþ RKUeBTükumar-GñkÉkeTsRkeBj

viTüasßan hatF½r GaKarEpñkeraKkumar

GaKar sIusIl PøImIg mnÞIreBTü m:akarIuyU

mnÞIreBTüsklviTüal½yLúgdug RksYgsuxaPi)al

Grafton Way, London WC1E 6AU, UK nIkUsüa/ sIuRBUs (Nicosia Cyprus)

saRsþacarü nIka kaBEBllInI

GUes<dal m:akC½r b:UlIKøInIkU/ )a:DIKøIGUn RkaenlI

vIy:a sV½rsa 35/ mILaNU 20122 GIutalI (Milano 20122 Italy)

CMnYy]btßmÖd¾mantMélBI evC¢> s> RKIsÞÚ nigelakRsI G> KarI GñkviTüasaRsþ

mnÞIrBiesaFn_ TaMgBIrnak;CabuKÁlikmCÄmNÐltaLasesmIu enAnIkUsüa

RksYgsuxaPi)alsIuRBUs Edlpþl;søay nigtaragmYycMnYnEdleRbIkñúg

esovePAenH.

CMnYy]btßmÖCMnajd¾mantMélBI evC¢> ehELn EBrI buKÁlik s>G>t

kñúgkarBinitüepÞógpÞat; nig kñúgkarerobcMsTÞanuRkménesovePAenH.

iv

v

kar]TÞis

sUm]TÞisesovePAenH cMeBaHGñkCMgWtaLasesmIuRKb;²Kña enABaseBj

BiPBelak EdlGñkTaMgenaH xJúM)ansÁal; nig)anCYb ehIyxøHeTotxJúMsÁal; Et

minTan;)anCYbenAeLIy RBmTaMgrab;ryBan;nak;epSgeTot EdlkMBugrs;enA

BaseBjBiPBelak EdlxJúMnwgKμan»kas)anCYb. karebþCJarbs;BYkeKedIm,I

rs;enA nigxMBuHBar )anpþl;eGayxJúMnUvcMNg; nigkMlaMgcitþ edIm,IcUlrYmkñúgkar

ts‘URbyuT§nwgCMgWenH. esckþIkøahand¾FMeFg ehIynigkarGt;Fμt;KμanTIbBa©b;

rbs;BYkeK)anbeRgonxJúMfaetIRtUvts‘URbyuT§edaymincuHcaj;EbbNa.

matikaerOg

buBVkfa xii

lixitBIGñkniBn§ xiv

CMBUkTI 1 Qam nig taLasesmIu 1

Qam - {sÞwgCIvit} 1

tYnaTIrbs;Qam 1

FatupSMrbs;Qam 3

QamTaMgmUl 4

RbePTQam ¼RkumQam 7

CMgWQam 9

CMgWtaLasesmIukMritF¶n; 10

CMBUkTI 2 Bn§útMNBUC nig taLasesmIu 11

EhSn (Genes) = Bn§ú 11

PaBminRbRktIkñúgeKalikaQamRkhmEdlnaMeGayekItCMgW

taLasesmIukMritF¶n; 15

eGm:UkøÚb‘Ín nig Edk 15

Bn§úEdlRKb;RKgkarsMeyaKkøÚb‘Ín 17

eraKsrIrsaRsþ rbs;CMgWtaLasesmIu (Pathophysiology of

Thalassemia) 20

kgVHExSEbtta (β - chains) ehIyelIsExSGal;hVa (α - chains) 20

kMNt;eraKvinicä½yCMgWtaLasesmuI 23

viFIsaRsþEpñkelahitviTüa EdleKeRbICaTUeTA

edIm,IeFVIeraKvinicä½ytaLasesmuIkMritF¶n; 24

vi

vii

karBüa)al Ebtta taLasesmuIkMritF¶n; 27

CMBUkTI 3 karBüa)aledaykarbBa©ÚlQam 28

etIeBlNaRtUvcab;epþImBüa)aledaybBa©ÚlQam 29

Fana[)ankarbBa©ÚlQammansuvtßiPaB 30

ehSNUTIbRkumQam (Blood group genotype) 31

etIQamGVIRtUvbBa©Úl 32

kMNt;rbbbBa©ÚlQam 37

vaytMélRbsiT§iPaBénrbbbBa©ÚlQam 39

karBüa)alRbtikmμbBa©ÚlQam 44

CMBUkTI 4 elIsbnÞúkCatiEdk nig karkMcat;CatiEdkecj 49

EdkenAkñúgsarBagÁkayGñkmansuxPaBl¥ 49

EdkkñúgCMgWtaLasesmIu 50

etIkarelIsCatiEdkbMpøajragÁkayya:gdUcemþc 52

Desferrioxamine (DFO) b¤ Desferal 53

etIeKcab;epþImBüa)alkMcat;CatiEdkeBlNa 55

meFüa)ayepSg²eTotkñúgkarcak;bBa©ÚlfñaM DFO 58

vaytMélRbsiT§iPaBénkarBüa)aledayfñaM DFO 61

eRbIkMrit Ferritin edIm,IkMNt;kMriteRbI DFO 62

CatiEdkkñúgTwkenam 65

plvi)akcMeBaHkareRbIfñaM DFO ehIyvabgðajeLIgEbbNa 65

karmanKP’ 71

karsMrbtamcMeBaHkarBüa)aledayfñaM DFO 71

fñaMkMcat;CatiEdk epSg²eTot 72

RbsiT§iPaBrbs;fñaM Deferiprone 72

plrMxanrbs;fñaM L1 73

plrMxanepSg²eTotrbs; Deferiprone 74

kareRbIfñaMkMcat;CatiEdkedaypÁúMcUlKña 76

fñaMfμI Deferasirox CaRbePTRKab;elbtammat; 77

pøÚveRbIR)as;fñaM 78

etI Deferasirox dMeNIrkardUcemþc 79

RbsiT§PaBrbs;fñaM 79

plrMxanrbs;fñaM Deferasirox 79

bMrameRbIR)as; (Contra-indications) 80

karmanépÞeBaH nig kareRbIfñaM Deferasirox 81

CMBUkTI 5 bBaðaevC¢saRsþbNþalmkBItaLasesmuI nig

karBüa)al 82

GeNþIkrIkFM 82

karkat;GeNþIkecj nig karbgáeraK 84

plvi)akcMeBaHebHdUg nig RkeBjbeBa©jkñúg (Endocrine) 86

plvi)akcMeBaHebHdUg 86

tamdanmuxgarebHdUg 88

plvi)akcMeBaHRkeBjbeBa©jkñúg 91

CMBUkTI 6 karbgáeraKkñúgCMgWtaLasesmuI 101

karbgáeraKbNþalmkBIkarbBa©ÚlQam 103

karbgáeraKedayvIrusrlakeføImRbePT B

(Hepatitis B = HBV) 104

rlakeføImRbePT C (Hepatitis C = HCV) 107

vIrusepSg²eTot 112

Pñak;garbgáeraKfμI 114

viii

ix

karRBYy)armμN_fμI cMeBaHPñak;garbgáeraKcas; 114

suvtßiPaBQam 115

suvtßiPaB nig manQameRbIR)as; enAbNþaRbeTskMBug

GPivDÆn_ 117

segçbbBaðaEdlGacekItman cMeBaHGñkCMgwtaLasesmuI

kMritF¶n;Edlmin)anBüa)al 118

CMBUkTI 7 taLasesmIukMritmFüm nigtaLasesmIuepSg²eTot 119

kareFVIeraKvinicä½y 121

karBüa)al taLasesmIukMritmFüm 123

karkMcat;CatiEdk 124

bBaðaEpñkevC¢saRsþkñúgCMgWtaLasesmIukMritmFüm 125

taLasesmIubNþalmkBI {eGm:UkøÚb‘ÍnxusFmμta} b¤

eGm:UkøÚb‘ÍnEdlmanrcnasm<½n§køay 128

lkçN³GUm:UhSIúháÚt rbs;eGm:UkøÚb‘ÍnxusFmμta 128

PaBpÁúMKñaén {eGm:UkøÚb‘ÍnxusFmμta} CamYy Ebtta

taLasesmIu 129

Gal;hVa ¬Alpha¦ taLasesmIu 130

CMBUkTI 8 rbbBüa)alEdl)aneFVI nig CMhan

naeBlGnaKt 131

karbnSaMxYrq¥wg 131

GVIeTACakarbnSaMxYrq¥wg ? 131

etImaneRKaHfñak;GVIxøH ? 132

etIeKGacbnßyeRKaHfñak;[enAticbMput)andUcemþc ? 133

CMhanepSg²eTot cMeBaHkarbnSaMxYrq¥wgEdlmanPaBsIuKña

BitR)akd ¬Cab;sac;Qam¦ 136

esckþIsnñidæan 137

bnSaMxYrq¥wgedayeRbIQamTgsuk 137

CMBUkTI 9 CMhanepSg²eToteq<aHeTArkkarBüa)al 139

»sfCMrujbegáIt eGm:UkøÚb‘ÍnTarkkñúgépÞ (HbF inducers) 139

Büa)aledayBn§ú (Gene therapy) 140

eraKvinicä½yrkPaBsIuKñaén HLA munkarbgákMeNIt 140

CMBUkTI 10 bBaðaecaTEpñkcitþsgÁm 141

cMeBaHGñkCMgW 141

cMeBaH«BukmþayGñkCMgW 141

cMeBaHGñkCMgW nig «Bukmþay 142

TTYlBt’man nig karKaMRT 143

GñkCMgWtaLasesmIukMritF¶n; Cakumar CaekμgCMTg; nig

CamnusSeBjv½y 144

CMBUkTI 11 Bt’bEnßm 147

karrs;ran nig KuNPaBCIvit 147

rbbGahar nig taLasesmIu 149

kILa nig taLasesmIu 149

visSmkal nig taLasesmIu 150

karGaBah¾BiBah¾ nig RKYsar 150

CMBUkTI 12 eGBIedmIsaRsþ nig karkarBarCMgWtaLasesmIu 152

eGBIedmIsaRsþ 152

karkarBar 152

sarsMxan;énkarkarBar 152

x

xi

rebobEdltaLasesmIucMlgCalkçN³tMNBUC 155

GñkpÞúktaLasesmIukMritRsal 156

ehtuGVIGñkcaM)ac;RtUvdwgfa etIxøÜnGñk CaGñkpÞúkCMgW b¤ Gt; 156

eGm:UkøÚb‘ÍnxusFmμta nig CMgWeGm:UkøÚb‘ÍnepSg²eTot 156

Gal;hVa taLasesmIu 158

EsVgyl;faetIrUbGñk CaGñkpÞúk b¤ Gt;eT 160

nrNaxøHKYreFVIetsþQam ? 163

PaBrYmExSelahitCamYyKña 163

CMerIssMrab;KUrRskrRbQmmuxnwgeRKaHfñak; 164

eFVIetsþTarkkñúgépÞrkCMgWtaLasesmIu 164

karbBaÄb;KP’ 167

sTÞanuRkm 169

Bt’mansMxan;² cMNIGahar nig CMgWtaLasesmIu 178

eRbI T2* sMrab;vas;kMritbnÞúkCatiEdkkñúgebHdUg 187

taragvaytMél nig karBüa)al 189

eKhTMB½rsMxan;² 199

Gas½ydæanTMnak;TMngsMxan;² nig Bt’man 201

buBVkfa

shB½n§GnþrCatiCMgWtaLasesmIu ¬s>G>t¦ RtUv)anbegáIteLIgkñúgqñaM 1986

kñúgbMNgelIkkMBs;eKalkarN_ karBar nig karEfTaMBüa)aleGay)anRtwmRtUv

cMeBaHCMgWtaLasesmIu CMuvijBiPBelak.

karbegáIt nig karBRgIksmaKmCatiCMgWtaLasesmIu ehIynigkarGPivDÆn_

elIkmμviFIGb;rMdl;RKUeBTüCMnaj EdlbMeBjkargarkñúgvis½yCMgWtaLasesmuI

edaybNþúHbNþalbnþ KWsßitenAkñúgbNþaeKalbMNgd¾sMxan; Edltamry³kar

enH s>G>t b:gR)afñasMerceKaledATaMgenaH. kñúgbribTenH s>G>t CYyBRgwg

dl;RkumGñkCMgW¼matabitafñak;Cati EdlBYkeKCaGgÁd¾sMxan; dak;sm<aFeTAelI

GaCJaFrsuxaPi)al eGayelIkkMBs;eKalneya)ayCati edIm,IkarBareGayman

RbsiTi§PaB nig karEfTaMBüa)aleGay)anRtwmRtUv cMeBaHCMgWtaLasesmuI.

smaKmCMgWtaLasesmuI eday\tmnÞilpgEdr manParkic©CaKnøwHkñúgkar

eXasna bBa©úHbBa©ÚlTak;TajGarmμN_CasaFarN³ CaEpñkd¾sMxan;mYyén

kmμviFINamYykñúgkarRtUtRtaCMgWtaLasesmIu.

s>G>t )anKUsbBa¢ak;pgEdrBIsarsMxan;énkarbegáItkmμviFIelIkkMBs;Gb;rM

edayerobcMsikçasila snñisiT fñak;Cati tMbn; nig CalkçN³GnþrCati ehIy)an

e)aHBum<pSaynUvÉksarCaRcIn Edl)anbkERbCaeRcInPasa cab;BIBt’man

mUldæansMrab;GñkCMgW matabita nig saFarNCnTUeTA edayRKbdNþb;GMBICMgW

karBüa)al nig karkarBar rhUtdl;GtßbTviTüasaRsþCMnajsMrab;RKUeBTü

EdlCab;Bak;B½n§kñúgkarEfTaMBüa)al CMgWtaLasesmIu.

esovePAenH nwgpþl;viPaKTany:agsMxan;dl;karxMRbwgERbgTaMgLaykñúgkar

pþl;CUnGñkCMgW nig matabita nUvRbPBfμI²d¾man tMél Bt’manBit Edlbgðaj

eLIgkñúglkçN³samBaØ² rMelcedayrUbPaB edayRKbdNþb;RKb;Tid§PaB

xii

xiii

CMgWtaLasesmuI/ epþImBImUlehtu nig FmμCatiénCMgW rhUtdl;karBüa)al

karkarBar TMhMrMCYlcitþ nig bBaðakñúgsgÁm.

s>G>t sUmsMEdgkardwgKuNCaxøaMgcMeBaHGñksMrbsMrYlviTüasaRsþrbs;

shB½n§ evC¢> Gg;RDULa eGElhVehrIuyU cMeBaHkarsMerckargaredayxitxM

y:agGs;BIsmtßPaBkñúgkarsresresovePAenH. CakarBit xJúMmanCMenOeday

mansgÇwmfakic©kary:aghμt;ct;kñúgkareFVIeGay)anfμI²Tan;ehtukarN_enHnwg

CYypþl;eGayGñkCMgW nig matabitanUvkþIsgÇwm nig karKaMRTbnþEdlBYkeK

RtUvkaredIm,IRbQmmux RbyuT§CamYyCMgWtaLasesmIu.

)a:NUs Gg;KøWhSÚs (Panos Englezos)

RbFanshB½n§GnþrCatiCMgWtaLasesmIu (TIF President)

lixitBIGñkniBn§

RbiymitþCaTIrab;Gan/

eBlEdlRbFanRkumRbwkSashB½n§GnþrCatiCMgWtaLasesmIu ¬s>G>t¦ )an

sMNUmBrxJúMeGayBicarNaniBn§esovePAmYyGMBICMgWtaLasesmuI edayepþat

eTAelItMrUvkarrbs;GñkCMgW nig matabita xJúM)ansÞak;esÞIr hak;dUcCakargarmYy

KYreGayrujra. b:unμanqñaMknøgeTAenH mankare)aHBum<pSayCaRcInRbkbeday

eCaKC½y nigmanRbCaRbiyPaB RtUv)ansresredayGñkviTüasaRsþBUEk

l,Il,ajEdlBUEkkñúgvis½y CMgWtaLasesmIu. cab;BIeBlenaHmkPaB

eCOnelOnkñúgRKb;Tid§PaBEdlTak;TineTAnwgkarRtYtRtaCMgWtaLasesmuI

ekItmanCaeRcIn nig RbkbedayGtßn½y.

xJúM)ansMerccitþTTYlykkargard¾lM)ak ehIycab;sresresovePAenHeday

bBa©ÚlnUvPaBeCOnelOnfμI²TaMgenaH b:uEnþRbkbedayehtupl. Cabzm

xJúMCab;citþCaxøaMgCamYy s>G>t ehIyebþCJaCYyelIkkMBs;vtßúbMNgrbs;

s>G>t. elIsBIenHeTot xJúMmanesckþIRsLaj;CaBn;eBkcMeBaHBiPBRKYsar

CMgWtaLasesmIu edaymanGarmμN_faxJMúCaEpñkmYykñúgcMeNamBYkeK.

xJúM)anBüayamy:aglM)akedIm,IbegáItesovePAenH CadMbUgsMrab;GñkCMgW nig

matabita Edlpþl;CUnnUvBt’manBit fμI²Tan;ehtukarN_GMBICMgWtaLasesmuI

karBüa)al nig karkarBar. ral;karxMRbwgERbgRtUv)aneFVIeLIgpgEdredIm,I

bgðajBt’manTaMgenHkñúgPasasamBaØ nig c,as;las;. karenHminEmnsut

EtgayRsYleFVIeLIy CaBiesskñúgCMBUkxøHEdlxJúMxVayxVl;fakarRbIR)as;Pasa

samBaØeBkGacmanplGaRkk;dl;Gtßn½yBit. eTaHy:agNak¾eday sgÇwmfa

sTÞanuRkmEdl)anpþl;CUnenAPaKxagcugénesovePAenH RBmTaMgrUbPaB nig

RkahVikCaeRcIn nwgCYyGñkGaneGay)anyl;TaMgRsugnUvGtþbTNa

xiv

xv

EdlminGacsresrCaPasasamBaØ)an.

GñkGannwgkt;sMKal;fa karBnül;bEnßmRtUv)anpþl;CUncMeBaHCMBUkedayELk.

karsMerccitþpþl;Bt’manlMGit RtUv)aneFVIkñúgPaKxøH² CMBUkRKbdNþb;PaKxøH

énrebobBüa)al EdlxJúMyl;favamansMxan;cMeBaHGñkCMgWsMrab;CIvitrs;enA nig

KuNPaBCIvitrbs;eK. kñúgEpñkxøHeTot dUcCakñúgPaKTak;TgnwgebHdUg

manBakübec©keTseBTüCaeRcInEdlxJúMminGacsMrYlCaPasasamBaØ

)aneRcIneLIy.

RKb;Bt’mankñúgesovePAenH suT§Et)andkRsg;ecjBIr)aykarN_Edl

e)aHBum<pSayCaeRcIn ehIyshB½n§GnþrCatiCMgWtaLasesmIuGacpþl;CUn

GñkGan)an tamkaresñIsMucMeBaHGtßbTNamYyEdlRtUv)anykmksikSakñúg

esovePAenH.

esovePAenH rYmCamYyesovePAelgEhÁm {elg nig eronGMBItaLasesmuI

(Play and learn about thalassemia)} nig esovePArUbkMEbøgmYyeTot

{GMBICMgWtaLasesmuI (About thalassemia)} EdlkMBugEterobcM b:uEnþnwg

ecjpSayqab;²enH nwgCYyGñkCMgWRKb;v½y nig matabitarbs;BYkeKsÁal;c,as;

CMgWtaLasesmIu ehIynigkarBüa)alva.

edaysgÇwmEdrfa kare)aHBum<pSayTaMgenHnwgbMeBjtYnaTIy:agman

GtßRbeyaCn_dl;GñkCMgW nig matabitarbs;BYkeKeGayTMlab;CamYyvaküs½BÞ

evC¢saRsþCaeRcInEdlBYkeKnwgCYbeBlTMnak;TMngCamYybuKÁlikeBTü b¤

GanesovePAviTüasaRsþ nig TsSnavdþInanaGMBICMgWtaLasesmuI.

GñkCMgWrauMér:kñúgbNþaRbeTsEdlmaneKalneya)ayKaMRTCasaFarN³

xvi

ehIymankarRtYtRtataLasesmuI nig CMgWBnæú (Genetic disorders) déTeTot

CaTUeTATTYl)ankarGb;rMl¥GMBICMgWrbs;BYkeK ehIyBYkeKEsVgrkBt’mansþIBI

karvivDÆn_fμI²CaRbcaM. k¾b:uEnþ GñkCMgWkñúgbNþaRbeTskMBugGPivDÆn_vij CaTUeTA

RtUvkarCMnYykñúgkarTTYl)anBt’manfμI²TaMgenaH Bt’manEdlcaM)ac;CYyBYkeK

RbyuT§CamYyCMgWeKpÞal;pg RBmTaMgBRgIkkarRbyuT§CMrujeGayrd§aPi)aldak;

eGaymaneKalneya)aynanasMrab;karBüa)aleGay)anRtwmRtUv.

CaTIbBa©b; esovePAenH)anrYmbBa©ÚlCMBUktUcmYy faetIRtUvkarBarkMeNItTark

BICMgWtaLasesmuIy:agdUcemþc vaCakmμvtßúcMbgrbs;TsSnvis½ysuxPaB

saFarN³ k¾dUcCal½kçx½NÐFanaKuNPaBBüa)alpþl;CUnGñkCMgW Edlman

Rsab;. kMeNItbEnßmnUvkumarmanCMgW nwgkat;bnßyRbPBFnFanEdlman

sMrab;pþl;karEfTaMBüa)aleGay)anRtwmRtUvdl;GñkCMgWEdlmanRsab;.

evC¢> Gg;RDULa eGElhVehrIuyU

1

CMBUkTI 1

Qam nig taLasesmIu

taLasesmIu (Thalassemia) KWCaCMgWmYyRkumEdleFVI[xøÜnmnusSeyIg

minGacplitQammanKuNPaBx<s;[)anbrimaNRKb;RKan;. dUceyIgnwg

)aneXIjenAxageRkamxøÜnmnusSrs;)an KWGaRs½yedaymanQam dUcenH

sMxan;RtUvyl;nUvbBaðaEdlmanplb:HBal;elIQam nig meFüa)ayEdl

xøÜnmnusSeyIgeqøIytb.

Qam - {sÞwgCIvit}

QamCavtßúravRTRTg;CIvit EdldwknaMsarFatuciBa©wmeTAkan; srIragÁ nig

Calika kñúgsarBagÁkay ehIynaMykmkvijnUvkaksMNl;sMrab;beBa©j

ecal. mnusSeBjv½yEdlmansuxPaBl¥ manQamRbmaN 5 - 6 lIRt

KWRbEhl 7 - 8° énTMgn;xøÜnTaMgmUl.

QameFVIdMeNIrBaseBjkñúgxøÜn edaysarebHdUgEdlCasñb;rujqøgkat;

bNþajbMBg;TaMgLayEdleKehAfa srésQam (Blood Vessels).

srésQamman 3 EbbxusKña KWsrésQamRkhm srésQamexμA nig

srésQamtUcl¥it (Capillaries) nImYy²suT§EtmanTMhM nig muxgar

xus²Kña ¬emIlrUb 1a, 1b¦ . srésQamTaMg 3 rYmKñaehAfa

RbB½n§srésQam (Circulatory system) ¬emIlrUb 1a¦ .

tYnaTIrbs;Qam

QambMeBjmuxgarsMxan;²CaeRcIn ³

(i) dwkCBa¢Ún GuksIuEhSn (Oxygen) ³ sarBagÁkayRtUvBwgEp¥kelIQam

edIm,IdwkCBa¢ÚnsarFatumkciBa©wmsMxan;²EdlxøÜnR)aNeyIgRtUvkar

1a red = oxygenated blood

blue = deoxygenated blood

1b

2

ehIyykeTAvijnUvkaksMNl;Bul EdlRtUvbeBa©jecalEdlxøÜnR)aN

eyIgminRtUvkar. ]TahrN_ edIm,ImanCIvitrs; nig bMeBjmuxgar)an

ekasika nig GtisuxumR)aNTaMgGs;RtUvkar GuksIuEhSn - Ca]sμ½nman

kñúgxül;EdleyIgdkdegðIm. Qamcab;ykGuksIuEhSnBI sYt ehIynaM

eTA[EpñkepSg²énsarBagÁkay.

(ii) cab;yk ]sμ½nkabUnik (Carbon dioxide)

Ca]sμ½nmYyepSgBIGuksIuEhSnEdlCakaksMNl;ekItecjBIekasika.

QamnaMkabUnDIGuksIutRtLb;mkbeBa©jecaleTAxageRkAvijenAsYt.

dUcKñaenHEdr Qamk¾RbEm:RbmUlkaksMNl;epSg²eTotdUcCa G‘uyer:

(Urea) nig GasIutG‘uyrik (Uric Acid) eTAbeBa©jecalenA tMrgenam nig

eføIm. cugbBa©b; kaksMNl;TaMgenHbeBa©jBIQameTAkñúgTwkenam nig

lamk ¬emIlrUb 1c¦.

(iii) dwkCBa¢ÚnsarFatuKImIBiess ehAfa G½rm:Un (Hormone) EdlRKb;RKg

muxgarénRbB½n§sarBagÁkaysMxan;² dUcCa RkeBjbeBa©jkñúg

(Endocrine gland) RbB½n§srIragÁbnþBUC nig pøÚvePT.

(iv) dwknaMsarFatuciBa©wmeTAEpñkepSg² énsarBagÁkaydUcCa RbUetGIun xøaj;

sár Edl)anmkBIkarbMEbkcMNIGaharenAkñúgRbB½n§rMlayGahar

1c 1d Exchanges between the blood and tissue

fluid across the capillary

3

¬emIlrUb 1d, 1e¦ .

(v) CYysarBagÁkayRbyuT§RbqaMgnwgCMgW nig karqøgeraKepSg² tamry³

ekasikaEdlbegáItEpñkRbB½nækarBar ehAfa RbB½næsuaMnwgeraK (Immune

system).

FatupSMrbs;Qam

QamRtUv)anplitenAkñúg xYrq¥wg (Bone marrow)

¬emIlrUb 1f¦ CaCalikaeXIjmanenAEpñkkNþal

énq¥wg (Central cavity). cMeBaHkumar ekasika

QammanplitenAtamCalikaCaeRcIn EtmnusS

FMvij manplitEtenAkñúgxYrénq¥wgk,al q¥wgkgxñg

q¥wgCMnI nig q¥wgRtKak.

1e RbB½n§rMlayGahar

1g ekasika

4

QamTaMgmUl

QamTaMgmUlpSMeLIgeday 2 Epñk ³ (i) EpñkminEmnCaRKab;Qam KWCaEpñk

KμanpÞúkRKab;Qam nig (ii) EpñkCaRKab;Qam KWEpñkpÞúksuT§EtRKab;Qam.

sarBagÁkayeyIg pSMeLIgBIPaKl¥itCaeRcInEsnekadi ehAfa ekasika.

ekasikaeRbob)annwgdMu\dæsg;GaKardUecñaHEdr. ekasikamanTMhMtUcemIlnwg

EPñkTeTmineXIjeT. CalikaPaKeRcInmanekasikas¥itpþúMCab;Kña b:uEnþkñúgQam

vij ekasikavaGENþt hUrcuHeLIg kñúgsarBagÁkay. ekasikanImYy²man 3

EpñksMxan;² KW EpñkkNþal eKehAfa sñÚl (Nucleus) EpñkCMuvijsñÚleKehAfa

sIutUpøas (Cytoplasm) ehIynigsMbkB½T§CMuvijeKehAfa Pñas (Membrane)

¬emIlrUb 1g¦. rcnasm<½n§tUcCagenHCaeRcIn mankñúgEpñkTaMgbIxagelI

ehIynImYy²manmuxgarBiessedayELkrbs;va. ya:gNak¾eday mYyPaK

FMkñúgekasikanImYy²suT§swgEtCaTwkrYmCamYyRbUetGIun xøaj; sár nuyekøGiuc

GasIut (Nucleic Acid) m:UelKulrlaykñúg

Twk (Dissolved molecules) GIuy:ugGsrIragÁ

(Inorganic ions). RbUetGIunCakMlaMgclkr

énekasikarbs;eyIg. kñúgsarBagÁkay

rbs;eyIg manRbUetGIunxus²Kña 100>000

RbePT. muxgarxøHrbs;RbUetGIunkñúgekasika

rYmman ³

* sagsg;rcnasm<½n§mYyPaKFMrbs;ekasika

* edIrtYCakatalIkr (Enzymes) sMrab;

RbtikmμKimI EdleFVI[CivitRbRBwtþeTA)an

* RKb;RKgTMnak;TMng rvagbNþaépÞsMbk

ekasika

* RKb;RKgTidæPaBBn§útMNrBUC (Expression

of genes)

* pþitykFatuBn§útMNBUC (Genetic material)

1f xYrq¥wg

5

(i) EpñkminCaRKab;QaménQam KWCaFaturavBN’elOgbnþic eKehAfa pøasμa

(Plasma) Edlmandl;eTA 55° énQamTaMgGs;. pøasμapSMeLIgBITwk nig

GMbilRBmTaMgRbUetGIunsMxan;²EdlvaEckcayBaseBj

sarBagÁkay dUcCa ³

� Gal;b‘uymIn (Albumin) CaRbUetGIuncMbgkñúgQam.

� køÚb‘uylIn (Globulins) rYmman hÁama:køÚb‘uylIn (Gamma

globulin) pSMeLIgedayGgÁbdibkçrab;mWun EdlCYysarBagÁ

kayRbyuT§RbqaMgnwgCMgW nig karqøgeraKepSg².

� hVIRb‘ÍNUEhSn (Fibrinogen) CYykñúgkMNkQamkMu[

QamhUrecjeBlmanrbYsNamYy.

(ii) EpñkCaRKab;Qamvij pSMeLIgedayekasika 3 RbePT ³ eKalikaQam

Rkhm b¤ eGrIRtUsIut (Erythrocytes) eKalika Qams b¤ LWkUsIut

(Leucocytes)/ pøaEkt b¤ RtumbUsIut (Thrombocytes) ¬emIlrUb 1h¦

eKalikaQamRkhm b¤ eGrIRtUsIut

sarBagÁkaymanpÞúkeKalikaQamRkhm (Red Blood Cells) RbmaN

4>500>000 - 5>000>000¼mm3 Qam - esÞIrEt 45° énbrimaNQam

srub. eKalikaQamRkhmmanGayumFümEvgCageKkñúgcMeNamRKab;Qam

déTeTot KW 100 - 120 éf¶.

muxgarcMbgrbs;eKalikaQamRkhm KWdwknaMGuksIuEhSneTABaseBj

sarBagÁkay edayP¢ab;GuksIuEhSneTAnwgsmasFatumYyehAfa eGm:UkøÚb‘Ín

(Hemoglobin) EdlbnÞab;mkvanaMGuksIuEhSneTAeGayekasikanImYy²

énsarBagÁkay. eKalikaQamRkhmmanpÞúkm:UelKuleGm:UkøÚb‘ÍnCaeRcIn

RbmaNCa 300 lan CaehtueFVI[QammanBN’Rkhm. tamBiteTA

eKalikaQamRkhmmanpÞúkeBjedayeGm:UkøÚb‘Ín EdleFVI[vaminGacpÞúk

GVIepSgeTot)andUcekasikaepSg²eTot dUcCa sñÚl (Nucleus) CaedIm

1h

1f

smasFatupSMrbs;Qam

6

¬emIlrUb 1h¦.

Pñas b¤ sMbkxageRkArbs;eKalikaQamRkhmmanPaBTn;dUcBBuHsab‘U.

karenHeFVIeGayvagayRsYlbt;Ebn RKb;Tis edayKμanEbk CaBiesseBlva

qøgkat;srésQamtUc²bMput (Capillaries) edIm,IdwknaMGuksIuEhSneTAkEnøg

NaEdlRtUvkar.

eKalikaQamRkhmmanpÞúkkatalIkrcMnYneRcInCa kabUnic GanIRdas

(Carbonic anhydrase) EdlrYmcMENksMxan;dl;kardwkCBa¢Ún]sμ½nkabUnik

BICalikaeTAsYt.

eKalikaQam>s b¤ LWkUsIut ¬emIlrUb 1h¦

vamanEt 1° énQam. BYkvamannaTICaGayuCIvitedayedIrtYCaExSbnÞat;

karBarCYrmuxRbqaMgnwgkarratt,atBIPñak;garbgáCMgW dUcCa )ak;etrI vIrus pSit

nig )a:ra:sIut. eKalikaQam>s (White Blood Cells) CaRkumekasikaepSg²

Kña RkumnImYy²rYmcMENktamviFIxus²KñaedIm,IRbqaMg nig karBarkarqøgeraK

nig karbMpøicbMpøajCalika. tamFmμta eKEbgEckBYkvaeTAtamlkçN³rUbragÁ

rbs;va ³

�� RkanuyLÚsIut (Granulocytes) b¤ ekasikaEdlmansñÚleRcIn

eKehAdUcenH edaysarsñÚlvamansPaBCaRKab; nig manEkøbeRcIn.

eKEbgEckRkumenHeTAtamBN’vaeBlbMBak;BN’ enAkñúgmnÞIrBiesaFn_ ³

- NWRtUhVIl (Neutrophils) ¬emIlrUb 1i¦ ¬man 72° éneKalika

Qam>sTaMgGs;¦ vamanBN’exov eBlbMBak;BN’.

- eGGUsIuNUhVIl (Eosinophils) ¬emIlrUb 1j¦ ¬man 1/5° éneKalika

Qam>sTaMgGs;¦ vamanBN’RkhmeBlbMBak;BN’.

- )asUhVIl (Basophil) ¬emIlrUb 1k¦ ¬man 0/5° éneKalikaQam>s

TaMgGs;¦ vamanBN’sVay eBlbMBak;BN’.

1i neutrophil

1j eosinophil

1k Basophil

1l monocyte

7

�� eKalikaQam>s epSg²eTotman ³

- m:UNUsIut (Monocyte) ¬emIlrUb 1l¦ ¬man 4° éneKalikaQam>s

TaMgGs;¦.

- LaMhVÚsIut (Lymphocyte) ¬emIlrUb 1h¦ ¬man 24° éneKalika-

Qam>s TaMgGs;¦.

eKalikaQam>s manTMhMFMCageKalikaQamRkhm EtvamancMnYnticCag

RbEhl 7>000¼mm3 énQam ehIyCIvitvak¾xøICagEdr manry³eBlEt 18

- 36 ema:gb:ueNÑaH .

pøaEkt (Platelets) mantYnaTIsMxan;EtmYyenAkñúgQam vaepþIm[mandMeNIr

kMNkQam ¬ERbQameTACaqñúkP¢it¦ karBarsarBagÁkaykMu[)at;bg;Qam

tamsrésQamEdldac;. pøaEkt KWCaeKalikaQamEdlmanTMhMtUcCageK

¬emIlrUb 1h¦. vamancMnYnRbmaN 200>000¼mm3 énQamehIyman

GayuRbEhl10 éf¶.

xusBIeKalikaQamRkhm BYkeKalikaQam>s nigpøaEkt suT§Etman sñÚl

nig sIutUpøas . eKalikaQamTaMg 3 RbePTenH ¬eKalikaQamRkhm

eKalika Qam>s nig pøaEkt¦ ekItecjBIekasikaedIm (Precursor) dUcKña

ehAfa eGma:tUb:UGIueyTic esþmEsl (Hematopoietic stem cell). ekasika

edImenHEbkCakUnecAelOnNas;. RKan;Etkñúgry³eBl 4 s)aþh_/ ekasika

edIm cMnYn 10 GacbMEbk)anCaRKab;QamRkhmcMnYn 30 Esnekadi RKab;

Qam>s cMnYn 30 ekadi nig pøaEktcMnYn 1/2 Esnekadi - EdlRKb;RKan;lμm

eTACMnYsral;RKab;QamkñúgsarBagÁkay.

RbePTQam ¼RkumQam

RkumQamcMbgman 4 RbePT ³ A, B, AB nig O kt;sMKal;edayRbePT

RbUetGIun b¤ ehAmüa:geTotfa cMNucsMKal; (Marker) b¤ Gg;TIEhSn (Anti-

gen) sßitenAelIPñaséneKalikaQamRkhm. QammnusSeyIgmñak;² cat;cUl

8

eTAkñúgRbePTNamYyénQamTaMg 4 RkumcMbgenH A, B, AB b¤ O KWfa

mnusSmñak;²maneKalikaQamRkhmEtmYyRbePTkñúgbNþaRkumTaMgenH ³

RkumQam A - eKalikaQamRkhmmancMNucsMKal; A (Marker A) elIPñas

rbs;BYkva.

RkumQam B - eKalikaQamRkhmmancMNucsMKal; B (Marker B) elIPñas

rbs;BYkva.

RkumQam AB - eKalikaQamRkhmmancMNucsMKal;TaMgBIr A nig B (A

and B Markers) elIPñasrbs;BYkva.

RkumQam O - eKalikaQamRkhmKμancMNucsMKal;TaMgBIr A nig B elI

Pñasrbs;BYkvaeT.

eKalikaQamRkhm GacpÞúkpgEdrnUvGg;TIEhSnmYyepSgeTot EdlKμan

Tak;TgnwgRkumQam KW Gg;TIEhSnerhSús [Rhesus (Rh) Antigen].

QamEdlmancMNucsMKal;erhSús (Rh Marker) eKehAfa erhSúsviC¢man

ÉQamEdlKμancMNucsMKal; erhSús eKehAfa erhSúsGviC¢man.

eK)anRsavRCavrkeXIjeKalikaQamRkhmCag 20 RbePTepSgeTot

b:uEnþmanEtRbePTxagelIeT EdlsMxan;bMput ehIyeKyl;c,as;.

manehtuplxøH²EdleKRtUvkardwgBIRbePTQamrbs;xøÜn. sarsMxan; KWsßit

enARtg;fabuKÁlmñak;GacnwgRtUvTTYlQamBIbuKÁlmñak;eTot dUcCakarbBa©Úl

QamCaedIm ¬emIltarag 1w¦. kñúgkarbBa©ÚlQam QamGñkpþl; ¬Gñkbri-

c©aKQam¦ nigQamGñkTTYl ¬GñkRtUvbBa©ÚlQam¦ RtUvEtsIuKña ¬cUlKña)an¦

edIm,IkMu[sarBagÁkayGñkTTYlQamRbqaMgnwgQamEdl)anbBa©ÚleTA.

dMeNIrénkarpÁÚrpÁg[sIuKñaénRkumQam nig Gg;TIEhSn erhSús rbs;Gñkbric©aK

CamYyGñkTTYl eKEtgEteFVIenAmnÞIrBiesaFn_énmCÄmNÐlpþl;Qam ehIy

kareFVIrebobenHeK[eQμaHfa RkUsμac; (Cross matching) b¤ karsakl,grk

PaBRtUvKña (Compatibility testing). RbsinebIRkumQam nig ktþaerhSúsmin

dUcKña sarBagÁkayGñkTTYlQamnwgyl;fa QamEdlbBa©ÚleTACaPñak;gar

1m what platelets do

9

ebotebon ehIysarBagÁkaynwgkMcat;vaecj. PaBRbwg

ERbgrbs;sarBagÁkay vaykMcat;QamBIeRkAEdlminsIu

KñaenaH nwgeFVI[ekItCaCMgWF¶n;F¶r ehIymandl;søab; ebI

eFVIeraKvinicä½y nigBüa)alminTan;eBlevla.

CMgWQam

manCMgWCaeRcInbNþalmkBImanPaBminFmμtaenAkñúgQam. PaBminFmμta

TaMgenH cat;fñak;eTAtamEpñkénQamEdlb:HBal; dUcCa CMgWeKalikaQam

Rkhm CMgWeKalikaQam>s b¤ CMgWkMNkQam.

CMgWeKalikaQamRkhm kñúgenaHmanCMgWtaLasesmIuCaedIm. CMgWmYYykñúg

bNþaCMgWeKalikaQamRkhmEdlCYbjwkjab;CageK KWeraKxVHeKalikaQam

Rkhm (Anemia) CaeraKEdlsarBagÁkayeyIgmaneKalikaQamRkhm b¤

eGm:UkøÚb‘ÍnmankMritTabxusBIFmμta. eraKsBaØaTUeTAéneraKxVHeKalikaQam

Rkhm KWPaBesøksøaMgGs;kMlaMgBlM edaysarQamBMu)andwknaMGuksIuEhSn

eTAsarBagÁkay[)anRKb;RKan;. RbePTeraKxVHeKalikaQamRkhmEdlCYb

jwkjab;CageKenaH KWbNþalmkBIxVHCatiEdk bNþal[xYrq¥wgminGacplit

cMnYneKalikaQamRkhm)anRKb;RKan;. GñkCMgWEbbenH CaTUeTAnwgCaes,Iy

edayRKan;EteRbIfñaMCatiEdkbMeBjbEnßm.

b:uEnþ man eraKxVHeKalikaQamRkhmepSg²eTot EdlvaF¶n;F¶rCag.

]TahrN_ eraKxVHeKalikaQamRkhmbNþalmkBIkarEbkRKab;Qam

(Hemolytic anemia) edaysarBagÁkayeyIgbMpøajeKalikaQamRkhm

kñúgkMritelOn. kñúgCMgWtaLasesmIukMritF¶n; (Thalassemia major) eKali-

kaQamRkhmekItPøamRtUv)anbMpøajPøam dUcenHxYrq¥wgminGacpliteKalika

QamRkhm[RKb;cMnYn mkCMnYs)aneLIy. CMgWTaMgenHmanlkçN³tMNBUCBI

«Bukmþaypþl;mk[kUn ehIyvaF¶n;F¶rNas;. Gs;CaeRcInqñaMmkehIyEdleK

KitfaminGacBüa)al)an ehIyGñkCMgWTaMgenaHnwgsøab;taMgBIv½yekμgeday

1w

10

eCosminrYc. Et\LÚvenH eyIgdwgfakarBüa)alRtwmRtUv GñkCMgWGacmanCIvit

Fmμta)an.

CMgWtaLasesmIukMritF¶n;

taLasesmIukMritF¶n; ehAmüa:geTotfa eraKxVHeKalikaQamRkhm

emDIETra:en (Mediterranean anemia) b¤ eraKxVHeKalikaQamRkhm-

kULI (Cooley’s anemia). eKFøab;EtKitfa vamanEtenACMuvijsmuRT

emDIETra:en GBa©wgehIy)anCaeKehAfa eraKxVHeKalikaQamRkhm

emDIETra:en b¤ taLasesmIu [Thalassemia (Thalassa + anemia)] Bakü

“Thalassa” )ann½yfa {smuRT} vamankMeNItBIPasaRkik rYmBakümk)an

esckþIfa eraKxVHeKalikaQamRkhmenAbNþaRbeTsCMuvijsmuRT - smuRT

emDIETr:aen. CMgWenHeKehAfa eraKxVHeKalikaQamRkhmkULI k¾)aneday

yktameQμaHevC¢bNÐitCMnajEpñkeraKkumar eFam:as kULI CaCnCati

GaemrikkaMgEdlrYmCamYyevC¢bNÐit EBrl_ lI )anerobrab; nig raykarN_

elIkdMbUg nUvlkçN³rUbPaBKøInikkñúgqñaM1927 eRkayBI)aneFVIkarsikSaelIGñk

CMgWTaMgLaymanedImkMeNItCaCnCati GIutalI.

taLasesmIukMritF¶n; CaRbePTtaLasesmIuEdlF¶n;F¶rCageK ehIyCaRbFan

bTcMbgénesovePAenH. taLasesmIumanCaTUeTAenAtMbn;ekþAkñúgBiPBelak.

eTaHbIya:gNak¾eday kareFVIcMNakRsukrbs;mnusS CMuvijBiPBelak)annaM

taLasesmIueTARKb;TIkEnøgEdlBImunBMuman. CaBiessbNþaRbeTsPaKxag

eCIg dUcCa Gg;eKøs shrdæGaemrik kaNada nig GaLWm:g; mankrNICMgWenH

ekIneLIgKYr[kt;sMKal; bNþalmkBIGenþaRbevsn_BIGWr:ubxagt,Úg nig GasIu.

11

CMBUkTI 2

Bn§útMNBUC nig taLasesmIu

taLasesmIu KWCaCMgWEdlTak;TgnwgBn§úehIytBUC )anesckþIfa qøgBI«Buk

mþay eTAkUntamry³ Bn§ú (Genes). vaminqøgtamQam xül; Twk b¤ qøg

tamkarb:HBal;ragÁkay b¤ pøÚvePTCamYyGñkCMgWeLIy vaminbgáedaykgVHcMNI

Gahar b¤ sßanPaBevC¢saRsþxVHGnam½yenaHEdr.

EhSn (Genes) = Bn§ú

RkumBn§ú KWCaBnøkBUCsMrab;sarBagÁkaymYyedayELkerog²xøÜn edaypþl;nUv

Bt’manCIvsaRsþTaMgGs;EdlcaM)ac;sMrab;RtYtBinitükarrIkFMFat; nig karvivtþn_

eBjmYyCIvitrbs;sarBagÁkayenaH vaCaRkumGgÁCIvsaRsþtMNBUC.

BinÞúnImYy²CaPaKsMxan;rbs;sarFatuKImImü:agehAfa edGuksIurIbUnuyekøGiuk

GasIud (Deoxyribonucleic acid = DNA).

DNA ¬emIlrUb 2a¦ Carcnasm<½n§mYydUckaMCeNþIr EdlmanemCeNþIrBIrRsb

KñaP¢ab;edaykaMCeNþIrCaeRcIn. kaMnImYy²mansarFatuKImI

BIrpþúMCab;KñaCaKUr eKehAfa )as (Bases). )asnImYy²

tagedayGkSrxus²Kña (C, G, A, T) . GkSr C sMrab;

sIutUsIun (Cytocine)/ G sMrab; háanIn (Guanine)/ A sMrab; GaednIn

(Adenine)/ T sMrab; TImIn (Thymine). )asTaMgbYnenHcab;KUrKñakñúg

lkçN³kMNt;c,as;las; ³ A KUrCamYy T ehIy C KUrCamYy G ¬emIlrUb

2a¦. BYk)astMerobKñatamlMdab;lMedayCakaMCeNþIrpþl;nUvkUttMNrBUC

(Genetic code) rbs;sarBagÁkay. DNA rbs;sarBagÁkaypÁúMcUlKñaCaeRcIn

eTA eK[eQμaHfa bNþMúBn§ú b¤ ehSenam (Genome) ¬emIlrUb 2b¦.

2a DNA

2a DNA

2b genome

12

ehSenamrbs;mnusSmanpÞúkBn§úeRcInBan;. DNA pþl;karENnaM[mankar

sagsg;m:UelKulTaMgGs; EdlTTYlxusRtUvdl;muxgarCIvsaRsþrbs;sarBagÁ-

kay dUcCa sagsg;RbUetGIun. dMeNIrénkarplitm:UelKulmYyecjBIBn§úTak;Tg

eTAnwgynþkarüCIvsaRsþkñúgekasika KW {karGan} Bt’manEdlpÞúkkñúgBn§ú.

Bt’manenaH)ancMlgcUlkñúgm:UelKulmYy Rsedognwg DNA eKehAfa RNA

[b¤ehAfa rIubUnuyekøGiuk GasIud (Ribonucleic Acid)]. dUc DNA Edr/ RNA

manpÞúk)asháanIn GaednIn nig TImIn (G, A and T) Et sIutUsIun )asTIbYnkñúg

RNA RtUvCMnYseday G‘uyra:sIul [Uracil (U)]. RNA xusBI DNA Rtg;fa RNA

manrcnasm<½n§EtmYy KμanBIrdUcCeNþIrenaHeT. muxgarrbs; RNA KWCaGñknaM

sar (mRNA) naMBt’manBI DNA eTAynþkarüCIvsaRsþrbs;ekasika EdlTTYl

xusRtUvkñúgkarbegáItplitplCIvsaRsþ dUcCa RbUetGIunCaedIm .

kñúgsarBagÁkaymnusStRmUv[manBn§úmYycMnYnFM edIm,IGnuvtþn_nUvmuxgarCIv

saRsþsμúRKsμajCaeRcIn. Bn§úTaMgenH)anRtUvrkSaCab;KñakñúgekasikaCaExS

DNA d¾EvgEdleKehAfa RkUm:UhSÚm (Chromosomes). ekasikanImYy²

rbs;mnusS ¬elIkElgEtekasikaemCIvitburs nig RsþI¦ manc,ab;cMlg

RkUm:UhSÚmBIr mYymkBImþay mYyeTotmkBI«Buk.

RbePTsarBagÁkayxusKña cMnYnRkUm:UhSÚmk¾xusKñaEdr. mnusSmanRkUm:UhSÚm

23 KUr b¤ 46 RkUm:UhSÚm ¬emIlrUb 2c¦. cMnYn 22 KUr ¬44 RkUm:UhSÚm¦ man

lkçN³dUcKñaTaMgePTRbus TaMgePTRsI eKehAfa GUtUsUm (Autosomes)

ÉmYyKUrenAsl;Ca RkUm:UhSÚmePT (Sex chromosomes) CaGñkkMNt;ePT.

kñúgRkUm:UhSÚmmYyKU²rbs;mnusSmñak;²manmYy)anmkBImþay/ mYyeTot)an

mkBI«Buk.

eRkaykarRsavRCavCaeRcInqñaM eKGacsÁal;Bn§úCaeRcInEdlkalNavaTTYl

rgkarb:HBal; vabgá[manCMgWepSg²ehAfa CMgWBn§ú (Genetic disorders).

2c RkUm:UhSÚm

13

CMgWBn§ú EbgEckeTACabYnRbePT ³

1> PaBminFmμtarbs;RkUm:UhSÚm ³ TaMgenHekIteLIgeBlRkUm:UhSÚmTaMgmUl

b¤ mYyEpñkFMrbs;RkUm:UhSÚm)an)at;bg; ekIneTV b¤ ERbRbYl .

2> CMgWrbs;Bn§úEtmYy ³ TaMgenHbgáeLIgeBlmankarERbRbYlpøas;bþÚrenARtg;

Bn§úbgá[mankarERbRbYl b¤)at;bg;karsMeyaKnUvplitpl

ecjBIkUdrbs;Bn§úenaH.

3> CMgWb:HBal;elIBn§úeRcIn ³ vaekItecjBIkarERbRbYl

pøas;bþÚrénBn§úCaeRcIn rEmgekItmkCamYyehtuplEpñk

briyakas. )anesckþIfa manktþaCaeRcInRtUvekItman

RsbKña munnwg[ekItmanCaCMgW.

4> CMgWTak;Tgnwg mItUkugRDI (Mitochondria) ³

CMgWbgáedaykarERbRbYlpøas;bþÚrkñúgrcnasm<½nætUcmYy EdlminEmnCa

RkUm:UhSÚm DNA eT vasßitenAkñúgsIutUpøasrbs;ekasika eKehAfa

mItUkugRDI vaCasßanIy_sþúkfamBlrbs;ekasika.

taLasesmIu KWCaGVIEdleKdwgfaCa CMgWrbs;Bn§úEtmYy vaqøgBI«Bukmþay

eTAkUn tamEbbEpnNamYyénEbbEpnTaMgbYnCamUldæanEdlRBHsgÇeQμaH

eRhÁK½r emnEdl (Gregor Mendel) )anerobrab;elIkdMbUgkñúgqñaM 1865.

EbbEpnTaMgbYn mandUcxageRkam ³

(i) GUtUsUm:al dUmINg; (Autosomal dominant)

(ii) GUtUsUm:al er:esssIuv (Autosomal recessive)

(iii) Giuc-lIg dUmINg; (X-linked dominant)

(iv) Giuc-lIg er:esssIuv (X-linked recessive)

BaküTaMgenHeKeRbIenAkñúgBn§úviTüa edIm,Ierobrab;lkçN³lT§plKøInik [ehVNUTIb

(Phenotype)] EdlekItecjBIPaBminFmμtarbs;Bn§ú [ehSNUTIb

2d mItUkugRDI

14

(Genotype)]. ebI ehVNUTIbekItecjBIehSNUTIbEtxagNamYy «Buk b¤ mþay

karenHCa GUtUsUm:al dUmINg;. ebIekItecjBIehSNUTIbTaMg«Buk TaMgmþay

enaHeKehAfa GUtUsUm:al er:esssIuv. dUcKñaenHEdr ebIehVNUTIb ekItecjBI

ehSNUTIbtamry³PaBminFmμtarbs;RkUm:UhSÚmkMNt;ePTEtmYy «Buk b¤ mþay

eKehAfa Giuc-lIg dUmINg; EtebIekItecjBIRkUm:UhSÚmkMNt;ePTTaMgBIr

TaMg«Buk TaMgmþay eKehAfa Giuc-lIg er:esssIuv.

taLasesmIu CaCMgWrbs;Bn§úEtmYyEdlqøgBI«BukmþayeTAkUn tamEbbEpn

tMNBUC GUtUsUm:al er:esssIuv.

CMgW GUtUsUm (Autosomal disease) GacekItTaMgePTRsI nig RbusdUcKña

edayehtufaPaBminRbRktIekItmanelI GUtUsUm (Autosomes) NamYy

EtminekItelIRkUm:UhSÚmEdlkMNt;ePTrbs;kumareT.

er:esssIuv (Recessive) mann½yfa kumarRtUvTTYlBn§úbgáCMgWBI«Bukpg nig

BImþaypg edIm,I[ekItCasPaBKøInikF¶n;F¶r ehAfa taLasesmIukMritF¶n;.

buKÁlNaEdlTTYlBn§úbgáCMgWBImþaypg BI«Bukpg eKehAfa GUm:UhSIúháÚt

(Homozygotes). kñúgkrNI Ebtta taLasesmIu ( β– Thalassemia)

eKehAfa GñkCMgW Ebtta taLasesmIu GUm:UhSIúháÚt (Homozygous β–

Thalassemia) eKGacehAmüa:geTotfa taLasesmIukMritF¶n;

(Thalassemia major)/ eraKxVHeKalikaQamRkhmemDIETra:en

(Mediterranean anemia) b¤ eraKxVHeKalikaQamRkhmkULI (Cooley’s

anemia).

buKÁlNaEdlTTYlBn§úFmμtamYyBImñak;én«Bukmþay ehIyBn§úmYyeTotbgáCMgW

BImñak;epSgeTotén«Bukmþay eKehAfa eGetr:UhSIúháÚt (Heterozygotes).

ehIydUcKñaenHEdr kúñgkrNI Ebtta taLasesmIu eKehAfa Ebtta

taLasesmIu eGetr:UhSIúháÚt (Heterozygous β – Thalassemia).

manBaküepSgeTotEdleKeRbIcMeBaHGñkpÞúkBn§úbgáCMgW EtGt;maneraKsBaØa KW

15

taLasesmIukMritRsal (Thalassemia minor b¤ Thalassemia trait).

GñkCMgWEbbenH nwgminekItCaeraKsBaØaGVIeT Etya:gNak¾eday eKnwgbBa¢ÚnBn§ú

bgáCMgWenaHeTAkUnrbs;eK.

PaBminRbRktIkñúgeKalikaQamRkhmEdlnaMeGayekItCMgWtaLa-

sesmIukMritF¶n;

dUc)anerobrab;kñúgCMBUkTI1/ taLasesmIu KWCaCMgWBn§útBUCénQam [cMCagenH

eTot KWCaCMgWmYybNþalmkBIPaBminRbRktIkñúgkarsMeyaKm:UelKul

eGm:UkøÚb‘Ín kñúgeKalikaQamRkhm.

eGm:UkøÚb‘Ín nig Edk

eGm:UkøÚb‘Ín ¬emIlrUb 2e¦ KWCaRbePTm:UelKulcMruHBiess EdlCaRbUetGIun

mankñúgeKalikaQamRkhm EdlmantYnaTIdwknaMGuksIuEhSneTAkan;kEnøg

EdlRtUvkarkñúgsarBagÁkay. eKalikaQamRkhmnImYy² manm:UelKul

eGm:UkøÚb‘Ín 300lan.

m:UelKuleGm:UkøÚb‘ÍnmYyEckCa 2 Epñk ³

(i) RbUetGIunmYy ehAfa køÚb‘Ín (Globin) begIát)anCaExSRbUetGIuncMnYnbYn

tMerobCaKUrnwgKña ¬emIlrUb 2e + f¦. vamanExSRbUetGIunmYycMnYn dUcCa

ExSRbUetGIunRbePT Gal;hVa (alpla2 = a2) nig ExSRbUetGIunEdlminEmn

RbePT Gal;hVa (β2,γ2, 2, 2,E2) tMerobKñaCaKUr a2β2, a2γ2, a2 2,

a2 2 nig a2E 2.

(ii) bNþMú EGm-Edk (Haem-Iron) Carcnasm<½n§ragCargVg;EdlsMeyaK enAkñúg

sIutUsul (Cytosol) nig mItUkugRDI (Mitochondria) rbs;ekasika.

m:UelKulEdkmYymankñúgbNþMú EGm-EdkCaGñkpþl;lT§PaB[mankardwk

CBa¢ÚnGuksIuEhSnkñúgsarBagÁkay. enHedaysarEtEdkgayP¢ab; nig gay

pþac;GuksIuEhSn edaybegáItCameFüa)ayd¾manRbsiT§iPaBkñúgkarnaM

GuksIuEhSneTACalika nig ekasika.

2e Haemoglobin

2f

16

mnusSFMFmμtamanEdk 4 Rkam enAkñúgxøÜn ehIy 75° ¬RbEhlCa 3 Rkam¦

RtUv)aneRbIedIm,IsMeyaKeGm:UkøÚb‘Ín. karplit nig sMeyaKeGm:UkøÚb‘Ín

TTYlkarRKb;RKgBIBn§úmYycMnYn KW BYk Bn§úGal;hVa (a- genes) sßitelI

RkUm:UhSÚmelx 16 cMENk Bn§úEbtta (β- genes)/ Bn§úháama: (γ- genes)

nig Bn§úEdlta ( - genes) sßitelI RkUm:UhSÚmelx 11. manBn§úbYn

kMNt;ExSGal;hVa (a- chains) nigmanBn§úBIrkMNt;ExSEbtta (B- chains)

¬emIlrUb 2g¦. edayminKitBIcMnYnBn§úEdlTTYlxusRtUvkñúgkarRtYtBinitükar

sMeyaKExSGal;hVa nig ExSEbtta KW ExSTaMgBIrRtUv)anplitkúñgbrimaN

esμIKñaya:gBitR)akd.

manRbePTeGm:UkøÚb‘ÍnepSg² )anmkBIkarpÁÚrpÁgExSRbUetGIunxus²Kña EdlRtUv

)anplitenAdMNak;kalnImYy²énCIvit dUcbgðajkñúgtaragxageRkam ¬emIlrUb

2h¦.

2g The αα- and

ββ- globin gene

clusters on

chromosomes

16 and 11,

respectively

2h

17

Bn§úEdlRKb;RKgkarsMeyaKkøÚb‘Ín

PaBminRbRktIBitR)akdEdlnaMeGayekIt Ebtta taLasesmIukMritF¶n;

KWenARtg;Bn§úEdlRKb;RKgkarplit ExSRbUetGIun Ebtta kñúg køÚb‘Ín rbs;

eGm:UkøÚb‘Ín.

CalT§plBIPaBminRbRktIrbs;Bn§úenH vaKμanplit ExSEbtta b¤ manbnþic

bnþÜc EdleFVI[eKalikaQamRkhmmanExSRbUetGIunEtmYyRbePT KW ExS

Gal;hVa. GtulüPaBenH raraMgdl;karpliteGm:UkøÚb‘ÍnFmμta EdlTamTar

[man ExSEbtta nig ExSGal;hVa edIm,IdMeNIr[)anRtwmRtUv. CalT§pl

karpliteKalikaQamRkhmEdleKehAfa eGrIuRtUb:UGIueyhSIús (Erythro-

poiesis) ¬ecjBIBaküRkik Erythra )anesckþIfa {eKalikaQamRkhm}/

poiesis )anesckþIfa {plit}¦ RtUvTTYlrgkar b:HBal;ya:gF¶n;F¶r.

dUc)anCMrabxagelI PaBminRbRktIrbs;Bn§úTTYlxusRtUvkñúgkarplit

ExSEbtta rbs;eGm:UkøÚb‘Ín KWbgáedaykarpøas;bþÚrEtmYyenAkñúgKUr)asmYy

¬karpøas;bþÚrcMNuc¦ b¤ edaybMENkTaMgLayrbs;Bn§ú)anbg; ¬rlub)at;¦.

2h The sites of

erythropoiesis

during development

and the different

globins produced at

each stage

18

eK)ansÁal;karpøas;bþÚr Cag 200 epSg²KñaEdlGacb:HBal;eTAdl;dMeNIr

karFmμtarbs;Bn§úsMrab; EbttakøÚb‘Ín (β- globin). GaRs½yedayRbePT

énkarpøas;bþÚr karb:HBal;karsMeyaK køÚb‘Ín mankMrittictYc b¤ xøaMgkøa

ehIynaMeGayekIttaLasesmIukMritRsal b¤ kMritF¶n;.

karpøas;bþÚrxøHmanplb:HBal;mFüm (β++) rhUtdl;b:HBal;bgÁÜr (β+)

eTAelIkarplit ExSEbtta/ rIÉkarpøas;bþÚrxøHeToteFVI[)at;karplit

ExSEbtta Etmþg (βo).

dUcenHmnusSEdlTTYlBn§ú β+ BI«Bukmþay TMngCavivtþn_eTArktaLasesmIu

kMritmFüm. pÞúyeTAvij GñkTaMgLayEdlTTYlBn§ú βo BI«Bukmþay nwgxVH

EbttakøÚb‘Ín esÞIrEtTaMgRsug ehIynwgvivtþn_eTArkTMrg;F¶n;F¶réntaLasesmIu

esÞIrEt 100°. eTaHya:gNak¾eday eBlTTYlBn§úcMruHcUlKña/ vaminCakar

gayRsYlkñúgkarTsSn_TayehVNUTIb EdlEsþgecjmkeLIy

dUcCa β+β++ b¤ β+βo b¤ β++β+o.

cMeBaH Ebtta taLasesmIu ( β - Thalassemia) ]TahrN_ lT§PaBbgá

GacCa ³

βo/βo : taLasesmIukMritF¶n; (Thalassemia major)

β+/ β+ : taLasesmIukMritF¶n; (Thalassemia major)

β++/β++ : taLasesmIukMritmFüm (Thalassemia intermedia - TMrg;mFüménCMgW)

β+/ β++ : taLasesmIukMritF¶n; ¼ kMritmFüm (Thalassemia major / intermedia)

ehSNUTIb ¼ ehVNUTIb (Genotype / Phenotype)

dUc)anerobrab;xagelI vamansarsMxan;Nas;kñúgkarEsVgrkkarpøas;bþÚrBn§ú

BiessedayELk (Genotype) EdlmanTMnak;TMngCamYyPaBF¶n;F¶rEpñkKøInik

énCMgW (Phenotype). karbUkpSMcUlKñaepSg²énBn§úxusKñaTaMgbI (β++, β+ nig

βo) EdlmankñúgtaLasesmIu eFVI[kMritF¶n;F¶rénCMgWxus²Kña. TnÞwmnwg

enHEdrenARbeTsepSg² manlkçN³énkarpSMBn§úcUlKña ehIynigPaBjwkjab;

19

énkarpøas;bþÚrbn§úedayELkrbs;eK.

]TahrN_ énkarpøas;bþÚr (Mutations) kMritF¶n; ¬0¦/ minsUvF¶n; ¬+¦/ mFüm

¬++¦ bgðajdUcxageRkam ³

β++ Mutations β+ Mutations βo Mutations

-101, -87, -88, -28 lVS1-110 lVS1-1

lVS1-106 lVS11-745 lVS11-1

lVS1-5 IVS1-[-25]

C39

ktþaBn§úmYycMnYnepSgeTot k¾GacCYybnßyPaBF¶n;F¶rénCMgW)anEdr dUcCa ³

(i) karsMeyaKExSGal;hVa (a - chains) fycuH. Ca]TahrN_ GñkCMgW

taLasesmIukMritF¶n;xøH Kat;TTYlkarpøas;bþÚrEdlbnßykarsMeyaK

ExSGal;hVaEfmBIelIkarfycuHénkarsMeyaKExSEbtta. kñúgkrNIenH

GtulüPaBrvagExSGal;hVa nig ExSEbttaminsUvxøaMg dUcenHCMgWk¾manTMrg;

minF¶n;F¶rEdr.

(ii) kMeNInkarsMeyaKExSháama: (γ - chains). QammnusSFM Fmμta man

eGm:UkøÚb‘ÍnTarkkñúgépÞ (Foetal hemoglobin = HbF) RbmaN 1°

ekItBIExSGal;hVamYyKUr nig ExSháama:mYyKUr (a2 γ2). cMeBaHGñkCMgW

taLasesmuI karplit ExSháama: (γ - chains) )anekIneLIgeday

sarBagÁkayxitxMsMrbsMrYldl;kgVHxatkarplit eGm:UkøÚb‘Ín Ga (HbA)

edaybegáInkarplit HbF. eTaHya:gNak¾eday karekIneLIgenHvaBMu

RKb;RKan;eTACMnYsmuxgarrbs; HbA Edl)at;bg;eLIy. manGñkCMgW

taLasesmuIxøHGacTTYlCatMNBUCpgEdrnUvlkçx½NÐmYyehAfa tMNBUC

GciéRnþy_én HbF (Hereditary Persistance of Fœtal Hemoglobin b¤

HPFH). eBlenaHkMrit HbF mankñúgkMritx<s;ehIymanCab; eBjmYyCIvit

rbs;mnusSFM. GñkCMgWk¾GacTTYlCatMNBUCpgEdrnUvkarpøas;bþÚr

20

(Mutations) EdlmanP¢ab;mkCamYynUvkareP£acBn§úrbs; háama:køÚb‘Ín (γ -

globin genes) ¬]TahrN_ γ Xmn polymorphism¦ b¤

P¢ab;mkCamYynUvlkçxNÐepSgeTotdUcCa β- Thalassemia

EdlGaceFVI[mankarekIneLIg karsMeyaK HbF elImnusSFM ¬BI 5 -

20°¦ ehIykarenHvabnßyPaBF¶n;F¶rrbs;taLasesmuIedaybnßy

GtulüPaBénExSkøÚb‘Ín (Globin chains).

eraKsrIrsaRsþ rbs;CMgWtaLasesmIu (Pathophysiology of

Thalassemia)

kgVHExSEbtta ( ββ- chains) ehIyelIsExSGal;hVa (a- chains)

kñúgGMLúgeBlmanépÞeBaH Qamrbs;TarkpÞúkeGm:UkøÚb‘ÍnRbePTBiess

müa:geQμaHfa eGm:UkøÚb‘ÍnTarkkñúgépÞ (Fœtal hemoglobin = HbF).

dUc)anCMrabxagelI/ HbF ekItBIExSGal;hVamYyKUr nig ExSháama:mYyKUr

(a2γ2). HbF enHbMeBjmuxgardwknaMGuksIuEhSn kñuúgsarBagÁkaydUc

eGm:UkøÚb‘ÍnFmμtaEdlbMeBjmuxgarkñúgxøÜnkumar nig mnusSFMEdr. eRkayBITark

RbsUt/ HbF bnþbMeBjmuxgarFmμtarhUtdl;TarkGayu 6 Ex eTIbvacab;epþIm

CMnYsbnþicmþg²edayeGm:UkøÚb‘Ínrbs;mnusSFM (HbA). HbA ekItBIExSGal;hVa

BIr nig ExSEbttaBIr (a2 β2).

cMeBaHtaLasesmIukMritF¶n; BMumankarplitExSEbttaeT b¤ manplit

EdrEttictYcbMput eFVI[KμankarsMeyaKecjCa HbA Fmμta)an ehIy

b:HBal;ya:gF¶n;F¶rdl;smtßPaBrbs;eKalikaQamRkhm kñúgkardwk

naMGuksIuEhSn. dUecñaH xøÜnkumareqIøytb edaybnþkarplit HbF.

Etya:gNak¾eday vaBMuGacplit)anbrimaNRKb;RKan; edIm,IeqøIytb

tamesckþIRtUvkarrbs;sarBagÁkaykumarEdlecHEtFMFat; nig

edIm,ICMnYsmuxgardwkCBa¢ÚnGuksIuEhSnrbs; HbA (a2 β2) )aneLIy.

karmanExSEbttabnþicbnþÜc b¤k¾KμanesaH k¾manplGviC¢manepSgeTot

21

Edr. eBlEdlsarBagÁkayeyIgbnþplitExSGal;hVaCaFmμta EtExSEbtta

minRKb;RKan;edIm,Icab;KUKña ExSGal;hVaecHEtekIneLIg²kkkujeRcIn. karkk

kujenHvarMxandl;karpliteKalikaQamRkhmrbs;sarBagÁkay edaykat;

bnßykarplitrhUtdl; 95°. edaymaneKalikaQamRkhmeBjGayuEt

bnþicbnþÜc sarBagÁkayecjCasPaBesøksøaMgxøaMg.

elIsBIenHeTot ExSGal;hVaelIslubkkpMþúkñúgeKalikaqamRkhmeBjGayu

kñúgcrnþQam eFVI[Pñasrbs;vagayxUcxat ehIyEbkFøayEdlritEteFVI[

PaBesøksøaMgkan;EtF¶n;F¶r. dMeNIrEbkeKalikaQamRkhmeKehAfa

eGm:UlIs (Hemolysis) ¬mkBIBaküRkik Hem )anesckþIfa {Qam}/ lysis

)anesckþIfa {Ebk}¦. eGm:UlIs bgá[ b‘ÍlIruyb‘Ín (Bilirubin) mankMrit

x<s;CagFmμta. b‘ÍlIruyb‘Ín CasarFatuKImIBN’elOgEdl)anmkBIkarrMlay

eGm:UkøÚb‘Ín (Hemoglobin) ehIyEdlRtUv)anbeBa©jedayeKalikaQam

RkhmxUcxatkñúgdMeNIrrbs; eGm:UlIs. b‘ÍlIruyb‘Ín mankMritx<s;eFVI[EPñk

nig Es,kGñkCMgWtaLasesmIukMritF¶n; mansPaBBN’elOg eKehAfa

“Icteric”.

EsþgecjCalT§plénkgVHeKalikaQamRkhm GñkCMgWtaLasesmIukMritF¶n;

mansPaBesøksøaMg/ Gs;kMlaMg ehIyebHdUgedIrjab;eRBaHkgVHGuksIuEhSn.

karenHnwgeFVI[ebHdUgrIk. GñkCMgWmankarFMFat;ragÁkayyWtya:v ehIyman

karlM)akeFVIskmμPaBragÁkay. PaBesøksøaMgmanCaRbcaM mineqøIytb

nwgkarBüa)al. kumareTACaexSay elVIy ehIymYrem:A nig yMxusFmμta.

kgVHeKalikaQamRkhmF¶n;F¶r k¾nwgjauMg[manynþkarkarBarmYycMnYn

ekIteLIgkñúgsarBagÁkay sMxan;CageK KWbegáInskmμPaBxYrq¥wg. dUc)anCMrab

ehIyfa eKalikaQamRkhmplitenAkñúgxYrq¥wg. edIm,IRbwgTb;Tl;nwgkar

fycuHéneKalikaQamRkhmkñúgcrnþQam xYrq¥wgbegáInskmμPaBelIsBIFmμta

RbmaNCa 30 dg edIm,IpliteKalikaQamRkhm[)aneRcIn. karbegáIn

skmμPaBenHk¾)anCMruj[q¥wg RtUvEtrIkFMEdr bgá[q¥wgk,almanTMrg;xusBI

Excess αα Globin Chains

� Major cause of anemia

� Precipitate in early nucleated

erythroid progenitors in marrow

� Lead to ineffective erythropoiesis

� Precipitate in mature red cells

� Lead to hemolysis

� Undergo proteolysis

-- absorbance

o..o..o radioactivity

-- absorbance

o..o..o radioactivity

22

Fmμta eFμjelIlyecjxøaMg ehIynwgkarrmYlénq¥wgCMnI nig q¥wgkgxñg .

q¥wgmansPaBesþIgCagFmμta (Osteopenia) ehIypuy CYnkalgaynwg)ak;.

eKalikaQamRkhmRtUveRcaHenAGeNþIk (Spleen) EdlCasrIragÁTTYlxusRtUv

kñúgkarkMeTcecalral;eKalikaQamRkhmEdlcas;RKb;GayuRtUvxUc.

Etya:gNak¾eday cMeBaHGñkCMgWtaLasesmuI GeNþIkRtUvra:b;rgkMeTceKalika

QamRkhmd¾eRcInsem,Im edaysarxYrq¥wgmanskmμPaBplitxøaMg.

CalT§pl GeNþIkxøÜnÉgRtUvEtskmμCagFmμta edayRbwgkMeTceKalika

QamRkhmEdlelIs ehIyvak¾cab;epþImrIkFM eKehAfa Hypersplenism.

bEnßmelIkarkMeTceKalikaQamRkhmEdlcas; GeNþIkEdlskmμxøaMg

k¾kMeTcpgEdrnUveKalikaQams/ pøaEkt nig eKalikaRkhmekμgx©I/ karenHva

rwtEteFVI[kgVHRKab;QamRkhmkan;EtF¶n;F¶reLIg. eKalikaQamRkhm

EdleFVIdMeNIrkñúgcrnþQamGñkCMgWtaLasesmIu CaeKalikamanragxusFmμta

edaysarxVHm:UelKul eGm:UkøÚb‘ÍnFmμta ehIyrEmgeTACab;kl;enAkñúgGeNþIk

eFVI[GeNþIkkan;EtFM. TIbBa©b; GeNþIkenHÉgb:unb:gcat;viFankarN_ Tb;kgVH

eKalikaQamRkhmrbs;sarBagÁkayedayplitRKab;QamRkhmedayxøÜn

ÉgEtmþg ¬karbegáItRKab;QamRkhmenAeRkAxYrq¥wg¦ karenHGeNþIkrwtEt

rIkkan;EtFM.

dUcenH karelIsExSGal;hVa ehIyKμantulüPaBcMnYnrvagExSGal;hVa nig

ExSEbtta CabuBVehtucMbgbgá[ekItCMgWtaLasesmuIkMritF¶n; CakarERbRbYl

muxgarEdlCab;Tak;TinCamYyCMgW.

sarBagÁkayxitxMTb;Tl;nwgkarkgVHeKalikaQamRkhmF¶n;F¶redayRsUb

ykCatiEdkBIcMNIGahar tambMBg;rMlayGaharkan;EteRcIn. eTaHya:genHkþI

vaRKan;EteFVI[sPaBkarN_kan;Etya:b;eTAeTot ³ GñkCMgWtaLasesmuITTYlrg

kgVHeKalikaQamRkhmedaysarkarplitRKab;QamRkhmBMuRKb;RKan; BMu

EmnedaysarkgVHCatiEdkeT. karRsUbCatiEdkkan;EteRcIn vaeFVI[sarBagÁ-

kayRbQmmuxnwgeRKaHfñak;fμI KWelIsbnÞúkCatiEdk.

23

kMNt;eraKvinicä½yCMgWtaLasesmuI

kumarekItmkmantaLasesmuI nwgminbgðajeraKsBaØa[eXIjeLIy. sUm,IEt

etsþmnÞIrBiesaFn_k¾braC½ykñúgkarrkeraKvinicä½yEdr CaBiessebIeKmin)aneFVI

etsþ«Bukmþay min)aneFVIetsþmuneBlTarkekIt ehIykñúgRKYsarKμankUnman

CMgWenH. ehtupllM)akeFVIeraKvinicä½ytaLasesmuIkñúgdMNak;kalGayudMbUg

rbs;kumaredaysarvaman HbF kñúgbrimaNRKb;RKan;edIm,IFana)annUv

tulüPaBkñúgcMnYnExSkøÚb‘Ín ¬Gal;hVa nig háam:a¦ EdlCaGñkbegáIt HbF

KWeFVI[kumartUc²eCosputBIdMeNIrGsmtßPaBkñúgkarpliteKalikaRkhm

dUc)anerobrab;xagedImmkehIy.

vaGacmanlT§PaBeFVIeraKvinicä½ytaLasesmuI enAGayudMbUg)aneday

meFüa)aybec©keTsm:UelKul EdlsMKal;nUvkarpøas;bþÚr (Mutations)

Edlkumar)anTTYlCalkçN³tMNrBUCBI«Bukmþay. eTaHya:gNak¾eday

kareFVIetsþenHTMngCaGnuvtþn_eBlsgS½yBiessNamYy Ca]TahrN_

eBlTarkekItBI«BukmþayEdlCa GñkpÞúkBn§úbgáCMgW (Carriers). CaGkusl

eTaHbICamankmμviFIBinitüRsavRCav (Screening programme) elITarkeTIb

ekItya:gl¥k¾edayk¾kareFVIetsþrkeraKvinicä½yCMgWtaLasesmuIkMritF¶n; swgEt

minGac[snñidæan)anenAdMNak;kalGayudMbUgeLIy. eTaHya:gNak¾eday

karBinitüRsavRCav GacmanRbeyaCn_kñúgkareFVIeraKvinicä½y rkRbePTkøay

epSg² dUcCa HbE b¤ HbS.

kñúgkrNIPaKeRcIn taLasesmuIkMritF¶n;GaceFVIeraKvinicä½y)anmunGayu 2 qñaM.

EttaLasesmuIkMritmFüm enAEtminGaceFVIeraKvinicä½y)ankñúgry³eBld¾yUr.

taragxageRkam bgðajBIlT§plénkarsikSaEdleFVIeLIgedayGñkRsavRCav

mYycMnYnelIbBaðaenH.

24

Gayu énkarbgðajeraKvinicä½yrbs;kumartaLasesmuIkMritF¶n; b¤ kMritmFüm

(from Modell and Berdoukas 1984)

Gayu ¬qñaM¦ taLasesmuIkMritF¶n; taLasesmuIkMritmFüm

<1 75 - 62° 4 - 11°

1-2 35 - 29° 11 - 30°

>2 11 - 9° 22 - 59°

viFIsaRsþEpñkelahitviTüa EdleKeRbICaTUeTAedIm,IeFVIeraKvinicä½y

taLasesmuIkMritF¶n;

(i) snÞTsSn_elahitviTüa ³ RKab;QamTaMgenHRtUv)anvas;Evgeday

]bkrN_eGLicRtUnic eKeRbIvaedIm,Ivas;TMhM nig maDeKalikaQamRkhm

nig brimaNeGm:UkøÚb‘Ín (Hb) EdlmankñúgeKalikaRkhm. kMNt;eraK

vinicä½ytaLasesmuI)aneBlNaTMhM nig maDeKalikaQamRkhm ehIynig

kMhab;eGm:UkøÚb‘ÍnkñúgeKalikaRkhmTaMgenaHmankarcuHfyya:gxøaMg eday

kMrit eGm:UkøÚb‘Ínrvag 2- 6 Rkam¼d>l ¬g/dl¦. xageRkamenH KWCa

snÞTsSn_ elahitviTüamYycMnYn EdleKeXIjmanTUeTAelIGñkCMgW

taLasesmuI ³

tMélmFüm KMlat ¬Range¦

Hb g/dl 6/8 3/9 - 9/3

¬brimaNmFümén Hb kñúgbrimaNQammYyrgVal;¦

MCH pg 20/9 15 - 26

¬TMgn;mFümén Hb kñúgeKalikaQamRkhmnImYy²¦

MCV fl 65/8 57 - 75

¬TMhMmFümrbs;eKalikaQamRkhm¦

MCHC g/dl 30/9 26 - 34

¬brimaNmFümén Hb kñúgbrimaNeKalikaQamRkhmmYyrgVal; ¦

25

cMnYneKalikaQam>s emIleTAhak;dUcCaekIneLIg edaysarvtþmand¾eRcIn

elIslb;éneKalikaQamRkhmekμgx©I ¬vaenAmansñÚl¦ Edl]bkrN_rab;

RKb;QamGacyl;RcLMfaCaeKalikaQam>seTAvij. eTaHya:gNak¾

eday karRcLMenHgaynwgEjk)anedayGegátEpñkmnÞIrBiesaFn_bnÞab;mk

eTot.

(ii) karBinitüQamedayBaselIkBa©k; (Blood film) nig rUbrageKalika

QamRkhm (RBC morphology) ³ BinitüedaymIRkUTsSn_ eKalikaQam

RkhmmansPaBesøkCag (Hypochromic) ¬emIlrUb 2k¦ ehIy

tUcCagFmμta (Microcytic). sMxan;bMputenaH PaKeRcInmanragxus

Fmμta xøHtUcxøHFM (Anisocytosis)/ xøHmanragreqjrqUj

(Poikilocyte).

(iii)eGLicRtUhV½rEr:séneGm:UkøÚb‘Ín (Hb electrophoresis) ³

enHCadMeNIrmYyEdlEjkRbUetGIunepSg² EdlbegáItCam:UelKul

eGm:UkøÚb‘Ín (Hb) dUcCa HbA, HbA2 nig HbF. eraKvinicä½ytaLasesmIu

GaceFVI)aneBlkMrit HbF x<s;CagFmμta ehIyGacERbRbYlenAcenøaH 20

- 90°. HbA2 EdlFmμtavamanrhUtdl; 3° éneGm:UkøÚb‘Ín ³ GacKμan

GacfycuH GacenAFmμta b¤ k¾ekIneLIgbnþic.

(iv)viFIviPaKm:UelKul (Molecular methods) ³ enHKWCameFüa)ayBiess

edIm,IbBa¢ak; b¤ TTYl)anBt’manBitR)akdkñúgkareFVIeraKvinicä½yedayGegát

elI DNA/ ]TahrN_edIm,IkMNt;karpøas;bþÚr (Mutations) EdlbgáCMgW.

eRkABIbBa¢ak;)annUveraKvinicä½y vak¾pþl;Bt’man[dwgpgEdrBIsPaBF¶n;F¶r

EpñkKøInikrbs;CMgW.

karGegátBinitüRKab;Qam k¾dUcCakarBinitükarpøas;bþÚrrbs;Bn§ú a/ β nig γ

KWCaCMhand¾sMxan;kMNt;eraKvinicä½ytaLasesmuIpg nig kMNt;karBüa)al

pg. eTaHbIeraKvinicä½y Ebtta taLasesmIukMritF¶n; EtgEtgayRsYl

2k

26

karlM)akk¾GacekItmanEdr CaBiessenAbNþaRbeTskMBugGPivDÆn_ Edl

manGRtaQWeRcIn dUcCaCMgWRKuncaj;GaceFVI[sμúRKsμajdl;kareFVIeraK

vinicä½y. ]TahrN_ RKuncaj;Gacbgá[xVHeKalikaQamRkhm nig GeNþIk

rIkFM ehIyeTaHCakarrkeXIjEpñkelahitviTüarbs;mnÞIrBiesaFn_vaxusKña

k¾eday vaGaccaM)ac;faRtUvBüa)alCMgWedayfñaMRKuncaj;sin munnwgeFVIkar

vaytMéleLIgvijnUvsPaB nig eraKvinicä½yrbs;CMgWtaLasesmIu.

CMgWepSg²eTot k¾Gacbgá[xVHRKab;QamRkhm nig GeNþIkrIkFMEdr ehIy

HbF ekIneLIgpgEdr ehIykareFVIeraKvinicä½yEjk KWCakarcaM)ac;eday

BinitüKøInik nigviPaKenAmnÞIrBiesaFn_bEnßm. vamansarsMxan;Nas;EdlRtUv

bBa¢ak;eraKvinicä½ytaLasesmuI[)anBitR)akdmuneFVIkarBüa)al.

CMgW eGm:UkøÚb‘Ín (Hb) kMritF¶n;epSg²

CMgW ExSGal;hVa køÚb‘Ín CMgW ExSEbtta køÚb‘Ín(a - globin chain disorders) (β - globin chain disorders)

a - thalassemias Sickle cell disorders

¬Gal;hVa taLasesmuI¦ ¬CMgW ekasikasIukxWl¦

� HbH disease � sickle cell anemia (HbSS)

� a - thalassemia hydrops foetalis � HbS/β - thalassemia

(= Hb Bart’s hydrops foetalis) � HbSC disease

� HbSD disease

� CMgW sIukxWl epSg²eTot

ββ - thalassemias

¬Ebtta taLasesmuI¦

� β - thalassemia major

¬Ebtta taLasesmuIkMritF¶n;¦

� β - thalassemia intermedia

¬Ebtta taLasesmuIkMritmFüm¦

� HbE/β - thalassemia

� CMgW taLasesmuI kMrepSg²eTot

27

karBüa)al Ebtta taLasesmuIkMritF¶n;

ry³eBl 3 TsvtSr_cugeRkayenH karBinitüGegátEpñkKøInik nigkarRsavRCav

)anbgðajfa taLasesmuIkMritF¶n;CaCMgWmYyGacBüa)al)an. karsikSa)an

bgðajfa karBüa)aledaybBa©ÚlQamCaeTogTat;nUvQamEdlBinitü)an

RtwmRtUv nig mansuvtßiPaBedaycMraj;ykCatiEdkya:gmanRbsiT§iPaB nig

eTogTat; )anbegáInKuNPaB nigkarrs;ranmanCIvitrbs;GñkCMgW)anya:geRcIn .

rebobBüa)alEdleKENnaMenH eKp©g;eTAelIkarRbyuT§nwgGRtakgVHeKalika

QamRkhmkñúgCMgWtaLasesmuI nig plvi)akrbs;va ehIynigp©g;eTAelI

karkarBarkMu[manelIsCatiEdkkñúgCalikabnþicmþg² EdlbNþalmkBICMgW

xøÜnÉgpg nigBIkarBüa)aledaykarbBa©ÚlQampg EdleRbIsMrab;Tb;Tl;

nwgkgVHeKalikaQamRkhm.

28

CMBUkTI 3

karBüa)aledaykarbBa©ÚlQam

karbBa©ÚlQamedayeTogTat; )anrYmcMENkd¾FMeFgdl;KuNPaB nig Gayu

kalCIvitGñkCMgWtaLasesmuIkMritF¶n; ehIyCaTidæPaBrYménkarBüa)al

taLasesmuI cab;taMgBITsvtSr_qñaM 1960.

ebIminmancMNat;karedayRbsiT§iPaBeT kgVHRKab;QamRkhmF¶n;F¶r nig

karrIkFMRCulénlkçN³xYrq¥wgrbs; taLasesmuIkMritF¶n; nwgnaMeTArk ³

� karrIkFMFat;yWtya:v

� karxusTMrg;minFmμtaénq¥wgmux nig q¥wgepSg²eTot

� q¥wgBukpuy nig kar)ak;q¥wg

� eføIm nig GeNþIkrIkFM

� karxUcxatdl;skmμPaBragkayFmμta

karbBa©ÚlQamya:geTogTat;elImUldæaneFVIeLIgGs;mYyCIvit GacTb;Tl; b¤

QaneTAdl;karBar)ankMu[ekItmaneraKsBaØa ¬rhUtdl;mankarBüa)al

taLasesmuIkMritF¶n;eGayCadac;NamYydak;[Gnuvtþn_¦. ya:gNak¾eday

manktþaCaeRcInRtUvEtykmkKitKUr eBlcab;epþImkarBüa)aledaykarbBa©Úl

Qam.

CMBUkenH nwgBiPakSaGMBI ³

1> etIeBlNaKYrcab;epþImkarBüa)aledaybBa©ÚlQam.

2> eFVIdUcemþcedIm,IFana[)ankarbBa©ÚlQamRbkbedaysuvtßiPaB.

3> etIeKRtUvbBa©ÚlQamRbePTGVI.

4> eFVIdUcemþceTIb)anQamsmRsbtamrebobrbbénkarBüa)al

edaybBa©ÚlQam .

29

etIeBlNaRtUvcab;epþImBüa)aledaybBa©ÚlQam

karBüa)aledaybBa©ÚlQamRtUvcab;epþImEteBlNa eraKvinicä½ytaLasesmuI

kMritF¶n;RtUv)anbBa¢ak;c,as;. dUc)anniyayxagelI/ eraKvinicä½ytaLasesmuI

kMritF¶n; Ep¥keTAelI ³

(i) etsþmnÞIrBiesaFn_/ ]TahrN_ viFIviPaKm:UelKul/ eGLicRtUhV½rEr:s

éneGm:UkøÚb‘Ín nig etsþmnÞIrBiesaFn_epSgeTot dUcCa High Pressure

Liquid Chromatography (HPLC).

(ii) karviPaKeTAelIBn§ú (Genetic analysis) edIm,IsÁal;RbePTénkarpøas;bþÚr

kñúgCMgWtaLasesmIu Ebtta nig Gal;hVa k¾dUcCavtþman Xmn1

restriction enzyme site EdlCakMNt;sMKal;mYyCYy[eyIgBüakrN_

BIsPaBF¶n;F¶énCMgW nig kMNt;BIrebobrbbBüa)alEdlsmRsbbMputcMeBaH

GñkCMgWnImYy².

PaBF¶n;F¶rénkgVHRKab;QamRkhm EtgEtRtUv)anvaytMéledayEp¥kelI

mUldæanénkMrit eGm:UkøÚb‘Ín (Hb) kñúgQam. eKKitCaRkamkñúgQammYy

edsIulIRt ¬d>l = 1¼100 lIRt¦. eGm:UkøÚb‘Ín gayRsYlvas;EvgkñúgmnÞIr

BiesaFn_edayeRbI ma:sIunrab;RKab;Qam. viFIcas;dUcCabec©keTs salI

(Sahli technique) k¾Gacvas;Evg eGm:UkøÚb‘Ín KYr[Tukcitþ)anEdr. kMrit

eGm:UkøÚb‘Ín Fmμta CaTUeTAeKKitfaenAcenøaH 13 - 16 Rkam¼d>l ¬g/dl¦

cMeBaHburs ehIy 11 - 14 Rkam¼d>l cMeBaHRsþI nig kumar. TaMgburs

TaMgRsþI eGm:UkøÚb‘Ín enAcenøaH 8 - 11 Rkam¼d>l tMNag[kgVHeKalika

QamRkhmkMritmFüm. ebIkgVHeKalikaQamRkhm

kMritF¶n; eGm:UkøÚb‘Ín ticCag 8 Rkam¼d>l.

GñkCMgWRtUvcab;epþImbBa©ÚlQamEteBlNataLasesmIu

RtUv)anbBa¢ak;tamry³eraKvinicä½yedaymnÞIrBiesaFn_ nig

karsikSam:UelKul ¬dUc)anerobrab;xagelI¦ ehIyeBl

30

Edl ³

� kMrit eGm:UkøÚb‘Ín (Hb) RtUv)ankt;RtaticCag 8 Rkam¼d>l kñúgGMLúgeBl

BIrdgbnþbnÞab;Kña XøatBIKñaCag 2 s)aþh_. CYnkalGñkCMgWGacmankMlaMg

BlM ehIyrIkFMFat;FmμtaeTaHCakMrit eGm:UkøÚb‘Ín enAcenøaH 6 - 7 Rkam¼d>l

k¾eday EtkarsMerccitþfaminbBa©ÚlQamkñúgeBlenH TamTarnUv

bTBiesaFn_KøInikd¾FMeFg nig karBinitüedayykcitþTukdak;.

� kMrit eGm:UkøÚb‘Ín elIsBI 7 Rkam¼d>l b:uEnþP¢ab;CamYysBaØarUbkay dUcCa ³

- TMrg;muxERbRbYl

- minFMFat; nig kareLIgTMgn;tictYc

- )ak;q¥wg

- karplitRKab;QamenAeRkAxYrq¥wg bgðajedayeføIm nig GeNþIkrIkFM

ebImanbBaðaTaMgenH karbBa©ÚlQamminRtUvBnüaeBleLIy.

Fana[)ankarbBa©ÚlQammansuvtßiPaB

QamRtUvEtmanRKb;RKan; b¤ gayrk)an

� edaykarbBa©ÚlQam KWCakarBüa)aleFVIeLIgGs;mYyCIvitcMeBaH

taLasesmuI GaC£aFrsuxaPi)alRtUvCMrujeKalneya)aypþl;QamedIm,I

Fana[)annUvkarpÁt;pÁg;Qamdl;GñkCMgW)anRKb;RKan; nig CaGciéRnþy_.

karBar[)ankMu[mankarcMlgPñak;garbgáeraK

� karbBa©ÚlQamRtUveFVIeLIgedaysuvtßiPaB tamEdlGaceFVIeTA)ansMrab;

GñkCMgW. enH)anesckþIfa eRKaHénPñak;garbgáeraKdUcCa vIrus/ )ak;etrI b¤

)a:ra:sIut EdlqøgtamQampþl;eTAGñkCMgW RtUveFVIya:gNa[mantictYc

bMput. edIm,Ikat;bnßyeRKaHfñak;qøgPñak;garbgáeraKtamry³karpþl;Qam/

GaC£aFrsuxPaBsaFarN³RtUv ³

31

- CMrujeKalneya)ayeRCIsykGñkpþl;QammansuxPaBl¥ nig CMrujesva

pþl;Qamedaysμ½RKcitþ nig CaeTogTat;. karpþl;Qamedaybg;R)ak;kas

RtUveCosvag ehIyeKminTamTar[GñkCMgWrkmitþPkþi b¤ bgb¥ÚnedIm,ICafñÚr

sMrab;karbBa©ÚlQam.

- Qamrbs;Gñkpþl; RtUvEtBinitürkPñak;garbgáeraKsMxan;²EpñkKøInik dUcCa

vIrusrlakeføImRbePT B nig C/ HIV1+2 ¬Pñak;garbgá[ekItCaCMgW

eGds¾¦ nig eraKsVay. enARbeTsmYycMnYn GacfacaM)ac;RtUvBinitürk

Pñak;garbgáeraKepSg²eTot dUcCaRKuncaj; cMeBaHtMbn;NaEdlvaman.

- RtUvFana[)anfa bNþaesvaCatipþl;QamrYmTaMgmnÞIrBiesaFn_ nig esva

epSg²eTotpþl;edaymCÄmNÐlpþl;Qam dUcCa karsþúk nig karbBa¢Ún

plitplQamRtUveqøIytbeTAnwgniyamGnþrCati.

EhSNUTIbRkumQam (Blood group genotype)

suvtßiPaBQamk¾Cab;Tak;TgCamYyktþaepSg² dUcCa RtUvFana[)anfaGñkCMgW

RtUv)aneFVIetsþsMrab;RbB½n§RkumQamsMxan;² tamEdlGaceFVIeTArYc.

]TahrN_ GñkCMgWRtUveFVIetsþrkRkumQamrbs;BYkeK ehIyRtUvdwgfa erhSús

(Rhesus = Rh) vIC¢man b¤ GviC¢man rYmCamYyRbB½n§RkumQamepSg²eTot

dUcCa Kell, Kidd nig Duffy . eBlcab;epþImbBa©ÚlQamPøam/ vaGaclM)ak

kñúgkarkMNt;RbePTRkumQamTaMgGs;Edlman. dUecñHvasMxan;RtUveFVIetsþrk

ehSNUTIbeKalikaQamRkhm munbBa©ÚlQamelIkdMbUg. ykl¥GñkCMgWRtUv

eFVIetsþrkvtþmanGgÁbdibkçfμI² cMeBaHeKalikaQamRkhmmunerogral;kar

bBa©ÚlQam. karpÁÚrpÁg (Matching) [)aneRcIntamEdlGaceFVIeTA)annUv

RbB½n§RkumQamGñkCMgW nig Gñkpþl; KW CameFüa)ayénkarkat;bnßyRbtikmμ

EdlmanCamYyRkumQamminsIuKña. eKalikaQamRkhmmanRbB½n§RkumQam

26 rYmmanGg;TIEhSn 600 xus²Kña. eBlEdleyIgminGacpÁÚrpÁg [)an

TaMgGs;eT/ ya:gehacNas;k¾RtUvxitxMpÁÚrpÁgRbB½n§RkumQamEdlman

jwkjyCageKEdr dUcCa ABO, RbB½n§ Rhesus nig Kell.

32

etIQamGVIRtUvbBa©Úl

QamEdlRbmUl)anBIGñkeFVIGMeNay eKehAfa QamTaMgmUl (whole

blood) EdleRkABImaneKalikaQamRkhm vak¾manpøasμa/ eKalikaQam>s

nig pøaEkt ¬sUmemIlvKÁsþIBIQam¦. RbmaN 450 m>l énQam ¬= 1

Ékta¦ EdlbUmBIGñkbric©aKQammñak;². eBlNapøaEkt/ eKalikaQam>s

nig ekasikaepSg²eTotRtUv)andkecj/ enAsl; RbmaN 250 m>l

eTotCaeKalikaQamRkhmeKehAfa mYyÉktaéneKalikaQamRkhm

Rckkñúgfg;TuksMrab;bBa©Úl. vtßúravepSg²eTotmandUcCa sarFatuTb;

kMNkQam (Anticoagulant) nig sarFatucBa©wmRKab;Qam k¾RtUv)anbEnßm

cUlkñúgfg;QamEdr ehIybrimaNvaGaRs½yelIviFIykQammkerobcM.

GñkCMgWtaLasesmIukMritF¶n; mankarxVHeKalikaQamRkhm. dUcenHGñkCMgW

EdlRtUvkarbBa©ÚlQam ykl¥KYrEtTTYlEteKalikaQamRkhmsuT§ehIy

KμanpÞúksarFatuepSgrbs;QamTaMgmUleLIy dUcCa )aøsμa/ eKalikaQam>s

nig pøaEkt. RbsinebIGñkCMgWTTYlQamTaMgmUl vamaneRKaHfñak;edayehtufa

RbB½n§srésQamnwgTTYlpÞúkelIscMNuH ehIyvivDÆn_eGaymanplvi)ak

epSg² dUcCa exSayebHdUg elIsCatiTwkkñúgtMbn;RTUg ¬ehImsYt¦. karyk

eKalikaQam>s nig pøaEktecjBIQamTaMgmUl k¾CYybnßyeRKaHfñak;énpl

EdleyIgmincg;)an dUcCaekþAxøÜnGMLúgeBl nigeRkaykarbBa©ÚlQam.

eTaHCasBaØaTaMgenHGacBüa)alk¾eday eKRtUvEtxitxMbeBa¢ósnUvplvi)ak

TaMgenH edaypþl;EtsmasFatuénQamNaEdlGñkCMgWRtUvkar.

etIeKTTYleKalikaQamRkhmdak;fg;dUcemþc

eKalikaQamRkhm RtUvEjkBIQamTaMgmUlenAÉmnÞIrBiesaFn_ tamry³

dMeNIrénkarbgVil (Centrifugation) EdlGacEjkecj)an pøasμa nig

eKalikaQam>s BIQamTaMgmUldl;Cag 70°. kñúgdMeNIrénkarbgVilQam/

eKalikaQamRkhm Ejkdac;BIeKedayenA)at Épøasμa/ eKalikaQam>s nig

33

pøaEktenAxagelI eFVI[gayRsYldkykecj. sUluysüúgmYycMnYnGac

bEnßmeTAelIeKalikaQamRkhm dUcCa sarFatuTb;kMNkQamEdlkarBar

ekasikakMu[s¥itCab;Kña/ sarFatuciBa©wm b¤ sarFatubEnßmEdlGacBnüaCIvit nig

EfrkSaKuNPaBeKalikaQamRkhm dUcCa SAGM (saline, adenine,

glucose, mannitol), PAGGS-M (phosphate, adenine, glucose,

guanosine, saline-mannitol), AS-3 (Nutricel system) nig ADSL

(adenine, dextrose, saline-mannitol) suT§swgCasUluysüúgbEnßmEdl

eKeRbI. CPD (citrate, phosphate, dextrosel), CPDA-1 (citrate,

phosphate, dextrose, adenine) nig CP2D (citrate, phosphate, double

dextrose) suT§swgBYkRbqaMgkMNkQam.

enAGWr:ubeKeRbIbBa©ÚlKñanUv CPD/SAG-M edIm,IrkSaCIvitrbs;eKalika

QamRkhm dl; 42 - 48 éf¶/ ÉenAshrdæGaemrikvij eKeRbIbBa©ÚlKña

CPDA-1/AS-3 b¤ eRbI CP2D/AS-3.

GñkCMgWtaLasesmuI RtUvTTYlkarbBa©ÚlQamedayeRbIeKalikaQamRkhm

suT§dak;fg; Cakarl¥QamEdlrkSaTukmin[elIsBI 7 éf¶. sUm,IenAkEnøgNa

EdlmaneRbIsarFatuciBa©wmeKalikaQamRkhmdUcerobrab;xagelIk¾eday/

eKminRtUvbBa©ÚlQam[GñkCMgWnUvQamEdlrkSaTukelIsBI 2 s)aþh_eLIy

BIeRBaHBMuTan;mankarsikSaKYr[eCOCak;NamYyEdlGHGagfa eKalika

QamRkhmEdlsþúkkñúgry³eBlyUr vapþl;RbeyaCn_kñúgkarBüa)alCMgW

taLasesmuIkMritF¶n;)anl¥dUceKalikaQamRkhmEdlsþúkkñúgry³eBlxøI

eLIy.

viFIepSg²eTotEdlGaceFVI[RbesIrnUvKuNPaB nig suvtßiPaBQam rYmman ³

i. lagsMGateKalikaQamRkhmEdldak;kñúgfg; ³

vtþmanRbUetGIunkñúgQambBa©ÚleGayGñkCMgW Gacbgá[manRbtikmμkñúgxøÜn

GñkCMgWIBIkMritmFümeTAxøaMgkøa)an. eTaHbIdMeNIrénkarbgVilQamdUc

erobrab;xagelI)andkecjCag 70° énpøasμa nig RbUetGIunBIQam

34

TaMgmUlk¾eday k¾KuNPaBéneKalikaQamRkhmdak;fg; GacnwgeFVI[

RbesIreLIg)aneRcInEfmeTot edaykarlagvakñúgTwkesrU:méRb (Saline

solution) edIm,Idk[)anGtib,rmanUvpøasμa nig RbUetGIun. CaTUeTA/ cMeBaH

GñkCMgWPaKeRcIn minTamTar[eFVIkarlageKalikaQamRkhmenaHeT.

karlageKalikaQamRkhmeKRtUveRbIeBlrvag 24 ema:g.

ii. sMGateKalikaQam>s BIfg;eKalikaQamRkhm ³

mKÁúeTsn_GnþrCati)anENnaMfa cMnYneKalikaQam>s RtUv[ticCag 1 x

106 kñúgmYyÉktaéneKalikaQamRkhm edIm,I[Rbtikmμbgáeday

eKalikaQam>s sßitkñúgkMritGb,rma. RbtikmμTaMgenHEdlEtgEtbgá[

manekþAxøÜn eKeCOfaedaysarEteKalikaQam>s beBa©jnUvsarFatuKImI

eQμaHfa exm:UKIn (Chemokines). elIsBIenHeTot karykeKalika

Qam>secj mann½yfa ykPñak;garbgáCMgWEdlmankñúgeKalikaQams

ecj dUcCa )ak;etrI Yersinia Enterocolitica, CMV, EBV, B19 nig HAV

¬sUmemIlenATMB½rBakübMRbYj¦ EdlCaPñak;garbgáCMgWedaymin)ansMGat

enAmCÄmNÐlpþl;Qam ehIykñúglkçxN½ÐxøH vanwgbgá[ekIteraKF¶n;F¶r.

viFIsMGateKalikaQams (Leucoreduction) man ³

¬k¦ kareRcaH (Filtration) ³ eKalikaQam>s nig Pñak;garbgáCMgWEdlman

Cab;CamYyvaGacRtUv)andkecjBIQamrbs;Gñkbric©aKya:gman

RbsiT§iPaBbMputedayeRbItMrgBiessEdlP¢ab;nwgfg;Qam. mKÁúeTsn_

GnþrCati)anENnaM[eFVIcMeraHQameRkayBIRbmUlQam)an rvag 4-8

ema:g edaycenøaHeBlenHeKsþúkvakñúgTUrTwkkksin. karBnüakñúgkareFVI

cMeraHenH GacTuk»kas[katalIkrkñúgeKalikaQam>s (Phagocytes)

eFVIkarkMeTc)ak;etrI (Phagocytosis) dUcCa )ak;etrI Yersinia

Enterocolitica EdlvaGaccUlkñúgeKalikaQam>s ehIyEbkkUnenAkñúg

enaH ¬emIlrUbenATl;mux¦. cMeraHGaceFVI)anenAmCÄmNÐlpþl;Qam b¤

enAERKrbs;GñkCMgWkñúgeBlbBa©ÚlQam. eTaHbIkareFVIcMeraHenAERKrbs;

35

GñkCMgWGaceFVI)anedaymanRbsiT§iPaBk¾eday EtvaGaclM)aknwgFana

)annUvkarRtYtBinitüKuNPaB.

kñúgb:unμanqñaMcugeRkayenH kareFVIcMeraHeKalikaQam>s (Leucofiltration)

kan;Etmansar³sMxan;bEnßmeTot CaBiesssMrab;GñkCMgW Ebtta

taLasesmuI v½yekμgEdl)aneFVI b¤ manEpnkarRtUveFVIkarbNþúHxYrq¥wg (Bone

marrow transplatation). enHk¾edaysareKalikaQams manP¢ab;mkCamYy

nUvvIrusmYyehAfa sIutUem:háaLÚvIrus (Cytomegalovirus) EdlGacbgáeraK

F¶n;F¶r b¤ rhUtdl;søab; cMeBaHGñkCMgWEdlmanRbB½n§karBarexSay (Immune

supressed) dUckrNIGñkCMgWbNþúHxYrq¥wg CaedIm.

eBlEdlmCÄmNÐlpþl;QamBMumanlT§PaBGnuvtþkarEjkpøasμa b¤ lag b¤

cMeraHQamtamviFIdUcerobrab;xagelI)an/ QamTaMgmUlEdlbUm)anBIGñkpþl;

RtUvsþúkTukkñúgTUrTwkkkenAsItuNðPaB 4oC ry³eBl 24 - 48 ema:g/ Tuk[

eKalikaQamRkhmrgenA)atfg;Qam ehIypøasμa/ eKalikaQam>s/ pøaEkt

nig FatuepSg²eTot EdlTaMgGs;enHeKehAfa RsTab;BN’etñat vaGENþt

mkEpñkxagelIEdleKGacykvaecj)angaybMput edayeRbI]bkrN_mnÞIr

BiesaFn_samBaØ. eTaHya:gNak¾eday/ viFIEbbenHmanRbsiT§iPaBticCag

1000 dg eRbobeFobnigkareRcaHQam.

RbsiT§iPaBénbec©keTsepSg²kñúgkarcMraj;eKalikaQam>secj

� RsTab;BN’etñatEdlykecjBIQam eRkayBITukeGayrgedayxøÜnÉg

kñúgTUrTwkkk/ karenHQamenAsl;maneKalikaQamsticCag 1,2 x

109/Ékta.

� eKalikaQamRkhmEdlEjk)anedaykarbgVilQam nig karlagkñúg

TwkesrU:méRb ¬Saline solution¦/ vamaneKalikaQams ticCag 1 x

107/Ékta.

� eKalikaQamRkhmEdl)anBIcMeraHeFVIenAÉERKGñkCMgW/ vamaneKalika

blood filter

phagocyte filled with

bacteria

36

Qam>s ticCag 5 x 106/Ékta.

� eKalikaQamRkhm Edl)anBIcMeraHeFVIenAÉmnÞIrBiesaFn_mCÄmNÐl

pþl;Qam/ vamaneKalikaQam>s ticCag 5 x 106/Ékta.

� eKalikaQamRkhmEdleRcaHmunykmksþúkTuk/ vamaneKalikaQam>s

ticCag 1 x 106/Ékta.

CakarRbesIr/ RKb;GñkCMgWtaLasesmIukMritF¶n;TaMgGs;RtUvTTYlkarbBa©Úl

QamEdlCaeKalikaQamRkhmsuT§ Edl)aneFVIcMeraHmunykmksþúkTuk/

)anlagsMGat/ )aneFVIkarpÁÚrpÁgPaBRtUvKñaya:geBjelj ehIymanbEnßm

sUluysüúg RbqaMgnwgkMNkQam nig sarFatuciBa©wmRKab;Qam. ya:gNa

k¾eday eBlNaKμanlT§PaBeFVIeTA)an eKRtUvBicarNadl;karerobcMQam

BiesssMrab;GñkCMgW EdlTamTarnUvlkçN³ dUcCa ³

1> eKalikaQamRkhm Edl)anlagsMGat (Washed RBCs)

RtUverobcMsMrab;GñkCMgWEdlxVH RbUetGIunBiesskñúgQam ehAfa IgA nig

sMrab;GñkCMgWEdl]sSah_man RbtikmμGalELhSIúF¶n;F¶r (Severe

allergic reactions) eBlBüa)aledaykarbBa©ÚlQam. GñkCMgWxøHKμan

kgVH IgA eT EtmanekþAxøÜndEdl²eBlbBa©ÚlQamcMeraH krNIenHk¾

TamTar[lageKalikaQamRkhmmuneBlbBa©ÚlEdr.

2> eKalikaQamRkhmRtCak;kk (Frozen b¤ cryo-preserved RBCs)

eRbIcMeBaHGñkCMgWEdlmanGg;TIEhSnkMrelIeKalikaQamRkhm eRBaH

QamBYkeKBi)akeFVIkarsakl,grkPaBRtUvKña (Cross-match) CamYy

QamGMeNay[)anc,as;las;. enAGWr:ub nig shrdæGaemrik/ eKalika

QamRkhmRtCak;kkEdl)anBIRkumQamkMr eKrkSavakñúgmCÄmNÐl

evC¢saRsþBiess edayrkSakMrit -60oC kñúgsUluysüúg Glycerol 40%

EdlCalkçx½NÐmYyGac[eKsþúkva Tuk)anrhUt 10qñaM.

3> eKalikaQamRkhm)aj;edaykaMrsIμ (RBCs treated with radiation

37

eRbIcMeBaHGñkCMgWEdlTTYlkarbNþúHxYrq¥wg b¤ GñkCMgWeRtomTTYlkarbNþúH

xYrq¥wg edIm,IeCosvagekItPaBF¶n;F¶rmYyehAfa CMgWTMnas;bnSaMsrIragÁ

(Graft versus host disease) ¬GanenAxageRkay¦.

4> eKalikaQamRkhmEdl)andkeKalikaQam>secj

(Leucoreduced RBCs) eRbIcMeBaHGñkCMgWEdl]sSah_manRbtikmμ

CamYyeKalikaQam>s eRkaykarbBa©ÚlQam. QamEbbenH k¾Tukpþl;

[pgEdrdl;GñkCMgWv½yekμg eTIbnwgeFVIeraKvinicä½y ehIymanKMeragRtUv

eFVIkarbNþúHxYrq¥wg edIm,IecosvagkarbgáeraKeday sIutUem:háaLÚvIrus

¬Cytomegalovirus = CMV¦/ k¾dUcCaTukpþl;[GñkCMgW Edl)anbNþúH

xYrq¥wgrYcehIy EtRtUvkarbBa©ÚlQam edIm,IeCosvagkarbgáeraKCafμIeday

sIutUem:háaLÚvIrus.

kMNt;rbbbBa©ÚlQam

KNnatMrUvkarbrimaNQam

brimaNQamEdlGñkCMgWRtUvkar nig kMritbBa©ÚlQamGaRs½yeTAelIGayu

GñkCMgW nig sPaBKøInik/ eTAelIsUluysüúgbEnßm edIm,IrkSaeKalika

QamRkhm/ eTAelIeGm:atURKIt (Hematocrite) rbs;QamGñkpþl; nigeTAelI

kMritcg;)anrbs; eGm:UkøÚb‘Ín.

PaKeRcInénGñkCMnajEpñkCMgWtaLasesmuI )aneGayGnusasn_fa karbBa©Úl

QamRtUveFVIedaylkçN³mFüm min[elIs/ CaFmμta GñkCMgWTTYleKalika

QamRkhmxab;kMrit 10 - 15 ml kñúgTMgn;mYyKILÚRkam/ bBa©Úlkñúg

ry³eBl 3 - 4 ema:g ¬Ep¥kelImUldæan eGm:atURKIt 75° éneKalika

QamRkhmrbs;Gñkpþl;Qam¦ ¬kMritbBa©ÚlQam¦ erogral; 2 eTA 5

s)aþh_ ¬KMlatbBa©ÚlQam¦. GñkCMgWminkat;GeNþIk RtUvkareKalika

QamRkhmsuT§RbmaN 180 ml¼KRk¼qñaM/ ÉGñkCMgWkat;GeNþIkvij RtUvkar

RbEhl 133 ml¼KRk¼qñaM.

38

krNIGñkCMgWmanbBaðaebHdUg b¤ krNIbBa©Úl

Qamcab;epIþmeBlkMrit eGm:UkøÚb‘Ín (Hb)

TabCag 5 Rkam¼d>l (g/dl) eBlenaHeK

bBa©ÚlQamkñúgbrimaNticedaykMrityWt

CagFmμta/ ]TahrN_ 2 - 5 ml¼KRk¼

em:ag. eKmantarag nig RkahiVk ¬emIlrUb

3a, 3b¦ edIm,ICYydl;buKÁlikeBTükñúgkar

KNnabrimaNQamBitR)akdEdlGñk

CMgWRtUvkar.

rbbbBa©ÚlQam KYrmaneKalbMNgrkSa kMrit eGm:UkøÚb‘Ín rbs;GñkCMgWeGay

enAcenøaH 9 - 10/5 Rkam¼d>l muneFVIkarBa©ÚlQam ehIyminRtUveGay

elIs 15 Rkam¼d>l eRkaykarbBa©ÚlQam. eRkamkarkMritEbbenH GñkCMgW

nwg)anTTYl ³

� rIkxYrq¥wgkñúgkMritGb,brima

� lUtlas;FMFat;Fmμta nig famBlragÁkayekIneLIg

� BMumankarrIkGeNþIk b¤ BnüaeBl)anyUrmineGayrIkGeNþIk

� kat;bnßykMhab;Qam Edlvagaybgáplvi)akeGaysÞHsrésQam

edaybNþúMRKab;Qam/ QWk,al nig QamRcmuH

� karelIsCatiEdkenAkñúgsrIragÁ)anfycuH

KMlatbBa©ÚlQam GacecaTbBaðadl;ktþa

epSg²Edr rYmman kargar b¤ karsikSarbs;

GñkCMgW nig cMgaypøÚvEdlGñkCMgWRtUveFVIdMeNIr

eTAmCÄmNÐlbBa©ÚlQam. Kit[Evgq¶ay

eTAkarkMNt;eBlevlabBa©ÚlQamRtUverobcM

[s½kþismnwgEbbbTrs;enArbs;GñkCMgWk¾dUc

CaeqøIytbtamtMrUvTamTarEpñkevC¢saRsþ .

3a Amount of donor blood, depending on

haematocrit, required to raise the

patient’s haemoglobin by 1 g/dl

3b Guidelines for choosing

how much blood to

transfuse

39

vaytMélRbsiT§iPaBénrbbbBa©ÚlQam

RbsiT§iPaBénkmμviFIbBa©ÚlQam eKEtgvas;EvgtamGRtaFøak;cuHkMrit

eGm:UkøÚb‘Ín (Hb) EdlminelIs 1 Rkam¼d>l¼s)aþh_ cMeBaHGñkCMgWkat;

GeNþIkecj nig 1/5 Rkam¼d>l¼s)aþh_ cMeBaHGñkCMgWmin)ankat;GeNþIk.

ebIeKeXIjfakMrit eGm:UkøÚb‘Ín Føak;cuHkñúgGRtaelOn/ eKGacRsavRCavnUv

buBVehtudUcteTA ³

� manbegáItGgÁbdibkçRbqaMgnwgeKalikaQamRkhmEdlbBa©Úl

(Alloimmunisation) ¬sUmGan RbtikmμTak;TgnwgkarbBa©ÚlQam¦.

� GeNþIkrIkFM (Hypersplenism) nig¼b¤ eføImrIkFM (Hepatomegaly).

]TahrN_ krNIGñkCMgWRtUvkareKalikaQamRkhmeRcInCag 200

ml¼KRk¼qñaM/ RtUvEtRsavRCavrklT§PaBrIkFMénGeNþIk.

� KuNPaBQamminsUvl¥ )anesckþIfa eKalikaQamRkhmmanCIvit

xøI ehIybMeBjmuxgarminsUvmanRbsiT§iPaB.

� mankarhUrQam ¬]TahrN_ enAeBaHevon¦.

� karbMpøajeKalikaQamRkhmmankarekIneLIg edaysarkar

eRbIfñaM ¬]TahrN_ Ribavirin¦.

� karbMpøajeKalikaQamRkhmmankarekIneLIg edaysarkar

bgáeraK ¬]TahrN_ CMgWRKuncaj;¦.

ykl¥eKRtUvvas;kMrit eGm:UkøÚb‘Ín mun nig eRkaykarbBa©ÚlQam edIm,IeFVIkar

vaytMélrbbkarBüa)al. ebIsineFVImin)aneT eKKYrEtvas;kMrit eGm:UkøÚb‘Ín

tamlT§PaBeFVI)anmYydgkñúgmYys)aþh_/ ral; 15éf¶mþg b¤ eBlNaGñkCMgW

TTYlkarbBa©ÚlQam.

40

RbtikmμTak;TgnwgkarbBa©ÚlQam

karbBa©ÚlQamGacbgá[manRbtikmμ EdleyIgmincg;)an b¤ plvi)akcMeBaH

GñkCMgW eKehAfa RbtikmμénkarbBa©ÚlQam. RbtikmμénkarbBa©ÚlQamCa

RBwtþikarN_eFVITukçEdlekItkñúgxøÜnGñkCMgWGMLúgeBl b¤eRkaykarbBa©ÚlQam.

RbmaN 4° én karbBa©ÚlQam ¬ehIyebIQammanKuNPaBminl¥/ vaGac

maneRcInCagenH¦ bgáeGaymanTMrg;xøHnUvRbtikmμ b¤ RbtiskmμEdleyIgmin

cg;)an. karbBa©ÚlQamCakarbBa©ÚlnUvsarFatuxageRkA ¬QamGñkpþl;¦ cUlkñúg

xøÜnGñkCMgW. dUcenHvaCakarsmehtupl EdlRtUvEtrMBwgTukfamanRbtikmμ

ekItecjBIRbB½n§karBarragÁkayrbs;GñkCMgW dUcKñanwgRbtikmμEdleKrMBwgTuk

eBlbNþúHsrIragÁ b¤ bNþúHxYrq¥wgEdr. tamBiteTA ral;nitiviFIevC¢saRsþman

plRbeyaCn_ k¾dUcCamaneRKaHfñak;xøaMgkøacMeBaHGñkCMgW EdlbuKÁlikeBTü

RtUveFVIkarvaytMéledayykcitþTukdak;.

RbtikmμEdlGacekItmancMeBaHGñkCMgW EdlTTYlkarbBa©ÚlQam EckCaBIr

RbePT ³

kk>> RbtikmμénkarbBa©ÚlQamedayTak;TgnwgRbB½n§karBarragÁkay

CakrNIRbB½n§karBarragÁkayrbs;GñkCMgWeqøIytbeTAnwgQam

EdlbBa©ÚleTA.

xx>> RbtikmμénkarbBa©ÚlQamedayminTak;TgnwgRbB½n§karBarragÁkay

CaRbtikmμEdlminEmnekItecjBIRbB½n§karBarragÁkayrbs;GñkCMgW .

RbtikmμTaMgBIrRbePTenH GacekIteLIgGMLúgeBl b¤ eRkaykarbBa©ÚlQam

¬RbtikmμRsYcRsav¦ ekIteLIgbnÞab;BIbBa©ÚlQam)an 2 b¤ 3 ml/ GMLúg

eBlbBa©Úl b¤ eRkaykarbBa©ÚlQam)anbBa©b; b¤ RbtikmμmansPaByWt

ekIteLIgeRcInéf¶ b¤ eRcIns)aþh_eRkaykarbBa©ÚlQam b¤ k¾yUrCagenH.

taragxageRkamenH ¬1/ 2/ 3¦bBa¢ak;BIRbtikmμRbePT ¬k¦ nig ¬x¦ Edl

GacekItmaneLIg ehIynigPaBjwkjb;rbs;va ¬tarag 3c, 3d, 3e¦.

41

¬kñúgEpñkenHmankareRbIR)as;Bakü bec©keTsevC¢saRsþeRcIn EdlnwgmankarlM)aksMrab;GñkGan

fñak;mFümEdlcaM)ac;RtUvemIlniymn½yBaküenAkñúgsTÞanuRkm/ sgÆwmfakarCUnniymn½yBakü

TaMgenHeFVI[GñkCMgWnigmatabitamanCMenOcitþxøaMgeLIgkñúgkareFVITMnak;TMngCamYybuKÁlikeBTü¦

taragelx 1 ³

RbtikmμénkarbBa©ÚlQamedayTak;TgnwgRbB½n§karBarragÁkay

Acute Frequency Delayed Frequency

¬RsYcRsav¦ ¬PaBjwkjab;¦ ¬BnüaeBl¦ ¬PaBjwkjab;¦

Hemolytic (intravascular) 1¼25>000 Alloimmune 1¼100[EbkeKalikaQamRkhm ¬kñúgsrésQam¦]

Anaphylactic 1¼50>000 Hemolytic (extravascular) 1¼2>500[EbkRKab;QamRkhm ¬eRkAsrésQam¦]

Febrile non-hemolytic 1¼100 Platelet refractoriness 1¼10¬ekþAxøÜn edayKμanEbkeKalikaQamRkhm¦

Allergy (Urticarial) 1¼100 Graft vs Host Disease (GVHD) rare

¬TMnas;bnSaMsrIragÁ¦ ¬kMr¦

Transfusion Related Acute Lung 1¼1>000Injury (TRALI)

¬rbYssYtRsYcRsavbNþalmkBIkarbBa©ÚlQam¦

3c

42

Acute Frequency Delayed Frequency

¬RsYcRsav¦ ¬PaBjwkjab;¦ ¬BnüaeBl¦ ¬PaBjwkjab;¦

Hemolytic (in vitro) Metabolic iron overload Long term

[EbkeKalikaQamRkhm ¬kñúmnÞIrBiesaFn_¦] ¬elIsCatiEdkkñúgemtabUlIs¦ ¬ry³eBlyUr¦

Metabolic : Infectious :¬Tak;TgnwgkarbgáeraK¦

Coagulopathy ¬CMgWvibtþikMNkQam¦ kMr 1. Bacterial ¬)ak;etrI¦

Hypothermia ¬sItuNðPaBcycuH¦ kMr Gram negative 1¼15>000Citrate toxicity ¬BulCatisuIRtat¦ kMr Gram positive 1¼2>000Hypocalcaemia ¬elIskMritCatikal;süÚm¦ kMr 2. Viral* ¬vIrus¦ 1¼15>000Hyperkalaemia ¬elIskMritCatib:UtasüÚm¦ kMr HBV 1¼60>000

HCV 1¼100>000HIV 1&2 1¼700>000HTLV 1&2 1¼650>000CMV 7¼100E-B 1¼200B19 kMr

3. Prions :Creutzfeld Jakob ?

4. Parasitic** ¬]> CMgWRKuncaj;¦ kMrEmbolic ¬sÞHsrésQamedayvtßúGVImYy¦ kMrCirculatory overload 1¼10>000¬elIscMNuHQamkñúgRbB½n§crnþQam¦

taragelx 2 ³

RbtikmμénkarbBa©ÚlQamedayminTak;TgnwgRbB½n§karBarragkay

* PaBjwkjyénkarcMlgvIrus ERbRbYlxøaMgeTAtambNþaRbeTsnana GaRs½yelIKuNPaBén

suxPaBsaFarN³ ehIynigesvabBa©ÚlQam nig eRbva:Lg;énPñak;garbgáCMgWTaMgenHtamtMbn;.

PaBjwkjyEdlbgðajkñúgtaragenH KWyktamr)aykarN_TUeTA enAGWr:ubkñúgb:unμanqñaM

cugeRkayenH.

** PaBjwjyénkarcMlg)a:ra:sIut vamanCaTUeTAenAbNþaRbeTskMBugGPivDÆn_.

taragelx 3 bgðajBIbuBVehtuénRbtikmμmYycMnYn Tak;TgnwgkarbBa©ÚlQam/ dMNak;kalEdl

RbtikmμekItmanjwkjab;bMput nig eraKsBaØaP¢ab;CamYy.

3d

43

taragelx 3 ³

buBVehtu/ eBlkekItRbtikmμ nig eraKsBaØaRbtikmμEdl Tak;TgnwgkarbBa©ÚlQam

Type of reaction Timing Cause Symptoms

¬RbePTRbtikmμ¦ ¬eBlkekIt¦ ¬buBVehtu¦ ¬eraKsBaØa¦

EbkeKalikaQamRkhmPøam² bnÞab;BIbBa©ÚlQam)an RkumQam ABO minRtUvKña ht;/ ENnRTUg/ ekþA/ jak;/ QWcegáH/

2 - 3 ml sMBaFQamFøak;cuH/ Shock/exSaytMrgenam

Anaphylactic “ kgVH IgA BIkMeNIt RkhmEs,k/ knÞalRtGak rmas;/ht;/ k¥Üt/raK/

QWRTUg/ sMBaFQameLIg/ )at;sμartI/ Shock

Air embolism “ xül;cUlkñúgRbB½n§srésQam k¥k/ ht;/ QWRTUg nig Shock

¬dMuxül;kñúgsésQamsYt¦

Sepsis enAcugbBa©b; b¤ eRkay )ak;etrIqøgtamQamEdlbBa©Úl ekþA/ jak;/ k¥Üt/ raK/ sMBaFQamFøak;cuH/

¬qøgemeraK)ak;etrIeTAkñúgQam¦ karbBa©ÚlQam)ancb; exSaytMrgenam/ Shock/ DIC

elIscMNuHkñúgRbB½n§Qam “ karbBa©ÚlQamkñúgel,Ón ht;/ esøksøaMg/ sMBaFQam)aj;ecj

elOneBk BIebHdUg ekIneLIg

TRALI (Transfusion Related “ RbtikmμrvagGgÁbdibkç ht;/ esøksøaMg/ k¥k/ sMBaFQamFøak;cuH

Acute Lung Injury) eKalikaQams énQambBa©Úl

¬rbYssYtRsYcRsavbNþalmk nig eKalikaQam>s rbs;GñkCMgW

BIkarbBa©ÚlQam¦

FNHTR (Febrile Non- “ RbtikmμrvageKalikaQam>s sItuNðPaBGñkCMgWekIneLIj 1oC b¤

Haemolytic Transfusion énQam bBa©Úl nig GgÁbdibkç elIs edayKμankarBnül;NamYy)an.

Reaction) eKalikaQams rbs;GñkCMgW.

¬ekþAxøÜn EtminmanEbkeKalika RbtikmμxøHeKeCOfa bNþalmkBI

QamRkhm¦ sarFatu cytokine pliteday

eKalikaQamskñúgeBlEdl

QamrkSaTukmunykmkeRbI.

Allergic (Urticaria) “ BNalmkBIsarFatuTas; knÞalRtGak/ mancMNucRkhm

¬knÞalRtGak¦ (Allergens) kñúgQambBa©Úl tUc²elI Es,k/ ehImedaydMu²

RbtikmμCamYyGgÁbdibkç

rbs;GñkCMgW b¤ GgÁbdibkç

énQambBa©Úl RbtikmμCamYy

srFatuTas;rbs;GñkCMgW

3e

44

3e taragelx 3 ³

buBVehtu/ eBlkekItRbtikmμ nig eraKsBaØaRbtikmμ EdlTak;TgnwgkarbBa©ÚlQam ¬bnþ¦

Type of reaction Timing Cause Symptoms

¬RbePTRbtikmμ¦ ¬eBlkekIt¦ ¬buBVehtu¦ ¬eraKsBaØa¦

GVHD (Graft vs Host Disease) 3 - 30éf¶ Antigens HLA rbs;GñkCMgWeTA ekþA/ rlakeBaHevon/ raKsuT§EtTwk/

¬TMnas;bnSaMsrIragÁ¦ eFVI[skmμ T-Lymphocyte rlakEs,k/ Es,keLIgRkhm nig

énQambBa©Úl CaBiess cMeBaH ³ karfycuHRKb;eKalikaQam

[i] GñkCMgWEdlmanRbB½n§ karBar ¬Pansicytopenia¦.

cuHexSay [ii] TarkkñúgépÞTTYlkar

bBa©ÚlQam [iii] TarkekItmkTTYl

karpøas;bþÚrQam [iv] GñkTTYl

QamBIsac;jati .

Alloimmune reaction eRcInéf¶ b¤ s)aþh_eRkay GñkCMgWbegáItGgÁbdibkçRbqaMgnwg ekþAxøÜnbnþicbnÞÜc/ jak;/

¬RbtikmμedaymanbegáItGgÁ karbBa©ÚlQam eKalikaQamRkhmxøH²EdlbBa©Úl Es,keLIgelOg/ Hb fycuH

bdibkçRbqaMgnwgeKalika bNþaleGayEbkRKab;QamRkhm minGacBnül;)an ¬eRKaHfñak;Qam

EdlbBa©ÚleTA. RkhmEdlbBa©Úl¦ ekItEbbenH

Gacfy)anedayqab;eFVIkar

bBa©ÚlQam[)anmunGayu)an 3

qñaM¦.

Haemolytic delayed 3 - 7 éf¶ dUcxagelIEdr EtGgÁbdibkçminGac

anamnestic response dwg)anedaykareFVIetsþmnÞIBiesaFn_

¬EbkeKalikaQamRkhm munkarbBa©ÚlQam ehIyRbB½n§

eRkayBIkareqøIytbeLIgvij¦ kaBar ragÁkayplitnUvbrimaNd¾eRcInén

GgÁbdibkçeBlbBa©ÚlQam.

karBüa)alRbtikmμbBa©ÚlQam

buernimitþ ¬eCaKvasna¦ énRbtikmμbBa©ÚlQamGaRs½yelIPaBF¶n;F¶rénRbtikmμekIteLIg.

teTAenHbgðajnUvrebobBüa)alRbtikmμmYycMnYn ³

45

plvi)akF¶n;F¶r ³

� RbtikmμbBa©ÚlQam eFVI[EbkRKab;QamRsYcRsav ¬Acute Haemolytic

Transfusion Reaction¦/ RbtikmμbNþaleGay Shock ¬Anaphylactic¦/

qøgemeraK)ak;etrIeTAkñúgQam ¬Sepsis¦ nig dMuxül;sÞHkñúgsésQam ¬Air

embolism¦ ³ eKRtUvbBaÄb;karbBa©ÚlQam. eKGacbBa©ÚlCatiTwktam

srésQam ehIyeRbIfñaMmYycMnYnedIm,IBüa)al b¤ karBarsßanPaBmYycMnYn

kMu[ekIteLIg dUcCa CMgWkMNkQamkñúgsrésQamRKb;TIkEnøg

¬Dissemination Intravascular Coagulopathy = DIC¦/ exSaytMrg

enam nig Shock .

� RbB½n§srésQamelIsbnÞúk GacBüa)aledayGukSIEhSn ¬O2¦nig

fñaMbBa©úHTwkenam.

� rbYssYtRsYcRsavbNþalmkBIkarbBa©ÚlQam ¬Transfusion Related

Acute Lung Injuey = TRALI¦ GaceFVI[FUres,Iyvij)an edayeFVIkar

KaMRTpøÚvdegðIm.

� \T§iBlénEbkeKalikaQamRkhmeRkayBIkareqøIytbeLIgvij nig

RbtikmμedaymanbegáItGgÁbdibkçRbqaMgnwgeKalikaQamRkhm

EdlbBa©Úl Gackat;bnßy)anedayeRbI Corticosteroids .

� TMnas;bnSaMsrIragÁ ¬GVHD¦ TamTarnUvkarBüa)alKaMRTd¾smRsb .

� karqøgvIrus RtUvBüa)altamRbePTvIrusEdlqøg .

plvi)akminF¶n;F¶r ³

� ekþAxøÜn EtminmanEbkeKalikaQamRkhm eKRKan;EteRbIfñaMbBa©úHkMedA.

� knÞalRtGak/ cMNucRkhmtUc²elIEs,kehIyrmas; Gackat;bnßy

)anedayfñaM Antihistamines.

46

karBarkMu[ekItmanRbtikmμbBa©ÚlQam

esvabBa©ÚlQam nig EpñkCab;Bak;B½n§nwgkarbBa©ÚlQam RtUvGnuvtþn_

eKalneya)aybBa©ÚlQam[)anhμt;ct; edayFanafa nitiviFIénkarbBa©Úl

Qamd¾l¥RtUv)aneKGnuvtþþn_ ehIysuxPaB nig suvtßiPaBGñkCMgWRtUv)anFana.

buKÁliksuxaPi)alRtUvRbkan;x¢ab;nUvsþg;da nig BiFIsaEdl)anbegáIteLIg.

karkMNt;GtþsBaØaNGñkCMgWxus ¬dUcCaxusRkumQam¦ mkTl;eBlenH

vaCakMhusEdlmaneRcInbMputEdlbNþal[søab;enAkñúgkarbBa©ÚlQam.

1> sMNakRbePTQam nig karBiesaFn_rkPaBsIuKñaénQam ¬Compatibility

testing¦ RtUvEtkMNt;GtþsBaØaNeGayc,as;las;. eQμaHeBj nig

éf¶ExqñaMkMeNItGñkCMgW RtUvsresrbBa¢ak;elIsøakbMBg;dak;Qam munnwg

bUmQam ehIyTinñn½ybMeBjelIRkdassñamsMrab;karbBa©ÚlQam

¬Transfusion Application Form¦ RtUvEtBinitü.

2> mRnþIsuxaPi)al RtUvEtepÞogpÞat;fa ral;]bkrN_sMrab;bBa©ÚlQam RtUv)an

kMBugeRbIeTAtamkarENnaMrbs;plitkrén]bkrN_enaH.

3> munnwgfg;QamNamYyP¢ab;nwg]bkrN_bBa©ÚlQam mRnþIsuxaPi)alRtUv

eFVIkarRtYtBinitüpÞal;nwgEPñknUvfg;QamrkemIlPsþútagénkarcMlgeraK

]TahrN_karERbRbYlBN’QameTACaRsKaMRkem:A/ mankMNk b¤ k¾Ebk

eKalikaQam ehIyepÞógpÞat;emIlfa QamBitCaminTan;hYs

kalkMNt;eRbI .

4> PaBsIuKñarvagQamGñkCMgW nig Qamkñúgfg;RtUvEtepÞógpÞat; edayBinitü

elIlixitbBa¢ak;RkumQamGñkCMgW nig RkumQamEdlbgðajelIsøakCab;

nwgfg;Qam.

5> GtþsBaØaNlMGiténQamkñúgfg;Edl)anbBa©ÚlRtUvcuHenAkñúgÉksar

GñkCMgW edIm,IfaebImankarcaM)ac; eKGacnwgdwgGñkeFVIGMeNayQam.

47

6> eKRtUvBinitüemIlGñkCMgW eBjGMLúgeBlkMBugbBa©ÚlQam CaBiess

dMNak;kaldMbUgEdlRbtikmμbBa©ÚlQamPaKeRcInGacnwgekIteLIg.

7>> edIm,IeCosvagkMu[b:HBal;dl;RbsiT§iPaBKøInik ehIymansuvtßiPaB nig

manPaBgayRsYlkñúgkarbBa©Úl/ smasPaBQam ¬Blood

components¦ RtUvbBa©ÚlkñúgefrevlakMNt; tamesckþIENnaM.

8> QamRtCak;bBa©ÚlelOnGacmaneRKaHfñak;. fg;QamkkeKRtUvEtkan;va

edayRbugRby½tñbMput eRBaHQamkñúgfg;GacRsYy ehIyGacgayeRbH

EbkenAsItuNðPaBTab.

9> RbsiT§iPaBénkarbBa©ÚlQam RtUvkMNt;edaykarkt;RtanUvtYelx

eKalikaQammun nig eRkaykarbBa©ÚlQam .

10> RbtikmμNamYyEdleKsegáteXIj eKkt;RtaTukedayykcitþTukdak;

ehIyraykarN_;. ral;plvi)akF¶n;F¶rTaMgGs; EdlekItmaneLIg RtUvEt

esIubGegát ¬RtUvbUmQameRkaykarbBa©ÚlehIy rYcbBa¢ÚnvaCamYy

plitplQamEdlminTan;)aneRbI ehIynigrbs;rbrbBa©ÚlQameTA

mCÄmNÐlpþl;Qam edIm,IRsavRCavrkPaBminsIuKña ¬Serological

Incompatibility¦ nig eFVIBiesaFn_bNþúH)ak;etrI.

11> kñúgkrNIRbtikmμbBa©ÚlQamekItmandEdl² RtUvEteFVIkaresIubGegát

rkvtþmanGgÁbdibkçmineTogTat; ¬Irregular antibodies¦ eRkABIRbB½n§

ABO nig Rh. eBlEdl FNHTR ekItmanmþgehIymþgeTot eKRtUv

eRbIQamEdlmaneKalikaQamstic.

12> sMxan;eKRtUvcgcaMfa plvi)akmYycMnYnekIteLIgmanlkçN³yWt dUcCa

karcab;epþImCMgWEdlqøgtamkarbBa©ÚlQam. krNIGñkpþl;Qam eKrk

eXIjfavIrusERbCaGviC¢manvij k¾GñkCMgWEdl)anTTYlQamGñkTaMgenaH

RtUvEtTTYlkartamdanEpñkevC¢saRsþ .

48

mCÄmNÐlpþl;QamRtUvEtmanRbB½n§Fanara:b;rgKuNPaB EdlFananUvKuNPaB

plitplQamEdleRbIR)as;. RbB½n§EbbenHRtUvFana[)anfa ³

1> eKRtUvGnuvtþeTAtameKalkarN_ Rbtibtiþplitpll¥ ¬Good

Manufacturing Practice¦ kñúgkareRCIserIsGñkpþl;Qam/ karbUmyk

Qam/ plitQam/ eFVIetsþmnÞIrBiesaFn_/ sþúk nig Eckcayplitpl

Qam.

2> RtUvkMNt;[)anc,as;nUvkic©kar nig karTTYlxusRtUvrbs;buKÁlikBak;B½n§

nwgskmμPaBTaMgenH.

3> RtUveRbIR)as;sMPar³[)anRtwmRtUv.

4> RtUv[manRbB½n§svNkmμépÞkñúg edIm,IgacvaytMél[)aneTogTat;nUvkar

Gnuvtþn_ nig RbsiT§iPaBénkarRtYtBinitüelIKuNPaB.

5> kMhusqÁgRtUvraykarN_[)aneBjelj nig eronsURtemeron

bTBiesaFn_.

mnÞIeBTüCaeRcInenAGWr:ub nig enAshrdæGaemrik manKN³kmμaFikarbBa©Úl

QamEdlrYmman tMNagénesvabBa©ÚlQam nig GgÁPaBKøIniksMxan;²Edl

manskmμPaBbBa©ÚlQamxøaMgkøa. KN³kmμaFikarEbbenH GacelIkkMBs;

EfmeTotnUvRbsiT§iPaBénkarGnuvtþn_karbBa©ÚlQameday ³

1> eFVIkMNt;eKalneya)aybBa©ÚlQam eTAtamskmμPaBKøInikkñúgtMbn;.

2> eFVIkarvaytMél[)aneTogTat; elIkarGnuvtþn_karbBa©ÚlQam.

3> eFVIviPaKRBwtþikarN_minl¥ EdlCab;Bak;B½n§CamYykarbBa©ÚlQam ehIycat;

viFankarN_EktMrUvCacaM)ac;.

49

CMBUkTI 4

elIsbnÞúkCatiEdk nig karkMcat;CatiEdkecj

dUc)anBnül;kñúgEpñkxagedImrYcmkehIy/ taLasesmIubgá[CatiEdkkkkuj

kñúgragÁkay. manpøÚvBIrEdlGñkCMgWtaLasesmIuRsUbCatiEdk KWBI

cMNIGahar nig edaykarbBa©ÚlQam. RbsinebIkarelIsCatiEdk

enHmin)ankMcat;ecjeT/ vaGacbgá[mankarxUcxatdl;srIragÁ

sMxan;² dUcCa eføIm nig ebHdUg. GñkCMgWtaLasesmIu RtUveRbIfñaM

BiessehAfa XILaT½r ¬Chelators¦ EdleTAcMraj;CatiEdkecj

BIragÁkay.

EdkenAkñúgsarBagÁkayGñkmansuxPaBl¥

mnusSeBjv½ymansuxPaBl¥ FmμtamanpÞúkCatiEdkRbmaN 4 Rkam

kñúgenaH 3 Rkam eRbIR)as;sMrab;plit eGm:UkøÚb‘Ín (Hb) kñúgeKalika

QamRkhm. dUcmanBnül;xagedIm eBleKalikaQamRkhm

cas;/ søab;eTAvijehIy eGm:UkøÚb‘Ín rbs;vaRtUvEbkCa FatupSMpÁMú KW

EGm nig køÚb‘Ín ¬Haem nig Globin¦. EdkEdlbeBa©jBI EGm

RtUv)andwkCBa¢Únedaym:UelKulRbUetGIunmü:agehAfa Rtg;EsV‘rIn

¬Transferrin¦ edIm,IykeTAeRbIeLIgvijsMrab;plit EGm rbs;eKalikaQam

RkhmfμI²eTot. sarFatuKImIEdlbegáItRbUetGIunkøÚb‘Ín eQμaHfa GamIuNUGasIud

¬Amino acid¦ RtUv)aneRbIeLIgvijedIm,IplitkøÚb‘Ín fμI²eTot. dMeNIrkarTaMg

enHmann½yfa kñúgmnusSeBjv½ysuxPaBFmμtamñak; sarBagÁkayeRbIEdkeLIg

vijesÞIrEtTaMgGs; edaybnSl;tictYcbMputsMrab;beBa©jecal. Caerogral;éf¶

EdkEt 1 mRk b:ueNÑaH RtUvbeBa©jecal PaKeRcIntamry³ Twkenam/ lamk/

tamEs,k ehIyebIRsþItamry³QamrdUv. EdkEdl)at;bg;enaH RtUvCMnYsmk

vijedayRsUbtameBaHevonBIcMNIGahar ¬emIlrUb 4a¦.

4a

50

EdkkñúgCMgWtaLasesmIu

kñúgCMgWtaLasesmIukMritmFüm nig kMritF¶n; Edlmin)anbBa©ÚlQam sarBagÁ

kayxitxMTUTat;cMeBaHPaBkgVHeKalikaQamRkhmF¶n;F¶r edaykarRsUbyk

CatiEdkya:geRcInEfmeTotelIsBIFmμtaBIeBaHevon ¬2 - 5 Rkam¼qñaM eRbob

eFobeTAnwg 0/0015 Rkam¼qñaM cMeBaHmnusSmansuxPaBl¥¦ edIm,IykeTA

pliteKalikaQamRkhmEfmeTot.

brimaNEdkRsUbbEnßmvaGaRs½yeTAelIPaBF¶n;F¶rrbs;kgVHeKalikaQam

Rkhm. kgVHeKalikaQamRkhmkan;EtF¶n;F¶r xYrq¥wgk¾kan;EtrIkFM edayva

xitxMeFVIya:gNapliteKalikaQamRkhm[)aneRcIn eBlenaHtMrUvkar

CatiEdkk¾kan;EtFMeFg. ktþaepSg²k¾cUlrYmcMENkkñúgkarkMNt;brimaN

EdkRsUbBIeBaHevonEdr. ]TahrN_ vItamInes CYybegáInkarRsUbCatiEdk rIÉ

TwkEt nig FBaØCatimYycMnYnbnßykarRsUbCatiEdk. eTaHbIya:gNak¾eday

pøÚvd¾sMxan;bMputkñúgkarbnßybrimaNEdkRsUbBIeBaHevon KWRtUvFanafa GñkCMgW

mankMrit eGm:UkøÚb‘Ín l¥. dUecñHsMxan;KWfa GñkCMgWRtUvkarbBa©ÚlQam[)an

eTogTat; edayrkSakMrit eGm:UkøÚb‘Ín [enAelI 9 Rkam¼d>l ¬vas;muneBl

bBa©ÚlQam¦. GñkCMgWEdlbBa©ÚlQammin)anRKb; GacRsUbCatiEdkbEnßm

BIeBaHevon 1 - 5 mRk¼éf¶ ¬b¤RbEhl 0/4 - 2 Rkam¼qñaM¦.

eTaHbIya:gNak¾eday RbPBd¾sMxan;énkarelIsbnÞúkCatiEdkcMeBaHGñkCMgW

TTYlkarbBa©ÚlQam KW tamry³QamEdlbBa©ÚlenHÉg. tamBiteTAbrimaN

CatiEdkEdlTTYltamry³karbBa©ÚlQammankMritFMeFgCagCatiEdkEdl

)anmkBIcMNIGahartamry³eBaHevon. dUcenHvaCakarsMxan;EdlfaGñkCMgW

TTYlkarbBa©ÚlQamCaRbcaMRtUveRbIfñaMkMcat;CatiEdk KWCafñaMEdleTAP¢ab;nwg

CatiEdk ehIybeBa©jvaecaleRkARbB½n§sarBagÁkay.

eKalikaQamRkhm 1 ml manCatiEdk 1/16 mRk. mYyÉktaQam

CamFümmanQameKalikaQamRkhmRbEhl 250 ml RtUvmanCatiEdk =

51

250 x 1/16 b¤enAcenøaH 200 - 290 mRk. CatiEdkEdlbeBa©jBI

eKalikaQamRkhmTaMgenHEdlxUcxat KWCaRbPBd¾sMxan;énCatiEdkEdl

kkpþúMenAkñúgsarBagÁkayénGñkCMgWEdlTTYlkarbBa©ÚlQameBjmYyCivit.

]TahrN_ GñkCMgWEdlTTYlQam 30 Ékta ¬30fg;¦ kñúgmYyqñaM nwgman

CatiEdkelIsRbEhl 6 Rkam kñúgmYyqñaM ¬ 200 x 30 = 6000 mRk = 6

Rkam¦ b¤ RbEhl 16 - 16 mRk¼éf¶ . sarBagÁkayBMuGacmanlT§PaB

beBa©jCatiEdkEdlelIskñúgbrimaNd¾eRcIndUcenHeT dUecñHvanwgkkBUn

kñúgCalika nig srIragÁepSg². RbsinebICatiEdkenH minRtUv)anyk

ecjedaymanGnþraKmn_BIfñaMeBTüeT vaGacCaeRKaHfñak;d¾xøaMg edaybgá

Caplvi)akF¶n;F¶rbMputkñúgCMgW Ebtta taLasesmIukMritF¶n;.

eraKsBaØaKøInikénkarelIsCatiEdk CaTUeTAekIteLIgenAGayuRbEhl

10qñaM eTaHbIeKeXIjmanPsþútagBI\T§iBlBulCatiEdkxøHcMeBaHeføImelIkumar

GayuticCagenHk¾eday. rbYsenAnwgeføImbgáedayelIsCatiEdk KW

sPaBcak;es£Hedaysrésevov ¬Fibrosis¦ edayvacab;epþImkñúgGMLúg

2qñaMénkarcab;epþImbBa©ÚlQam. rbYsF¶n;F¶r KW RkineføIm ¬Cirrhosis¦

GacekIteLIgmun Gayu 10qñaM RbsinebIKμankarBüa)albM)at;karelIsCati

EdkeT CaBiesscMeBaHGñkCMgWmanrlakeføImRbePT eb nig¼b¤

RbePT es. CMgWebHdUg EdlCabuBVehtumYyjwkjab;CageK

bNþal[søab;kñúgCMgW taLasesmIukMritF¶n; RtUveK)andwgfava

ekIteLIgkMLúgeBl 10qñaM cab;taMgBIcab;epþImbBa©ÚlQam eTaH

CaCMgWexSayebHdUgekItmanTal;EtkarbBa©ÚlQamry³eBl

15qñaM b¤ eRcInCagenHk¾eday.

karelIsCatiEdk k¾CamUlehtusMxan;bMputpgEdr naMeGayyWtya:vdl;kar

cas;TMuEpñkpøÚvePTcMeBaHGñkCMgWtaLasesmIu TaMgePTRbus nig ePTRsI edayva

b:HBal;cMnYnRbEhlCaBak;kNþal. elIsBIenHeTAeTot karelIsbnÞúk

CatiEdk Gacbgá[mankarlM)akcMeBaHRsþIkñúgkarmanépÞeBaH ¬manRbEhl

4b Increase of transfusional

iron as needs for blood

transfusions increase with

age

(From Cohen and Schwartz -

Chelation therepy in chronic iron

overload, 977, symposia special-

ists, Inc., Box 610397, Miami

Florida)

52

25° énkrNI¦ ehIyCamUlehtujwkjykñúgkarvivtþn_eGayekItCMgWTwkenam

Ep¥meTAGnaKt.

kñúgry³eBlyUreTA elIsCatiEdkbgá[manplvi)akelIq¥wg nig xUcxatdl;

srIragÁsMxan;²epSgeTotdUcCa RkeBj Thyroid nig Parathyroid. dUecñH

ehIyGñkCMgWRtUvTTYlkarBüa)albM)at;karelIsCatiEdk EdlnwgkkpþMúkñúg

ragÁkay ehIyb:HBal;F¶n;F¶relIKuNPaB nig GayuCIvitrbs;GñkCMgW.

etIkarelIsCatiEdkbMpøajragÁkayya:gdUcemþc

eBlEdlCatiEdkkkpþúMkñúgragÁkay eTaHbNaþalmkBICMgWtaLasesmIu

xøÜnvapÞal; b¤ mkBIkarbBa©ÚlQam b¤ k¾TaMgBIrkrNI/ RbUetGIunsMxan;EdldwknaM

EdkkñúgQam KW Transferrin mansPaBEq¥tnwgCatiEdk ¬Saturated¦.

edayKμan Transferrin NamYyenATMenr EdkesrIEdlKμanP¢ab;nwgRbUetGIun

eFVIdMeNIrenAkñúgQamEdlCaeRKaHfñak;xøaMgcMeBaHsarBagÁkay. Edkk¾kkpþúM

pgEdrkñúgCalikaedayP¢ab;nwgm:UelKulRbUetGIunsMrab;sþúk ehAfa Ferritin

¬emIlrUb 4c¦ nig Haemosiderin. EdkEdlsþúkkñúgRbUetGIunTaMgenH vaBMusUv

eRKaHfñak;dUcEdksßitkñúgPaBCaesrIeT. eTaHya:gNak¾eday sarBagÁkay

eyIgeFVIkarbMEbk Ferritin nig Haemosiderin Cab;rhUt EdlbnÞab;mkeFVI

karbeBa©jEdkmYycMnYnsßitkñúgPaBesrI. CatiEdkGacnwgRtUv)anbeBa©jBI

RbUetGIunsþúkEdr eBlNaGñkCMgWFøak;xøÜnQW.

CatiEdkEdlenAsl;Edlmin)anP¢ab;nwg Transferrin edaysarKμan

Transferrin sMrab;P¢ab; KWCaEdkEdlKμanPaBnwgnr )anesckþIfavaGacgay

nwgTTYlyk b¤ pþac;ecalbnÞúkGviC¢manEdleKehAfa eGLicRtug

¬Electron¦. eBlEdkekIn eGLicRtug mYy/ vabþÚrBImanbnÞúkviC¢manbI

¬CaRbePTEdkEdleKsÁal;fa 3+ b¤ ferric iron¦ eTACaEdkmanbnÞúkviC¢manBIr

¬CaRbePTEdkEdleKsÁal;fa 2+ b¤ ferrous iron¦. eBlEdkpøas;BIsPaB

2+ eTA 3+ vabegáItnUvsarFatueRKaHfñak;mYy ehAfa ra:DIkal;esrI ¬Free

4c Ferritin molecule

53

4d Desferrioxamine B, the

active substance of

Desferal

radicals¦ EdlGacbgáeGayxUcxatFMeFgcMeBaHCalikasarBagÁkay.

dMeNIrEdleKsÁal;c,as; edaybegáItCara:DIkal;esrI eKehAfa Rbtikmμ

Fenton EdlCaRbtikmμKImImYy EdlsMraydUcxageRkamenH KW karbegáItCa

ra:DIkal;GIuRduksIul ¬HO¦ ³

O2 + Fe3+ � O2 + Fe2+

H2O2 + Fe2+ � OH- + HO- + Fe3+

Desferrioxamine (DFO) b¤ Desferal

edaysarBagÁkayeyIgBMumanmeFüa)ayRbsiT§iPaBNaedIm,IkMcat;Edkecj

viFIEtmYyKt;edIm,IkMcat;CatiEdkelIsecj KW eRbIfñaM ehAfa XILaT½rEdk

¬Gñkcab;ykEdkecj¦ edaybegáItCasmasFatuCamYyEdk

EdlGacbeBa©jecalBIsarBagÁkayeyIgtamry³Twkenam

nig lamk .

Desferrioxamine (DFO) KWCafñaMkMcat;CatiEdkTImYy EdlRtUv

)aneKbegáIteLIgdMbUgenATsvtSn_qñaM 1960 ehIyRtUv)an

beBa©jenAelITIpSarenAedImTsvtSn_qñaM 1970 sMrab;karBüa)alCMgW

taLasesmIukMritF¶n;. DFO ¬emIlrUb 4d¦ eFVIkartamviFIBIirEbb ³ TI1-

dMeNIrRbRBwtþieTAmanPaBsnSwm² kñúgenaH DFO)anP¢ab;eTAnwgFatuEdkbegáIt

)anCasarFatumYyehAfa Ferrioxamine ¬emIlrUb 4e¦ EdlbnÞab;mk)an

beBa©jBIsarBagÁkay. tYnaTITI2rbs; DFO KWRtUvbnßysPaBBulrbs;Edk

kñúgsarBagÁkayedaycab;CamYyra:DIkal;esrI. dMeNIrRbRBwtþiEbbenHman

sPaBelOn.

DFO )ancab;ykEdkBIRbPBBIrsMxan;kñúgsarBagÁkay. RbPBTI1 KWkar

xUcxaténeKalikaQamRkhm RbPBenHmanrhUtdl; 70° énEdk Edl

cab;ykeday DFO ehIybeBa©jeTAeRkAxøÜntamry³Twkenam. RbPBTI2

énEdk Edl DFO cab;yk KWEdkmkBIeføIm EdlCasrIragÁsþúkEdkFMbMputkñúg

4e Ferrioxamine B

54

sarBagÁkayeyIg. EdkEdlsþúkTukkñúgeføImRtUv)anbeBa©j kalNasarFatu

BIr ¬Ferritin nig Haemosiderin¦ EbkkñúgekasikaeføIm ¬Hepatocyte¦.

bnÞab;mk DFO kñúgekasikaeføIm P¢ab;CamYyEdkenaH muneBlRtUvbeBa©jeTA

eRkAsarBagÁkaytamry³lamk. DFO mincab;ykEdkEdl)anP¢ab;CamYy

Transferrin rYcehIyeT.

m:UelKul DFO nImYy²P¢ab;CamYyGatUmEdkmYy edIm,IbegáIt)an

Ferrioxamine . enH)anesckþIfa RbsinebI DFO manRbsiT§iPaB 100°

enaH 1 mRk DFO nwgbeBa©jecalEdk)an 93 mRk. eTaHbIya:gNa

vaminGacBitdUecñaH)aneT/ kMritRbsiT§iPaBBwgEp¥kelIkMritfñaM nig

pøÚvEdleKeRbIfñaM/ brimaNsþúkénEdk/ kMritvItamIn es (Vitamin C)

enAkñúgxøÜn nig kMritEdlGñkCMgWGnuvtþtamevC¢bBa¢aeRbIfñaMkMcat;CatiEdk.

tYnaTIrbs;vItamInes ¬Vitamin C¦

vItamInes KWCaPñak;garkat;bnßymYy KwfavaGacCYy[EdkbþÚrBI Fe3+

eTAEdk Fe2+ . enHvasMxan;Nas;eRBaHEdk Fe2+ GaceFVIdMeNIr)an

gayRsYlkñúgsarBagÁkayCagEdk Fe3+ ehIyEdk Fe2+ k¾CaTMrg;mYy

EdlgayP¢ab;nwgXILaT½r dUcCa DFO CaedIm. edayehtuenHehIyEdk

Fe2+EdlCaTMrg;Edk Ferrous rEmgeKehAfa EdkEdlGackMcat;ecj)an

rIÉEdk Fe3+ EdlCaTMrg;Edk Ferric KWCaEdkKμanclna eKeXIjmanenA

kEnøgsþúkkñúgsarBagÁkay maneføImCaedIm edayvaP¢ab;CamYyRbUetGIunsþúk

dUcCa Ferritin nig Haemosiderin. viFImYyedIm,IbegáInEdkbeBa©jeday

RbUetGIunTaMgenH KWkareRbI vItamInes bEnßmCamYyfñaM DFO.

GñkCMnajpþl;dMbUnμanfa GñkCMgWtaLasesmIKYrEtelbfñaMvItamInes

erogral;éf¶ ykl¥kñúgeBlEtmYyCamYyfñaM DFO. eTaHbIya:gNak¾eday

CaTUeTAeKENnaMfa GñkCMgWcab;epþImelbfñaMvItamInesbEnßm EteBlNaeK)an

TTYl DFO BIr b¤ bI s)aþh_ehIy.

55

kMritvItamInes eRbIRbcaMéf¶ EdleKENnaMenaHKW 50 mRk sMrab;ekμgGayutic

Cag 10qñaM ehIy 100 mRk sMrab;ekμgFMCag 10qñaM. ehIykMriteRbIenH

minRtUvelIs 200 mRk¼éf¶eT. GñkCMgWEdlmanrbbcMNIGahareTogTat;

jaMuRkUc b¤ TwkRkUcRss; GacmincaM)ac;elbvItamInesbEnßmk¾)an. ]TahrN_

RkUcFMmYyEpømanpÞúkvItamInes 75 mRk ÉTwkRkUcRc)ac;Rss; 100 ml

manpÞúkvItamInes 50 mRk.

minxusBIfñaMBüa)al nig fñaMbEnßmepSg²Edr/ kMriteRbIRtUvéllkedayBiPakSa

CamYyevC¢bNÐit. sMxan;Nas;RtUvcMNaMfa vItamIneseRcIneBkGacmanplBul

elIGñkCMgW edayvaeFVI[EdkCaeRcInmanclna. dUecñH vItamInesbEnßmGacman

pleRKaHfñak; CaBiesscMeBaHGñkCMgWEdlmin)anTTYlfñaM DFO edayEdkman

clnaeLIgedaysarvItamInes CaEdkesrIehIyvabgáeRKaHfñak;dl;Calika.

etIeKcab;epþImBüa)alkMcat;CatiEdkeBlNa

GñkCMgWtaLasesmIukMritF¶n; RtUvcab;epþImBüa)aleday DFO EteBlNaeK

)anTTYlkarbBa©ÚlQamya:geTogTat;. DFO minsMrab;edaHRsaybBaða

TaMgLayEdlbNþalmkBIkarbBa©ÚlQamminRKb; dUcCa kgVHRKab;Qam

Rkhm/ karERbRbYlq¥wg/ rIkGeNþIk nig rIkeføImeLIy. eKminRtUvpþl; DFO

dl;GñkCMgWEdlmin)anbBa©ÚlQam b¤ bBa©ÚlQambnþicbnþÜc luHRtamanGayu

elIs 10qñaM b¤ manPsþútagénkarelIsCatiEdk.

CaRkitüRkmTYeTA GñkCMgWRtUvcab;epþImBüa)aledayfñaMkMcat;CatiEdkeBlNa

eK)anbBa©ÚlQam 10 - 20dg b¤eBlkMrit Ferritin eLIgelIs 1000 μg/l.

reboblayfñaM/ karrkSaTuk nig kareRbIR)as; Desferrioxamine

Gs;ry³eBlCag 30qñaM énbTBiesaFn_KøIniksMrab;RKUeBTü nig GñkviTüasaRsþ

edIm,I)ankarRBmeRBogKñaelImeFüa)ayl¥bMputkñúgkareRbI DFO edIm,IkMcat;

CatiEdkBIsarBagÁkay[manRbsiT§iPaB ehIyeCosvag)annUvplBulNamYy

bNþalmkBIkareRbIfñaMeRcInhYseBk.

56

edaysarm:UelKul DFO manTMhMFM/ sarBagÁkayRsUbyk DFO )anbnþic

bnþÜctamry³eBaHevon. dUecñHpøÚvl¥bMputkñúgkareRbI DFO KWcak;bBa©Últam

eRkamEs,k b¤ cak;bBa©ÚltamsrésEv:n. TnÞwmnwgenHEdr eday DFO

beBa©jeTAvijBIQammanlkçN³elOn [Bak;kNþalCIvit ¬Half-life¦ rbs;va

10 - 15naTI ] fñaMRtUvEtbBa©ÚlyWt²kñúgry³eBlmYyedIm,ITTYl)annUv

kMhab;efrmYyenAkñúgQamkñúgry³eBlEvgtamEdlGaceFVIeTA)an. eKeRbI

DFO tamry³sñb;BiessmYy Edlcak;bBa©ÚlfñaMcUleRkamEs,kyWt²

ry³eBl 8 - 12ema:g ya:gehacNas; 6éf¶ kñúgmYys)aþh_. kMriteRbIBit

R)akdsMrab;GñkCMgWnImYy² KWKNnaelImUldæanénGayu bnÞúkCatiEdkkñúg

sarBagÁkay nig sPaBKøInik. CaTUeTAkMriteRbICamFümsMrab;kUnekμgmin[

elIs 20 - 40 mRk¼KRk énTMgn;xøÜn edaysarkMriteRbIx<s; GacBnüWtkarrIk

FMFat;rbs;kumar. kMriteRbICasþg;da sMrab;mnusSeBjv½y KW 30 - 50

mRk¼KRk énTMgn;xøÜn.

reboblayfñaM DFO

DFO Caem:SAs¶ÜtBN’s manCaÉkta 500 mRk b¤ 2 Rkam. ÉktanImYy²

manP¢ab;mkCamYydbEkvtUcmYymanemS:ABN’s EdleKRtUvlay[eTACa

l,ay 10° muneRbI. ]TahrN_edIm,I)anl,ay 10° énfñaM DFO BIdbfñaM

cMNuH 500 mRk eKRtUvbEnßmTwklay 5 m>l. TwklayeKGacrkTij)anBI

»sfsßannana. bnÞab;mkeKTuk[emS:Avarlaykñúgry³eBl 2 - 3naTI.

sMxan;eKminRkLúkdbfñaMeT edIm,ICYy[fñaMrlayeKGacRKan;EtbgVilRbemol

dbfñaMkñúg)atéd b¤ elIépÞturabesμI. eBlemS:AfñaMrlayGs;ehIy eKyksIuraMg

bUmehIydak;cUlkñúgsñb;cak;edIredayfμBil sñb;sgßt;elIsIuraMgyWt² rujfñaMcUl

eTAkñúgsarBagÁkaykñúgry³eBlkMNt;mYy KWcenøaHBI 8 - 12 ema:g. sIuraMg

nwgcak;Gs;fñaMeTAtamcMnYnema:gEdleyIgkMNt;. sñb;mYycMnYnEdleKeRbI

jwkjab;CageKbMput mansMelg[sBaØaRbsinsIuraMgsÞH b¤ Gs;fñaM.

57

karrkSafñaM DFO

l,ayfñaM DFO EdleK)anlayrYc GacsþúkkúñgbnÞb;mansItuNðPaB 23ºC

kñúgry³eBlCaGtibrima 24ema:g. ebIGakasFatuekþA caM)ac;RtUvrkSavaenA

kEnøgRtCak; edayTukvakñúgTUrTwkkkenAsItuNðPaB 4ºC ebIeKminTan;eRbIva

PøameT. l,ayfñaMEdlTukkñúgTUrTwkkk GacTuk)anrhUt 5éf¶. eTaHbI

ya:gNak¾eday ebIl,ayfñaMenaHkøayCal¥k; nig manBN’minl¥ eKRtUve)aHva

ecalPøam. ykl¥bMput muneRbIeKRtUvykl,ayfñaM DFO mkeRkATUrTwkkk

BIrema:g edImIi[vamankMedAesμIkMedAsarBagÁkay. sMrab;karcak;eRkamEs,k

nig tamsrés eKGaclayTukmuneday»sfkarI kñúglkçxN½ÐmYys¥atKμan

emeraK ¬Sterile condition¦ ehIy DFO layrYcGacrkSaTuk)anrhUtdl;

BIrs)aþh_enAsItuNðPaB 4ºC b:uEnþ DFO layTukmunedayGñkCMgWxøÜnÉg ehIy

minEmnCa»sfkarI l,ayfñaMminGacTuk[elIsBI 1 - 2 éf¶)aneT edayehtu

favaGacqøgemeraK. karcak;l,ayqøgemeraK Gacbgá[manRbtikmμ.

karcak; DFOeRkamEs,k

edIm,Icak; DFO eRkamEs,k sIuraMgEdlpÞúkfñaM DFO RtUv[smlμmdak;cUl

kñúgsñb;)an ehIybnÞab;mkPÞab;cUlkñúgbMBg;)aøsÞicesþIg EdltMrg;eTAmÞúl

ya:gtUcmYy/ mÞúlenHehIyEdlcak;cUleTAeRkamEs,k. fñaM DFO RtUvcak;

bBa©ÚleTAkñúgcenøaHEs,k nig sac;dMu b¤ k¾xøaj;EdlsßitenAeRkamEs,k.

CaeRcInqñaMcugeRkayenH sñb;sMrab;cak;fñaMkan;EttUceTA²/ RsalCag nig s¶at;

CagRbePTsñb;mun. enH)aneFVI[sñb;sMrab;cak;fñaMenH gayRsYleRbIkñúg

lkç½Ns¶b;s¶at;kñúgeBléf¶ Gaclak;eRkamsMelokbMBak;)an. eTaHbIya:gNa

k¾eday GñkCMgWCaeRcIncUlcitþeRbIsñb;cak;fñaMenHkñúgeBlyb; edIm,IkMu[rMxandl;

skmμPaBkargareBléf¶rbs;eK.

kareRbIfñaM DFO CaeTogTat; CacMENkd¾sMxan;bMputkñúgkarEfrkSaGñk

CMgWtaLasesmIu[mansuxPaBl¥. b:uEnþkarBüa)alkMcat;CatiEdkeday DFO

58

CakarlM)ak KWfavaeFVI[QW/ eRbIR)as;eBlevlaeRcIn ehIylM)aksMrbsMrYl

CIvitRbcaMéf¶. dUecñHsMxan;RKb;²rUbEdlcUlrYmEfTaMGñkCMgW rYmman evC¢bNÐit/

Kilanubdæak-yika/ «Bukmþay sMxan;bMputenaH KwGñkCMgWxøÜnÉgEtmþg rYmKñark

meFüa)ayeFVIya:gNa[karBüa)alkan;Etgay tamEtGaceFVIeTA)an.

CaBiesskumarFM² GacvaytMélx<s;cMeBaHkarxitxMeFVI[karBüa)alman

lkçN³s¶at;es¶omeRcIneLIg. eKRtUvEtxitxMpgEdr edIm,Ibnßy[dl;

Gb,brimanUvRbtikmμkEnøgcak; b¤ plvi)akepSg²eTotEdlCab;Tak;TgnwgfñaM

DFO ¬GanenAxageRkay¦.

meFüa)ayepSg²eTotkñúgkarcak;bBa©ÚlfñaM DFO

eRkABIkareRbIfñaMtampøÚveRkamEs,k dUcerobrab;xagelI enAmanpøÚvmYycMnYn

eTotEdleKGaceRbIfñaM)an EtpøÚvnImYy²manTaMgKuNsm,tþi nig KuNvibtþi.

karcak;bBa©ÚltamsésQambnþCab; 24 em:ag

viFIenHKWeRbI DFO cak;cUltamsrésQamEvn. bec©keTsenHGacseRgÁaH

CIvitGñkCMgWEdlTTYlrgkarelIsCatiEdkF¶n;F¶r ehIymanplvi)akcMeBaH

ebHdUgmkCamYy edaybnßykarBulmkBICatiEdkkñúgsarBagÁkayeday

kMcat;CatiEdkecj)aneRcInCag nig elOnCagviFIepSg² kñúgeBleKkMBugcak;

bBa©ÚlfñaMEtmþg. eTaHya:gNak¾eday karcak;bBa©ÚltamsrésCab;GacnaM

mkCamYynUveRKaHfñak;F¶n;F¶rCagEdr CaBiesskarbgáeraK nig kMNkQam

bgáedayExSbBa©ÚlQam Edlpþl;»kas[emeraKeRCotcUltamsrésEvn)an

¬sUmGanEpñkxageRkamsþIBIkareRbI DFO bBa©ÚltamsrésEvn Cab;rhUt¦ .

dUecñHeKRKan;EteRbIviFIenHkñúgkrNIGñkCMgWbgðajnUv ³

[i] elIsCatiEdkmanlkçN³F¶n;F¶r/ )anesckþIfa kMrit Ferritin ekInCab;elIs

BI 2500 mIRkURkam¼l ¬μg/l¦ nig¼b¤ kMhab;CatiEdkkñúgeføIm elIsBI 15

mIRkURkam¼l TMgn;eføImsuT§ EdlkMNt;edaykarRcwbsac;eføImBinitü.

59

[ii] plvi)akelIebHdUg bNþalmkBIelIsCatiEdk.

[iii] GñkCMgWCaRsþImanKMeragfanwgmanKP’ ehIymankMrit Ferritin kñúgesr:Umx<s;

nig¼b¤ kMhab;CatiEdkkñúgeføImx<s; ¬LIC = Liver Iron Concentration¦.

[iv]karcak;bBa©ÚltamsrésEvnCab; GacRtUveFVIEdrcMeBaHGñkCMgW EdlcaM)ac;

RtUvkarkMcat;ecjCatiEdk edayminKitBIkMrit Ferritin b¤ kMrit LIC .

]TahrN_ munkarbnSaMxYrqw¥wg b¤ GñkEdlmanrlakeføImRbePT es ¬C¦

skmμ ehIyrauMér:.

karbBa©ÚltamsrésEvnCab;énfñaM DFO KWRtUv[kMrit 50 mRk¼KRk¼éf¶/ [

bnþCab;Etmþg 7 éf¶kñúgmYys)aþh_ tammeFüa)ayénRbB½n§bBa©ÚlfñaMBiess

mYYyeKehAfa kaetETrP¢ab;eTAsésEvnFM ¬In-dwelling Catheter¦ .

kaetETr RbePTenHmantMéléfø ehIyGñkCMgWEdleRbIvaTamTarnUvkartam

danBIRKUeBTüedayykcitþTukdak;eGay)anditdl;. karbgáeraKF¶n;F¶r KWCa

plvi)akjwkjab;bMputEdlekItmanCamYyviFIkMcat;CatiEdkEbbenH ehIyCYn

kalmankMNkQameTotpg ¬Thrombosis¦. fñaMBiess ¬fñaMRbqaMgnwg

kMNkQam¦ RtUv)aneKcak;bBa©ÚledIm,IkarBarkMu[mankMNkQam ehIy

Rby½tñRbEygrkSaEs,kCMuvij kaetETr eGays¥atedIm,IkarBarkarbgáeraK.

GñkCMgWRtUvEtrkSaEs,keGays¥at ehIymkBieRKaHCamYyRKUeBTüPøam ebIsin

maneraKsBaØa jak; b¤ ekþA b¤ manTTYlGarmμN_faQW b¤ RkhmenAelIEs,k.

karcak;bBa©ÚlfñaM DFO tamsésQam 8 - 12 em:ag

pÞúyBIkarcak;bBa©ÚltamsrésQamCab;ry³eBl 24ema:g dUc)anBnül;

xagelI/ karcak;bBa©Úl DFO tamsrésQam ry³eBl 8 - 12 ema:g

CaCMerIsmYyepSgeTot kalNakarcak;eRkamEs,kmanbBaðaecaTF¶n;F¶r b¤

bBaðaecaTenAnwgkEnøgcak;. kMriteRbI nig ry³eBleRbIdUcKñanwgkarcak;bBa©Úl

eRkamEs,kEdr KW 40 - 50 mRk¼KRk¼éf¶ ry³eBl 8 - 12 ema:g

eRcInCag 5éf¶ kñúgmYys)aþh_. eTaHya:gNak¾eday viFIenHvaBMuman

60

RbsiT§iPaB dUckarcak;bBa©ÚltamsrésQamry³eBl 24ema:g Cab;enaHeT

ebIkrNIelIsCatiEdkvaF¶n;F¶r ehIymanplvi)akcMeBaHebHdUgpgEdr.

vamansarsMxan;Nas;edaycaMfa kareRbItamsrésQamnUvfñaM DFO RtUvman

karRbugRby½tñ ehIyeRbIEtkrNIcaM)ac;. eRbIry³eBlEvg viFIenHGacbgá

eRKaHfñak;dl;srésQamEvnEdlmansar³sMxan;sMrab;karcak;bBa©ÚlQam

ehIymaneRKaHfñak;bgáeraKkMritx<s;.

karcak;bBa©ÚlfñaM DFO tamsésQamGMLúgeBlbBa©ÚlQam

enAmnÞIreBTümYycMnYn/ DFO RtUvcak;bBa©ÚltamsrésQamdMNaleBl

CamYykarbBa©ÚlQam. eTaHya:gNak¾eday eKminRtUvykfñaM DFO

eTAlaycUlpÞal;EtmþgkñúgQamEdlkMBugbBa©ÚleGayGñkCMgWenaHeT eRBaHva

GaccMlgemeraKdl;Qam b¤ bgá[manRbtikmμ. CaTUeTA eKminRtUvbEnßm

sarFatuNamYycUlkñúgQambBa©Úl[GñkCMgWeT elIkElgEt)anbgðajtam

viTüasaRsþfa vaminbgá[maneRKaHfñak;NamYyenaHeT.

edIm,IbBa©Úl DFO tamsrésQamGMLúgeBlbBa©ÚlQam/ brimaNfñaM DFO

EdlRtUveRbI RtUvykmklayCamYyesr:UméRbFmμta ¬Normal saline¦.

m¢úlrbs;fg;esr:UmEdlmanpÞúkfñaM DFO RtUvedatbBa©ÚlCaEbb “Y” CamYy

fg;Qam ehIyRtUvbBa©Úl[GñkCMgWkñúgry³eBlRbEhl 4 ema:g. eKGaceRbI

sñb;GMLúgeBlbBa©ÚlenHk¾)an RbsinebIeKcg;. CYnkal eBlevlaRtUvkar

edIm,IbBa©ÚlfñaM DFO mYykMriteRbI[manRbsiT§iPaB manry³eBlyUrCag

ry³eBleRbIsMrab;bBa©ÚlQam.

sMxan;RtUvkMNt;cMNaMfa DFO EdleRbIEtkñúgGMLúgeBlbBa©ÚlQam/

)anesckþIfa 2 - 4 s)aþh_bBa©ÚlfñaM DFO mþg/ vamanRbsiT§iPaBtictYc

Nas;. ya:gNak¾eday enHCaCMerIsEtmYyKt; kñúgkrNIfñaM DFO mankar

xVHxat. müa:gvijeTot mnÞIreBTümYycMnYneRbIfñaM DFO bBa©Últamsrés

QamGMLúgeBlbBa©ÚlQam[GñkCMgW CakarbEnßmeTAelIkarBüa)alRbcaMéf¶

61

ya:geTogTat; kñúgkic©RbwgERbgedIm,IeFVI[)anRbesIrnUvRbsiT§iPaBTUeTAénkar

Büa)alkMcat;CatiEdk.

karcak;bBa©ÚlfñaM DFO tamsac;dMu

eBlcab;epþImeRbIfñaM DFO dMbUgedIm,Ibüa)alCMgWtaLasesmIu Kwcak;vabBa©Úl

tamsac;dMu. eTaHya:gNak¾eday eK)anrkeXIjfa viFIEbbenHBMusUvman

RbsiT§iPaBdUckarcak;bBa©ÚleRkamEs,k b¤ tamsrésQameLIy.

karcak;tamsac;dMu eKenAEteRbIkñúgkarNImYycMnYn ]TahrN_enARbeTsEdlfñaM

DFO mantMéléføeBk ehIymancMnYntictYc b¤ krNIKμansñb;sMrab;cak;.

manPsþútagkan;EteRcInbBa¢ak;fa GñkCMgW)anTTYlfñaM DFO xøH eTaHCatam

viFINak¾eday k¾RbesIrCagkarmin)anTTYlfñaMesaH. dUecñHebIKμanCMerIseTenaH

eKGaccak;fñaM DFO tamcak;dMuk¾)an. bec©keTsmYyeTot KW eKlayfñaM 500

mRk CamYyTwklayfñaM 5 ml ehIycak;sac;dMuéd b¤ sac;dMueCIg. kMriteRbIfñaM

enHeKEckCaBIr ehIyeRbI 2dgkñúgmYyéf¶ enHCaviFImYyEdlGñkRsavRCav

mYycMnYn)anbgðajnaeBlfμI²enHfa vamanRbsiT§iPaB.

vaytMélRbsiT§iPaBénkarBüa)aledayfñaM DFO

meFüa)aymYyénkarvaytMélRbsiT§iPaBénrbbkarBüa)alGñkCMgW KWkarvay

tMéleTAelIbrimaNCatiEdkEdlsþúkkñúgeføIm nig kñúgCalikaepSg²eTot

énsarBagÁkay. eføImmanlT§PaBGacpÞúkCatiEdk)aneRcIn )anCag 70°

énCatiEdkEdlsþúkTukkñúgsarBagÁkay KWRbEhl 20 Rkam. CatiEdkEdl

elIskñúgxøÜn vasþúkTukenAeføIm/ ebIeføImeBj KWvasþúkTukenACalikaepSg²

eTot. EdkEdlsþúk KWP¢ab;CamYyRbUetGIun Ferritin nig Haemosiderin dUcKña

nwgEdkEdlenAkñúgQamEdrEdlvaP¢ab;CamYyRbUetGIun Transferrin. man

Ferritin tictYcdac;ecjBIeføImcUlkñúgcrnþQam karvas;kMrit Ferritin kñúg

Qam KW eRbIedIm,IkMNt;bnÞúkCatiEdk.

62

kMritFmμtarbs; Ferritin kñúgQam ¬b¤ Serum ferritin¦ mandl; 250

mIRkURkam¼l ¬μg/l¦ sMrab;burs nig enAcenøaH 10 - 120 mIRkURkam¼l

sMrab;RsþI. 1 mIRkURkam ¬1μg¦ én Ferritin kñúgQamcat;faesμInwgEdk 8

mIRkURkam sþúkenAkñúgxøÜn. GñkCMgWtaLasesmIukMritF¶n; EdlmankMritCatiEdl

x<s;eRcInenAkñúgxøÜn eKrMBwgfamankMrit Ferritin x<s;ya:gsMebImenAkñúgQam.

eTaHbIkMrit Ferritin GacCaGñkeGaykarN_mYyKYr[Tukcitþ)anénEdksþúkTuk

kñúgeføIm EtvaminGac[eyIgTsSn_Tay)anc,as;las;BIbnÞúkCatiEdkenAkñúg

srIragÁepSg²eTot dUcCaebHdUg b¤ bnÞúkCatiEdkkñúgsarBagÁkayTaMgmUl

)aneLIy. elIsBIenH ktþaepSg²eTot dUcCa karrlak/ karbgáreraK

edayvIrus b¤ )ak;etrI/ CMgWeføImraMér:/ CMgWrlaksnøak; nig kgVHvItamInes Gac

b:HBal;elIkMrit Ferritin kñúgQam edaybBa¢ak;faCatiEdkmankMritx<s;Cag b¤

TabCag sPaBBitCak;Esþg.

ya:gNak¾eday kMrit Ferritin kñúgQamRtUvcat;TukfaCakMNt;sMKal;Cak;Esþg

bMputénplvi)akEdlGacekItmaneLIgedayCab;Tak;TgCatiEdk. ]TahrN_

karsikSamYycMnYn)anbgðajfa krNIkMrit Ferritin enAeRkam 2500

mIRkURkam¼l ¬μg/l¦ Cab;rhUtCaeRcInqñaM/ eRKaHfñak;cMeBaHGñkCMgW eGayman

plvi)akelIebHdUgmantictYc. dUecñH eKalbMNgKW RtUvrkSa Ferritin [enA

cenøaH 1000 - 2000 mIRkURkam¼l ehIyRtUvBinitükMrit Ferritin ya:gehac

Nas; 3Exmþg kñúgeKalbMNg[dwgfa etIkarBüa)alkMcat;CatiEdkrbs;

GñkCMgWRtUvEfmfy b¤ ya:gNa. vasMxan;pgEdr RtUvRtYtBinitülT§PaBBul

edayfñaM DFO EdlGacekItmaneBleRbIvakñúgkMritx<s; nig eBlEdlkMrit

Ferritin Føak;eRkam 1000 mIRkURkam¼l.

eRbIkMrit Ferritin edIm,IkMNt;kMriteRbI DFO

eKGaceRbIkMrit Ferritin edIm,IkMNt;kMriteRbIsmRsbrbs;fñaM DFO sMrab;

GñkCMgW edayeRbIsmIkadUcxageRkam EdleKehAfa snÞsSn_Büa)al

63

“Therapeutic Index” (TI) .

kMriteRbImFümRbcaMéf¶ ¬mRk¼KRk = mg/kg¦*

TI =

kMrit Ferritin kñúgQam ¬m>Rk¼l = mg/l¦

eKaledA KW RtUvrkSatMél TI [enAeRkam 0/025 RKb;eBlevla.

* kMriteRbImFümRbcaMéf¶én DFO KW KNnaedayKuN kMriteRbI EdleRbIkñúg

karBüa)alnImYy² CamYynwgcMnYnsrubkMriteRbIkñúgmYys)aþh_ Ecknwg 7

cMnYnéf¶éns)aþh_.

kñúgb:unμanqñaMcugeRkayenH eK)anbegáItetsþepSg²eTotmYycMnYnEdlGac

vas;EvgEdkkñúgsarBagÁkayRbkbedayPaBKYrTukcitþ)an. CaerOy²eK

GnuvtþetsþTaMgenH CamYykarvas;Evg Ferritin kñúgQam edIm,Ipþl;rUbPaBBit

R)akdEfmeToténkarpÞúkCatiEdkkñúgragÁkay CaBiess eføIm nig ebHdUg .

kMhab;EdkkñúgeføIm (Liver Iron Concentration = LIC)

karvas;kMhab;CatiEdkkñúgeføIm Tak;Tgnwg karRcwbykCalikaeføIm ¬Liver

biopsy¦ehIyvas;brimaNEdkkñúgsac;eføImEdlRcwbenaH.

karRcwbykCalikaeføIm KWeFVIeLIgedays‘km¢úlBiesstameBaHcUleTAeføIm

CaTUeTAeKeRbIfñaMs<wkRtg;kEnøgcak; ehIyeKcUlcitþeRbI eGkU ¬Ultrasound¦

CYynaMpøÚvykCalikaeføImmYyEpñktUc. karvaytMélbnÞúkCatiEdk)anRtwmRtUv

mYyEpñkBwgEp¥kelIbrimaN ¬ya:gehacNas; 1 mRk TMgn;eføIms¶Üt¦ nig

KuNPaB ¬)anesckþIfa KμanRkineføIm b¤ Kμan PaBcak;es£Hedaysrésevov¦

énCalikaeføImEdlykmkBinitü dUecñHsMxan; KWPaBsÞat;CMnajrbs;buKÁlik

eBTüEdlGnuvtþkargarenH. eTaHCakarRcwbykCalikaeføIm manGRtapl

vi)akTab eBlEdleyIgeFVIenAmnÞIreBTüman eGkU CYytMrg;Tisk¾eday k¾RtUv

TamTarnUvkaryl;RBmBIGñkCMgW nig¼b¤ «BukmþayGñkCMgW¼GaNaBüa)alEdr.

64

karRsavRCav)anbBa¢ak;fa krNIkMritCatiEdkkñúgeføImrkSa[enAeRkam 7

mRk¼Rkam énsac;eføIms¶Üt)anCab;lab;enaH eRKaHfñak;énbBaðaecaT

EpñkebHdUg b¤ eføIm mantictYc. ebIkMritelIs 15 mRk¼Rkam énsac;eføIms¶Üt

enaHvaGacmaneRKaHfñak;x<s;énkarsøab;ebHdUg. eTaHbIya:gNa karRsavRCav

enHkMBugbnþedIm,IeGaydwgfa etIkMritkMhab;EdkkñúgeføImdUcxagelIenH

GacbBa¢ak;KYr[Tukcitþ)anGMBIeRKaHfñak;xøaMg b¤ tic cMeBaHebHdUg b¤ y:agNa.

dUckñúgkrNIén Ferritin kñúgQamEdr bNþaGñkviTüasaRsþ)anecaTsYrfa

etIbec©keTsenHqøúHbBa©aMgya:gRtwmRtUvnUvbnÞúkCatiEdksrubkñúgsarBagÁkay

ehIyBiessCagenHeTotCatiEdkenAebHdUg b¤ ya:gNa. karRsavRCavfμI)an

bgðajfa karvas;EvgKYr[TukcitþbMputénCatiEdkkñúgxøÜn KWTTYl)aneday

KuNkMhab;EdkkñúgeføIm CamYyktþamYy KW 10/6 mRk¼KRk énTMgn;xøÜn.

karRcwbykCalikaeføImGacpþl;Bt’manpgEdr cMeBaHr)ayCatiEdk rvagBYk

ekasikaeføImdUcCa Hepatocytes nig ekasika Kuffer edaybBa¢ak;)anEdrBI

karrlak b¤ karxUcxatcMeBaHeføIm dUcCaRkineføIm b¤ PaBcak;es£Heday

srésevov.

viFIfμI²EdleFVImineGayQWcab; kñúgkarvaytMél[)nRtwmRtUvnUvbnÞúkCatiEdk

RtUv)aneKbegáItpgEdr dUcCa]bkrN_ SQUID (Superconducting Quantum

Interface Device) EdleRbIKuNsm,tþiGmma:ej:Ticrbs;Edk

¬Paramagnetic properties¦ edIm,Ivas;kMhab;rbs;vakñúgsarBagÁkay. vaeRbI

Ednma:ej:TicmYyEdlmankMlaMgRbEhlEdkqk;EdleKeRbIkñúgTUrTwkkk.

munnwgeRbI]bkrN_enH GñkCMgWRtUvedkeGayRsYlelIERKry³eBl 2 - 3 naTI

ehIyeKeRbI eGkU CYykMNt;TItaMgrbs;eføIm bnÞab;mkeKrujERKGñkCMgWcUleTA

cMBIeRkam]bkrN_ SQUID. Epñkrbs;ma:sIun SQUID Edlb:HdgxøÜnGñkCMgW

KWCa)aLúgmYyeBjedayTwkekþA]NÑ² EdleKdak;BIelIeBaHEpñkxagelIeday

snSwm². kñúgeBlEdlma:sIunkMBugvas;EvgCatiEdkkñúgeføIm GñkCMgWRtUvGt;

degðImry³eBl 2 - 3 dg/ kñúgmþg² 2 - 3 vinaTI. GñkCMgWGayueRkam 6qñaM

4f SQUID (Liver iron

measurement system)

65

GacRtUvkareRbIfñaMguyedkedIm,I[vaenAes¶óm. GñkCMgWTMgn;ticCag 10 KRk b¤

GñkCMgWFat;duHeBaH eKKitfamin)anpll¥ sMrab;etsþenHeT. CaGkusl

bec©keTsenHmantMéléfø TamTarnUvbec©kviTüax<s; ehIybc©úb,nñenHman

eRbIEtenAmCÄmNÐlR)aMEtb:ueNÑaHenAGWr:ub nig enAGaemrikxageCIg.

eTaHya:gNak¾eday cMeBaHGñkCMgWBIbNþaRbeTsnana ebIRKUeBTüBüa)alKitfa

caM)ac; eKGaceFVIkarNat;TukmunedIm,I)anBinitüedayma:sIun SQUID enHEdr.

viFIepSgmYyeTotedIm,Ivas;bnÞúkCatiEdkkñúgeføIm EdlkMBugEtRsavRCav

enAeLIy KWeRbI MRI (Magnetic Resonance Imaging) EdlBwgEpñkpgEdr

eTAelIKuNsm,tþiGmma:ej:Ticrbs;Edk . kMhab;EdkkñúgeføImdwg)aneday

viFIenH ¬MRI, T2*¦ bgðajnUvTMnak;TMngsmehtupl nig Tukcitþ)an CamYy

kMhab;CatiEdk)anBIkarviPaKKImIcMeBaHsac;eføImEdlRcwb. KuNRbeyaCn_d¾

manskþanuBlénbec©kviTüaenH KWfa MRI maneRbIeRcIn TUeTACag. eK)an

bgðajfa MRI Ca]bkrN_ mansar³sMxan;kñúgkarvaytMélbnÞúkCatiEdkkñúg

ebHdUgpgEdr.

CatiEdkkñúgTwkenam

viFIepSgmYyeTotEdlmanCayUrmkehIykñúgkarvaytMélbnÞúkCatiEdk KWvas;

CatiEdkkñúgTwkenamkñúg 24ema:g edIm,IvaytMélRbsiT§iPaBénkarBüa)al

kMcat;CatiEdkedayfñaM DFO. ya:gNak¾eday xøwmsarEpñkKøInikrbs;etsþenH

vamankMNt; eRBaHvaERbRbYlBImYyéf¶eTAmYyéf¶ kñúgkarbeBa©jCatiEdkeTA

kñúgTwkenamrbs;fñaM DFO.

plvi)akcMeBaHkareRbIfñaM DFO ehIyvabgðajeLIgEbbNa

RbtikmμEs,kenAkEnøgcak;fñaM

RbtikmμenAkEnøgcak;jwkjab;CageKbMput KW rma:s;/ Rkhm/ ehIm/ e)a:g/

dMe)A/ QW nig karrMxanTUeTA. cMnucKnøwHdUcxageRkamGacCYybnßyRbtikmμ

dUcxagelI)an ³

66

[i] eCosvagkars‘km¢úlEk,rsrésQam b¤ srésRbsaTsMxan;² edIm,Ibnßy

CaGtibrimanUveRKaHénkarxUcxat nig¼b¤ karhUrQam.

[ii] RtYtBinitüemIlfaetIfñaM DFO rlaykñúgbrimaNTwkRKb;RKan;Edr b¤ eT

¬Twklay 5 ml sMrab;fñaM DFO 500 mRk¦. ebIcaM)ac; eKbEnßmTwk

layedIm,I[varavEfmeTot.

[iii] RtUvbþÚrTIkEnøgcak;. eBaHrEmgCaTIkEnøgcak;d¾l¥bMput. GñkCMgWxøHcUlcitþ

cak;edIméd b¤ ePøA. eTaHya:gNak¾eday RbtikmμenAkEnøgcak;vaekIteLIg

mYyry³eBl eTaHTIkEnøgNak¾eday sMxan;RtUvdUrkEnøgcak;epSg²Kña.

kareRbI DFO yUr²eTA vaGacbgá[mandMue)a:genACMuvijkEnøgcak;. karenH

eKGackarBar)an edaybgVilCMuTIkEnøgcak; b¤k¾/ kñúgkrNIxøH minTaMgGs;

eT/ edaybMeBjnUvsarFatuKImI Hyluronidase bnþiccUlkñúgbMBg;cak;

)aøsÞic mundMeLIgsñb;cak;fñaM.

[iv] eKGacbnßykarQWcab;edaylabERkmfñaMs<wkdUcCa Emla rvag 30 -

60naTI muncab;epþImkarBüa)aleday DFO. ehImGacbnßyehImeday

s¥MtwkekþA]Nð²eRkayBIcak;fñaM DFO . sMrab;kareLIgRkhm/ rma:s;/

ehIm b¤ dMe)A/ evC¢bNÐitxøH eKeRbIERkm Heparin b¤ Fusicor.

[v] el,Óncak;bBa©ÚlfñaMk¾RtUvRtYtBinitüpgEdr edaysarfakarehIm GacekIt

maneLIgeBleKcak; DFO elOneBk.

[vi] evC¢bNÐitGaceFVIkarsMerccitþpgEdr eGayBYk Antihistamine dl;GñkCMgW

muncak;fñaM DFO b¤ kñúgkrNIF¶n;F¶r eKGacbEnßm Hydrocortisone 5 -

10 mRk eTAkñúgl,ayfñaM DFO.

[vii] mansñb;sMrab;cak;fñaMbBa©ÚleRcInRbePTenATIpSar ehIyGñkCMgWGaceRCIs

ykRbePTNamYymkeRbIsmnwgesckþIR)afñarbs;eK. sñb;cak;fñaMman

)aLúg KWCaRbePTfμICageKbMput. vatUcCag/ RsalCag ehIys¶at;Cag

RbePTcas;. vaeFVIeGaycMeNjeBlevlapgEdr ehIyGacfasmrmü

67

CageKsMrab;GñkCMgW eRBaHl,ay DFO RtUv)anlayTukmuneday»sfkarI

kñúglkçxN½ÐmYys¥atKμanemeraK. edaysarKuNsm,tþiTaMgGs;enHehIy

sñb;cak;fñaMEbbenHGacCYyeFVI[RbesIreLIgnUvkarGuvtþn_rbs;GñkCMgW

cMeBaHkarBüa)alkMcat;CatiEdk. eTaHya:gNak¾eday/ tMéléfø)aneFVI[

kareRbIR)as;sñb;cak;fñaMEbbenH minmanPaBTUlMTUlayeLIy.

[viii]GñkCMgWGacsakl,gm¢úlepSg² edayBikSaBIkarEfm b¤ fynUvRbePT

xus²KñaCamYyevC¢bNÐit b¤ Kilanub,dæak-yika nig GñkCMgWepSgeTot.

manGñkCMgWCaeRcInniymm¢últUc ehIyRsalehAfa m¢úlemGMe)Aelx

25 b¤ tUcCagenH cak;eTAelIépÞEs,kenAmMu 45 GgSa. GñkCMgWepSgeTot

cUlcitþm¢últUcEbbCa m¢úledatrUbPaBCab;CBa¢aMg ¬Thumbtack¦

EdleKcak;EkgeTAnwgEs,k ehIybiTCab;nwgEs,kedaybg;s¥itBiess .

RbsinebIviFIdUc)aneBalxagelI BMu)anCYyGñkCMgWBIRbtikmμTak;TgfñaM DFO eT

eKRtUvBicarNakarBüa)aledayfñaM DFO edaybBa©ÚltamsrésQam b¤

fñaMkMcat;CatiEdkepSgeTot.

RbtikmμF¶n;F¶rCamYyfñaM DFO

RbtikmμF¶n;F¶rCamYyfñaM DFO CakarkMr. eraKsBaØarYmman karTTYlGarmμN_

TUeTAminRsYlxøÜn/ hwugRtecok/ vilmux/ eLIgRkhmeBjxøÜn b¤ ehIm nig

Bi)akdkdegðIm CYnkalrYmCamYyekþAxøÜn/ Qwsac;dMu. eKRtUvbBaÄb;fñaM DFO

ebImaneraKsBaØadUcxagelIekIteLIg.

RbtikmμTUeTATaMgGs;enH manninñakarekIteLIgPøam² cMeBaHGñkCMgWEdleTIb

cab;epþImBüa)aleday DFO. cMENkÉGñkCMgWEdlTTYlkarBüa)aleTogTat;

nwgsMKal;eXIjeraKsBaØaekIteLIgbnþicmþg². kñúgkrNINak¾eday kar

Büa)alcMeBaHRbtikmμF¶n;F¶r CaviFImYyEdleKehAfa bnßyPaBqab;TTYl

Rbtikmμ ¬Desensitisation¦ EdlkñúgenaHGñkCMgWRtUveKcak; DFO kñúgbrimaN

tictYclayCamYy Hydrocortisone rYcbegáIn DFO bnþicmþg²rhUtdl;

68

brimaNeRcIn. karBüa)alenHRtUvGnuvtþeRkamkarRtYtBinitüBIRKUeBTü

ya:gditdl; CaerOy² eKeFVImþgehIymþgeTot rhUtdl;)at;eraKsBaØa ehIy

rEmgTTYl)aneCaKC½y. ya:gNak¾eday RbsinebIkarBüa)alenHmin)an

eCaKC½y GñkCMgWKYrEtQb;eRbIfñaM DFO ehIyBicarNakareRbIfñaMkMcat;CatiEdk

epSgeTot dUcCa Deferiprone ¬sUmGanenAEpñksþIBIkareRbI Deferiprone¦.

plvi)akcMeBaHkMriteRbIfñaM DFO minRtwmRtUv

plvi)akxageRkamenH sMxan;bNþalmkBIkareRbIfñaM DFO kñúgkMritx<s;elIGñk

CMgWkumar nig GñkCMgWEdlmankMrit Ferritin kñúgQamTab ¬)anesckþIfaman

elIsCatiEdkbnþicbnþÜc¦.

� bBaðaRtecok ¬BulcMeBaHRtecok¦ rYmmansMelgeralT×kñúgRtecok/

fycuHPaKxøHénkarsþab;B¤ CaBiess enAeBleRhVkg;x<s;.

� bBaðaEPñk ¬BulcMeBaHEPñk¦ rYmmanxVak;man;/ RBilEPñk/ kMritKMehIj

fycuH ¬Visual acuity¦/ fycuHKMehIjBN’/ eLIg)ayEPñk/ nig karrMxan

énEPñkepSg²eTot.

CaTUeTAfñaM DFO bgá[manbBaðaRtecok nig EPñk kalNaeKeRbIvakñúgkMritx<s;

kalNam:UelKul DFO eFVIdMeNIrkñúgQamedayKμan)anP¢ab;nwgEdk. ya:gNa

k¾eday GñkCMgWTwkenamEp¥m b¤ GñkCMgWkMBugBüa)aledayfñaMrMgab;GarmμN_

¬Psychotrope¦ GacmaneRKaHeGayekItbBaðaEbbenH eTaHCaeKTTYlkMritfñaM

DFO RtwmRtUvk¾eday eRBaHfasßanPaBTaMgenHeFVI[brimaNeRcInénfñaM DFO

eTAdl;RbB½n§srésRbsaTkNþal. GñkCMgWEdlekItbBaða

Rtecok nig EPñk CaTUeTAeKENnaM[Qb;eRbI DFO mYyry³

sin ehIycab;epþImkarBüa)alvij kñúgkMriteRbITabeBlplvi)ak

)anFUres,Iy b¤ )at;eTAvij. RbsinebI)antamdanBIedImTI

plvi)akEbbenHGacRKb;RKg)an ehIynwgFUrRsalmkvij.

dUcenHsMxan;RtUvRtYtBinitüGñkCMgWTTYlfñaM DFO [)aneTogTat;

4g Difference parts

of the brain have

different jobs to do

69

kñúgenaHmankarBinitüevC¢saRsþ[)aneBjelj rYmCamYykarvas;lT§PaB

sþab;lWrbs;Rtecok ¬Audimetric test¦ nig lT§PaBemIleXIjrbs;EPñk

¬Ophtalmologic test¦.

� karrIkFMFat;yWt nig karERbRbYlq¥wg ³ kMriteRbIfñaM DFO kMritx<s;elI

GñkCMgWmankMrit Ferritin Tab GacBnüakarrIkFMFat;. ktþaeRKaHfñak;rYm

man GayuekμgeBlcab;epþImBüa)al ¬ticCag 3 qñaM¦ ehIykMriteRbI DFO

x<s;CagkarENnaMeGayeRbI ¬> 35 mg/kg cMeBaHkumartUc²¦. karbnßy

kMriteRbImkvij GaceFVIeGaykarrIkFMFat;vilmkFmμtavij dUcmunkar

Büa)aledayfñaM DFO.

\T§iBlfñaM DFO eTAelIkarrIkFMFat; rYmmandgxøÜn b¤ édxøIminsmamaRtKña rYmCa

mYykarxUcxatq¥wg b¤ snøak;EdleKeFVIeraKvinicä½y)anedaykarftviTüúsaRsþ .

ktþaepSg²eTot dUcCabnÞúkCatiEdkk¾Gacb:HBal;dl;karrIkFMFat; kñúgCMgW

taLasesmIu Edr. ya:gNak¾eday vagayRsYlnwgdwg)anfaetIkaryWtya:v

kñúgkarrIkFMFat; bNþalmkBIfñaM DFO eRbIkñúgkMritx<s; b¤ ya:gNa. ebIbNþal

mkBIfñaM DFO GñkCMgWnwgmineqøIytbnwgkarBüa)aleday G½rm:UnlUtlas;

¬Growth Hormone¦ eT. GñkCMgWKYrTTYlkarRtYtBinitü[)aneTogTat;

sMrab;karERbRbYldUecñH eRBaHfavaminFUres,IymkvijeTebIvaekItehIy.

> karbgáeraKedayemeraK Yersinia enterocolitica ³ karbgáeday

)ak;etrI Yersinia enterocolitica k¾eXIjmanCaTUeTACamYykareRbIfñaM

DFOEdr. ral;GtisuxumR)aNmanCIvitTaMgGs; RtUvkarFatuEdkedIm,IrIk

FMFat; kñúgenaHman )ak;etrI/ )a:ra:sIut nig Pñak;garbgáCMgWepSgeTot.

GtisuxumR)aNPaKeRcInmanrcnasm<½n§BiessEdlpþl;lT§PaB[vaTTYl

ykEdk)an. ya:gNak¾eday Yersinia vasßitkñúgRkum)ak;etrImYyEdlBMu

manmeFüa)aypÞal;xøÜnedIm,ITTYl)anEdk dUeqñHvaBwgEp¥kelI receptor

rbs; ferrioxamine EdlCasmasFatuekIteLIgeRkayBIEdkP¢ab;CamYy

fñaM DFO. DFO CaPñak;gardwkCBa¢ÚnFmμCatimYyrbs;Edk EdleKehAfa

70

Siderophore ¬mkBIBaküRkik “Sidero” )ann½yfa Edk nig “Phoro”

)ann½yfa dwkCBa¢Ún¦ dUcenHCalT§pl DFO KaMRT[mankarrIkFMFat;

én)ak;etrI Yersinia edaypþl;[)ak;etrIenH nUvCatiEdk. karbgáeraK

eday Yersinia GacmanlkçN³F¶n;F¶rxøaMgcMeBaHGñkCMgWelIsCatiEdk

ehIyeRKaHfñak;enHkan;EtFMeFg eBleKkMBugEteRbIfñaM DFO. dUecñHsMxan;

eKRtUveFVIeraKvinicä½ykarbgáeraKeday Yersinia [)anelOn ehIy

Büa)alvaedayfñaMpSH[)ansmRsb ¬sUmGanenACMBUksþIBIkarbgáeraK¦.

eraKsBaØaénkarbgáeraKrYmman QweBaH/ raK/ QWsnøak;/ ekþAxøÜn/

QWbMBg;k. ebImanekIteraKsBaØadUcenH eKRtUvbBaÄb;fñaM DFO rhUtdl;

)at;eraKsBaØa ehIyvKÁeBjénkareRbIfñaMpSH)anbBa©b;. mankarbgáeraK

F¶n;F¶r Edlbgáeday)ak;etrIepSg²eTot mandUcCa Klebsiella b¤ BYkpSit

¬Mucormycosis¦ GacBak;B½n§EdrnwgkareRbIfñaM DFO b:uEnþkarbgáeraK

TaMgenH vaminsUvjwkjab;dUcRbePT Yersinia eT. eKRtUvBinitüGñkCMgW

edayykcitþTukdak; ehIyRtUvrMlwkGñkCMgW[)anCaRbcaMfa RtUveTACYb

BikSaCamYyRKUeBTü kñúgkrNImanekþAxøÜnminGacBnül;)an. GacfaebI

caM)ac; RtUvbBaÄb;fñaM DFO sin rhUtdl;eBleraKvinicä½yNamYy )aneKrk

eXIjc,as;.

plvi)akd¾kMrcMeBaHfñaM DFO

eKminRtUvcak; DFO eday)aj;eGaylOncUlkñúgxøÜneT eRBaHvaGacbgá[

eLIgRkhmelIEs,k/ cuHsm<aFQam/ ebHdUgedIrjab; ehIymanrhUtdl;

snøb; ¬Shock¦. karxUcxatdl;tMrgenam nig bBaðasYtF¶n;F¶r

¬Bi)akdkdegðIm¦ RtUv)aneKraykarN_fa ekItmaneBlNacak;bBa©Últam

srésQam edaykMriteRbIx<s; 10 mRk¼KRk¼ema:g b¤ eRcInCagenH.

71

karmanKP’

karsikSaCapøÚvkarBMuTan;)anbBa¢ak;BIsuvtßiPaBénfñaM DFO GMLúgeBlman

épÞeBaHenAeLIy. bc©úb,nñenH dMbUnμand¾l¥cMeBaHGñkCMgWmanépÞeBaH EdlRtUv

karkMcat;CatiEdk KWRtUveCosvageRbIfñaM DFO GMLúgeBl 3Ex dMbUgénkar

manKP’. GñkCMgWmanépÞeBaH ehIymanelIsCatiEdkxøaMgkøa b¤ manbBaða

ebHdUgF¶n;F¶r/ RtUveRbI DFO kñúgkMritTab 20 - 30 mRk¼KRk¼éf¶

enARtImascugeRkayénKP’ ehIyKμanKuNvibtþiGVIeLIy. eKGaceRbI DFO

bnþeTotTnÞwmKñanwgkarbMe)AedaH.

karsMrbtamcMeBaHkarBüa)aledayfñaM DFO

plGaRkk; nig karQWcab;bNþalmkBIkareRbIfñaM DFO mann½ysMxan;fa

eKRtUvEtpþl;karKaMRTelIkTwkcitþdl;GñkCMgW edIm,I[Kat;Rbkan;x¢ab;kar

Büa)al. evC¢bNÐit/ Kilanub,dæak-yika nig GñkmanGaCIBepSgeTot dUcCa

GñkcitþsaRsþ/ mRnþIsgÁmkic© k¾dUcCa«Bukmþay RtUvpþl;dl;GñkCMgWRKb;v½y

CaRbcaMnUvkþIsgÇwm nig elIkTwkcitþeGaybnþkarBüa)aledayfñaM DFO BImYy

éf¶eTAmYyéf¶. TnÞwmnwgenHEdr eKminRtUvePøcnUvqnÞHd¾FMeFg edIm,Ies;enA

teTAeTot ehIyrs;edayPaBRbesIr EdlGñkCMgWmñak;²RtUvBRgwgxøÜneLIg

edIm,ICMnH[)annUvkMritTamTarEbbenH.

karBüa)alfñaM DFO mankarlM)ak/ eRbIeBlevla/ Qwcab; nig mantMéléfø

ehIyRtUvRbkan;x¢ab;Gs;mYyCIvit. eTaHbIvaCakarBüa)alCYyseRgÁaHCIvit

k¾eday/ DFO BMu)anpþl;[GñkCMgWnUvGarmμN_fa )anTTYlplRbeyaCn_Pøam²

dUceRkaykarbBa©ÚlQamenaHeT. pÞúyeTAvijKuNRbeyaCn_énkarkMcat;

CatiEdkvaGacbgðaj[eXIjkñúgry³eBlyUr kñúgkarkarBar b¤ Büa)alpl

vi)akF¶n;F¶rCaeRcInbNþalmkBIelIsbnÞúkCatiEdk. edaysarEtplRbeyaCn_

karBüa)aledayfñaM DFO BMubgðaj[eXIjPøam manGñkCMgWCaeRcInCYbbBaða

elIsCatiEdk sUm,IEtenARbeTsGPivDÆn_sMbUrfñaM DFO k¾eday enHedaysar

EtGñkCMgWKitfaBi)akGnuvtþn_tamkarBüa)al. eBlEdlGñkCMgWQandl;v½y

4h

72

Cag 10qñaM nig eBjv½y rEmgFujRTan;cMeBaHkarBüa)al eRBaHvaGacrMxan

dl;skmμPaBRbcaMéf¶/ GaCIB/ kic©karpÞal;xøÜn nig / kic©karsgÁm. dUecñH

eBlEdlenARbeTskMBugGPivDÆn_ GRtaGñkCMgWmanCIvitmankMritTab

CaTUeTAbNþalmkBIxVHxatfñaM nig sñb;sMrab;cak;fñaM/ enARbeTsGPivDÆn_vij

bBaðaTMngbNþalkmBIxVHPaBRbkan;x¢ab;tamkarBüa)aleTAvij. eRkABIcMNuc

sMxan;EdlRtUvpþl;fñaM nig sñb;sMrab;cak;fñaM EdlGñkCMgWRtUvkarsMrab;

karBüa)al b¤ k¾RtUvFana[)anfaGñkCMgWRtUvkan;x¢ab;eTAtamkarBüa)al/

GñkCMgWenARtUvkarCYyeRCamERCg nig elIkTwkcitþBIsmaKmCatiCMgW

taLasesmIu/ buKÁlikeBTü/ «Bukmþay nig GñkCMgWepSgeTot. TIF

¬shB½n§GnþrCati CMgWtaLasesmIu¦ k¾CaRbPBmYysMxan;pþl;karKaMRT

edayCYybBa©úHbBa©ÚlrdæaPi)al[CYydl;karBüa)al nig kmμviFIkarBar.

fñaMkMcat;CatiEdk epSg²eTot

RbsiT§iPaBrbs;fñaM Deferiprone (L1, Ferriprox®, Kelfer®)

edIm,IkMNt;RbsiT§iPaB nig karBarKuNvibtþibNþalmkBIeRbIfñaM Deferiprone

(L1) eKRtUvGnuvtþCMhandUcteTA ³

[i] eKRtUvvas;kMrit Ferritin kñúgQamerogral; 3Ex mþg.

[ii] eKRtUvrab;RKab;QamTaMgGs;erogral;s)aþh_ edaytamdancMnYneKalika

Qam>s.

[iii] erogral; 3 Ex mþg eKyksMNakTwkenamkñúg 24 ema:g edIm,IkMNt;bnÞúk

CatiEdkkñúgTwkenam. karkMNt;bnÞúkCatiEdkenHmanlkçN³sMxan;Biess

kalNaeKeRbIfñaM L1 eRBaHbrimaNCatiEdkesÞIrEtTaMgGs;beBa©jtam

Twkenam xusBIfñaM DFO.

[iv] karviPaKCIvKImI ¬Biochemistry¦ GMBImuxgareføImRtUvEteFVIerogral;Ex.

4h L1 molecule

73

[v] KYrvas;kMritCatisgá½sI erogral; 3 - 6Ex.

RKUeBTüBüa)al CaGñkdwgBIsPaBrbs;GñkCMgWc,as;las; KW CaGñksMerceTAelI

cMnYndg nig lMdab;lMedayetsþtamdan. ebIGaceFVI)aneKRcwbsac;eføImmk

Binitü ¬k¦/ eRbI]bkrN_ SQUID ¬x¦/ eRbI MRI ¬K¦ Gacpþl;Bt’man

c,as;las;BIkMritbnÞúkCatiEdkkñúgeføIm nig CalikaebHdUg.

GMLúg BIrbIqñaMcugeRkayenH Tinñn½ysMxan;²mYycMnYnmaneRcIneLIg²Tak;Tgnwg

RbsiTi§PaB nig suvtßiPaBfñaM Deferiprone . RKUeBTüBüa)alCMgWtaLasesmIu

CaeRcIn )aneFVIkarviPaKTinñn½yTaMgGs;EdlmanTak;TgnwgRbsiT§iPaB RBmTaMg

PaBgayRsYltamdaneBleKeRbIfñaMenH.

eK)aneFVIkarvaytMélfa cab;taMgBIkarsakl,gKøInikelIkdMbUgbMputmk cMnYn

GñkCMgWsrubCag 15000 nak; )anTTYlfñaM Deferiprone enACag 50 RbeTs

TUTaMgBiPBelak mYyPaKFMsuT§EtCaGñkCMgWtaLasesmIu elIsbnÞúkCatiEdk.

bc©úb,nñenHfñaM Deferiprone )anRtUvcuHbBa¢IkarenACag 50 RbeTsTUTaMg

BiPBelak. b:uEnþenAminTan;mankaryl;RsbBI rdæ)alcMNIGahar nig

»sf ¬FDA¦ enAeLIyeT. rhUtdl;Ex mIna qñaM 2007 eK)ancuHfñaM

Deferiprone kñúgGtßbTviTüasaRsþ 495 c,ab; kñúgkarsakl,gKøInikcMnYn 60

¬13 eFIVedaymineRCIserIs¦ nig cuHkñúgGtßbTepÞógpÞat;EpñkviTüasaRsþcMnYn

78 c,ab;.

plrMxanrbs;fñaM L1

eKsMKal;eXIjfa GñkCMgWmYycMnYntUcRtUvbBaÄb;kareRbIfñaM Deferiprone

bNþalmkBIkarBul. plb:HBal;sMxan;bMputrbs;fñaM L1 KWkarfycuHeKalika

Qam>s CaBiessKW Neutrophils EdledIrtYnaTIsMxan; kñúgkarkarBarsar-

BagÁkayTb;Tl;nwgkarbgáeraK.

karfycuHlμmén Neutrophils ¬500 - 1500¼mm3¦ eKehAfa

74

Neutropenia, ÉkarfycuHxøaMg ¬< 500¼mm3¦ eKehAfa Agranulocy-

tosis. eyagtamkarsikSamYycMnYn EdlvaytMélcMeBaHkareRbIR)as; L1,

]sSah_eXIj Neutropenia Cag/ rIÉ Agranulocytosis ekItmankñúgkrNI

tictYc. ya:gNak¾eday krNITaMgBIrenHGacvilRtLb;mkFmμtavij ebIGñk

CMgWQb;eRbI L1. dUecñHvasMxan;Nas;cMeBaHGñkCMgWeRbIfñaM L1 RtUvBinitücMnYn

eKalikaQam>s ya:gehacNas;erogral; 7 eTA 10 éf¶ mþg. eKRtUvBinitü

GñkCMgW[)anjwkjy ERkgmanbgðajeraKsBaØaénkarbgáeraKNamYy.

sBaØadMbUg KWekþAxøÜn/ QWbMBg;k b¤ sBaØaepSgNamYyeToténkarbgáeraK/

GñkCMgWRtUvQb;eRbI Deferiprone Pøam ehIyBinitüQamlMGit nig pþl;kar

Büa)al[)ansmRsb. krNIPaKeRcIncMnYneKalikaQam>s vilRtLb;mk

Fmμtavij ehIy Deferiprone Gaccab;epþImvijmþgeTot)an. eTaHya:gNa

k¾eday eKcab;eRbI L1 vij Tal;EteKalikaQams esμI b¤ elIsBI

3000¼mm3/ cMnYn Neutrophils esμI b¤ elIsBI 1000¼mm3 hIycMnYn

pøaEkt esμI b¤ elIsBI 100 000¼mm3.

edaymanKuNvibtþiEbbenH eKRtUvBicarNaedayykcitþTukdak; munnwgeRbIfñaM

L1 cMeBaHGñkCMgWtaLasesmIukMritF¶n; ehIymanCMgWrlakeføIm B nig¼b¤ C

EdlsuT§EtTamTarkarBüa)aleday Interferon pgEdr.

plrMxanepSg²eTotrbs; Deferiprone

[i] GñkCMgWmYycMnYnEdleRbI L1 TTYlnUvkarQWcukcab; nig rlakenAtam

snøak; dUcCak,alCgÁg; keCIg Ekgéd RteKok nig¼b¤ cegáH. BYkeKGac

mansPaBrwgxøÜn nig Bi)akeFVIclnaepSg². eTaHbIeKBMuTan;yl;)anc,as;

BIbuBVehtubgá[manplrMxanenHk¾eday EteKKitfavaGacmkBIkarrlak

¬Inflammation¦ EdlGacbgáedayBYkCatiEdkEdlfñaM L1 RbmUlBIkEnøg

epSg²mkpþúMenAsnøak;. karbnßykMriteRbI b¤ bBaÄb;fñaM L1 plrMxanenH

nwg)at;eTAvij. karQWcab;snøak; Gacbn§ÚbnßyedayeRbIfñaMbM)at;karQW

cab; ¬Analgesic¦. ebIkarbnßykMriteRbIfñaM L1 nig¼b¤ eRbIfñaMbM)at;kar

75

QWcab;enAEtmin)anFUres,Iy eKRtUvbBaÄb;eRbIfñaM L1 Etmþg. mü:ageTot

ebIkarQWsnøak;ehIymanehImeTot CaFmμtaeKtMrUv[GñkCMgWQb;eRbIfñaM

Edr. eKGaccab;epþImeRbI L1 eLIgvijmþgeTot edaykMriteRbITabCagmun

ehIytamdaneGayditdl; eRBaHvagaynwgbgáeGayQW nig ehImsnøak;

ekItmanmþgeTotEdr.

[ii] bBaðaRkBHeBaHevon dUcCa ceg¥ar/ vilmux/ k¥Üt/ raK nig QWeBaH ekItman

elICMgWxøHEdleRbI L1. eraKsBaØaTaMgenHrEmgminsUvxøaMgkøa ehIymin

TamTarkarBüa)aleT. fñaM ¬bM)at;CatiGasIutkñúgRkBH/ bM)at;k¥Ütceg¥ar¦

CaTUeTAbnßyeraKsBaØaTaMgenH. elIsBIenHeTot elbfñaM L1 CamYycMNI

GaharGacCYybnßyk¥Ütceg¥arEdr.

[iii] GñkCMgWeRbI Deferiprone GacnaMeGaykgVHCatixniC nig sgá½sI ¬Zinc¦

edaysarfñaMenHvaGacP¢ab;nwgelah³FatuepSg²eTot eRkABIelah³Cati

Edk dUcCa sgá½sI EdlCasarFatuEdlsarBagÁkayeyIgRtUvkarsMrab;

dMeNIrkarmYycMnYn dUcCakarrIklUtlas;ragÁkay nig Es,k.

karfycuHkMritsgá½sI eKsMKal;eXIjmanelIGñkCMgWxøH BiessGñkmanCMgW

TwkenamEp¥m. bBaðaTaMgenHeKGacEksMrYlmkvijedaypþl;sgá½sIbEnßm.

vaCakarcaM)ac;pgEdr edayFanafakar)at;bg;elah³FatuepSg²eTot

edaysarkareRbIfñaM L1 RtUv)aneKpþl;b®gÁb;mkvij. TisedAénkarkMcat;

CatiEdkkñúgCMgWtaLasesmIukMritF¶n; KWbeBa©jecalEtCatiEdkEdlelIs

bgá[Bul ehIymaneRKaHfñak;Etb:ueNÑaH ehIyrkSaeGay)anBYkelah³

FatusMxan;²epSgeTotkñúgsarBagÁkay.

[iv] karBuleføIm ³ karsikSaenAmCÄmNÐlCaeRcInbgðajfamankarekIneLIg

kMritGg;sIumrbs;eføIm dUcCa ALT CaTUeTAmanlkçN³beNþaHGasnñ ehIy

ekItjwkjyelIGñkCMgWrlakeføImRbePT C. lT§pletsþmuxgareføIm

ERbRbYlcuHeLIg TamTar[GñkCMgWmYycMnYntUcRtUvEtQb;eRbI L1. man

ÉksarmYy)anbgðajfa sPaBcak;es£Hedaysrésevov ¬Fibrosis¦

76

enAeføImGacvivtþn_eTAmuxelOneBleRbI L1 CagkareRbIfñaM DFO ehIyeK

min)anepÞógpÞat;karGegátTaMgenH enAkñúgkarsikSamYycMnYneTot nig

karvaytMélkñúgry³eBleRkaymkenaHeT. eTaHCaya:gNak¾eday krNI

GñkCm¶WmanCMgWrlakeføImRbePT C skmμ eKGacsegáteXIjmankarekIn

eLIgsPaBcak;es£HedaysrésevovenAeføIm enAdMNak;kaleRkaymk

eTot .

kareRbIfñaMkMcat;CatiEdkedaypÁúMcUlKña

kñúgbNþaqñaMcugeRkayenH mCÄmNÐlevC¢saRsþmYycMnYn)aneRbIrbbBüa)al

pÁÜbKña KW Desferrioxamine nig Deferiprone edIm,I[)anlT§pll¥. eKal

KMnitenHKWfa fñaMkMcat;CatiEdkTaMgBIrGaccab;ykCatiEdkBIRbPBepSg²Kña.

kñúgrebobdUcenHskmμPaBfñaMmYyRtUvbMeBjbEnßmedayfñaMmYyeTot. fñaMmYy

CaGñkdwkCBa¢ÚnCatiEdkBIekasikaeTAkñúgQam EdlenATIenaHfñaMTI 2 )anTTYl

nigP¢ab;ehIybeBa©jmkeRkAsarBagÁkay. ynþkarEbbenHeRbob)annwg

{plekItBIRtl;tm,aj ¬Shuttle effect¦}. viFIsaRsþénkarBüa)aleday

eRbIfñaMpÁÜbenH kMBugsßitkñúgkarsikSaenAeLIy. Etya:gNak¾eday lT§plén

kareRbIfñaMpÁÜbbgðajfa GñkCMgWelIsbnÞúkCatiEdkF¶n;F¶r b¤ elIsbnÞúkCatiEdk

enAebHdUg ¬edayEp¥kelIlT§pl MRI T2*¦ kareRbIfñaMpÁÜbKñaBitCaman

RbsiT§iPaB. lT§plenaHbgðajfa mankarFUres,Iymuxgarrbs;ebHdUg

¬muxgarftebHdUgxageqVg Ep¥ktam T2* nig karEk)aneLIgvijsPaBcgVak;

ebHdUgedIrmineTog¦. dUecñH kareRbIfñaMpÁÜbKña

k¾pþl;plRbeyaCn_dl;karrs;ranmanCIvit

rbs;GñkCMgWpgEdr CaplmYymankarepÞógpÞat;

edaykarsikSaelIkarrs;ranmanCIvitrbs;Gñk

CMgWEdleK)ane)aHBum<pSay.

4j Shuttle effect

77

fñaMfμI Deferasirox CaRbePTRKab;elbtammat;

bc©úb,nñenH fñaMkMcat;CatiEdkepSgeTotmYycMnYnkMBugsßitkñúgkMNak;kal

RsavRCav BiessfñaMkMcat;CatiEdkeRbIelbtammat;)an. manfñaMmYyb:ueNÑaHén

bNþafñaMTaMgenH)anQancUldMNak;kalsakl,gKøInIk KWfñaM ICL670 ehA

müa:geTotfa Deferasirox ¬b¤maneQμaHBaNiC¢kmμfa Exjade®¦. Rkumh‘un

plitfñaMenH KWRkumh‘unEdlplitfñaM Desferrioxamine.

eRkayBI)anbBa©b;karsakl,gKøInik mkdl;eBlenHeKkMNt;)annUvsuvtßiPaB

RbsiT§iPaB nig kMritfñaMsmRsbrbs;mnusS. enAkñúgkmμviFId¾FMbMputénkarsak

l,gKøInIk cMeBaH Deferasirox EdleFVIeLIgelIGñkCMgW KWmanGñkCMgW)ancUlrYm

dl;eTACag 1000 nak;. mkdl;eBlenH manbTBiesaFn_ 5 qñaMehIybnÞab;

BIkaryl;RBmCapøÚvkarmk.

manCag 70 RbeTskñúgBiPBelakehIy Edl)anpþl;karyl;RsbcMeBaHfñaM

Deferasirox dUcCa ³

� shrd§Gaemrik ¬FDA¦

� RbeTssVIs

� GWru:b

� kaNada

� GURsþalI

� RbeTsGara:b;rYm

� GIuRsaEGl

� eRbsIul nig

� GahSg;TIn

enAbNþa 50 RbeTs fñaM Deferasirox )andak;[eRbIR)as;dl;GñkCMgW eday

minkMrittwgrwgdl;karecjevC¢bBa¢NaeLIy. mkdl;RtImasTImYyénqñaM

2007 fñaMenHman[eRbIR)as;enAbNþaRbeTsepSgeTotCaeRcIn.

4k ICL670A molecule

78

mkdl;eBlenH eKvaytMélfa enATUTaMgBiPBelakmanGñkCMgWRbmaNCa

15000 nak; TTYlkarBüa)aledayfñaM Deferasirox .

Deferasirox eRbIsMrab;Büa)alelIsbnÞúkCatiEdkraMér:bNþalmkBI ³

k> GñkCMgW Ebtta taLasesmIu cab;BIGayu 2 qñaMeLIgeTA ehIyTTYlkar

bBa©ÚlQameRcIndg.

x> GñkCMgWTTYlrgsPaBesøksøaMgepSg² nig GñkCMgW Ebtta taLasesmIu

EdlbBa©ÚlQamjwkjab; b¤ eBlNakarBüa)alkMcat;CatiEdkedayfñaM

Deferrioxamine minGaceFVIeTAekIt b¤ k¾eKKitfaenAminRKb;Tan;RKan;.

karsMerccitþcab;epþImeRbI Deferasirox eRscEtenAelIkarvaytMélrbs;RKU

eBTüBüa)al. kñúgkMrit fñaM 20-30 mRk¼KRk¼éf¶ Deferasirox kMcat;Cati

EdkecjBIeføImRbhak;RbEhlnwgkareRbIfñaM Desferrioxamine kñúgkMrit 35-

40 mRk¼KRk¼éf¶. bc©úb,nñenHBMumankareRbIfñaM Deferasirox pÁÜbCamYykMcat;

CatiEdkEpSgeToteT eRBaHKμankarsikSapþl;Bt’mansMrab;kareRbIfñaMpÁÜbdUcenH

eT.

Deferasirox manlT§PaBpþl;karkarBar)an 24 ema:g edayelbEtmþgkñúg

mYyéf¶erogral;éf¶. sUmkMNt;cMNaMfa muncab;epþImkarBüa)alkMcat;CatiEdk

edayfñaM Deferasirox eKRtUvkMNt;kMrit Ferritin kñúgQammun. elIsBIenH

muxgareføIm nig tMrgenamk¾dUcCamuxgarRtecok nig EPñk RtUvEtBinitüCamun

sin. kñúgGMLúgBüa)alk¾RtUvBinitütamdandUcKñaenHEdr. enHKWeyageTAtam

karENnaMsMrab;kareRbIR)as;fñaMenHBIRkumh‘unplitfñaM nig eTAtamkarENnaM

rbs;evC¢bNÐit.

pøÚveRbIR)as;fñaM

Deferasirox manTMrg;CafñaMRKab;EdlrlaykñúgTwk b¤ TwkRkUcRc)ac; b¤ TwkEpø

e)a:m. fñaMenHelbmunBisarGarharya:gehacNas; 30 naTI eBlvarlay

79

Gs;PøamkñúgTwk. eKminelbfñaMenHkñúgsPaBminrlay b¤ CaemSAeLIy.

kñúgmYyéf¶eKeRbIEtmþgkñúgkMrit 20-30 mRk¼KRk.

etI Deferasirox dMeNIrkardUcemþc

Deferasirox eTAP¢ab;nwgGatUmEdkkñúgTMrg; Fe3+ . dUecñHGatUmmYyrbs;

Fe3+ RtUvP¢ab;nwgmU:elKul Deferasirox BIr. bNþMú ¬Complex¦ EdlekIt

ecjBIkarP¢ab;m:UelKulEdk nig mU:elKul Deferasirox enHRtUvbeBa©jecal

tamlamk. edaysarvaeFVIemtabUlIsyWtkñúgeføIm fñaMenHvasßitkñúgQam

ry³eBlCaeRcInema:g ¬Bak;kNþalCIvitrbs;vamanBI 9-11 ema:g¦. dUecñH

kMritfñaMelbmYydgkñúgéf¶GacmanRbsiT§iPaBry³eBleBj 24 ema:g.

RbsiT§PaBrbs;fñaM

eK)anbgðajfa kMritfñaM 20 mRk¼KRk GaceFVI[kMrit Ferritin kñúgQamenA

efrRbmaN 2000 μg/l rIÉkMritfñaM 30 mRk¼KRk GacbnßykMrit Ferritin

cuHdl; 1000 μg/l kñúgGMLúgry³eBlmYyqñaMénkareRbIfñaMCab;rhUt. karRcwb

yksac;eføImmkBinitübgðajfa fñaM)ankMcat;CatiEdkecjBIeføImBiesseBleRbI

kMrit 30 mRk¼KRk. müa:geTot Tak;TinnwglT§PaBrbs;fñaMkñúgkarkMcat;Cati

EdkecjBIebHdUg karBiesaFn_edaybNþúHekasika bgðajfa fñaMenHcUleTAkñúg

ekasika ehIycab;ykCatiEdkecjBIekasika. ya:gNak¾eday mkTl;nwg

eBlenH karBiesaFn_EpñkKøInikBMuTan;)anbBa¢ak;[)aneBjeljnUvkarrkeXIj

TaMgenHenAeLIyeT. eKrMBwgdl;karsikSaTUlMTUlay nig Evgq¶ayCagenHt

eTAeTot.

plrMxanrbs;fñaM Deferasirox

kñúgs)aþh_dMbUgénkareRbIfñaM eKGacCYbRbTHeraKsBaØaraKruHeFVITukçeBaH. eraK

sBaØaenHqab;)at;eTAvij ehIyCakarkMrEdlevC¢bNÐitcaM)ac;RtUvbnßykMritfñaM.

GaceFVIeGayekþAxøÜn nig pþasaymYyry³eBl. GñkCMgWRbEhl 10 ° Gac

80

CYbsPaBknÞÜlrmas;elIEs,k EdlGacraldaleBjxøÜn b¤ enA)atéd b¤ )at

eCIg mYyPaKFMekItmanGakar³enHkñúgGMLúgeBl 15 éf¶ dMbUgénkareRbIfñaM.

eRkaymkrEmg)at;eTAvijbnþicmþg²edayBMucaM)ac;bBaÄb;fñaM b¤ bnßykMrit

fñaMeT.

eRkABIenH man 1° eKsegáteXIjkMrit Creatinine ekIneLIg ¬Creatinine

CaRbUetGIunmYyEdlmanTMnak;TMng ya:gsñiteTAnwgmuxgarrbs;tMrg;enam¦.

PaKeRcInkrNIEbbenH vamanTMnak;TngnwgkMritfñaMeRbI. müa:geTot manGñkCMgW

mYycMnYntUcbu:eNÑaH EdlkMrit Creatinine ekItx<s;hYskMritFmμta. PaKeRcIn

kMrit Creatinine vilRtLb;mkkMritFmμtavij eBleKbnßykMritfñaM. )an

esckþIfa GñkCMgWPaKeRcInEdleKrkeXIjsPaBEbbenH minKYrekItkarRBYy

)armÖeT EtCakarbBa¢ak;fa vaRtUvtamdanemIlmuxgartMrgenam[)anjwkjab;

KW[)anral;Ex. müa:geTot eKminRtUvpþl;fñaMenHcMeBaHGñkCMgWEdlexSaytMrg

enam nig¼b¤ kMBugQaneTArkkarexSaytMrgenam.

eKkMreXIjPaBminl¥énGg;sIumrbs;eføIm ¬kMeNIt Transaminase¦. dUcKña

enHEdr ebIeføImmandMeNIrkarminRsYleTAehIy RKUeBTüBüa)alKWCaGñksMerc

faKYr b¤ minKYreRbIfñaMenH ehIydMeNIrkarGg;sIumrbs;eføImRtUvBinitü[)an

ral;Ex. PaBvibløasekItenARtecok nig EPñkk¾eKeXIjelIGñkCMgWmYycMnYn

tUcEdr. ya:gNak¾eday dUcKñanwgkareRbIfñaM Desferrioxamine Edr eKRtUv

BinitüRtecok nig EPñk[)anral;qñaM.

bMrameRbIR)as; ¬Contra-indications¦

kareRbIfñaMepSgeTotkñúgeBlCamYyKñanwgfñaM Deferasirox fñaMmYycMnYn dUcCa

Rifampicin b¤ Phenobarbitone b¤ Phenytoin GaceFVI[kMhab;fñaM

Deferasirox fycuHkñúgQam. enHeFVI[RbsiT§iPaBfñaMfycuHEdr. ebIcaM)ac;

RtUveRbIfñaMdUcxagelI KWRtUvmankarENnaMBIRKUeBTüBüa)al.

kareRbIfñaMvItamInesdMNalKñanwgfñaM Deferasirox BMuTan;mankarsikSaenA

81

eLIy. eTaHya:gNa BMuTan;eXIjmanplb:HBal;NaeTeBleKeRbIvItamInes

200 mRk CalkçN³mUldæanRbcaMéf¶. müa:geTot

eKRtUveCosvagkarelbdMNalKñanUvfñaMbM)at;CatiGasIudmanpÞúk GaluymJÚ:m

¬Alumimium¦ dUcCa Alumimium hydroxide ¬Maalox nig¼b¤ Gaviscon¦

eRBaHBMumankarsikSaBIkarpÁÜbEbbenHeLIy. ebIcaM)ac;RtUvEteRbIfñaMenH enaHeK

ENnaM[eRbIvaeRcInema:geRkayfñaM Deferasirox.

karmanépÞeBaH nig kareRbIfñaM Deferasirox

edaykgVHTinñn½y/ eKhammin[eRbIfñaM Deferasiox kñúgGMLúgeBlmanKP’

eLIy elIkElgEtRKUeBTüBüa)alyl;facaM)ac;sMrab;GayuCIvit.

82

CMBUkTI 5

bBaðaevC¢saRsþbNþalmkBItaLasesmuI nig karBüa)al

karBüa)altaLasesmuIkMritF¶n; mankarvivtþn_y:agxøaMgkñúgry³eBlBIrTsvtS

cugeRkayenH EdleFVI[GRtars;ranmanCivitekIneLIgkMritx<s; Rbkbeday

KuNPaBCIvitGñkCMgWmanPaBRbesIrxøaMgkøa CaBiessGñkCMgWenARbeTselak

xaglic. ya:gNak¾eday GñkCMgWtaLasesmuIkMritF¶n;GacTTYlrgnUvbBaða

evC¢saRsþmYycMnYn kñúgenaHxøHbNþalmkBICMgWpÞal; ÉmYycMnYneTotbNþal

mkBIkarBüa)almanlkçN³minRtwmRtUv b¤ min)anditdl; rYmmankarbBa©Úl

Qammin)anRtwmRtUv xVHsuvtßiPaBQam nig xVHlT§PaBeRbIfñaM DFO.

GeNþIkrIkFM

GñkCMgWtaLasesmuIkMritF¶n;PaKeRcInmanbBaðaecaTelIGeNþIk ¬Spleen¦

EdlCasrIragÁmYyTn;BN’RsKaM TMhMb:unkNþab;éd sßitenAeqVgéd xageRkam

sac;dMusnÞHRTUg nig eRkamq¥wgCMnI ¬emIlrUb 5a¦. GeNþIkFmμtamanpÞúk

eKalikaQamRkhm 20 - 30 ml EtGñkCMgWEdlmankgVHRKab;Qam

RkhmmFüm nig F¶n;F¶r ry³eBlyUrEdlbNþalmkBIkarbBa©ÚlQam

minRtwmRtUv GeNþIkGacpÞúkQamrhUtdl; mYylIRt b¤ eRcInCagenH.

enHbNþalmkBIGeNþIkplitRKab;QambEnßmkñúgdMeNIrmYyeKehAfa

Extra-medullary erythropoiesis KWCakarplitRKab;Qam

enAeRkATItaMgFmμtarbs;vakñúgxYrq¥wg kñúgkic©RbwgERbgCYysarBagÁkay

edIm,ICMnHPaBkgVHRKab;QamRkhm.

GeNþIkbMeBjmuxgarsMxan;²mYycMnYn kñúgenaHrYmmankarkarBarsarBagÁkay

Tl;nwgkarbgáeraK edaykareRcaHQamkMcat;mIRkub/ )ak;etrI nig )a:ra:sIut

EdlebotebonsarBagÁkay ¬emIlrUb 5a¦. GeNþIkk¾CaGñkTTYlxusRtUv

83

pgEdr kñúgkarkMeTcecaleKalikaQamRkhmv½ycMNas;dl;Gayu edaykar

bMEbkvaedIm,Iyk køÚb‘Ín nig Edk BI eGm:UkøÚb‘Ín rbs;va edIm,IeRbIeLIgvij

pliteKalikafμI. cMeBaHGñkCMgWtaLasesmuIkMritF¶n; dMeNIrykCatiEdk nig

køÚb‘Ín edIm,IpliteLIgvijenH minRbRBwtþeTAdUcFmμtaeT. pÞúyeTAvijEdk)an

kkpþúMkñúgGeNþIk b¤ EdkRtUvbeBa©jeTAkñúgcrnþQam ehIybBa¢ÚnRtLb;eTA

GeNþIkvij. elIsBIenHeTAeTot eKalikaQamRkhmPaKeRcIn EdlGñkCMgW

taLasesmuIplit)an manrUbragÁxusFmμta ehIybNþal[vas¥itCab;kñúg

GeNþIk. CalT§pl GeNþIkrIkFMeTA² CYnkalk¾eFVI[eBaHGñkCMgWrIkFMtamenaH

Edr.

edIm,IxitxMeqøIytbtamtMrUvkarEdlekIneLIg GeNþIkritEtskmμxøaMgeLIg

eFVIeGayGeNþIkrIkFM. kñúgdMeNIrenHpgEdr k¾mankarbMpøajeKalikaQam

RkhmFmμtaEdl)anmkBIkarbBa©ÚlQam CaehtueFVI[GñkCMgWRtUvkarQam

ecHEtekIneLIgral;eBlbBa©Úlmþg² b:uEnþkarbBa©ÚlBMuGacman\T§iBleTAelI

PaBkgVHRKab;QamRkhmeT. GeNþIkskmμxøaMg k¾bMpøajsmasFatuepSg²

eTotrbs;QamEdr dUcCa eKalikaQams nig paøEkt.

eKminGacEkkunsPaBGeNþIkrIkFM)aneT. dUecñHebIeKdwgfa GeNþIksßitkñúg

sPaBmYybgáeRKaHfñak;dl;sarBagÁkaydUceBalxagelI eKRtUvEtkat;vaecj

eKehAfa Splenectomy Etkarkat;GeNþIkecjminGacBüa)altaLasesmuI

)aneT vaRKan;EtedaHRsaybBaðamYycMnYnb:ueNÑaH EdlbgáedaysarGeNþIk

rIkFM. karsMerccitþkat;GeNþIkecj RtUveFVIeRkayBI)anBicarNaedayykcitþ

Tukdak;eTAelIktþaevC¢saRsþmYycMnYnrYmmancMNucsMxan;dUcxageRkam ³

[i] GeNþIkrIkFM )anesckþIfamanRbEvgbeNþayEvgCag 6 sm ehIybgá[

mankarrMxan.

[ii] GñkCMgWecHEtRtUvkarbBa©ÚlQamekIneLIg eTaHKμanbBaðaecaTEpñkevC¢ -

saRsþepSgeTotk¾eday )anesckþIfa eBlNabrimaNtMrUvkarQamekIn

5b phagocyte filled with

bacteria

a neutrophil that

detected the

bacteria nearby

84

1/5dg b¤ elIsBI 200 - 220 m>l¼KRk¼qñaM éneKalikaQamRkhm

edIm,IrkSa[)ankMrit eGm:UkøÚb‘Ín mFüm.

[iii] GñkCMgWRtUvmanGayuelIsBI 5qñaM . dUc)anCMrabehIy GeNþIkedIrtYsMxan;

kñúgkarkarBarsarBagÁkayTl;nwgkarbgáeraK. dUcenHkarkat;GeNþIkecj

vabegáIneRKaHfñak;karbgáeraKF¶n;F¶r. elIkumarGayuticCag 5qñaM eRKaHfñak;

mankMritx<s;xøaMg eRBaHRbB½n§karBarrbs;vaenAminTan;maMTaMenAeLIy.

\LúvenHeKcat;fa karkat;GeNþIkecj CadMeNIrEpñkvHkat;suT§saF BMuTak;Tg

nwgeRKaHfñak;FMdMuEdleKeXIjenAGtItkaleT. eKk¾)anbegáItpgEdrnUv

bec©keTskat;GeNþIkEtmYycMENk ¬minTaMgmUl¦ b¤ bec©keTs

Embolisation kñúgkarRbwgERbgrkSamuxgarkarBarsarBagÁkayrbs;GeNþIk .

karGeNþIkecj nig karbgáeraK

karRBYy)armÖcMbgbMput eRkayBIkat;GeNþIkecj KWeRKaHfñak;énkarbgáeraK

F¶n;F¶r. man)ak;etrICaeRcInecaTCabBaðFMeFgcMeBaHGñkCMgWkat;GeNþIkecj

edaybgáeraKF¶n;F¶rGac[)at;bg;Civit. )ak;etrIEbbenH jwkjybMput KWBYk

emeraK Streptococcus nig Meningococcus.

eRKaHfñak;mankMritx<s;Nas; RbsinGñkCMgWmanGayuticCag 5qñaM ehIykan;Et

x<s;Nas;eTot ebIGayuticCag 2qñaM. eRKaHfñak;enAEtx<s;eBjmYyCIvit

eRkayBIkat;GeNþIk Biess 1 - 4qñaM eRkayBIvHkat;.

manbec©keTs 3 ya:g edIm,IkarBar b¤ bnßyCaGtibrimanUveRKaHfñak;bgáeraK

cMeBaHGñkCMgWkat;GeNþIkecj ³

[1] Immunoprophylaxis : kareFVI[manPaBsaMuedaycak;fñaMbgáarBYk

Pneumococcus, Haemophilus influenza nig Meningococcus.

karcak;fñaMbgáar FmμtaeKeFVIenARbmaNCa 2s)þah_ munkarvHkat;ehIy

mþgeTotenAeRkaykarvHkat; eyageTAtameKalkarN_ENnaM.

85

[2] Chemoprophylaxis : fñaMpSH FmμtaeKeRbIfñaM Penicilline elbtammat;

125 mRk elbBIrdgkñúgmYyéf¶ cMeBaHekμgGayuticCag 2qñaM ehIy

250 mRk elbBIrdgkñúgmYyéf¶ cMeBaHekμgGayuelIs 2qñaM. ebIGñkCMgW

minGaceRbI Penicilline )an eKGaceRbIfñaMpSHepSgeTot)an. y:agNa

k¾eday ry³eBlénkareRbIERbRbYlBIkrNImYy eTAkrNImYy. ]TahrN_

RKUeBTüxøH[GñkCMgWkat;GeNþIkecj eRbIfñaMpSHGs;mYyCIvit ÉRKUeBTüxøH

eTot[eRbIrhUtdl;GñkCMgWmanGayu 18qñaM ehIyGñkxøHeTot[eRbIEt

ry³eBl 2qñaM eRkayBIkat;GeNþIkecj. eTaHCakrNINak¾eday GñkCMgW

nwgTamTarnUvkarvaytMélCaeTogTat; edIm,Ifana[)anfa eRKaHfñak;RtUv

)anrkSa[enAkMritGb,brma.

[3] karGb;rMENnaM ³ Gb;rMENnaMGñkCMgW nig «BukmþayGñkCMgWGMBIeRKaHfñak;

énkarbgáeraKNamYy ehIyelIkTwkcitþBYkeK[RbugRby½tñcMeBaHsBaØa

NamYyénkarbgáeraKEdlGacekItmaneLIg ]TahrN_ ekþAxøÜn/ minRsYl

kñúgxøÜn b¤ Qwsac;dMu TaMgenHmansar³sMxan;Nas;. GñkCMgW nig «Bukmþay

GñkCMgW EdlmanKMerageFVIdMeNIrq¶ay RtUvyl;dwgpgEdr GMBICMgWqøgepSg²

EdlmanenAtMbn;epSg²kñúgBiPBelak.

[4] RtUvRbugRbyt½ñcMeBaHcMnYnpaøEktekIneLIg ³ karekIneLIgcMnYn

paøEkt GacekItmaneRkayBIkat;GeNþIkecj )anesckþIfa ekInelIsBI

800 000¼mm3. sPaBenHGacRKb;RKg)anedayeRbI Aspirine

50 - 100 mRk¼éf¶ rhUtdl;paøEktRtLb;mkkMritFmμtavij.

niyayCarYm/ CMhanl¥bMputKWRtUveCosvagbBaðaTak;TgnwgGeNþIk[)aneday

qab;cab;eRbIQammansuvtßiPaBehIy)anviPaKRtwmRtUv eBleraKvinicä½ydwgfa

CaCMgWtaLasesmuIkMritF¶n; edayrkSakMrit Hb [elIsBI 9 - 10 Rkam¼d>l

karenH GaceFVI[GeNþIkminqab;rIkFM b¤ minrIkFMk¾man ehIyeCosvag)ankar

kat;GeNþIkecj. elIsBIenHeTot karrkSaGeNþIk[enATMhMFmμta CYyFana

dl;RbsiT§iPaBénkarbBa©ÚlQam.

86

plvi)akcMeBaHebHdUg nig RkeBjbeBa©jkñúg (Endocrine)

GñkCMgWtaLasesmuIkMritF¶n; CaerOy²CYbbBaðaebHdUg/ eføIm nig RkeBj

beBa©jkñúg/ TaMgGs;enHrEmgbgáedaybBaðaelIsbnÞúkCatiEdk. b:uEnþvamin

dUcbBaðaebHdUgeT ¬GanenAEpñkxageRkam¦ GñkCMgWEdlTTYlQammin

RKb;RKan; b¤ min)anTTYlkarbBa©ÚlQam kMrmanbBaðaeføIm nig RkeBjbeBa©j

kñúgNas; mYycMENkedaysarsPaBTaMgenHekIteLIgcMeBaHGñkCMgW)anbBa©Úl

QameRcIn EtTTYlfñaMkMcat;CatiEdktictYc ehIymYycMENkeTotedaysar

GñkCMgWEdlbBa©ÚlQammin)anRKb;RKan;rs;enAmin)anyUr edIm,I[bBaða

TaMgenaHekIt)an.

plvi)akénkarelIsCatiEdk

ebHdUg exSayftebHdUgeRkam TaMgsgxag

¬Biventricular failure¦

cgVak;ebHdUgedIrmineTogTat; ¬Arrhythmia¦

RkeBj Pituitary Hypogonadotrophic – Hypogonadism

Bukq¥wg ¬Osteoporosis¦

RkeBjbeBa©jkñúg TwkenamEp¥m

Hypothyroid hypoparathyroid

eføIm sPaBcak;es£Hedaysrésevov RkineføIm/ CaBiess

ebImanrlakeføImRbePT es skmμ

plvi)akcMeBaHebHdUg

plvi)akcMeBaHebHdUg manCaTUeTAcMeBaHGñkCMgWbBa©ÚlQamminRKb;RKan; b¤

KμankarbBa©ÚlQam. GñkCMgWEbbenHmanPaBkgVHRKab;QamRkhmraMér:

RBmCamYykarkkpþúMénCatiEdkenAebHdUgecHEteRcIneTA² TaMgenHeFVI[ebHdUg

RbwgeFVIkarEfmeTot ehIyGaceFVI[exSayebHdUg.

87

mYyPaKFMénGñkCMgWbBa©ÚlQamminRKb;RKan; b¤ KμankarbBa©ÚlQam

minGacQandl;GayuxÞg; 20qñaM)aneLIy edaysarCMgWebHdUgCa

mUlehtucMbgbgá[søab;.

GñkCMgW)anbBa©ÚlQamRKb;RKan; Etmin)anTTYlkarkMcat;CatiEdk

manRbsiT§iPaB edaysarGñkCMgWmincg;eRbIfñaM DFO [eTogTat;tam

karENnaM b¤ edaysarfñaM DFO mingayrk)an ¬b¤ mantMéléføeBk¦

GacCYbbBaðaebHdUg bNþalmkBIelIsbnÞúkCatiEdk. dUc)aneBal

BIxagedImmkehIy karbBa©ÚlQam)annaMeTACamYynUvbrimaNCatiEdkbEnßm

CaeRcIncUlkñúgsarBagÁkay ebImin)ankMcat;ecj vanwgeTAkkpþúMenARKb;srIragÁ

TaMgGs;kñúgenaHmanebHdUg. CatiEdkelIskkpþúMenAebHdUgeRcIneTA² rMxandl;

dMeNIrkarFmμtarbs;va eFVI[vamansmtßPaBtictYcTb;Tl;nwgkarbgáeraK b¤

CMgWepSgeTot. CalT§pl sac;dMuebHdUgGacexSay eFVI[vaRc)ac;QameTAkñúg

sarBagÁkaymin)anxøaMg ¬emIlrUb 5c¦. GñkCMgWEdlbBa©ÚlQam)anRKb;RKan;

Etmin)ankMcat;CatiEdkecj[)anRKb;RKan; CaTUeTAnwgsøab;edaysarbBaða

ebHdUgenAGayuCag20qñaM.

EpñkepSg²mYycMnYnrbs;ebHdUgRtUvb:HBal; edayCatiEdkEdlelIs dUcCa

eRsamebHdUg/ sac;ebHdUg/ va:l;ebHdUg/ b¤ CalikabBa¢ÚncrnþGKÁisnIebHdUg.

EpñknImYy²enH manmuxgarepSg²KñaEdlGacb:HBal;edaysarelIsCatiEdk

edaybegáItCaeraKsBaØaepSg² rYmman cgVak;ebHdUgedIrmineTogTat;

¬Arhythmia¦/ karrlavijénsac;dMuebHdUgminRbRktI ¬Diastolic

dysfunction¦/ karkRnþak;rujQamxusRbRktI ¬Systolic dysfunction¦/

karRcalsÞHénCatiTwkkñúgCalika ¬karRCabTwkcUleRsamsYt/ eRsamebHdUg/

eRsameBaH[CaTacTwk/ ehImGvyv³¦ nig sBaØaexSayebHdUgepSg²eTot.

dUcKñaenHEdr CMgWebHdUgF¶n;F¶r bNþalmkBIelIsCatiEdk GacekItmaneLIg

edayKμaneraKsBaØaNamYyk¾man. dUecñHsMxan;GñkCMgWRtUvBinitüsuxPaB [)an

eTogTat;CamYyRKUeBTüCMnajCMgWebHdUgcab;taMgBIGayu 10qñaMeTA eBlEdl

88

minTan;eFVITukçemø:H edIm,IrkeraKsBaØa[Tan;eBlevla dUcCa j½rRTUg/ xSl;K/

ht;/ QWcugdegðIm/ hat;R)aNqab;ht; b¤ k¾ehImenACMuvijkeCIg b¤ enAEpñk

epSgeTotragÁkay. ebIkalNaeraKsBaØaEbbdUecñHekItehIy CMgWebHdUg

Gacfa )anvivtþn_dl;dMNak;kalF¶n;F¶rrYceTAehIy. y:agNak¾eday eTaH

muxgarebHdUg)anRtUvb:HBal;F¶n;F¶rk¾eday karBüa)alkMcat;CatiEdkxøaMgkøa

GacCYysþaebHdUgmksPaBFmμtavij)an.

tamdanmuxgarebHdUg

GñkCMgWtaLasesmuI KYrRtUvBinitüvaytMéleBjeljEpñkebHdUg ya:gehac

Nas;[)anmþgkñúgmYyqñaM - eRcIndgRbsinebImanbBaðaebHdUgehIy.

etsþdUcxageRkamenH CaEpñkd¾sMxan;sMrab;karBinitüRbcaMqñaM ³

� karBinitüragÁkayGñkCMgW nig Rbvtþi ¬kñúgenaHmankarerobrab;BIsBaØa

NamYy¦ .

� ftkaMrsμIGiucenARTUg ¬Chest x-ray¦. eTaHbIbc©úb,nñmanbec©keTs

pþl;Bt’manya:geRcInsMrab;GñkCMnajebHdUg k¾karftkaMrsμIGiucenA

RTUgenAEtCa]bkrN_d¾mansar³sMxan; kñúgeBlEdlviFITMenIbepSg

eTotBMumaneRbIR)as; edayvapþl;Bt’mansMxan;sþIBITMhMebHdUg/ sYt

RBmTaMgkarRBman[dwgBIdMuplitRKab;QamRkhmenAeRkAxYrq¥wg

EdlGacekItmaneLIg.

� ECG ¬vas;GKÁIsnIebHdUg¦ P¢ab;CamYylMhat;R)aN KWCaetsþmYy

nwgbgðajGakar³cgVak;ebHdUgedIrmineTogTat; b¤ dMeNIrkarminl¥én

ftebHdUgeRkam.

� ECG - ry³eBl 24ema:g. karP¢ab;]bkrN_tamdanBiess

¬Holter¦ Cab;xøÜnGñkCMgWry³eBl 24ema:g GaceGaydwg)anBIPaB

minFmμtaNamYyéncgVak;ebHdUgEdl ECG FmμtaminGacdwg.

5c A summary of the

homeostatic control of

the heart.

89

� eGkUebHdUg. enHKWCa]bkrN_mYymansar³sMxan;Nas; KwvabBa¢ak;BITMhM

ftebHdUg nig dMeNIrkarEpñknImYy²rbs;ebHdUg .

� MUGA scan . enHKWCakarBinitümYyeRbIsarFatu Radioisotope Edl

pþl;Bt’manbEnßmsþIBIdMeNIrkarrbs;ebHdUg. karBinitüenHGaceFVIeLIg

eBlGñkCMgWsMrak eTaHya:gNa Bt’manc,as;las;CagenHGacTTYl)an

ebIeKBinitüeBlGñkCMgWeFVIlMhat;R)aN.

edIm,IkarBar b¤ edIm,IRKb;RKgCMgWebHdUgelIGñkCMgWtaLasesmuIkMritF¶n; eKRtUv

Rbkan;ykviFankarN_mYycMnYn ³

� GñkCMgWEdlminTan;manplvi)akelIebHdUgenAeLIy RtUvpþl;Qam[)an

RKb;RKan;edIm,IrkSakMrit eGm:UkøÚb‘Ín (Hb) [enARbhak;RbEhlkMritEdl

eKENnaM KW 9/5 - 10 Rkam¼d>l (g/dl).

� GñkCMgWEdlmanplvi)akelIebHdUgehIy RtUvTTYlQam[)anRKb;RKan;

edIm,IrkSakMrit eGm:UkøÚb‘Ín muneBlbBa©ÚlQam [enAkMrit 10 - 11

Rkam¼d>l edIm,IFananUvkarpþl;GuksIuEhSn[sac;dMuebHdUg)anl¥.

kñúgeKalbMNgeCosvagbnÞúkF¶n;F¶relIebHdUg eKENnaMGñkCMgW[TTYl

QamEdlmankMhab;eKalikaQamRkhmkñúgbrimaNtictYcEteRcIndg.

krNImansPaBexSayebHdUg eKGacpþl;fñaMbBa©úHTwkenameBlbBa©Úl

Qammþg² eRkamkarRtYtBinitürbs;RKUeBTü.

� GñkCMgWmanbBaðaebHdUg b¤ elIsbnÞúkCatiEdk RtUvGnuvtþtamkmμviFIkMcat;

CatiEdkd¾xøaMgkøamYy/ kñúgenaHmanlT§PaBbBa©ÚlfñaM DFO bnþCab;

24ema:g ¬cak;eRkamEs,k b¤ bBa©ÚltamsrésedayeRbI kaetETrP¢ab;eTA

sésEvnFM¦ kñúgkMriteRbI 50 - 60 mRk¼KRk¼éf¶. rbbBüa)alenH

karBarnUvkarxUcxatNamYycMeBasac;dMuebHdUgenAeBlxagmux edaysar

CatiEdkEdlelIs RBmTaMgkarBarsac;dMuebHdUgBIkarbMpøicbMpøajCaRbcaM

rbs;ra:DIkal;esrI .

90

� karsikSafIμ²)anbgðajfa kareRbI DFO nig L1 bBa©ÚlKñaGacbnßy)an

ya:gelOnnUvbnÞúkCatiEdkenAebHdUg ehIyeFVI[RbesIrdl;dMeNIrrbs;

ebHdUg.

\LÚvenHeK)anrkeXIjfa karkMcat;CatiEdk[)anxøaMgkøa GaceFVI[)an

FUres,IyCMgWebHdUgF¶n;F¶relIGñkCMgWtaLasesmuI. b:uEnþ karRtUvBüa)al

kMcat;CatiEdkedIm,IkarBarCamun vaRbesIrCagcab;Büa)aleBlmanCMgW

ebHdUgekIteLIgrYceTAehIy. karvaytMélkMhab;CatiEdkkñúgCalika

ebHdUgmankarlM)ak. b:uEnþkarGPivDÆn_cugeRkay kñúgkareRbIbec©keTs

MRI CameFüa)ayd¾l¥bMputsMrab;eFVIkarvas;EvgbnÞúkCatiEdkkñúgebHdUg.

niyay[xøIeTA karBüa)alplvi)akelIebHdUgcMeBaHGñkCMgWtaLasesmuI

kMritF¶n; BwgEp¥keTAelIkarBüa)al[eBjTMhwgkMcat;CatiEdkEdlkkpþúM

kñúgCalika bEnßmeTAelIfñaMpøÚvkareRbICYyKaMRTsac;ebHdUgEdlexSay

dUcCa ³

k> fñaMEdleFVI[RbesIrdl;skmμPaBRc)ac;Qamrbs;ebHdUg. fñaMTaMgenH

sßitkñúgRkum EdlehAfa Angiotensin Converting Enzyme b¤ ACE

inhibitors.

x> fñaMbBa©úHTwkenam EdlCYysPaBht;cMeBaHGñkCMgWexSayebHdUg.

K> fñaMEdlCYyEkkuncMeBaHcgVak;ebHdUgedIrmineTogTat; ¬Agent anti-

arrhythmique¦ .

� CMgWepSg²eTotdUcCa plvi)akcMeBaHRkeBjbeBa©jkñúg Hypothyroidism

ehIynig Hypoparathyroidism b¤ k¾kgVHvItamInes GacbgáCMgWsac;dMu

ebHdUgpgEdr EtCMgWTaMgenHGac)anFUres,Iyvij edaykarBüa)al

mUlehtuedImrbs;va )anesckþIfa karBüa)alplvi)akénRkeBjbeBa©j

kñúg b¤ Büa)alkgVHvItamInes.

91

plvi)akcMeBaHRkeBjbeBa©jkñúg

RbB½n§RkeBjbeBa©jkñúg rYm

manRkeBjCaeRcIn ³ RkeBj

Pituitary, RkeBj Thyroid,

Parathyroids, RkeBj

Adrenals, nig ekasika

Ebtta ¬β¦ rbs;lMEBg/

RBmTaMgBgsVasrbs;burs/

GUEvrrbs;RsþI. RkeBjTaMg

Gs;enHTTYlxusRtUvkñúgkar

plit nig beBa©jG½rm:Un/ BYk

vagayTTYlrg\T§iBlBulénCatiEdkEdlelIskkpþúMkñúgekasikarbs;va Edl

rMxandl;karplitG½rm:Un. dUecñH plvi)akeTAelIRbB½n§RkeBjbeBa©jkñúg

CabBaðamanCaTUeTAcMeBaHGñkCMgWtaLasesmuI eTaHbIGñkCMgW)ancab;epþImBüa)al

kMcat;CatiEdk)anRtwmRtUvBIedImTIénCIvitk¾eday. CMgWRkeBjbeBa©jkñúg rYmman

karrIkFMFat;manPaByWtya:v/ RkeBjv½y/ CMgWTwkenamEp¥m/ Hypothyroidism,

Hypoparathyroidism ehIycMeBaHmnusSFMvij exSaymuxgarpøÚvePT.

karrIkFMFat; (Growth)

RbmaN 30 - 50° én

GñkCMgWtaLasesmuIkMritF¶n;

TTYlrgvibtþirIkFMFat; Edl

bNþalmkBIktþamYycMnYn.

kgVHRKab;QamRkhmraMér:/

rIkGeNþIk/ elIsbnÞúkCati

Edk/ BulfñaM DFO, Hypo-

thyroidism, PaBeBjv½y/

92

yWty:av/ Hypoparathyroidism, nig CMgWeføImraMér:/ TaMgGs;enHCa

plGviC¢maneTAelIkarrIkFMFat; RBmTaMgkgVHG½rm:UnsMrab;rIkFMFat; b¤

PaBsauMeTAnwgskmμPaBrbs;G½rm:UnenaH/ PaBgaybMErbMrYlBn§ú/ kgVH

cMNIGahar nig karkgVl;kñúgGarmμN_.

enAbNþaRbeTsEdlGñkCMgWBMu)anTTYlkarBüa)alRtwmRtUv/ kgVH

RKab;QamRkhmraMér: nig cMNIGaharminRKb;RKan; KWCabuBVehtu

cMbg bNþal[Gak;xandl;karrIkFMFat; ÉbNþaRbeTsEdlGñkCMgW

)anbBa©ÚlQamRKb;RKan;EtGnuvtþn_karBüa)alkMcat;CatiEdk

min)anl¥/ karelIsbnÞúkCatiEdkCamUlehtusMxan;bgá[Gak;xandl;karrIk

FMFat;. y:agNak¾edaycMeBaHGñkCMgWEdlTTYlkarBüa)alRtwmRtUv eday

bBa©ÚlQam)anRKb;RKan; nig kMcat;CatiEdk)anl¥/ kMriteRbIx<s;rbs; DFO

bgá[Buldl;q¥wg TIbMputeFVI[Gak;xandl;karrIkFMFat;.

karBüa)aleGaymanRbsiT§iPaB cMeBaHbBaðarIkFMFat; BwgEp¥kelIkarvaytMél

[)anc,as;las;BImUlehtubgárbs;va. dUecñHcab;taMgBIedImdMbUgénkumarPaB

rhUtdl;v½yCMTg; eraKvinicä½yRtUvEteFVIeLIgedaykarBinitüKøInIkedayykcitþ

Tukdak; nig eTogTat; RBmTaMgetsþmnÞIrBiesaFn_epSg². karvaytMélcMeBaH

karbeBa©jG½rm:UnsMrab;rIkFMFat; nig skmμPaBEsþgecjrbs;va)anpþl;lT§pl

pÞúyKñaeTAvij tMrUveGaykarBüa)aledayG½rm:UnenH cMeBaHEtGñkCMgWNaEdl

bgðajfamankgVHG½rm:Un ehIyeqøIytbKYrCaTIeBjcitþeTAnwgkarBüa)al.

PaBRkeBjv½y nig Hypogonadism

enAeBlcab;epþImeBjv½y/ Hypothalamus EdlCaEpñkmYyénxYrk,alenA

Ek,rRkeBj Pituitary cab;epþImbeBa©j G½rm:UnmYyeQμaHfa Gonadotrophin-

Releasing Hormone (GnRH) EdleTAeP£acRkeBj Pituitary [plit nig

beBa©j Gonadotrophins EdlmandUcCa ³ Follicle Stimulating Hormone

(FSH) nig Luteinizing Hormone (LH). G½rm:UnTaMgenHeFVIskmμPaBeTAelI

5d Incidence (%) of

endocrine complications

in thalassaemia major

(from De Santis et al 1995

multicentre study on endocrine

complications

in thalassaemia major, Clin

Endocrinol 42:581-86)

93

RkeBjePT KWBgsVascMeBaHburs nig GUEvr cMeBaHRsþI edIm,I[varIkFMFat;

ehIyplitbeBa©jG½rm:UnePT KW Testosterone cMeBaHekμgRbus/ Estradiol nig

Progesterone cMeBaHekμgRsI. G½rm:UnePTTaMgenHRtUv)andwknaMkñúgsarBagÁkay

RKb;RKgelIkarvivtþn_énsrIragÁePTRbus nig ePTRsI RBmTaMgsmtßPaBbnþBUC.

RkeBj Pituitary EdlCaGñkplit FSH nig LH qab;TTYl\T§iBlxøaMgNas;BI

pleRKaHfñak;rbs;EdkesrI eFVIeGayfycuHlT§PaBrbs;RkeBjenHkñúgkar

plitG½rm:Un.

PaBRkeBjv½y nig Hypogonadism KWCaplvi)akeRcInCageK eTAelIRkeBj

beBa©jkñúgEdlTak;TgnwgEdk enHebItamkarsikSaesÞIrEtTaMgGs;enAtam

bNþaRbeTsmYycMnYn. PaBRkeBjv½yenH eKeGayniymn½yfaCaGvtþman

énkarvivtþn_pøÚvePT dUcCa KμankarrIkFMénsudn;cMeBaHekμgRsIeBldl;Gayu 13

qñaM/ KμankarrIkFMénBgsVas cMeBaHekμgRbuseBldl;Gayu 14 qñaM.

ebIKμansBaØaeBjv½yeT eBldl;Gayu 16 qñaM GñkCMgWRtUvvinicä½yfaman

Hypogonadism KwekμgRbus BgsVas nig lwgÁenAEttUc

ÉekμgRsIsudn;KμanvivtþehIyKμanrdUvdMbUg ¬Primary amenorrhoea¦.

sPaBEbbenHrEmgbgáPaBkgVl;EpñkpøÚvcitþ ¬Psychological stress¦.

RbsiT§iPaBBüa)alcMeBaHPaBRkeBjv½y GaRs½yelIkarvaytMélBImUlehtu

bgá[)anc,as;las;. karelIsCatiEdk GacrMxandMNak;kalNamYyénkar

vivtþn_EpñkpøÚvePTdUc)anerobrab;xagelI edayb:HBal;xus²KñaBImñak;eTAmñak;.

dUecñH krNInImYy²tMrUveGayeFVIeraKvinicä½yedayRbugRby½tñ edayEp¥kelIkar

BinitüKøInikeGayl¥itl¥n;. karBüa)alkMcat;CatiEdkRtwmRtUv mannaTIsMxan;

kñúgkareFVI[RbesIrnUvplvi)akTaMgenaH. elIsBIenHeKeRbIfñaM Sexual steroid

¬Testosterone cMeBaHekμgRbus nig Oestrogen cMeBaHekμgRsI¦ edIm,ICYy

CMrujkarrIkFMFat;RsbKñanwgkarvivtþn_lkçN³pøÚvePT nig begáInTMhMsrIragÁpøÚv

ePT.

RbB½n§RkeBjbeBa©jkñúg enAEtgayTTYlrg\T§iBlénkarelIsCatiEdk eTaHCa

94

GñkCMgW)anTTYleBjv½yFmμtarYcehIyk¾eday eRBaHCatiEdkEdlekIneLIg

eBleRkaymkeTot GacenAEtbgákarb:HBal;dl;RkeBj Pituitary b¤ RkeBj

ePT dEdl kñúgkrNIEbbenH rdUvrbs;RsþIGac)at;vij ¬Secondary ame-

norrhoea¦ ÉbursvijGacGsmtßPaBpøÚvePT ¬Secondary Importence¦

karplitTwkkamfycuH ehIysmtßPaBpþl;kMeNIt ¬Infertility¦ k¾fycuHEdr.

Hypothyroidism

RkeBj Thyroid sßitenARtg;k ehIyedIrtYsMxan;kñúgkarFana[)annUvkar

vivtþn_Fmμtarbs;xYrk,alkñúgbNþaqñaMdMbUgénCIvit ehIyeRkaymkedIrtY

kñúgkarvivtþn_ nig rIkFMFat;TUeTA. Thyroxin CaG½rm:Unplit nig beBa©j

edayRkeBj Thyroid rYmcMENkdl;kMritfamBlTUeTA nig emtabUlIs

¬Metabolism¦ énbuKÁlmnusSmñak;². b:uEnþeBlEdlEdkeTAkkpþúMenA

RkeBjenH naMeGayfylT§PaBplitG½rm:Un Thyroxin ehIyekItCasPaB

mYyehAfa Primary hypothyroidism.

GñkCMgWTTYlrg Primary hypothyroidism GacTTYlGarmμN_rgarxøaMg nig

gguyedk ehIyCaerOy²GñkCMgWbgðajnUvsPaBemIuk²EpñkragÁkay nig pøÚvcitþ

ehIyeLIgTMgn;. karb:HBal;F¶n;F¶rcMeBaHRkeBj Thyroid bgáedaykarkkpþúM

CatiEdk k¾Gacb:HBal;dl;muxgarebHdUgpgEdr. b:uEnþ hypothyroidism

minEmnsuT§EtmaneraKsBaØaKøInikeT dUecñHeKeFVIeraKvinicä½y)andwgc,as;eday

saretsþmnÞIrBiesaFn_eTogTat; ¬TSH, T3 nig T4¦ vaerogral;qñaM eRkayBI

GñkCMgWQancUldl;Gayu 10qñaM .

eBletsþmnÞIrBiesaFn_bBa¢ak;faman hypothyroidism ehIy ¬TSH ekIn Et

T4 esrIenAFmμta b¤ cuHfy¦ eBlenaH eKBüa)aledayG½rm:Un Thyroxin

eTaHbIGñkCMgWmaneraKsBaØaKøInik b¤ Kμank¾eday.

95

Hypoparathyroidism

RkeBj Parathyroid manTaMgGs;bYn EdlP¢ab;eTAnwgRkeBj Thyroid.

muxgarsMxan;rbs;RkeBjTaMgenH KW RKb;RKgkMritkal;süÚm ¬Calcium¦

kñúgsarBagÁkaytamry³ Parathyroid hormone (PTH) EdlCaG½rm:Unplit

nig beBa©jedayRkeBjenH. PaBelIsbnÞúkCatiEdk b¤ sPaBkgVHRKab;Qam

Rkhm eFVI[b:HBal;dl;muxgarrbs;RkeBj Parathyroid ehIy[ekItCa

hypoparathyroidism. karenHbgá[kMritkal;süÚmkñúgQamFøak;cuH sPaB

mYyEdleKehAfa Hypocalcaemia EdlbnÞab;mkeTot manplb:HTgÁic

eTAelIkMritsarFatuKImIsMxan;epSgeTot KW pUs½Vr.

kMritkal;süÚm nig pUs½Vr manTMnak;TMngeTAnwgeraKsBaØaKøInikmYycMnYn. kMrit

kal;süÚmTabGacbgá[manGarmμN_ QW-rYy enAtaméd nig eCIg CYnkal

bgá[rmYlRkeBI nig rwgsac;dMu. karelceLIgnUvsPaBRbkac;TUTaMgxøÜnR)aN

nig PaBGak;xanénmuxgarrbs;ebHdUgGacCaeraKsBaØacugeRkay. kartam

danEpñkmnÞIrBiesaFn_eTAelIkMrit kal;süÚm/ pUs½Vr nig PTH kñúgQamGac

CYykñúgkareFVIeraKvinicä½y.

karBüa)aledayeRbI kal;süÚm nig vItamIn ed ¬Vitamin D¦ GacEkkunPaB

minRbRktIén emtabUlIs enH)an. kñúgkrNIkMr EdlekItmankarrwgsac;dMu

F¶n;F¶redaysarkMritkal;süÚmkñúgQamFøak;cuHxøaMg eKGaccak;kal;süÚmbBa©Úl

tamsrés.

CMgWTwkenamEp¥m ¬Diabetes mellitus¦

PaKeRcInénplvi)akcMeBaHkarelIsCatiEdkrauMér: mandUcCa CMgWeføImraMuér:/

karbgáeraKedayvIrus nig ktþaBUC. TaMgenH Cakar)at;bg;tulüPaBsárEdlcug

bBa©b;vaGaceFVI[ekItCaCMgWTwkenamEp¥m. CMgWTwkenamEp¥m eKeGayniymn½y

fa CakareLIgCatisárkñúgQameBlminTan;hUbGahar elIs 126 mRk¼d>l

(mg/dl) b¤ eBlhUbGaharehIy elIs 200 mRk¼d>l Ékar)at;PaBTb;Tl;

96

CamYysárvijmanniymn½yfa Cakar)at;bg;lT§PaBrbs;ekasika β rbs;

lMEBg kñúgkarbeBa©j Insulin eGay)anbrimaNsmRsb edIm,IeqøIytbnwg

CatisárEdlcUleTAkñúgxøÜn. esÞIrEtBak;kNþalénGñkCMgWtaLasesmIukMritF¶n;

suT§EtTTYlrgnUvkar)at;PaBTb;Tl;CamYysár É 10 - 30° eTotekItCa

CMgWTwkenamEp¥m eBlNamYykñúgCIvitrbs;eK. RbvtþiRKYsarekItCaCMgWTwkenam

Ep¥m CaBiesskñúgcMeNambgb¥ÚnExSrdMbUg )anesckþIfaCa «Buk b¤ mþay

enaHeRKaHekItCMgWTwkenamEp¥mmankMritx<s;.

TwkenamEp¥mekIteLIgeBlEdl emtabUlIs rbs;

CatisárRtUvkat;pþac;ehIysárminGaccUlkñúgekasika

edIm,Ipþl;famBl EdlvaRtUvkaredIm,IbMeBj

muxgar. srIragÁTTYlxusRtUvcMeBaH emtabUlIs

Catisár KWlMEBgEdlsßitenAEk,rRkBH Edltam

ry³ekasika β vaplit Insulin EdlCaG½rm:UneRbIsMrab;eFVIemtabUlIsCatisár.

FatuEdkGaceFVI[xUcxatekasika β TaMgenH dUecñHlT§PaBeRbIR)as;sárrbs;

sarBagÁkayRtUvkat;bnßyeFVI[sárCujeRcInkñúgQam. GñkCMgWTwkenamEp¥m

kñúgTMrg;mFüm eKehAfa kar)at;PaBTb;Tl;CamYysár ¬Glucose

intolerance¦ KμanbgðajsBaØaKøInikeT eKeFVIeraKvinicä½y)anedayetsþmnÞIr

BiesaFn_.

karRsavRCav)anbgðajfa GñkCMgWtaLasesmIu munQaneTAekItCMgWTwkenam

Ep¥m RtUvqøgkat;dMNak;kal )at;PaBTb;Tl;CamYysár sin ehIykñúgkMLúg

eBlenHkarplit Insulin mankarekIneLIgya:gEsþgEtmþg CakareqøIytb

nwgskmμPaBvaEdlxUcxat. dMNak;kalenHeKehAfa karsaMueTAnwg Insulin

¬Insulin resistance¦ TamTarnUvkartamdanedayykcitþTukdak; GñkCMgWRtUv

GnuvtþtamrbbGahar[)anRtwmRtUv ebIcaM)ac;RtUvsMrkTMgn; RBmTaMgkMcat;

CatiEdk[)anxøaMgkøa.

kar)at;PaBTb;Tl;CamYysár nig CMgWTwkenamEp¥m RtUveFVIeraKvinicä½ytam

97

ry³etsþmnÞIrBiesaFn_rkkMritCatisárkñúgQamenAdMNak;kalepSg² mun nig

eRkaykarjaMu. ]TahrN_ kMritCatisárkñúgQamesμI b¤ elIs 7 mmol/l ¬b¤

126 mg/dl¦ enAeBlRBwkminTan;jaMuGVITaMgGs; RtUveKkMNt;famanCMgW

TwkenamEp¥m. kMritCatisárkñúgQamelIs 11 mmol/l ¬b¤ 200 mg/dl¦ enA

2 ema:geRkayBIcak;bBa©ÚlCatisárkñúgxøÜn k¾kMNt;)anfa CaCMgWTwkenamEp¥m

)anEdr. kMritCatisárkñúgQamBI 8 - 11 mmol/l ¬140 - 200 mg/dl¦ enA

2 ema:geRkayBIjaMusár 75 Rkam eKehAfa etsþkMNt;PaBFn;RTaMcMeBaHsár

bBa¢ak;BIkar)at;PaBTb;Tl;CamYysár. etsþrkkar)at;PaBTb;Tl;CamYysár

RtUveFVImYyqñaMmþgRKb;GñkCMgWTaMgGs; eBleKQancUldl;GayuCag 10qñaM.

kñúgkrNICMgWTwkenamEp¥m/ TMrg;F¶n;F¶rxøaMg CakrNImankarb:HBal;F¶n;F¶r

dl;karplit Insulin EdlGñkCMgWTamTarkarcak; Insulin eRkamEs,k

erogral;éf¶ edIm,IeFVI[kMritCatisárkñúgQammkFmμtavij. TwkenamEp¥m

CaCMgWGacBüa)al)an EtkarBüa)alCabnÞúkbEnßmmYyeTot. dUecñHkarKaMRT

BIRKUeBTü RKYsar nig mitþPkþi CakarsMxan;. GñkCMgWTwkenamEp¥m RtUvEtBinitü

emIlkMritCatisárkñúgQamenApÞH bIeTAbYndgkñúgmYyéf¶ edayeRbI]bkrN_

vas;kMritCatisár ehAfa Glucometer. lT§plénkartamdanenApÞHenH)an

CYyRKUeBTükñúgkaréllkkMritfñaM Insulin eTAtamesckþIRtUvkarrbs;GñkCMgW.

etsþKøInik nig mnÞIrBiesaFn_epSgeTotRtUveKGnuvtþn_edIm,IvaytMélplvi)ak

rbs;CMgWTwkenamEp¥m KW Binitümuxgarrbs;tMrgenam nig ftrUbPaBemIlépÞ

)atRkBH EdlCaBYksrIragÁgayb:HBal;bMputkñúgCMgWTwkenamEp¥m. eKRtUvelIk

TwkcwtþGñkCMgWtaLasesmuI [Rbkan;x¢ab;nUvkarBüa)aleTogTat;edayfñaM DFO

edayehtufa kareRbIfñaMenH)anRtwmRtUvGacCYykat;bnßy)aneRcInnUveRKaH

ekItCMgWTwkenamEp¥m.

CMgWBukq¥wg ¬Osteoporosis¦

q¥wgesþIgehIypuyeKeXIjmanCaTUeTAelIGñkCMgWtaLasesmuI bNþalmkBI

ktþaCaeRcIn dUcCa kgVHRKab;QamRkhm/ xYrq¥wgskmμxøaMg/ cMNIGaharman

5e Typical sites of

osteoporotic fractures

98

kMritkal;süÚmTab/ kMritCatiEdkkñúgq¥wgekInx<s;/ kgVHcMNIGahar/ PaBRkeBj

v½y b¤ Hypogonadism nig bBaðabNþaRkeBjbeBa©jkñúgepSg²eTot RBmTaMg

ktþaBUC TaMgGs;enHGacrYmcMeNkdl;karkekItCMgWBukq¥wg ¬emIlrUb 5e¦.

GñkmanCMgWq¥wg rEmgmanskmμPaBragkayGn;fy ehIyGvyv³bþÚrTMrg;xøaMg

ehIyeFVI[)ak;q¥wgF¶n;F¶r.

CaTUeTAeKeFVIeraKvinicä½y CMgWq¥wgedayvas;dgsIuetq¥wgenAtMbn;q¥wgkg nig

q¥wgRtKakedayeRbI DEXA ehIyebItMrUv[eFVI eKeRbIkartamdaneday

mnÞIrBiesaFn_epSg²eTot. GgÁkarsuxPaBBiPBelakkMNt;fa

fydg;suIetq¥wg ¬Osteopenia¦ eBlNadgsIuetq¥wgRtUvkat;bnßydl;BinÞú ³

-1 eTA -2.5 eRkamkMritFmμta ehIyBukq¥wg ¬Osteoporosis¦ eBlEdl

ma:s;q¥wgTabCag -2.5 ¬emIlrUb 5f¦ .

karBüa)alCMgWq¥wg sMxan;KWepþateTAelIkarkarBar tamry³karbBa©ÚlQam

[)aneTogTat; karkMcat;CatiEdk[)anl¥/ Büa)alCMgWRkeBjbeBa©jkñúg/

eFVIlMhat;R)aN[)aneTogTat;. edIm,IkarBarkMu[Bukq¥wg GñkCMgWEdlman

fydg;suIetq¥wg RtUvQb;Ck;)arI/ jaMuGaharsMbUrCatikal;süÚm ehIyelb

vItamIn D bEnßm RBmTaMghat;R)aN[)aneTogTat;. elIsBIenHGñkCMgW

Hypogonadism RtUvTTYlG½rm:UnePTedIm,IkarBarkMu[Bukq¥wg. ebIBukq¥wgekIt

ehIy kareRbIfñaMmYycMnYn dUcCa Biphosphonates (Pamidronate,

Aledronate) pþl;plRbeyaCn_xøHEtb:ueNÑaH .

karpþl;kMeNIt nig bnþBUC ¬Fertility

and reproduction¦

RsþImanCMgWtaLasesmuI GacmanépÞeBaH

edaysuvtßiPaB. b:uEnþkarsMerccitþman

épÞeBaH RtUvEtBicarNaeGayhμt;ct;

edayKUrRskrRtUvRbwkSaCamYyRKUeBTü.

5f Example of DEXA results

on a patient with osteo-

porosis (z score-5.9)

99

RsþIEdlmanbMNgcg;manépÞeBaHRtUveFVIkarvaytMél[)aneBjeljcMeBaH

sPaBpøÚvcitþ nig sPaBKøInikrbs;Kat; . GñkCMgWtaLasesmuI ePTRsIEdlman

rdUvFmμta GacmanépÞeBaHedayÉkÉg. EtGñkEdlKμanrdUvdMbUg b¤ )at;rdUv

nwgRtUvkarBüa)aledayG½rm:UnedIm,IeP£ac[mankarplitemCIvitRsþI ehIyvaRCuH

Føak;cUlmkkñúgs,Ún. GñkCMgWtaLasesmuI ePTRbusEdlmanEtTwkkamEtKμan

emCIvit CaerOy²eqøIytbnwgkarBüa)aledayG½rm:UnbBa©ÚlKñakñúgry³eBl

mYyqñaM. eBlNaeKdwgfaGñkCMgWmanépÞeBaHehIy eKRtUvRbkan;viFankarN_

mYycMnYn dUcteTA ³

1> eKRtUvEtbBaÄb;kareRbIfñaM DFO eBlEdldwgfamanépÞeBaHPøam edaysar

Et\T§iBlrbs;fñaMeTAelITarkminTan;dwgc,as;enAeLIy. ya:gNak¾eday

tamkarsikSaelIstV fñaMenHmanplb:HBal;F¶n;F¶reTAelITarkstV. ebIsin

GñkCMgWmanépÞeBaH mankarelIsCatiEdkxøaMgkøa b¤ manbBaðaebHdUgF¶n;F¶r

enAdMNak;kalcugeRkayénépÞeBaHeKKYreRbIva kñúgkMritTab 20 - 30

mRk¼KRk¼éf¶.

2> RsþImanépÞeBaHRtUvbBa©ÚlQamkñúgbrimaNtic² Etjwkjab;CagFmμta

edIm,IrkSa Hb [sßitkñúgkMritKYrcg;)an ¬10 - 15 Rkam¼d>l¦ ¬g/dl¦.

3> muxgarrbs;ebHdUgRtUvEttamdan[ditdl;. karRsavRCavbgðajfa

RsþIEdlcab;manépÞeBaHkñúgeBlEdlkMrit Ferritin Tab/ muxgarrbs;

ebHdUgmanPaBRbesIrCageBlEdlkMrit Ferritin x<s;.

4> eKRtUvRtYtBinitüGñkCMgW ERkgekItmanCMgWTwkenamEp¥m b¤ CMgWRkeBj

beBa©jkñúgepSg²eTot.

kñúgbNþaqñaMcugeRkayenH manGñkCMgWtaLasesmuICaeRcIn Edl)anTTYlkar

Büa)albBaðabgákMeNIt)anRtwmRtUv)ansMerckñúgkarmankUnEdlmansuxPaB

l¥. KUrRskrEdlédKUrTaMgBIrnak;manCMgWtaLasesmuIkMritF¶n; ehIyman

bMNgcg;)ankUnEdlmansuxPaBl¥/ emCIvitburs b¤ RsþI Edl)anmkBIGñkeFVI

100

GMeNaysuxPaBFmμta RtUveKeRbIkñúgdMeNIr[mankarPBRbsBV

bgákMeNIt ¬Fertilisation¦ EdlRtUveFVIenAeRkAsarBagÁkaykñúgmnÞIr

BiesaFn_ rYcehIyemCIvitRsþIEdl)anPBRbsBVCamYyemCIvitbursehIy

enaH RtUveKykbBa©Úlkñúgs,Únrbs;RsþI. karBüa)al nig kartamdanGñk

CMgWEbbenH TamTarkarykcitþTukdak;CaRkum BIbNþaRKUeBTüÉkeTsCa

eRcIn rYmman GñkÉkeTsEpñkelahitviTüa/ ÉkeTsEpñkkumar/ ÉkeTs

EpñkRkeBjbeBa©jkñúg nig ÉkeTsEpñkeraKRsþI.

snñidæanmk/ bc©úb,nñenHkarbnþrBUCcMeBaHGñkCMgWtaLasesmuIkMritF¶n; nig

mFüm KWCasPaBBitCak;EsþgmYy. ]TahrN_ enARbeTssIuRBUs kñúgcMeNam

RsþItaLasesmuI 62 nak; ¬50 nak;kMritF¶n; nig 12 nak;kMritmFüm¦ manGayu

mFüm 25qñaM sMerc)ankñúgkarmanépÞeBaHdl;eTA 90° kñúgenaH ³

> 14 nak; tamry³karCYyeRCamERCg[manépÞeBaH KW eFVI[mankarRCuH

emCIvitRsþImkkñúgs,Ún/ karPBRbsBVénemCIvitkñúgmnÞIrBiesaFn_ ¬In vitro

fertilisation = IVF¦ nig kardak;bBa©ÚlemCIvitburs ¬Insemination¦ .

> ekIt)anTarkmansuxPaBl¥ 87 nak; kñúgenaH 69 nak; RKb;Ex/ 12 nak;

minRKb;Ex ¬Edlman 4 épÞeBaHkUnePøaH nig 1épÞeBaHkUn 3¦. man 7

rlUt ehIysøab;ekIt 2 krNI. eKBMueXIjmanplvi)akF¶n;F¶rkñúgkar

RbsURteT ehIyGñkCMgW 9 nak;manplvi)akeTAelIebHdUgmYyry³xøI

b:ueNÑaH.

101

CMBUkTI 6

karbgáeraKkñúgCMgWtaLasesmuI

CMgWtaLasesmuIkMritF¶n; maneRKaHfñak;bgáeraKx<s;edaysar ³

� kgVHeKalikaQamRkhm

� karkat;GeNþIkecj

� elIsbnÞúkCatiEdk

� karbBa©ÚlQam

� kareRbIfñaM Desferrioxamine

kgVHeKalikaQamRkhm ¬Anemia¦

krNIGñkCMgWKμankarbBa©ÚlQamTal;EtesaH b¤ bBa©Úlmin)anRKb;RKan;/ kgVH

RKab;QamRkhmCabuBVehtud¾sMxan;bMputbgá[manCakarbgáeraKF¶n;F¶r dUcCa

rlaksYtCaedIm. eTaHbIbBaðaenHvakMrmanenARbeTselakxaglic eRBaH

gayrkQambBa©Úl)anRKb;RKan; EtenARbeTskMBugGPivDÆn_mYycMnYnvij

karbBa©ÚlQamminRKb;RKan; CabBaðaEtgekItmanCaeTogTat; ehIykarbgáeraK

EbbdUecñHGacenAEtekItmanteTAeTot.

karkat;GeNþIkecj ¬Splenectomy¦

GñkCMgWEdlmin)ancab;epþImkarBüa)aledaybBa©ÚlQamRKb;RKan;)anTan;

eBlevla b¤ GñkCMgWEdl)ankat;GeNþIkecj RbQmmuxnwgeRKaHfñak;xøaMgkøa

cMeBaHkarbgáeraKF¶n;F¶r dUcCa rlaksYtedayBYkemeraK Streptococcus,

Haemophilus Influenza nig Neisseria Meningitides suT§EtCa)ak;etrI

EdlmansMbkB½T§CMuvijkarBarxøÜn. manBYk )ak;etrI/ vIrus nig )a:ra:sIutepSg²

eTotk¾GacbgáeraKF¶n;F¶rcMeBaHGñkCMgWEdlkat;GeNþIkecjEdr. enHedaysar

GeNþIkCaGñkkarBarsarBagÁkayTl;nwgkarbgáreraK.

102

elIsCatiEdk ¬Iron load¦

GñkCMgWEdl)anbBa©ÚlQamRtwmRtUv b:uEnþkMcat;CatiEdkmin)anl¥/ edaysar

mankarlM)aknwgrk)anfñaM Desferrioxamine b¤ k¾karGnuvtþn_tamkar

eRbIR)as;min)anditdl;/ k¾GacgayeRKaHfñak;bgáeraKy:agF¶n;F¶rEdr. enHk¾

edaysarPñak;garbgáeraKrIkFMFat;edaysarFatuEdk ³ FatuEdkkan;Etx<s;

kñúgxøÜn enaHPñak;garbgáeraKkan;EtrIkFMFat; begáIncMnYn ehIybgáeraKF¶n;F¶r.

eraKEdleKsÁal;c,as;CageK KW bgáeday Yersinia Enterocolitica.

)ak;etrIenHmindUc)ak;etrIepSgeToteT vaCa)ak;etrIBiessEdlKμanynþkar

pÞal;xøÜnedIm,IRbmUlyk nig eRbIR)as;EdkBIbrisßanCMuvijxøÜnva)aneT. cMeBaH

buKÁlmansuxPaBl¥ )ak;etrITaMgGs;enHKμaneRKaHfñak;eT ehIyKμansBaØaKøInik

b¤ manEtbnþicbnþÜc. EtcMeBaHGñkCMgWtaLasesmIukMritF¶n; EdlelIsCatiEdk

esrI b¤ EdkP¢ab;nwgm:UelKulfñaM Desferrioxamine, BYk Yersinia rIkFMFat;

begáIncMnYnya:gelOn ehIybgáeraKF¶n;F¶r KMramkMEhgdl;Civit.

fIVebImankarsikSaCaeRcIn sþIBItYnaTIrbs;EdkkñúgkarbgáeraKeday)ak;etrIk¾

eday Etk¾mankarsikSaCaeRcInEdr eTAelItYnaTIrbs;EdkkñúgkarbgáeraK

edayvIrus ¬dUcCarlakeføIm nig eGds_¦ edayBinitüeTAelIrebobEdlEdk

b:HBal;eTAelIkarvivtþn_eTAmuxénkarbgáeraKTaMgenH nig kareqøIytbcMeBaH

karBüa)aledayeRbIfñaMtamkarENnaM. lT§plénkarGegát)anbgðajfa

cMeBaHGñkCMgWtaLasesmuIkMritF¶n;/ karelIsbnÞúkCatiEdk GaceFVIeGayeCaK

vasnaénkarrlakeføImruaMér:RbePT B nig C kan;EtGaRkk;eTA ehIykar

Büa)alrlakeføImenHmanlT§plminsUvl¥. dUecñH RbsiT§iPaBénkarkMcat;

CatiEdk hak;bIdUcCaedIrtYnaTIsMxan; kMNt;eCaKvasnaénkarrlakeføImraMuér:

bgáedayvIrus.

eK)anbgðajpgEdrfa cMeBaHGñkCMgWtaLasesmuIkMritF¶n;/ karbgáeraKeday

vIrus HIV manlkçN³F¶n;F¶rxøaMgeLIg eBlNaeKeRbIkMritfñaMkMcat;CatiEdk

DFO ticCag 40 mRk¼KRk b¤ eBlNakMrit Ferritin kñúgQamelIs 1935

103

mIRkURkam¼l ¬μg/l¦.

niyayCarYm CatiEdkGacedIrtYnaTIsMxan;kñúgkarbegáInPaBF¶n;F¶rénkarbgáeraK

cMeBaHGñkCMgWtaLasesmuIkMritF¶n; eRBaHEdkGac ³

[i] CacMNIGaharsMrab;karFMFat;Pñak;garbgáeraK

[ii] CacMNIGaharsMrab; Gg;sIum ¬Enzyme¦ EdlRTRTg;karbegáIncMnYnén

GtisuxumR)aN

[iii] kMcat;sarFatuKImIsMxan; ehAfa Gg;TIGuksIudg; ¬Antioxidants¦ EdlCa

sarFatuKImIkarBarekasika Tb;Tl;nwgkarrlak ¬Inflammation¦

[iv] eFVI[xUcRbePTekasikamYycMnYn EdledIrtYsMxan;kñúgkar

karBarsarBagÁkay Tl;nwgkarbgáeraK.

karbgáeraKbNþalmkBIkarbBa©ÚlQam

QamRtUv)anTTYlsÁal;fa CaRbPBsMxan;énPñak;garbgáeraK EdlGaccMlg

eTAGñkCMgWtamry³karbBa©ÚlQam. dUcenH eTaHCaQamGacCYyseRgÁaHCIvit

vak¾GacbgáRbtikmμmYycMnYnEdleyIgmincg;)an rYmmankarbgáeraKF¶n;F¶r

KMramKMEhgGayuCIvit.

GtisuxumR)aNtUc²CaeRcIn Gacrs;ranmanCIvitmYyry³eBlkñúgQam eday

bgáeraKdl;GñkCMgWeBlQamRtUv)anbBa©Úl. kñúgcMeNamenaHmanrlakeføIm B

nig C, HIV-1 nig HIV-2 TaMgenHCaPñak;garbgáeraK EdlGacmanGakar³raMuér:

F¶n;F¶r. enAGWr:ub nig GaemrikxageCIg/ esvabBa©ÚlQaml¥ kmμviFIcak;fñaMbgáar

karRtYtBinitüQam)anBIGñkeFVIGMeNayQam nig esvasuxPaBsaFarN³

manKuNPaBx<s; CaTUeTA)aneFVI[karcMlgeraKTaMgenH naeBlbc©úb,nñCakarkMr

KWfa karbgáeraKeday HBV, HCV nig HIV RtUv)ankat;bnßyesÞIrEtdl;

kMritsUnüenAkñúgRbeTsTaMgenH. b:uEnþenARbeTskMBugGPivDÆn_CaeRcIn kar

bgáeraKenAEtekItman eRBaHesvabBa©ÚlQamminsUvmanKuNPaB esvaEfTaM

suxPaBminl¥ FnFanenAmankMrit nig mankarBuHBarnwgGaTiPaBsuxaPi)al

104

epSg²eTot. CalT§plGñkCMgWtaLasesmuIkMritF¶n; enARbeTskMBugGPivDÆn_

enAEtmankarqøgeraKtamry³karbBa©ÚlQam. rlakeføImRbePT B nig¼b¤ C

manlkçN³F¶n;F¶rEpñkKøInik cMeBaHGñkCMgWtaLasesmuIkMritF¶n; edaysar

rlakeføImTaMgenH GaceFVI[kan;EtF¶n;F¶reLIgcMeBaHCMgWeføIm bNþalmkBI

CatiEdkelIs EdlmanCaTUeTAkñúgCMgWtaLasesmuI bNþalmkBICMgWxøÜn

ÉgpÞal;Etmþg b¤ mkBIkarBüa)alkMcat;CatiEdkmin)anl¥. CMgWeføImCabuBVehtu

rYménGRtamanCMgW nig søab; CaBiesskñúgcMeNamGñkCMgWtaLasesmuI Edl

manv½ycMNas; enAbNþaRbeTs]sSah_kmμ nig GñkCMgWesÞIrEtRKb;v½y

enARbeTskMBugGPivDÆn_.

karbgáeraKedayvIrusrlakeføImRbePT B (Hepatitis B = HBV)

rlakeføImraMuér:RbePT B enAEtCabBaðasuxPaBsaFarN³ F¶n;F¶renARbeTs

kMBugGPivDÆn_CaeRcIn eTaHbImanfñaMbgáarmansuvtßiPaB nig RbsiT§iPaBk¾eday

RBmTaMgetsþRbkbedayKuNPaBx<s;Cak;lak;manlk;enATIpSaredIm,I

RtYtBinitüQamGMeNayk¾eday.

105

karcMlgeraK

eRkABItamry³karbBa©ÚlQam/ vIrusrlakeføImRbePT B Gacqøgtam

pøÚvepSg²eTot dUcCa karrYmePT/ qøgBImþayeTAkUnkñúgGMLúgeBl

manépÞeBaH enAeBlTarkekIt b¤ eBlbMe)AedaHTark. EtGñkCMgW

taLasesmIukMritF¶n; vIrusrlakeføImRbePT B jwkjybMputqøg

tamry³karbBa©ÚlQam.

karcMlg HBV GackarBar)anedayGnuvtþn_eKalneya)aysmRsbeday ³

[i] eRCIserIs nig eFVIetsþGñkeFVIGMeNayQam

[ii] cak;fñaMbgáar HBV nig kmμviFIsMGatemeraK

[iii] karBarkarqøgBImþaymanCMgWeTATarkeTIbekIt

savtarrbs; HBV

RbmaN 5 - 10° énGñkCMgWEdlqøg HBV nwgmanemeraKenHGs;mYy

CIvit )anesckþIfaBYkeKnwgkøayCaGñkpÞúk HBV raMuér:edaymaneRKaHfñak;

xøaMgkøaGacekItCMgWeføIm. cMeBaHkumar/ ebIKμanviFankarN_karBareT 90°

énkumar EdlekItBImþaymanemeraK nwgkøayCaGñkpÞúkvIrusrlakeføImGs;

mYyCIvit. kñúgenaHmanRbmaN 25 - 30° manpÞúkvIrusrlakeføImraMuér:

RbePT B ehIybnþicmþg²nwgekItCMgWeføIm. PaKryénGñkCMgWtaLasesmIu

kMritF¶n;EdlmanCMgWrlakeføImRbePT B ehIyvivtþn_eTACaCMgWeføImF¶n;F¶r

mankarERbRbYlBIRbeTsmYyeTARbeTsmYy GaRs½yedaykarGnuvtþn_

viFankarN_karBar dUcCa karcak;fñaMbgáar HBV, eRCIserIs nig sMraMgGñk

pþl;Qam ehIysMxan;bMputenaH GaRs½yelIGRtaekIt HBV kñúgtMbn;.

eyagtamTinñn½y)anecjpSay/ man 2 - 35° énGñkCMgWtaLasesmuI

kMritF¶n;TUTaMgBiPBelak CaGñkpÞúkrlakeføImRbePT B ehIycenøaH 20 -

90° manPsþútagmnÞIrBiesaFn_bgðajfa manqøgvIrusrlakeføIm B kñúgeBlNa

mYykñúgCIvitrbs;BYkeK ¬qøgeraKkñúgGtItkal¦ . enARbeTsGPivDÆn_ GñkCMgW

mYyPaKFMEdlpÞúkCMgWrlakeføImRbePT B suT§swgEtmanv½ycMNas; KW)anqøg

106

eraKmunkarbegáIt nig kareFVI[RbesIrénkarcak;fñaMbgáar nig eKalneya)ay

eFVIetsþQam. b:uEnþenARbeTskMBugGPivDÆn_ GñkCMgWRKb;GayubnþqøgvIrus

rlakeføIm.

enAeBleKeFVIeraKvinicä½yfa CaCMgWtaLasesmuIkMritF¶n; munnwgcab;epþIm

bBa©ÚlQam GñkCMgWRtUveFVIetsþBinitü HBV )anesckþIfa Kat;CaGñkpÞúk

emeraKrlakeføImraMuér: b¤ knøgmkmanrlakeføImRbePT B.

RbsinebIGñkCMgWKμanGVIeT eKRtUvEtcak;fñaMbgáar edayminKitBIGayueLIy.

GñkCMgWtaLasesmuIkMritF¶n;TaMgGs; RtUveFVIetsþtamdan HBV erogral;

qñaM. etsþTaMgenHrYmman Gg;TIEhSnelIépÞekasika ¬HBsAg¦/ GgÁbdibkç

RbqaMg HBV ¬Anti-HBs¦/ Gg;TIEhSn e ¬eAg¦/ GgÁbdibkçRbqaMg eAg

¬anti-HBe¦ nig GgÁbdibkçRbqaMgnwgsñÚl ¬anti-HBc¦. ebIcaM)ac; eKGac

eFVIetsþGñkpÞúk HBV raMuér: ¬emIlrUb 6a¦ [)anjwkjab;edIm,IvaytMél

eBlevlad¾smRsbbMputsMrab;cab;epþImkarBüa)al. karelIsbnÞúkCatiEdk

elIGñkCMgWtaLasesmuIkMritF¶n; ehIymanrlakeføImraMuér:RbePT B KWktþabEnßm

mYyEdlrYmcMENkd¾xøaMgkøaeFVI[xUceføIm ehIykarBüa)alkMcat;CatiEdk

d¾manRbsiT§iPaB CakarsMxan;xøaMgNas;.

karBüa)alrlakeføImrauMér:RbePT B

karBüa)alrlakeføImraMuér:RbePT B maneKaledAkat;bnßy nig lubbM)at;

HBV ehIykarBarplvi)akBIkarrlakeføIm. kñúgbNþaqñaMcugeRkayenHkar

Büa)alrlakeføImRbePT B mankarrIkcMerInKYr[kt;sMKal;. »sfRbqaMg

vIrusEdleKeRbITUeTArYmman Alpha - recombinant interferon EdleK)an

eRbIyUrmkehIy. Alpha - interferon CasarFatuKImImYymanlT§PaBEkERb

RbB½n§karBar RtUv)anbgðajfaGacCYy[GñkCMgWrlakeføImskmμraMuér:Ca)an

25 - 40°. »sfRbqaMgvIrusEdleTIbnwgbegáItfμI KW Lamivudine.

Lamivudine (EpivirTM - HBV, 3TC) eRbIEtÉg b¤ pÁúMKñaCamYy Interferon

)aneFVI[karBüa)al HBV raMuér:manPaBRbesIrya:geRcIn eTaHbIeKKitfa

6a Serological profile of

chronic HBV infection

107

vamanKuNvibtþiedaykarekItnUvPaBsaMu kñúgcMnYnGñkCMgW 14 - 32° kñúgqñaM

dMbUg ehIy 67° kñúg 4 qñaMeRkay . fñaMfμImYyeTot manlk;elITIpSarsMrab;

karBüa)alrlakeføImraMuér:RbePT B ¬CHB¦ KWfñaMelb Adefovir dipivoxil

EdlbgðajfamanRbsiT§iPaBdUc Lamivudine Edr kñúgkarkMcat;vIrus ehIyKμan

RBYy)armÖBIPaBsaMu. fñaMl¥²epSgeTot dUcCa [i] Interferon EbbfμIman

RbsiT§iPaBl¥CageKehAfa pegylated Interferon nig [ii] Entecavir. TaMgBIr

RbePTenHkMBugsßitkñúgkarsikSaenAeLIysMrab;karBüa)alrlakeføImRbePT

B. karsMerccitþeBlNaRtUvBüa)al nig CMerIsfñaM b¤ eRbIfñaMbBa©ÚlKña RtUveFVI

eLIgedayRKUeBTüBüa)al edayshkarN_ya:gCitsñiT§CamYyGñkCMnajEpñk

CMgWeføIm. ya:gNak¾eday CarYmebIFnFan nig lT§PaBGacrkfñaM)an kareRbI

fñaMTaMgenHeTAtammKÁúeTsn_Edlmankaryl;RsbCaGnþrCati )aneFVI[RbesIr

ya:gFMeFgcMeBaHkarBüa)alCMgWrlakeføImraMuér: ehIyCYykat;bnßyya:geRcIn

nUveRKaHvivtþin_eTAkan;CMgWeføImF¶n;F¶r dUcCa RkineføIm ¬sþarvij)an nig sþarvij-

min)an¦ nig mharIkeføIm.

rlakeføImRbePT C (Hepatitis C = HCV)

HCV naMeq<aHeTArkkarrlakraMuér:Gs;mYyCIvitCag 80° énGñkqøgCMgW. vamin

dUcrlakeføImRbePT B eT/ HCV mingaycMlgtampøÚvepSgeRkABIkar

bBa©ÚlQameLIy. enAminTan;manva:k;saMgmansuvtßiPaB nig RbsiT§iPaB

RbqaMg HCV enAeLIyeT EteTaHya:gNak¾manetsþmnÞIrBiesaFn_

EdlGacdwgya:gc,as;BIvIruskñúgQamEdr. dUecñHmeFüa)ayEtmYyKt;

manRbsiT§iPaBkarBarkarqøg HCV nig kat;bnßyGRtaCMgW KW RtUvFana[

)anfa QamEdlpþl;RtUv)aneFVIetsþedayRbugRby½tñ. eTaHbIya:gNa

k¾eday pÞúyBIvIrusepSg² HCV manry³eBlsMgMyUr KwfaeBlvacUldl;

kñúgQam vasMgMes¶óry³eBlyUr CYnkalminGacdwgedayeFVIetsþGgÁbdibkç

¬antibody tests¦ EdleKeRbITUeTAbMputenAtammCÄmNÐlpþl;QamPaK

eRcIn. kñúgGMLúgeBlenaH/ QamRtUv)anbBa©Úl ehIyGñkCMgWk¾TTYlkarqøg.

etsþrk HCV )anTTYlkarykcitþTukdak;ya:gxøaMgkñúgkic©RbwgERbgedIm,I

HCV virus

Envelope

Nucleocapsid protein

Single-strandedRNA

Approximatly10,000 nucleotides

50-60mm diameter

108

bnßyry³kalcMlgCMgW ¬eKehAmüa:geTotfa “ window phase “ b¤

immunosilent period¦ [)aneRcIntamEdl

GaceFVI)an ehIybnßyCaGtibrimanUvkarqøg

HCV tamry³Qam.

savtarrbs; HCV ¬emIlrUb 6b¦

RbmaN 20° énGñkpÞúk HCV raMuér:nwgekItCMgW

eføImFn;Rsal ¬Fibrosis¦ kñúgenH 20° Gac

vivtþn_CaCMgWeføImF¶n;F¶rCag dUcCa RkineføIm nig

mharIkeføIm. vivtþn_eTACaRkineføImrvag10qñaM RbmaN 10 - 20° énGñkCMgW

rlakeføImraMuér:RbePT C. rlakeføImRbePT C CamUlehtucMbgcMeBaHbnSaM

eføIm ¬bþÚrefIøm¦.

man ehSNUTIb (Genotypes) TaMgGs; 6 RbePT ¬rUb

6c¦/ RbePTnImYy² mankarEbgEcktMbn;PUmisaRsþ nig

sPaBKøInikxus²Kña. cenøaH 10 - 80° énGñkCMgW

taLasesmuIkMritF¶n;TUTaMgBiPBelak manqøgvIrusrlak

eføImRbePT C ehIyvaenHCamUlehtusMxan;bgá[manCMgW

eføImraMuér:F¶n;F¶rcMeBaHGñkCMgWTaMgenH. dUcrlakeføImRbePT B Edr/ karelIs

CatiEdkkñúgxøÜn GñkCMgWEdlmin)ankMcat;CatiEdk b¤ kMcat;EdrEtmin)anRtwmRtUv

KWCaktþabEnßmEdlGacrYmcMENkxøaMgkøadl;karxUcxateføIm. dUecñHsMxan;

GñkCMgWtaLasesmuIkMritF¶n;TaMgGs;EdlmanvIrus HCV RtUvTTYlkar

Büa)alkMcat;CatiEdk[)anRtwmRtUv ehIyeFVIetsþmnÞIrBiesaFn_ya:g

BiesstamdanrlakeføImRbePT C erogral; qñaM ¬emIlrUb 6d¦. karman

HCV nig HBV rYmKña Edl]sSah_ekItmanelIGñkCMgWtaLasesmuI

kMritF¶n;EdlqøgtamkarbBa©ÚlQam GacrYmcMENkya:gxøaMgdl;karvivtþn_eTA

muxya:gelOneTArkCMgWeføImF¶n;F¶r.

Natural history

of HCV infection6b

Global geographic

distribution of HCV

genotypes (Zein, 2000)

6c

109

karBüa)alrlakeføImraMuér:RbePT C ¬CHC¦

kalBIdMbUgeLIy karBüa)alrlakeføImraMuér:RbePT C rYmmankareRbI

fñaMmYymuxRbePT recombinant α-Interferon fVIebIvaeqøIytbnwgkar

Büa)almankMritTab ¬10 - 25°¦. Etya:gNak¾eday kñúgbNþa

qñaMcugeRkayenHkarBüa)alrlakeføImRbePT C ruaMér:mankarvivtþn_

KYr[kt;sMKal;. bc©úb,nñkalenH karBüa)alEdleKENnaMrYmman

recombinant α-Interferon rYmCamYyfñaM Ribavirin EdlCafñaMelb

tammat;manKuNsm,tþiRbqaMgnwgvIrus. b:uEnþkarBüa)aleday

Ribavirin bgáEbkRKab;QamRkhm eRBaHfñaMenHvaeFVI[mankarfycuHKYr[kt;

sMKal;nUvFatupSMsMxan;mYyrbs;eKalikaQamRkhm KW ATP ¬Adenosine

Triphosphate¦ EdlTTYlxusRtUvdl;karrs;ranmanCIvitrbs;eKalikaQam

Rkhm. GñkCMgWtaLasesmuIkMritF¶n;EdleRbI Ribavirin GacnwgCYbRbTHkar

EbkRKab;QamRkhmeRcIn ehIyGacRtUvkarbBa©ÚlQameRcInEfmeTot

¬ekInRbmaN 30°¦ EdlvanwgTamTarkarBüa)alkMcat;CatiEdkxøaMgeLIg

EfmeTot edIm,IkMcat;CatiEdkEdlelIs. fμI²enH/ man Interferon RbePTfμI

RbesIrCagmuneQμaHfa pegylated Interferon RtUv)aneKeRbIEtÉg b¤ pÁúMnwgfñaM

Ribavirin EdleFVI[GRtaeqøIytbnwgkarBüa)al)anRbesIreRcIn ehIy)anpþl;

CMerIsBüa)alepSgmYyeTotsMrab;GñkCMgWrlakeføImRbePT C ruaMér:. RbePT

nig ry³eBlénkarBüa)alGaRs½yeTAelI ehSNUTIb énvIrusrlakeføIm

RbePT C EdleKsÁal; kñúgenaH type-1 eKKitfa mankarlM)akBüa)alCageK.

karBüa)alRtUvsMercedayQrelIkarRbwkSaCamYyGñkCMnajEpñkelahitviTüa.

rebobrbbBüa)alfμI)anbegáInGRtaeCaKC½yBIkMritTabRtwmEt 10- 25°

edayeRbIEt recombinant α-Interferon EtmYymux rhUtdl;kMritCag 60°

cMeBaHRbePTeRkABI type-1 ehIyrhUtdl; 48° sMrab; type-1.

karBüa)alRbqaMgnwgvIrus HBV nig HCV Gs;esah‘uyéføNas; ehIyCa

Gkusl kareRbIR)as;fñaMTaMgenHkñúgRbeTskMBugGPivDÆn_CaeRcInenAmankMrit.

Healthy Liver

Fibrosis

Cirrhosis

Serological course of HCV

infection evoluting to

chronicity; other antibody

patterns are possible

6d

110

karRbugRby½tñTUeTAmYycMnYnTak;Tinnwgkarqøg HCV nig HBV KWRtUveCosvag

kareRbIR)as;rYmKñanUvRcas;duseFμj/ LamekaBukmat; nig vtßúeRbIR)as;pÞal;xøÜn

epSgeTotEdlGacmut)an. HBV gayqøgCag )ann½yfavagayqøg BImñak;

eTAmñak;Cag HCV, b:uEnþkarcak;fñaMbgáarRbqaMgvIrus)anRtwmRtUv GaclubbM)at;

eRKaHqøgesÞIrEtTaMgRsug.

Human immunodeficiency virus (HIV)

vIrus huIv (HIV) CaGtisuxumR)aNtUcmYy ¬emIlrUb 6e¦ EdlbgáCaCMgW eGds_

(AIDS = Acquired Immunodeficiency Syndrome).

HIV sßitkñúgRkumvIrusmYymaneQμaHfa “Retroviruses” EdlmanlkçN³

CIvsaRsþBiessEdlRKb;RKgviFIbegáIncMnYnkUnecArbs;va nig RKb;RKglkçN³

sm,tþirbs;vaenAxagkñúgekasikaénsarBagÁkayEdlvavayRbhar. eKalika

QamsEdleKsÁal;faCa Lymphocytes (CD4) Caekasikamansar³sMxan;

bMputrbs;sarBagÁkayEdlGacP¢ab;CamYyvIrusenH ¬emIlrUb 6f¦. vIrusenH

cUleTAkñúgekasikaenH edayeRbIynþkarmYyEdlGnuBaØati[vakøayCaEpñkén

DNA rbs; CD4 enaH ehIybegáItrUbragÁxøÜnvapÞal;CaRbcaMenAkñúgsarBagÁkay

énbuKÁlEdlmanCMgW KW begáIncMnYnkUnecAenAkñúgekasika Lymphocytes rYc

bMpøajekasikaenaHecaleTA ¬emIlrUb 6f¦. Lymphocytes edIrtYnaTIsMxan;

enAkñúgRbB½n§karBarsarBagÁkay b:uEnþeBlNamankarQøanBaneday

Retrovirus, BYk Lymphocytes nwg)at;bg;lT§PaBkarBarsarBagÁkay

Tl;nwgkarbgáeraK. ebIKμankarBüa)aleT CMgWnwgFøak;kñúgsPaBF¶n;F¶rkñúg

ry³eBlRbEhl 7 - 11qñaM eTaHCaeraKsBaØaKøInik nig PaBF¶n;F¶rGacekIt

qab;CagenHk¾eday ehIyKμankarkMNt;eBlc,as;las; sMrab;karcab;epþIm

énsPaBCMgWF¶n;F¶reLIy. kñúgdMNak;kaldMbUg BMumanbgðajsBaØaKøInik b¤

sBaØamnÞIrBiesaFn_eT ¬eKehAfa KμaneraKsBaØa¦. eBleraKsBaØaekItman b¤

lT§plmnÞIrBiesaFn_ enaHbBa¢ak;famankarxUcxatF¶n;F¶rcMeBaHekasika ehIy

karlUtlas;rbs;vIrusmankMritelOn mann½yfaCMgW)anQaneTAdl;dMNak;

6e

6f

111

kalkMritF¶n; ehAfa CMgWeGds_.

mUlehtusMxan;bNþal[søab; cMeBaHGñkCMgWeGds_ Edlmin)anTTYlkar

Büa)al KWkarbgáeraKF¶n;F¶r eBlEdlRbB½n§karBarKμanRbsiT§iPaB. esÞIrEt

RKb;Pñak;garbgáCMgWNak¾eday nwgbgá[mankarbgáeraKF¶n;F¶r rhUtdl;)at;bg;

CIvit cMeBaHGñkCMgWeGds_Edlmin)anBüa)al kñúgenaH Pneumocystic carinii

KWCabuBVehtujwkjybMputbNþal[søab;.

HIV qøgBIbuKÁlmñak;eTAbuKÁlmñak;tamry³Qam/ karrYmePTKμaneRbIeRsam b¤

BImþaymanCMgWeTAkUnEdleKehAfa Vertical route of transmission.

dUckrNI HBV nig HCV Edr/ karBarkarcMlg HIV tamry³Qam rYmman

kareFVIetsþQamedaymnÞIrBiesaFn_ edayRbugRby½tñral;QamGMeNay ehIy

esvapþl;QamRtUvEtmanKuNPaB. karcMlg HIV tamry³pøÚvepSgeTot

Gackat;bnßy)anya:geRcInedayeCosvagkarrYmePTédKUreRcIn ehIyKμan

eRsamGnam½ykarBar. dUckrNI HCV Edr/ minTan;manva:k;saMgman

RbsiT§iPaB nig suvtßiPaBRbqaMg HIV enAeLIyeT.

manfñaMCaeRcInRtUv)aneKykmkeRbItaMgBIqñaM 1996 CaBYk Anti-retrovirals

¬fñaMRbqaMgnwgBYk retrovirus¦ Edl cUleTAeFVIskmμPaBenAdMNak;kalepSg²

¬emIlrUb 6g¦ eBlEdlvIruscUl nig ecjBIekasika b¤ GMLúgeBlvIrusva

Life cycle of HIV showing

opportunities for drug

intervention

6g

112

eFVIkarbMEbkxøÜnenAkñúgekasikaEdlvavayRbhar. edayeRbIbBa©ÚlKñanUvfñaM

BIrmux/ bImux b¤ eRcInCagenH GnuelameTAtammKÁúeTsn_GnþrCati EdlGñk

CMnajkñúgvis½yenHeFVIkaresIerIfμICanic©/ fñaMTaMgenH)aneFVI[mankarpøas;bþÚr

muxmat;RbvtþiedImrbs;CMgW. cab;taMgBIfñaMTaMgenHRtUv)andak;[eRbImk/ TaMgkar

rs;ranmanCIvit nig KuNPaBCIvitrbs;GñkCMgW HIV manPaBRbesIrya:g

FMeFg. Cag 80° énkrNIqøgBImþaymanCMgW eTATarkkñúgépÞ b¤ eTAkUnekIt

mkehIy RtUv)ankarBaredayeCaKC½yedayeRbI Anti-retrovirals pgEdr.

ya:gNak¾eday dUckñúgkrNIBüa)al HCV nig HBV Edr fñaMeRbIkñúg

karBüa)al HIV mantMéléføxøaMgNas;Edr. GñkCMgWmYyPaKFMrs;enARbeTs

RkIRk eTaHbImankarxitxMRbwgERbgya:gNa k¾fñaMdUcenHenAmankMNt; ehIyCa

erOy²mantMéléføminGacTijeRbI)an. rhUtmkTl;eBlenH eTaHbITinñn½y

ecjpSaybBa¢ak;BIGRtaTabénkarqøg HIV kñúgcMeNamGñkCMgWtaLasesmuI

kMritF¶n;k¾eday k¾tYelxTMngCamankarekIneLIgx<s; eBlEdleKTTYl)an

Tinñn½yBIRbeTsCaeRcIncUlKña.

manGtisuxumR)aNepSg²CaeRcIneTotEdr EdlGacqøgtamry³Qam eTaHCa

vaminGacbgáeraKraMuér:cMeBaHGñkCMgWtaLasesmIukMritF¶n; b¤ GñkCMgWkgVHRKab;

QamRkhmedaykarEbkRKab;Qam CaBiessGñkEdlmanRbB½n§karBar

cuHexSayxøaMgkøa ]TahrN_ eRkayBIkarvHkat;pSaMsrIragÁk¾eday k¾Gtisuxum

R)aNTaMgenHGacbgákarlM)akEpñkKøInikEdr.

vIrusepSg²eTot

Human Parvovirus B-19

Human Parvovirus B-19 (HPV-B-19) CavIrusepSgmYyeTotEdlGacqøg

tamry³Qam ¬eTaHCaQamminEmnCapøÚvcMlgsMxan;k¾eday¦. \T§iBlb:H

Bal;sMxan;bMputrbs;vIrusenHeTAelIGñkCMgWtaLasesmuI b¤ CMgWkgVHRKab;Qam

Rkhm edaykarEbkRKab;QamepSgeTotdUcCaCMgW Sickle cell KWfavaGac

bgáeGayp¥akkarplitRKab;QamRkhmmYyry³ EdleKehAfa vibtþi

113

beNþaHGasnñénkarplitRKab;Qam ¬Transient Aplastic Crises¦.

dMNak;kalRsYcRsavbgáeday HPV B-19 sMKal;edaykarFøak;cuH eGm:UkøÚb‘Ín

(Hb) Pøam² ehIynwg)at;bg; eKalikaQamRkhmedIm enAtamsésQam

KW)at;BYk Reticulocytes enHÉg. kñúgkrNIeKeFVIeraKvinicä½yTan;eBlevla

eKEtgBüa)alGñkCMgW edaykarbBa©ÚlQamRss; EdlmanpÞúkGgÁbdibkç

RKb;RKan;sMrab;RbyuT§nwgvIrus. b:uEnþGñkCMgWEdlexSayRbB½n§karBar dUcCaGñk

CMgWEdlpSaMxYrq¥wg b¤ GñkCMgWmanpÞúkvIrus HIV CaedIm eBlEdlqøgvIrus HPV

B-19 GacekItmansPaBF¶n;F¶r CYnkalmanplvi)akEpñkKøInikraMuér:.

Human Cytomegalovirus

vIrusmYyEdlecaTeRKaHfñak;FMeFgcMeBaHGñkCMgWexSayRbB½n§karBarKW Human

Cytomegalovirus (CMV). vIrusenHbgáCMgWF¶n;F¶rcMeBaHGñkCMgWpSaMsrIragÁ

ehIyrEmg[)at;bg;CIvitebIeFVIeraKvinicä½y nig Büa)alminTan;eBlevla.

lkçN³sMxan;rbs;vIrusenH KwvamanlT§PaBP¢ab;xøÜnvaCaGciéRnþy_kñúgekasika

EdlvaQøanBan ¬sMxan;KWeKalikaQams¦ edayeFVI[eKalikas BuHkeRBa¢al

eLIgedayminGacdwgmun)an ehIybgá[manCMgWeBlNamYy b¤ CMgWsacuH

saeLIgF¶n;F¶r. dUecñHsMxan;GñkCMgWtaLasesmuIkMritF¶n; CaBiessGñkEdl

eRtomeFVIkarbNþúHxYrq¥wg b¤ GñkEdlkMBugdMeNIrkarbNþúHxYrq¥wg RtUvbBa©ÚlQam

Edl)aneFVIcMeraHEdleK)anykecjnUveKalikaQams nig Pñak;garbgáCMgW

epSg²eTotEdlmanCab;CamYyva rYmman CMV CaedIm.

CMgWRKuncaj nig CMgW Chagas

CMgWRKuncaj;eRkaykarbBa©ÚlQam nig CMgW Chagas ¬CaCMgWbgáedayBYk

):ar:asIutmü:agrs;kñúgQameQμaH Trypanosoma¦ RtUv)aneKsÁal;Cag

50qñaMmkehIy. RbePT Plasmodium EdlCaPñak;garbgáCMgWRKuncaj; nig

Trypanosoma cruzi EdlCaPñak;garbgáCMgW Chagas GacmanCIvitkñúg

smasFatuQamkøaes nig rhUtdl;kñúgpøasμakk ya:gehacNas;k¾)an

BIrs)aþrh_Edr.

114

CalT§pl/ eKRBYy)armÖxøaMgcMeBaHeTscrN_eTAkan;RbeTsEdlmanekItCMgW

CaRbcaM ehIykMeNInGenþaRbevsn_ BIRbeTsekItCMgWeTARbeTsKμanekItCMgW

GacbegáInkarcMlgCMgWRKuncaj; nig CMgW Chagas. GgÁkarsuxPaBBiPBelak/

RkumRbwkSaGWr:ub/ GaC£aFrsuxaPi)al nig esvapþl;QamCatirbs;shrdæ

Gaemrik )anrYmKñadak;ecjnUvbNþasþg;da ¬niyam¦ kñúgeKalbMNgkarBarCMgW

RKuncaj; nig CMgW Chagas eRkaykarbBa©ÚlQam rYmmankareFVIetsþQam

EdlCaGMeNay.

Pñak;garbgáeraKfμI

ehAfa Pñak;garbgáeraKfμI CaPñak;garEdleKrkeXIjenAcenøaHqñaM 1995 -

98 rYmmanvIrusbgárlakeføImepSgeToteRkABI RbePT B nig C enaHKWrlak

eføImRbePT G ¬HGV b¤ GBV-C¦/ SEN-V nig TTVirus. eTaHbIman

Éksarc,as;las;bBa¢ak;BIkaeqøgvIrusTaMgenHtamry³karbBa©ÚlQamk¾eday

k¾tYnaTIvIrusTaMgenHkñúgkarbgá[ekItCMgWeføIm enABMuTan;c,as;enAeLIy.

karRBYy)armμN_fμI cMeBaHPñak;garbgáeraKcas;

CMgW Creutzfeld-Jakob (CJD) nig TMrg;fμIrbs;va b¤ ExSRsLayva (vCJD)

EdlCaTMrg;ekItelImnusSecjBI Bovine Spongiform Encephalitis (BSE)

EdlmanenAelIstVeKaRkbI CaCMgWpþac;CIvitedayb:HBal;eTAelIRbB½n§RbsaT.

CMgWenHbgáedayRbUetGuInbgáCMgWmü;:ag ¬Prion protein¦ RsedognwgvIrus Et

minEmnCavIrusBitR)akdeLIy ehIyrcnasm<½n§rbs;vamankarlM)aknwgeFVI

cMNat;fñak; edayeFobnwgPñak;garbgáeraKepSg²eTot ¬mIRkub/ )ak;etrI nig

)a:ra:sIut¦. eRkamlkçxN½ÐxøHminc,as;las; eKdwgfasarBagÁkaymnusS)an

bþÚrRbUetGuInbgáCMgWenaHeTACaPñak;gareRKaHfñak;mYy bgá[ekItCaCMgWsrés

RbsaTpþac;Civitedayvivtþn_eTAmuxyWt². eTaHeK)ansÁal;CMgWenHCaeRcInqñaM

mkehIyk¾eday EtsarsMxan;énCMgW CJD nig ExSRsLayrbs;va eTIbEtelc

ecjeFøaeLIgenABak;kNþalTsvtSr_ 1990 eBlEdleKBinitüelIGñksøab;

115

ehIyeK)anrkeXIjCaeRcInkrNI BiessenAcRkPBGg;eKøs bnÞab;mkman

enARbeTsepSg²eTotEdr EtkñúgcMnYntictYc. enAExFñÚ 2001 man 113

keNIenAcRkPBGg;eKøs/ 4 krNIenARbeTs)araMg nig 1 krNIenA

saFarNrdæeGoLg; nig 1 krNIenAekaHhugkug.

RKb;krNITaMgGs;énExSRsLayfμIrbs; CJD EdlmankñúgÉksarrhUtmkTl;

eBlenH eKKitfabNþalmkBIGñkCMgW)anhUbplitplecjBIstVeKaRkbIman

CMgW. dUcKñaEdr karcMlgeraKRtUv)aneKdwgfaekItmancMeBaHGñkCMgWEdl)an

TTYlG½rm:UnmYycMnYn)anmkBImnusS ¬G½rm:UnlUtlas;¦ nig GñkEdleKbnSaM

eRsamxYrk,al nig bnSaMRsTab;kBa©k;EPñk. eKmanr)aykarN_pgEdr sþIBIkar

qøgtamry³sMPar³Edlmin)ansMGatl¥eRkaykarvHkat;EpñksrésRbsaT

nig rebobftskmμPaBxYrk,al. BMumanr)aykarN_sþIBIkarqøgtamry³kar

bBa©ÚlQameLIy. edaysarPaBF¶n;F¶rénCMgW nig vtþmanénRbUetGuInbgáCMgW

kñúgCalikaTwkrég/ RbeTselakxaglicmYycMnYn CaBiesscRkPBGg;eKøs

)ancMNayFnFanya:geRcInelIeKalneya)aykarBarkarqøgCMgWtamry³

Qam. eKalneya)aymYyénbNþaeKalneya)ayEdleKGnuvtþ KW kareFVI

cMeraHQamCaskl KWeFVIcMeraHral;QamEdlRbmUl)an tamry³enaH

eKalikaQamsmYyPaKFMbMput ¬Lymphocytes¦ RtUv)anykecj.

suvtßiPaBQam

ral;kic©RbwgERbgCaGnþrCati KWepþateTAelIkic©karBarkarcMlgPñak;garbgáeraK

tamry³karbBa©ÚlQam. bNþaktþaEdlrYmcMENkdl;suvtßiPaBQamrYmman ³

[i] begáIteKalneya)ayEdlmaneKalbMNgFana[)ankareFVIGMeNay

QamedayKμanbg;R)ak; elIeKalkarN_sμ½RKcitþ ehIyeTogTat; BIGñkeFVI

GMeNayQam.

[ii] esvapþl;QammanKuNPaBx<s; rYmmankareFVIetsþmnÞIrBiesaFn_Edl

caM)ac;cMeBaHQamGMeNay edayrk HIV, HBV, HCV, Syphilis nig

Pñak;garbgáeraKepSg²eTotEdlmankñúgtMbn;.

116

[iii] begáItviFankarN_suxaPaBsaFarN³ dUcCa karcak;fñaMbgáar nig CMhan

epSg²kñúgkarsMGatemeraK.

kñúgbNþaqñaMcugeRkayenH eTaHtMéléføya:gNak¾eday k¾bNþaRbeTs

]sSahkmμ)anykmkeRbIpgEdr nUvbec©kviTüaBiessEpñkmnÞIrBiesaFn_ DNA

eKehAfa Nucleic Acid Testing (NAT) edIm,IBinitüQam Edleq<aHeTAkat;

bnßyKYr[cab;GarmμN_EfmeTot nUveRKaHénkarcMlgvIrus EdlmanlkçN³

F¶n;F¶rEpñkKøInik.

karcMlg)ak;etrI

eRkABIvIrus/ GtixumR)aNepSg²eTotdUcCa )ak;etrI nig )a:ra:sIut GacmanCIvit

nig begáIncMnYnkñúgQamqøgeTAGñkCMgWtamkarbBa©ÚlQam. tamBiteTAGRta

énkarqøgtamQamrbs;)ak;etrImanBI 50 - 250 dg x<s;CagvIrus.

CaerOy² karqøgekIteLIgtamviFIsamBaØ ehIyGaceCosvag)anya:ggay.

]TahrN_ RbPBcMlgeRcInCageK KWkarxVHGnam½y KwfaEs,kGñkeFVIGMeNay

QamsMGatmin)anl¥muncak;m¢úlbUmQam. eBlEdlm¢úlmutcUlkñúgEs,k

va)annaM)ak;etrIBIépÞEs,kcUleTAkñúgQamEdleyIgRtUvbUmyk.

bNþaCMhanfμI² edIm,I)ansuvtßiPaBQam

kñúgkic©RbwgERbgmYy edIm,Ibnþkat;bnßylT§PaBcMlgeraKedayPñak;garbgá

eraKRKb;RbePTTaMgGs;/ eK)anbegáItbec©kviTüafμI²kñúgeKalbMNgkMeTc

ecalnUv DNA rbs;BYkPñak;garbgáeraK eBlNaeKbBa©ÚlvaeTAkñúgfg;Qam

GMeNay. viFIEbbenHeKehAfa Pathogene inactivation. vaCaviFId¾skmμ

dMbUgbMputedIm,IsuvtßiPaBQam edayvaeFVIeGayemeraKElgmanskmμPaB

teTAeTot eTaHeK)andwg b¤ mindwgfamanemeraKenAkñúgfg;Qam b¤ Gt;.

karRsavRCav nig karsikSaEpñkKøInikénbec©kviTüaenH )anmkdl;dMNak;kal

cugeRkay ehIybec©kviTüamYykMBugRtUv)anyl;RBm ehIyecjlk;elITIpSar

enAGWr:ub cMeBaHpøaEkt/ EtvtßúbMNgcugeRkay KWRtUveRbIbec©kviTüaenH cMeBaH

117

RKb;smasFatuepSg²eTotrbs;Qam rYmmaneKalikaQamRkhmpgEdr.

suvtßiPaB nig manQameRbIR)as;enAbNþaRbeTskMBug

GPivDÆn_

enARbeTskMBugGPivDÆn_CaeRcIn eRKaHfñak;bNþalmkBIkarbBa©ÚlQamman

PaBFMeFgeRcInCagbNþaRbeTs]sSahkmμ. enAbNþaRbeTskMBugGPivDÆn_

bBaðasMxan;KwkareRbIR)as;QamxN³EdlKuNPaBesvapþl;Qam dUcCa

eKalneya)ayCMerIsGñkpþl;Qam nig kmμviFIcak;fñaMbgáar minTan;)anQan

dl;sþg;daEdleKcg)andUcenAGWr:ub nig shrdæGaemrik. karxVHxatEpñk

hirBaØvtßú karRbECgKñaEpñkGaTiPaB nig GesßrPaBneya)ay TaMgGs;enH)an

eFVI[yWtya:vdl;dMeNIrGnuvþn_sþg;dax<s;énsuvtßiPaBQam. eyagtamTinñn½y

EdlRbmUl)anedayGgÁPaBEpñksuvtßiPaBQamrbs;GgÁkarsuxPaBBiPBelak

)an[dwgfa 80° énRbCaBlrdæTUTaMgBiPBelak rs;enAkñúgRbeTskMBug

GPivDÆn_ b:uEnþBYKeKTTYl)anmindl; 40° pgnUvkarpÁt;pÁg;QamsrubRbcaMqñaM

rbs;BiPBelakEdlmandl; 75 lanfg;. RbEhlCa 16° b:ueNÑaHénkar

pÁt;pÁg;Qam EdlRbmUl)anenAbNþaRbeTskMBugGPivDÆn_ )anmkBIGñkbric©ak

eFVIedaysμ½Rkcitþ Kμanbg;luykak; xN³Edl 43° énQam min)aneFVIetsþ

)aneBjeljeLIy. CalT§pl eKrMBwgfaenARbeTskMBugGPivDÆn_CaeRcIn

ekItmankarcMlgeraK HIV, HBV nig HCV tamry³karbBa©ÚlQam ehIy

kñúgenaHRbeTsmYycMnYnmankMritx<s;. GaC£aFr nig GgÁkarsuxPaBCati nig

GnþrCatiTaMgGs; RtUvxitxMRbwgERbgeRcInEfmeTot edIm,IeFVI[suvtßiPaBQam

kan;EtRbesIr nig manQameRbIR)as;RKb;RKan;kñúgRbeTsTaMgenaH.

118

ERbRbYlTMrg;q¥wgrbs;mux

¬q¥wgfÁamelIrIke)a:g¦

srIragÁTTYlkarb:HBal;edaykarbBa©Úl

QamminRKb;RKan;

srIragÁTTYlkarb:HBal;edaykarkMcat;CatiEdk

minRKb;RKan;

RkeBj Pituitary ¬b:HBal;karFMFat;/ srIragÁ

ePT/ RkeBj Adrenal, RkeBj Thyroid¦

RkeBj Parathyroid ¬Hypoparathyroidism

[naMekIt Hypocalcemia¦

ebHdUg ¬exSayebHdUgb:HBal;xøaMgCageK¦

eføIm ¬rIkeføIm¦

Rbdab;ePT ¬mankarlM)akkñúgkarrIkFMFat;

nig dMeNIrkar¦

Bukq¥wg/ QWq¥wg/ QWsnøak; ¬Tak;Tgnwg

Hypoparathyroidism ¦

Pancreas ¬TwkenamEp¥m¦

Pigmentation ¬Es,k sPaBeLIgRbepH

enAcugRkck/ Ekgéd/ CgÁg;/ EPñkeKal¦

RkeBj Thyroid ¬kMr¦

Qam

¬kgVHRKab;QamRkhm¦

)ak;q¥wg

* bNþalmkBIkarrIxYrq¥wg

Epiphyse q¥wg femoral

xageRkambiTmunkalkMNt;

Hypersplenism

(Thrombocytopenia)

segçbbBaðaEdlGacekItmancMeBaHGñkCMgwtaLasesmuIkMritF¶n; Edl

min)anBüa)al

119

CMBUkTI 7

taLasesmIukMritmFüm nig taLasesmIuepSg²eTot

taLasesmIukMritmFüm CasPaBevC¢saRsþmYyEdlGñkCMgW)anTTYltBUCBI

mþay nig «BuknUv Bn§úEbtta (B - gene) EdlmanCMgW ¬)ann½yfaGñkCMgWCa

GUm:UhSIúháÚt cMeBaH Ebtta taLasesmIu¦ b:uEnþGñkCMgWTaMgenHmaneraKsBaØa

KøInikRsalCagtaLasesmIukMritF¶n;. GñkCMgWtaLasesmIukMritmFüm varkSa

)ankMrit eGm:UkøÚb‘Ín kñúgQamenAcenøaH 6 - 9 Rkam¼d>l (g/dl) ehIyGacmin

caM)ac;bBa©ÚlQamCaeTogTat;eLIy.

ya:gNak¾eday karRsavRCavCaeRcIn)anbgðajfa taLasesmIukMritmFüm

mansBaØaKøInikeRcInya:g kñúgenaH sBaØaxøHGacmanlkçN³F¶n;F¶r. kñúgkrNI

F¶n;F¶rbMput GacbgðajsBaØaKøInik nig sBaØamnÞIrBiesaFn_enAGayuBI 2 - 6 qñaM

fVIebIkarrIkFMFat; nig karlUtlas;ragÁkaymansPaByWtCagFmμtak¾eday

k¾GñkCMgWGacrkSa)annUvKuNPaBCIvit edaymincaM)ac;bBa©ÚlQameTogTat;dUc

krNItaLasesmIukMritF¶n;eLIy. kñúgkrNIminsUvF¶n;F¶r GñkCMgWminbgðajeraK

sBaØaNamYyeLIy rhUtdl;eBjv½y KWRKan;EtmansPaBxVHRKab;QamRkhm

bnþicbnþÜcb:ueNÑaH ¬8 - 10 Rkam¼d>l¦ ehIykMrnwgbBa©ÚlQamebIKμan

bBaðaGVIenaHeT.

GeNþIkGacmanTMhMFM ¬Splenomegaly¦ dUckrNICMgWtaLasesmIukMritF¶n;Edr

edaysareKalikaQamRkhmEbkelOn ehIykkCujenAkñúgGeNþIk ehIyCYn

kalbgá[sPaBkgVHRKab;QamRkhmkan;EtF¶n;F¶rcMeBaHCMgWtaLasesmIu

kMritmFüm. krNIEbbenH GñkCMgWGacnwgRtUvbBa©ÚlQamya:geTogTat;.

karkat;GeNþIkecj GacEkkun)annUvplvi)akenH Etya:gNak¾edaykarsMerc

citþeFVIdUcenHCakarF¶n;F¶r EdlRtUvmankarcUlrYmpþl;mtieyabl;BIGñkCMnaj

edayBicarNaeTAelIplb:HBal;EdlGacekItmaneLIg dUcCalT§PaBénkar

120

bgáeraK EdlCaRCugepSgeToténsuxPaBGñkCMgW eRkABIkarCYyseRgÁaHsPaB

xVHRKab;QamRkhm.

dUc)anelIkeLIgkñúgCMBUkTI2/ buBVehtucMbgEdlbgá[maneraKsBaØakñúgCMgW

taLasesmIukMritF¶n; KWedaysarkarkkCujkñúgeKalikaQamRkhmnUv

ExSGal;hVa (a) esrI begáIt[manGtulüPaBrvag ExSGal;hVa nig

ExSRbUetGIunEdlFmμtaCaédKUrrbs;va KW ExSEbtta (β). edayvaÉkeka

EtÉg/ ExSGal;hVa )ancUleTArMxan esÞIrEtRKb;dMNak;kalén vdþeBjv½y

¬Cycle of maturation¦ rbs;eKalikaQamRkhm bgáeGaymansPaBxVH

RKab;QamRkhmF¶n;F¶r nig sBaØaepSg²eTot dUc)anCMrabxagedIm.

dUckarGHGagxagelIenH/ manehtupl[eyIgrMBwgfa eraKsBaØarbs;

taLasesmIunwgminsUvF¶n;F¶r kalNa ExSGal;hVa EdlelIsRtUv)anbnßy

mkvij. karGegáteTAelIm:UelKul)anbgðajfa lkçx½NÐmYycMnYnEdlExS

Gal;hVacuHfy mandUcCa ³

[i] vtþman Bn§úEbtta (β+ gene) EdlGacplit ExSEbtta xøHEdr eTaHCa

plit)anticCagFmμta/ ExSEbtta enHnwgeTAcab;KUrCamYy ExSGal;hVa

dUcenHva)anbnßy ExSGal;hVaesrI EdlKμanKUr. karpøas;bþÚr

(Mutations) cMeBaH Bn§úEbtta (β+ gene) EdlnaM[ecjCalT§pl

KøInik minsUvF¶n;F¶rxøaMg CYnkalenaH eKRtUvdak;nam[fa β++.

[ii] karxUcxatcMeBaHBn§ú EdlTTYlxusRtUvdl;karplit ExSGal;hVa eFVI[

ExSGal;hVamYycMnYnRtUv)at;bg; rYck¾eFVI[RbesIreLIgvijnUvtulüPaB

rvag ExSGal;hVa nig ExSEbtta.

[iii] Bn§úháama: ¬γ - genes¦ begáInskmμPaBCagmun edaybegáInkarplit

ExSháama: ¬γ - chains¦ EdlGaceTAP¢ab;CamYYy ExSGal;hVa edIm,I

begáItCa eGm:UkøÚb‘ÍnTark [Foetal haemoglobin (a2γ2)] dUcenHvaCYy

bnßy ExSGal;hVaesrI EdlmaneRKaHfñak;. sßanPaBEdlGacCYydl;

karplit ExSháama: man ³ CMgW β- taLasesmIu edayman eGm:UkøÚb‘Ín

121

TarkCaGciéRnþy_ nigkarpøas;bþÚrkEnøgNamYyCayfaehtuenA Bn§úháama:

EdleKehAfa Xmnl.

dUc)anbgðajxagelIenH RKUeBTüGacelIkkMBs;cMeNHdwgrbs;eKcMeBaHsPaB

GñkCMgW tamkaryl;nUvEbbEpnxUcxatBitR)akdcMeBaH DNA rbs;GñkCMgW .

dUecñHbnÞab;mkeTot vakan;Etgay[eyIgdak;ecj)annUvkmμviFIBüa)al

smRsbsMrab;GñkCMgWmñak;². ebIGaceFVIeTA)an viFIBinitüm:UelKulEbbenH

kMBugEtbgðajnUvCMnYyminGackat;éfø)an cMeBaHkarBüa)alCMgWtaLasesmIu.

kareFVIeraKvinicä½y

kñúgkareFVIeraKvinicä½yCMgWtaLasesmIukMritmFüm sMxan;RtUvrk[)annUvBt’man

KøInik nig mnÞIrBiesaFn_mYycMnYnedIm,I[eyIgEjk[)anBItaLasesmIu

kMritF¶n;. b:uEnþenHminEmnecHEtgayRsYlenaHeT b¤ eFVI)anRKb;eBlenaHeT

eTaHbImankarrIkcMerInEpñkbec©keTsmnÞIrBiesaFn_xagm:UelKulk¾eday.

eTaHya:gNa cMNucsamBaØ nig manGtßRbeyaCn_sMrab;eFVIkarEbgEck

eraKvinicä½ymandUcteTA ¬tarag 7a¦ ³

122

CasnñidæanmkBaküfa taLasesmIukMritmFüm KWeKeRbIedIm,Ierobrab;eraKsBaØa

KøInik nig EpñkelahitsaRsþ cMeBaHGñkCMgWEdlmanTMrg;RsalCag Ebtta

taLasesmIu eGetr:UhSIúháÚt (Heterozygous b - Thalassemia) b:uEnþva

F¶n;F¶rCagTMrg; taLasesmIu eGetr:UhSIúháÚt.

kMritF¶n; kMritmFüm kMritRsal

sBaØaKøInik cab;epþImBIkumarPaB cab;epþImeBleRkay KμaneraKsBaØa

GeNþIkrIkFM ++++ +++ - ++++ 0 - +

xan;elOg +++ + - +++ 0 - +

bMErbMrYlq¥wg ++++ ++ - ++++ 0

bMErbMrYlmux ++ - ++++ 0 - ++++ 0

sBaØaEpñkelahitsaRsþ

PaBesøksøaMg ++++ ++ - +++ 0 - +

eKalikaQamRkhm � � N -�

Microcytosis + + +

NRBC ++ - ++++ + - ++++ 0

sBaØaEpñkCIvKImIsaRsþ

HbF 10 – 95 + % 10 – 95 + % N b¤ < 10%

HbA2 N b¤ � N b¤ � N b¤ � (> 3,5%)

eraKsBaØabgðajCMgWtaLasesmIu

7a

(Proceding from a conference,

MA Nov. 14, 1996

123

karBüa)al taLasesmIukMritmFüm

kñúgCMgWtaLasesmIukMritmFüm/ sMnYrsMxan;bMputKWfaetIeBlNaEdlRtUvcab;

epþImBüa)aledaybBa©ÚlQam. sßanPaBevC¢saRsþmYycMnYn dUcxageRkam

enHGacbNþalmkBIkgVHRKab;QamRkhmraMuér: ehIyCaehtuplBitR)akd

sMrab;eKcab;epþImBüa)aledaykarbBa©ÚlQam ³

� karrIkFMFat;manPaByWtya:v

� q¥wggaynwg)ak;

� plvi)akcMeBaHebHdUg

� ERbRbYlTMrg;q¥wgrbs;mux

� skmμPaBragÁkayFmμtafycuH

� GeNþIkskmμrIkFMxøaMg

dUckñúgkrNICMgWtaLasesmIukMritF¶n;Edr/ sMxan;eKRtUvtamdanGñkCMgW[)an

eTogTat;tamry³karBinitüevC¢saRsþ nig mnÞIrBiesaFn_ kñúgeKalbMNgqab;

)andwgTan;eBlevla kalNamanplvi)akNamYyEdlekIteLIg. elIsBIenH

edaysarGñkCMgWtaLasesmIukMritmFümcab;epþImkarbBa©ÚlQamenAeBl

EdlmanGayueRcInbnþic mindUckrNICMgWtaLasesmIukMritF¶n; dUcenHsMxan;

RtUvykcitþTukdak;eTAelIRbtikmμEdlGacekItmanCayfaehtu ¬Rbtikmμ

Alloimmunisation¦ dUc)anerobrab;BIxagedIm edayRbtikmμEbbdUcenHEtg

ekItmaneLIgeBlbBa©ÚlQamcab;epþImenAGayuFM. dUcenH/ sMxan;Qamrbs;

GñkCMgW nig Qamrbs;Gñkpþl; RtUvEtRkumQamdUcKña nig pÁÚrpÁg[sIuKña

ral;eBlmuneFVIkarbBa©ÚlQammþg². vasMxan;pgEdr edaykt;sMKal;fa

RsþICaGñkCMgWtaLasesmIukMritmFüm ehIymanépÞeBaH GacRtUvkarbBa©Úl

QamEdr.

124

karkMcat;CatiEdk

dUckñúgkrNICMgWtaLasesmIuF¶n;F¶rEdr/ CatiEdkelIskñúgCMgWtaLasesmIu

kMritmFüm GacbNþalmkBI ³

[i] karpliteKalikaQamRkhmKμanRbsiT§iPaB

[ii] karEbkeKalikaQamRkhm

[iii] brimaNEdkRsUbBIeBaHevonekIneLIg

mankarsikSaeRbobeFobmYycMnYnb:ueNþaHGMBIkarkkpþúMCatiEdk cMeBaHGñkCMgW

taLasesmIukMritmFüm. b:uEnþkarsikSamYy)anbgðajfa Caerogral;qñaMman

CatiEdkkkpþúMkñúgxøÜnGñkCMgWtaLasesmIukMritmFüm 2 - 5Rkam KWesμI 0/1

mRk¼KRk¼éf¶. )anesckþIfa CatiEdkRtUv)anRsUbBIcMNIGaharx<s;CagFmμta

20 - 70°. dUecñH kñúgkrNIPaKeRcIn eRkayBIGayuCag 10 qñaMeTA/ GñkCMgW

taLasesmIukMritmFümgaynwgmanplvi)akbNþalmkBIEdkswgEtdUcKña

nwgGñkCMgWtaLasesmIukMritF¶n; Edl)anTTYlkarbBa©ÚlQamCaeTogTat;.

karlM)akkñúgkarsMercfa eBlNaRtUvcab;epþImkMcat;CatiEdkBIkñúgxøÜnCMgW

taLasesmIukMritmFüm KWkMNt;edaysPaBelIsCatiEdkBitR)akdkñúgxøÜn

GñkCMgW eRBaHfakMrit Ferritin kñúgQam minGacpþl;rgVas;rgVal;BitR)akdeLIy

EdlvadUcKñanwgkrNICMgWtaLasesmIukMritF¶n;Edr. ehtuenHkarENnaM[eFVIkar

vaytMélkMhab;CatiEdktammeFüa)ayRcwbsac;eføIm ¬liver biopsy¦ b¤

tamry³viFIfμIEdlgaydwg ehIymin[GñkCMgWQWcab; dUcCa SQUID b¤ MRI.

eBlEdlsMerccitþfa cab;epþImkarBüa)alkMcat;CatiEdk/ eKENnaM[eRbIfñaM

DFO dUckñúgkrNICMgWtaLasesmIukMritF¶n;Edr. EtcMeBaHGñkCMgWtaLasesmIu

kMritmFümeKKYreRbIkarcak;eRkamEs,kmin[elIs 2 b¤ 3éf¶kñúgmYys)aþh_.

eKENnaM[tamdankarBüa)alkMcat;CatiEdkcMeBaHGñkCMgWtaLasesmIukMrit

mFüm eGaydUcKñanwgkrNIGñkCMgWtaLasesmIukMritF¶n;Edr.

125

edayGñkCMgWtaLasesmIukMritmFümRsUbykCatiEdkBIeBaHevoneRcInCag

Fmμta GñkCMgWRtUveCosvagcMNIGaharNaEdlsMbUrCatiEdk ¬]TahrN_

és<exμA eføImstV nig RbePTsENþkmYycMnYn¦ RBmTaMgkarelbCatiEdk

bEnßm. pwkTwkEtexμACamYycMNIGahar GacCYykat;bnßybrimaNCatiEdk

RsUbtameBaHevonEdr.

bBaðaEpñkevC¢saRsþkñúgCMgWtaLasesmIukMritmFüm

[1] bMErbMrYlqw¥wg> xYrqw¥wgskmμxøaMg bNþalmkBIsarBagÁkayxitxMplit

RKab;QamRkhm edIm,ITb;Tl;nwgsPaB kgVHRKab;QamRkhm eFVI[q¥wg

mansPaBrmYlxusTMrg;edIm puyehIyesþIg rMxandl;karrIklUtlas;

ehIyeFVI[GñkCMgWgayrgkar)ak;q¥wg. b:uEnþbBaðacMeBaHq¥wgF¶n;F¶rGacCMnH

)antamry³karBüa)aledaybBa©ÚlQameGay)aneTogTat;.

PaBBukq¥wg (Osteoporosis)> GñkCMgWRtUveKelIkTwkcitþ[hat;R)aN

nigbegáInCatikalsüÚb Calcium kñúgcMNIGahar edIm,IeCosvagCMgWq¥wg

F¶n;F¶r ¬PaBBukq¥wg¦. RKab;fñaM Calcium nig vItamIn ed (Vitamin D)

Gacpþl;KuNRbeyaCn_bEnßm. RtUveCosvagkarCk;)arI evC¢bNÐitxøH)an

bgðajBIGtßRbeyaCn_BIkareRbI Biophosphonates elbtammat; b¤

cak;bBa©ÚltamsrésQam/ b:uEnþtYnaTIrbs;vakñúgkarRbyuT§nwgPaBBukq¥wg

BMuTan;RtUv)aneKbBa¢ak;enAeLIy.

[2] karmanskmμPaBxøaMg b¤ karrIkFMénxYrqw¥wg nig hVÚlikGasIut (Folic

acid)> edaysarxYrq¥wgGñkCMgWtaLasesmIukMritmFüm RbwgeFVIkarxøaMg

edIm,IRbyuT§nwgPaBkgVHRKab;Qamrbs;sarBagÁkay edayxMplit

eKalikaQamRkhm[)aneRcIn/ GñkCMgWRtUvkarbEnßmbrimaNvItamIn

mYycMnYn CaBiess hVÚlikGasIut. kgVH hVÚlikGasIut GaceFVI[PaB

xVHeKalikaQamRkhmkan;EtF¶n;F¶rcMeBaHGñkCMgWtaLasesmIukMrit

mFüm. hVÚlikGasIut eKeXIjmankñúgFmμCati dUcCa sac;/ bEnøébtg.

126

b:uEnþbrimaNbEnßm Kwelb 1RKab;¼éf¶ vaGacbMeBjesckþIRtUvkarbEnßm

rbs;GñkCMgW .

[3] RKYsRbmat;> GñkCMgWtaLasesmIukMritmFümekItRKYsRbmat;

¬Cholelithiasis¦jwkjyeRcInCagFmμta. RKYsRbmat; ekItecjBI

plitplsMNl; ¬Bile pigment = sarFatuBN’TwkRbmat;¦ Edl)an

mkeBleKalikaQamRkhmEbk/ ehIysarFatuenHeTAkkpþMúkñúgfg;Twk

Rbmat;EdlGacbgá[sÞHfg;TwkRbmat; ehIybNþal[cukeBaH. vtþman

RKYskñúgfg;TwkRbmat;GacbBa¢ak;)anedayBinitü eGkU ¬Ultrasound¦.

ebIcukeBaHenaHCab;rhYtmin)at; eKGackat;fg;TwkRbmat;enaHecal.

[4] dMe)AeCIg> GñkCMgWtaLasesmIukMritmFüm CajwkjyekItdMe)ACMuvij

keCIg CaBiessGñkCMgWv½ycMNas; bNþalmkBIcrnþQamnaM GuksIuEhSn

min)anl¥eTAEp¥kxøHénsarBagÁkay. dMe)AenHmingayCasH ehIyBi)ak

Büa)alNas;. b:uEnþkarbBa©ÚlQameTogTat;edIm,IbegáInkMrit eGm:UkøÚb‘Ín

(Hemoglobin) nwgCYYypÁt;pÁg; GuksIuEhSn [)anRbesIreTACalikadMe)A

enaH RBmTaMgviFankarsamBaØ²dUcCa edkelIkeCIg nig RbGb;eCIg[x<s;

CagebHdUg[)an 1 - 2ema:g kñúgmYyéf¶ ehIyBak;eRsameCIgkarBar

keCIg TaMgenHGacCYysMral)anxøH. fñaMxøH dUcCa Zinc sulphate RbePT

RKab; CYnkalGacCYy)anEdr/ RBmTaMgfñaM Hydroxyurea eRbIEtÉg b¤

pÁúMcUlKñaCamYyfñaMepSgeTot GacbegáInkMrit HbF dUcCa Erythropoietin

nig Butyrates.

[5] plvi)akeTAelItMrgenam> bBaðecaTEpñkevC¢saRsþepSg²eTot EdleK

raykarN_mkfaekItmankñúgcMeNamGñkCMgWtaLasesmIukMritmFümKwkar

xUcxatcMeBaHtMrgenam EdlGacbNþalmkBIkarelIsCati Uric acid

kñúgQam. Uric acid KWCakaksMNl;sMxan;bMputbNþalmkBIxYeq¥wg

skmμxøaMg. fñaM Allopurinol GacCYykat;bnßybrimaN Uric acid

EdlRtUv)anplitelIs.

127

[6] kMeNInpøaEkt ¬Thrombophilia¦> plvi)akepSgeTotKW eRKaHekIt

kMNkelIPñassrésQam ¬Thrombosis¦ bNþalmkBI pøaEkt

¬sUmemIlEpñk Qam¦ kkCujkñúgsrésQambegáItCadMukMNk ¬Clots¦

EdleFVI[sÞHcrnþQamrt;Fmμta ehIybnßykarpþl;GuksIuEhSneTAciBa©wm

ekasika nig Calika. karrab;cMnYnpøaEktCaeTogTat; GnuBaØati[

evC¢bNÐitkMNt;)anfaRtUveRbIfñaMRbqaMgnwgkMNkpøaEkt RbsinebIpøaEkt

ekIn b¤ eRbIfñaMRbqMagnwgkMNkQam ¬Anti-coagulants¦ ebImanKMerag

RtUveFVIkarvHkat; b¤ ekItman Thrombosis .

[7] karpliteKalikaQamRkhmenAeRkAxYrq¥wg> mindUcGñkCMgW

taLasesmIukMritF¶n;EdlRtUvTTYlkarbBa©ÚlQamCaeTogTat;taMgBI

Gayuekμgmkemø:HedIm,IbM)at;skmμPaBrbs;xYrq¥wgmin[xøaMgkøa/ GñkCMgW

taLasesmIukMritmFümBMuTTYlkarbBa©ÚlQamCaeTogTat;dUcenHeT

GBa©wgvabnþpliteKalikaQamRkhmkñúgkMritx<s; rYmTaMgkarplitenA

kEnøgeRkAxYrq¥wg sMxan;enAtMbn;edImRTUg nig Ek,rq¥wgkgxñg. karft

edaykaMrsμI Guic ¬X-rays¦ GacbgðajeGayeXIjCalikabegáItRKab;

QamEdlekItCadMu²enAtamtMbn;TaMgenaH.

karplitRKab;QamenAEk,rq¥wgkgxñg Gacbgá[manplvi)akdl;RbsaT

EdlekItmaneLIgeBlNavasgát;BIxageRkA eTAelIxYrq¥wgkgxñg.

skmμPaBEbbenHGacdwg)antamry³ftkaMrsμI Guic b¤ eRbIviFIgay[

eXIjc,as;dUcCa MRI. CafμImþgeTot/ sßanPaBEbbdUcenH rEmgRKb;RKg

)antamry³karBüa)aledaybBa©ÚlQamEdlnwgbM)at;karbegáIt

RKab;QamenAeRkAxYrq¥wg ehIyCalT§pl bnßy)annUvdMubegáItRKab;Qam

TaMgenaH. krNIsßanPaBEpñkRbsaTmanlkçN³F¶n;F¶r eKRtUvkarviFan

karxøaMgCagenH dUcCa karBüa)aleday viTüúsaRsþ ¬Radiotherapy¦.

[8] plvi)akeTAelIeføIm nig ebHdUg> kgVHeKalikaQamRkhmraMuér:

k¾GacbgábBaðaeTAelIebHdUgEdr xN³eBlEdlebHdUg nig eføIm

128

GacTTYlrgplxUcxatBIkarelIsbnÞúkCatiEdk. sßanPaBTaMgBIrGac

RKb;RKg)an dUckñúgkrNItaLasesmIukMritF¶n;Edr.

taLasesmIubNþalmkBI {eGm:UkøÚb‘ÍnxusFmμta} b¤ eGm:UkøÚb‘Ín

Edlmanrcnasm<½n§køay

{eGm:UkøÚb‘ÍnxusFmμta (Abnormal hemoglobin)} manrcnasm<n§½ nig

lkçN³mindUc eGm:UkøÚb‘ÍnFmμta eLIy. eGm:UkøÚb‘ÍnxusFmμta sMxan;²man ³

� eGm:UkøÚb‘Ín S (HbS)

� eGm:UkøÚb‘Ín C (HbC)

� eGm:UkøÚb‘Ín E (HbE)

� eGm:UkøÚb‘Ín D (HbD)

� eGm:UkøÚb‘Ín Lepore

eGm:UkøÚb‘Ín TaMgGs;enHcMlgtamtMNBUC dUcCMgW Ebtta taLasesmIu Edr.

]TahrN_ GñkTaMgLayNaEdlTTYltMNBUC Bn§ú ¬gene¦ mYykMNt;)an

eGm:UkøÚb‘ÍnFmμta ehIyBn§úmYyeTotkMNt;)aneGm:UkøÚb‘ÍnxusFmμta S, C, E b¤

D GñkTaMgenaHRtUveKehAfa GñkpÞúk HbS, HbC, HbE b¤ HbD eTAtamenaH.

GñkpÞúkTaMgenaH eKGacdwg)anedayeFVIetsþmnÞIrBiesaFn_ dUcKñanwgGñkpÞúk

Ebtta taLasesmIu Edr.

lkçN³GUm:UhSIúháÚt rbs;eGm:UkøÚb‘ÍnxusFmμta

buKÁlTaMgLayGacTTYltMNBUC eGm:UkøÚb‘ÍnxusFmμta ¬S, C, E b¤ D¦ mYyBI

nrNamñak;én «Buk b¤ mþay ehIynig eGm:UkøÚb‘ÍnxusFmμta ¬S, C, E b¤ D¦

mYyepSgeTotBImñak;epSgeTotén «Buk b¤ mþay. b:uEnþmanEtGñkEdlTTYl

tMNBUC HbS BI«Bukpg nig BImþaypgeTEdlmansßanPaBKøInikKYr[kt;

sMKal; eKehAfa GUm:UhSIúháÚt ¬Homozygote¦ edaysar HbS b¤ ehAfa

CMgWkgVHeKalikaQamRkhm RbePT Sickle Cell. PaKeRcInénkrNI

129

GUm:UhSIúháÚt edaysar HbC, D b¤ E Kμanbgá[ekItCaCMgWeT ehIyKμansar³

sMxan;EpñkKøInikEdr.

PaBpÁúMKñaén {eGm:UkøÚb‘ÍnxusFmμta} CamYy Ebtta taLasesmIu

karpÁúMcUlKñaEdleKeXIjjwkjyCageKén Ebtta taLasesmIu CamYy

eGm:UkøÚb‘ÍnxusFmμta man ³

> HbS/ β – taLasesmIu

> HbE/ β – taLasesmIu

HbS/β – taLasesmIu - vabgáCasßanPaBKøInikmYymanlkçN³Rsedognwg

CMgW Sickle Cell CagCMgWtaLasesmIukMritF¶n; b¤ kMritmFüm.

HbE/β – taLasesmIu - enHCasßanPaBEdlmaneRcInCageKbMputrbs;

{eGm:UkøÚb‘ÍnxusFmμta} b¤ eGm:UkøÚb‘ÍnkøaypÁúMCamYyCMgWtaLasesmIu nig

manGRtaekIteRcInCageKenAGasIuGaeKñy_. sPaBpÁúMcUlKñaEbbenH k¾bgðaj

[eXIjpgEdrnUveraKsBaØaKøInik b¤ PaBF¶n;F¶r eRcInEbbeRcInya:g.

tameraKsBaØaKøInik/ eKEck β – taLasesmIu /HbE Ca 3 kMrit eyageTA

tamPaBF¶n;F¶réneraKsBaØa ³

> β – taLasesmIu /HbE kMritRsal - eXIjman 15° énGñkTTYlrg

karb:HBal;TaMgGs;enAtMbn;GasIuGeKñy_. RkumGñkCMgWEbbenHminekItman

bBaðaKøInikeT ehIyrkSakMrit Hb rvag 9 - 12 Rkam¼d>l (g/dl).

> β – taLasesmIu /HbE kMritmFüm - GñkCMgWmYycMnYnFMkñúgRkumenH

ekItmaneraKsBaØaKøInikRsedognwgGñkCMgW β – taLasesmIukMritmFümEdr

ehIymankMrit Hb efr enAcenøaH 6 - 7 Rkam¼d>l.

> β – taLasesmIu /HbE kMritF¶n; - GñkCMgWkñúgRkumenHmaneraKsBaØa

KøInikF¶n;F¶rdUcCMgWtaLasesmIukMritF¶n;Etmþg ehIymankMrit Hb cuHTabdl;

4 - 5 Rkam¼d>l. GñkCMgWTaMgenHRtUvBüa)aldUcGñkCMgWtaLasesmIu

kMritF¶n;Edr.

130

karbBa©ÚlQam ³ GñkCMgWRbePTenHRtUvtamdan[)anditdl; kñúgGMLúgeBl

bBa©ÚlQam edIm,IeCosvagplvi)akEpñkevC¢saRsþsMxan;² dUcCa eLIgsMBaF

Qam/ Rbkac; nig hUrQamenAxYrk,al. edIm,IRKb;RKgkareLIgsMBaFQam

EdlGacbNþal[søab;edaykarhUrQamenAxYrk,al eKGaceRbIfñaMTb;Tl;

kareLIgsMBaFQam. GñkCMgWEdlKμankgVHRKab;QamRkhmF¶n;F¶reT eKmin

RtUvbBa©ÚlQameLIy.

Gal;hVa taLasesmIu

Bn§ú rbs; Gal;hVakøÚb‘Ín (a-globin genes) rbs;mnusSmanBIrKUr manTItaMg

sßitenAelIRkUm:UsUmelx 16. karfycuHén ExSGal;hVa (a-chains)

EdlRtUv)ansMeyaK ekItmaneLIgkalNaPaKxøHrbs;ExS DNA EdlmanBn§ú

rbs;Gal;hVakøÚb‘Ín mYy b¤ TaMgBIr RtUv)anrlb;)at;.

- ebIrlb;Bn§ú cMnYnmYy/ GñkCMgWRtUveKcat;fa RbePT es¶oms¶at; b¤

CaGñkpÞúk aa/a-

- ebIrlb;Bn§ú cMnYnBIr/ eKcat;fa bgðjtMruy aa/-- b¤ a-/a-

- ebIrlb;Bn§ú cMnYnbI/ enaHeKcat;fa manCMgW Hb/H bNþalmkBImanExS

RbUetGIunEbtta (B-chains) eRcInelIslb;. CaCMgWmYysMKal;edaykarEbk

eKalikaQamRkhmkMritmFüm/ GeNþIkrIkFM ¬nig vibtþiEbkeKalika

QamRkhmRsYcRsav eBleRbIfñaMmYycMnYn b¤ cak;fñaM¦.

- ebIrlb;Bn§ú cMnYnbYn/ manCMgW Hydrops foetalis ¬b¤ Hb Barts¦ ekIteLIg

)anesckþIfa Tarksøab;kñúgépÞ.

131

CMBUkTI 8

rbbBüa)alEdl)aneFVI nig CMhannaBalGnaKt

karbnSaMxYrq¥wg

karbnSaMxYrq¥wg ebITTYleCaKC½y Gacpþl;karBüa)al

)anCadac; cMeBaHGñkCMgWtaLasesmIukMritF¶n;.

karbnSaMxYrq¥wg sMrab;CMgWtaLasesmIu RtUv)aneKcab;

epþImeFVItaMgBIqñaM 1981 rhUtmkdl;eBlenH manCag

1500 krNI.

GVIeTACakarbnSaMxYrq¥wg ?

xYrq¥wgKWCaCalikaes<at sßitenAkñúgq¥wg ¬emIlrUb 8a¦

EdlplitekasikaTaMgGs;EdlmankñúgQam. enAGayu

v½yCMTg; xYrq¥wgmankñúgq¥wgCMnI/ q¥wgsnÞHRTUg/ q¥wgk,al/

q¥wgRtKak nig q¥wgkgxñg b:uEnþenAeBleTIbekItBIépÞmþay

eKeXIjxYrq¥wgmanenAtamq¥wgepSg²eTotEdr. xYrq¥wgmanpÞúkekasikaedIm

¬emIlrUb 8b¦ EdlecjBIenaH ekItecjCaeKalikaQamRkhm/ eKalika

Qams nig pøaEkt.

karbnSaMxYrq¥wg KWCakarykxYrq¥wgBIbuKÁlEdlmansuxPaBl¥

¬ehAfa Gñkbric©ak¦ edIm,Ipþl;eTA[GñkCMgW ¬ehAfa GñkTTYl

bnSaM¦. xYrq¥wgKμansuxPaBl¥rbs;GñkCMgWTTYlkarbMpøicbMpøaj

dMbUgedayfñaM b¤ CYnkaledaykaMrsμIviTüúskmμ (Irradiation) kñúg

dMeNIrmYyEdleKehAfa sPaBeCosminput xYrq¥wgEdll¥

rEmgykecjBIq¥wgRtKakrbs;Gñkbric©ak bnÞab;mkpþl;va[eTAGñkCMgWkñúg

sPaBravedaybBa©ÚlkñúgcrnþQam RsedogKñanwgkarbBa©ÚlQameTogTat;

8a

8b

132

dUecñaHEdr ¬emIlrUb 8c¦. eBlcUldl;kñúgcrnþQamrbs;GñkTTYl/ ekasika

xYrq¥wgGñkbric©ak eFVIdMeNIreTAkan;q¥wgFM² EdlenATIenaHvacab;epþImplit

ekasikaQamFmμtamansuxPaBl¥. dMeNIrenHRtUveRbIry³eBl 2 - 3

s)aþh_.

etImaneRKaHfñak;GVIxøH ?

eTaHCadMeNIrenHGacTTYleCaKC½yeRcInk¾eday vak¾maneRKaHfñak;F¶n;F¶rEdr.

dUecñHmunnwgGnuvtþbnSaM eKRtUvBicarNaedayykcitþTukdak;GMBIeRKaHfñak; nig

plRbeyaCn_cMeBaHkrNInImYy². edIm,ITTYl)anlT§pll¥ xYrq¥wgRtUvEtpþl;

edaybgb¥ÚnRbus b¤ RsI EdlmanCalikasIuKñaeBjelj.

TMraMEtxYrq¥wgEdlbNþúHGacCMnYsxYrq¥wg rbs;GñkTTYlbnSaM)anenaH varEmgekIt

manplvi)ak dUcCakarbgáeraK nig karhUrQam. plvi)akelIsBIenHeTA

eTotenaH KWCMgWTMnas;bnSaM ¬Graft versus host disease = GVHD¦ kalNa

xYrq¥wgEdlbBa©ÚleTAyl;fasarBagÁkayGñkCMgWKWCavtßúeRkAcMElk edaybegáIt

CabBaðacMeBaHEs,k eBaHevon nig eføIm kñúgsPaBRsal/ F¶n;F¶r b¤ rhUtdl;

søab;k¾man.

bNþaGñkCMnaj)anraykarN_fa eRkayBI)aneFVIbnSaMxYrq¥wg/ bBaðaTaMgLay

Tak;TgnwgCMgWtaLasesmIu dUcCa karelIsbnÞúkCatiEdk/ rlakeføImraMuér:/

bBaðaebHdUg nig RkeBjbeBa©jkñúg mansPaBgayRsYlRKb;RKgeRcInCagmun

rIÉsrIragÁEdlTTYlrgkarxUcxat CYnkalGacCasHes,Iyvij. eTaHCaya:g

Nak¾eday eKRtUvbnþtamdanGñkCMgW edayykcitþTukdak;eBjmYyCIvit.

eRkayBI)aneFVIbnSaMxYrq¥wg FatuEdkEdlelIskkkujkñúgsarBagÁkayGñkCMgW

muneBleFVIbnSaMRtUvEtkMcat;ecj. eKGaceFVI)anedayeRbIfñaMkMcat;CatiEdk b¤

edayRKan;EtbgðÚrQamecjerogral;BI 1 - 2s)þah_. Cakarl¥bMput eKGac

bUmQamykecjBIsrésEv:n 6 m>l kñúgmYyKILÚRkamTMgn;xøÜn erogral;

RbEhl 14éf¶ .

133

sMxan;RtUvyl;fa karbnSaMxYrq¥wg RKan;EtBüa)alxYrq¥wgb:ueNÑaH. dUecñHGñkCMgW

EdlmanKMeragmanépÞeBaHRtUvcgcaMfa Bn§ú rbs;eKenAEtmanCMgWtaLasesmIu

dEdl Edlnwgpþl;bnþeTAkUnrbs;eK)an.

etIeKGacbnßyeRKaHfñak;[enAticbMput)andUcemþc ?

k> CMerIsGñkbric©ak> meFüa)aysMxan;bMputedIm,IrkSaeRKaHfñak;[enAtic

KWRtUv[manPaBsIuKñaBitR)akd ¬exact match¦ rvagCalikaGñkCMgWCamYy

CalikaGñkbric©ak.

Gñkbric©akl¥bMput EdlBitCaman HLA sIuKñaCamYyGñkRtUveFVIbnSaMxYrq¥wg

KWbgb¥ÚnePøaH. PaBsIuKñaEk,rbMputTI2 KWbgb¥ÚnbegáItEdllkçN³ HLA rbs;

eKmanPaBsIuKñaya:geRcInnwg HLA rbs;bgb¥ÚnRbus b¤ RsIrbs;GñkEdlRtUveFVI

bnSaMxYrq¥wg. »kasGacrk)annUvPaBsIuKñaBIbgb¥ÚnRbus b¤ RsI KW 4 man 1

¬25°¦ vaBi)akNas;nwgrkGñkmanPaBsIuKñaenHenAeRkArgVg;RKYsar. Bn§úEdl

kMNt;RbePTCalikaeKehAfa Human Lymphocyte Antigen (HLA) genes

EdlR)ab;[eyIgdwgBIbNþaRbePTCalika EdlEsþgeLIgenAelIeKalikaQam

s TaMgLay. kñúgcMeNam Antigen TaMgenH/ HLA-A, HLA-B, HLA-C nig

HLA-DR edIrtYnaTIsMxan;CageKkñúgkarpÁÚrpÁg[manPaBsIuKñasMrab; bnSaMxYr

q¥wg. man Antigens cMnYn 59 enATItaMg HLA-A, cMnYn 118 enATItaMg

HLA-B, cMnYn 36 enATItaMg HLA-C nig cMnYn 137 enATItaMg HLA-DR.

RbB½n§enHTaMgmUlman HLA-loci CaeRcIneTot RBmTaMgmanBn§úCag 500 eday

begáIt[manlT§PaBpÁúMcUlKña)aneRcInekad kñúgcMeNammnusSkMNt;mYy.

ya:gNak¾eday edayBn§úTaMgenHmanTMnak;TMngnwgKña nig bnþeTAkUnecAkñúg

lkçN³CasMNuMenaH/ bgb¥ÚnRbus nig RsI RtUvTTYlCalkçN³tMNBUC nUvsMNMu

NamYyénsMNMuTaMgBIr BI«Buk nig mþayTaMgsgxag eFVI[»kasénsMNMuman

lkçN³dUcKñadl;eTA 25°. »kasCaTUeTA/ ya:gehacNas;manbgb¥Ún

RbusmYy b¤ RsImYyEdlmanxYrq¥wgsIuKña b:uEnþKμanCMgWtaLasesmIukMritF¶n;/

134

GaRs½yeTAelITMhMRKYsarmanbgb¥Úntic b¤ eRcIn. CamFümmanGñkCMgWRbmaN

30° b:ueNÑaHEdlEsVgrkkareFVIbnSaMxYrq¥wg nwgrk)annUvGñkbric©akxYrq¥wg

EdlmanlkçN³sIuKñaeBjelj.

dUckarKitRsém:/ karlM)akkñúgkarpÁÚrpÁg[manPaBsIuKña énxYrq¥wg)anBIGñk

bric©akEdlKμanCab;sac;Qam ¬eRkArgVg;RKYsar¦ kan;EtmanPaBFMeFgEfm

eTot. karKNnaCaRTwsþIbgðajfa PaBxusKñaénTMrg; HLA BIbuKÁlmYyeTA

buKÁlmYyepSgeTot EdlKμanCab;sac;Qam manemKuNxusKñadl;eTA

mYyBan;lan. CakarsMxan;/ RtUvFana[manPaBsIuKñaebHbiTénxYrq¥wgGñkeFVI

bric©ak nig GñkRtUvTTYlkarbnSaMxYrq¥wg edayehtufa»kasTTYleCaKC½y

manticNas; kalNaPaBsIuKñaenHmankMrittic. dUcenH karEsVgrkPaBsIuKña

BIGñkbric©akeRkArgVg;RKYsar eFVIeLIgEteBlNaeKBMuGacrk)annUvPaBsIuKña

kñúgrgVg;RKYsar nig smaCikRKYsarCab;sac;QamCamunsin. elIsBIenHeTot

Gñkbric©akxYrq¥wgRtUvmanGayuya:gticcab;BI 3qñaMeLIgeTA edIm,IeGaymuxgar

xYrq¥wgrbs;eKmanPaBcas;TMuRKb;RKan;.

kic©RbwgERbgkñúgbNþaqñaMcugeRkayenH)anRbmUlpþúMeTAelIkareFVI[RbesIrdl;

bec©keTspÁÚrpÁg[manPaBsIuKña edIm,IGacpSaMxYrq¥wgrvagGñkeFVIbric©akminCab;

sac;QamehIymanPaBsIuKña (Matched Unrelated Donors = MUDs)

CamYyGñkRtUveFVIbnSaMxYrq¥wg/ tamry³enH vapþl;lT§PaBkan;EteRcInkñúgkarrk

Gñkbric©ak. eK)anbegáItnUvbBa¢Irkt;RtalkçN³rbs; HLA énGñkeFVIbric©ak

CMuvijBiPBelakEdlsμR½Kcitþ[eKeFVIetsþ

edaymanrayeQμaH nig Gas½ydæanrbs;

BYkeK. enAeBleKeXIjfa GñkRtUveFVI

bnSaMxYrq¥wgman HLA sIuKñaGacTTYlyk

)an/ PøamenaH eKCUndMNwgeTAGñkbric©ak

ehIyGnuvtþkarbnSaMxYrq¥wgPøamEtmþg.

KMnitbegáIt[manbBa¢IrGñkeFVIbric©akxYr

q¥wgEdlminCab;sac;Qam ekIteLIg

8c

135

dMbUgenAqñaM 1987 ehIybc©úb,nñenHmanGñkbric©aksuxPaBl¥cuHeQaμH

RbmaNCa 3/5lannak; CaBiessenAshrdæGaemrik nig GWr:ub. eTaHbIya:g

Nak¾eday eTaHCa)anGnuvtþkargarmYycMnYnTak;TgnwgGñkbric©akminCab;sac;

Qamk¾eday eKenARtUvkarRsavRCavCaeRcIneTot edIm,Ikat;bnßyeRKaHfñak;

Tak;TgnwgkarbnSaMxYrq¥wgEdlmankMritx<s;CagkarykxYrq¥wgBIGñkbric©akkñúg

cMeNambgb¥ÚnbegáIt EdlmanPaBsIuKña .

x> CMerIsGñkRtUveFVIbnSaMxYrq¥wg ¬GñkCMgW¦> GñkCMgWmYycMnYnCYbeRKaHfñak;

BIkarbnSaMxYrq¥wgkñúgkMritx<s;CagGñkCMgWepSgeTot. eK)ansÁal;ktþabgá

eRKaHfñak;mYycMnYn ³

� eføImrIkFM - FMCag 2 s>m

� eføImxUcxat - sPaBcak;es£Hedaysrésevov ¬Fibrosis¦ b¤ eføImman

søaksñamdMe)A

� karRKb;RKgsPaBelIsbnÞúkCatiEdkmin)anl¥

QrelIktþaTaMgenH eKeFVIcMNat;fñak;GñkCMgWCa bI RbePT ehIyRbePTnImYy²

manGRtaTTYleCaKC½yxus²Kña. cMNat;fñak; I manktþabgáeRKaHfñak;EtmYy/

ÉcMNat;fñak; III manktþabgáeRKaHfñak;TaMgbIEtmþg.

ktþabgáeRKaHfñak;mYyeTotdac;EtÉg KWGayuGñkCMgW/ lT§plénkarbnSaMxYrq¥wg

elIGñkCMgWGayuelIsBI 16qñaMeLIgeTA mansPaBya:b;yWunCagGñkCMgWv½yekμg

¬sUmemIltaragxageRkam¦ ³

kumar ¬16qñaM¦ cMnYn GRtars; KμanRBitiþkarN_manCIvit ° GVIekIteLIg °

cMNat;fñak; I 121 95 90

cMNat;fñak; II 272 85 81

cMNat;fñak; III 125 78 59

eBjv½y ¬17-22qñaM¦ 70 70 66

[Pesaro Experience 1995]

136

Cakarl¥ GñkCMgWbnSaMxYrq¥wg KYrmanGayueRkam 16qñaM rYmCamYyeføImman

suxPaBFmμta manelIsbnÞúkCatiEdktictYc ehIymanbgb¥ÚnRbus b¤ RsI

Edlman HLA sIuKñaBitR)akd. dUc)anbBa¢ak;kñúgtaragxagelI GRtars;ran

manCIvitmankMritTab cMeBaHGñkCMgWEdleFVIbnSaMxYrq¥wgenAGayuelIsBI 16qñaM.

CMhanepSg²eTot cMeBaHkarbnSaMxYrq¥wgEdlmanPaBsIuKñaBit

R)akd ¬Cab;sac;Qam¦

bNþaevC¢bNÐitkMBugRbtibtiþkaredIm,IeFVI[GRtaeCaKC½y)anRbesIr nig kat;

bnßyplvi)akbNþalmkBIkarbnSaMxYrq¥wg)anBIGñkbric©akmanPaBsIuKñaEt

minCab;sac;Qam ¬MUDs¦/ BIsmaCikkñúgRKYsarmanPaBsIuKña EdlminEmn

Cabgb¥ÚnbegáIt nig BI«BukmþayEdlminmanPaBsIuKña. TidæPaBmYy KWRtUvpøas;

bþÚrkarBüa)al ¬sPaBeCosminput¦ edIm,Ikat;bnßyeRKaHfñak;ekItecjBI

nitiviFIénkarbnSaM. eTaHya:gNa lT§plTTYl)anmanPaBERbRbYl ehIy

eRKaHfñak; nig plcMeNjRtUvEtBicarNaedayQrelImUldæankrNInImYy²

¬sUmemIltaragxageRkam¦.

lT§pl énkarbnSaMxYrq¥wg )anBIGñkbric©akepSg²kñúgCMgWtaLasesmIu

� GñkCMgWtaLasesmIukMritF¶n; cMnYn 29nak; Gayu 1/1 - 33qñaM ¬GayumFüm 6qñaM¦

- 6nak; mansac;jatiman HLA dUcKñaebHbiT

- 2nak; mansac;jatiKμanPaBsIuKña

- 13nak; manbgb¥ÚnbegáItKμanPaBsIuKña

- 8nak; man«BukmþayKμanPaBsIuKña

� minTTYl ¬Rejection¦¼braC½y 55° ¬minTak;TgnwgKμanPaBsIuKña¦

� TMnas;bnSaMRsYcRsav ¬Acute GVHD¦ I-IV 47°

� TMnas;bnSaMraMuér: ¬Chronic GVHD¦ 38°

� GRtaenArs;manCIvit ¬FU 7/5qñaM¦ 65° ¬21° KμanRBitiþkarN_GVIekIteLIg¦

� søab;edaykarbnSaM 34° ¬50° TMnas;bnSaM/ 30° karbgáeraK¦

[Gaziev et al, Pesaro BMT 2000]

137

esckþIsnñidæan

karbnSaMxYrq¥wg edayykBIbgb¥ÚnbegáItEdlmanPaBsIuKñaeBjelj pþl;»kas

GacCHsHes,Iyya:gx<s; ehIyeRKaHfñak;bNþal[søab;edaysarnitiviFIén

karbnSaMmankMritTabEt 5° cMeBaHGñkCMgWEdlRtUv)aneRCIserIsl¥. kargar

kñúgTisedAbnßyeRKaHfñak;BIkarbnSaMenAEtbnþeTAmuxeTot rYmmankareFVI[

RbesIreLIgnUvlT§plbnSaMxYrq¥wg)anmkBIGñkbric©ak EdlminEmnCabgb¥Ún

begáItEtmanPaBsIuKñaeBjelj.

bnSaMxYrq¥wgedayeRbIQamTgsuk

bec©keTseFVIbnSaMxYrq¥wgmYyepSgeTot KW eFVIetsþBinitülkçN³ HLA rbs;

TarkEdlsßitkñúgépÞmþayrbs;kumarmanCMgW. ebITarkkñúgépÞman HLA sIuKña

CamYynwgkumarenaH/ eKGaceFVIbnSaMedayeRbIQamTgsukCMnYsxYrq¥wgeTAkñúg

xøÜnkumarEdlmanCMgW. QamTgsuk KWCaQamEdlsßitkñúgsuk ¬Placenta¦

nig Tgsuk ¬umbilical cord¦ eRkayBITarkekItrYc vaCaRbPBsMbUreday

ekasikaedIm ¬Stem cells¦ EdlTTYlxusRtUvdl;karpliteKalikaQam.

eTaHya:gNak¾eday EdnkMNt;sMxan;cMeBaHbec©keTsenH KwfaQamTgsuk

rbs;Gñkbric©akRtUvmanpÞúkekasikamansñÚl ¬Nucleated Cells¦ kñúgcMnYn

Gb,brimamYy ¬>1x107¦ kñúgmYyKILÚTMgn;énGñkRtUveFVIbnSaMxYrq¥wg. elIsBI

enHeTot/ eføIm/ tMrgenam/ ebHdUg nig sYt rbs;GñkCMgWRtUvmandMeNIrkar

l¥pgEdr.

karTTYleCaKC½ydMbUgbMput cMeBaHkarbnSaMedayeRbIQamTgsuk RtUv)aneK

GnuvtþtaMgBICag 10qñaMmun. bnÞab;BIenaHmk RbtibtiþkarEbbenHcMnYn 500

RtUv)aneFVITUTaMgBiPBelak. bec©keTsenHk¾)anTTYleCaKC½ypgEdrkñúgkar

Büa)alGñkCMgWtaLasesmIukMritF¶n;mYycMnYn. eTaHya:gNak¾eday eKenARtUv

karRsavRCavbnþeTot edIm,IeFVI[GRtaTTYleCaKC½ykan;EtRbesIreLIg .

138

bnSaMedayQamTgsuksMrab;CMgWtaLasesmIu

cMnYnkumar

bnSaM 10

enArs;manCIvit 10

CasHes,Iy 4

taLasesmIuminCasHes,Iy 4

[Rocha et al. 1998, Eurocord]

KuNRbeyaCn_EdlGacekItmanBIkareFVIbnSaMEbbdUcenHman ³

� QamTgsukminsUvmanGtisuxumR)aNbgáeraK

� eRKaHfñak;CMgWTMnas;bnSaM ¬GVHD¦ mankMritTab eRBaH T-lymphocytes

EdlbgáTMnas;bnSaMminTan;GacdMeNIrkar)aneBjelj enAeBlTark

eTIbnwgekIt ¬enHeKRtUvEtbBa¢ak;cMeBaHGñkCMgWtaLasesmIu¦

� PaBsIuKñaén HLA minsUvTamTarxøaMgdUckrNIbnSaMxYrq¥wgFmμta epSgeTot

enaHeT

mkTl;eBlenH/ minTan;manlT§plRKb;RKan;edIm,Ibgðajfa etIKuNRbeyaCn_

TaMgenHGacEsþgecjCaplsMercya:gRbesIr b¤ ya:gNaenaHeT.

bc©úb,nñenH/ kargarkMBugdMeNIrkaredIm,IbegáItRbB½n§mCÄmNÐlsþúkQam

Tgsuk dUcKñanwgRbB½n§mCÄmNÐlpþl;Qamedaysμ½RKcitþnaeBlbc©úb,nñenH

Edr. kñúgkrNIGnuvtþkarEfTaM)anRtwmRtUv/ QamTgsukGacrkSaTukenA

sItuNðPaB -19oC )anry³eBl 20qñaM. mCÄmNÐlQamTgsukd¾FMbMput KW

mCÄmNÐlpþl;QamTIRkug jÚy:k ¬New York Blood Center¦ EdlRbmUl

)anKMrUQamCag 7000 fg; ehIy)anCYydl;krNIbnSaMCag 200 krNI.

bc©úb,nñenH/ edIm,ITTYl)an nig eFVIbnSaMQamTgsukBImCÄmNÐlpþl;Qam

TIRkug jÚy:k eKRtUvcMNay 28 000 duløashrdæGaemrik.

RbeTsGaLWm:g;/ )araMg/ cRkPBGg;eKøs/ GIutalI/ EblhSik/ hULg; nig

RbeTseGs,aj k¾)anbegáItmCÄmNÐlpþl;QamTgsukpgEdr.

139

CMBUkTI 9

CMhanepSg²eToteq<aHeTArkkarBüa)al

»sfCMrujbegáIt eGm:UkøÚb‘ÍnTarkkñúgépÞ (HbF inducers)

bNþaGñkviTüasaRsþ kMBugEteFVIkarRsavRCavya:gCab;lab; rkmeFüa)ay

epSg²eTot edIm,IBüa)alCMgWtaLasesmIu edayrkmeFüa)ayBüa)alEdl

mineFVITukçxøaMg maneRKaHfñak;tic nig Gs;R)ak;kasticCagviFIBüa)albc©úb,nñ.

CMhanfμIPaKeRcIn)ancab;GarmμN_eTAelIkarkat;bnßyGtulüPaB rvagExS

køÚb‘Ín. Ca]TahrN_ fñaMmYycMnYnRtUv)aneKsÁal;faGacbegáInkarplitExS

RbUetGIunepSgeTot dUcCaExSRbUetGIunháama: ¬γ-chains¦ EdleRkayBI)an

cab;KUrCamYyExSGal;hVa begáIt)anCa eGm:UkøÚb‘ÍnTarkkñúgépÞ HbF (a2y2)

dUecñHva)anbnßyExSGal;hVaesrI. fñaMTaMgenHeTAbnßyskmμPaBxYrqw¥wg ehIy

eP£aceGayplit ExSháama:. fñaMTaMgenHrYmman 5-azacytidine, Cytosine

arabinoside, Hydroxyurea, Erythropoietin, nig ExSRsLay Butyric

acid. TaMg 5-azacytidine nig cytosine arabinoside )anbgðajnUv\T§iBl

BulcMeBaHGñkCMgWtaLasesmIu ehIytaMgBIenaHmkeK)anbBaÄb;kareRbIR)as;

fñaMTaMgenH ebIeTaHbIExSRsLayrbs;vafμI ehIyminsUvBulkMBugRtUv)anRsavRCav

k¾eday. eKsegáteXIjeCaKC½ymYycMnYnCamYy Hydroxyurea nig

Erythropoietin eTaHbIrhUtmkTl;eBlenH KuNRbeyaCn_KwmancMeBaHGñkCMgW

taLasesmIukMritmFüm eRcInCagGñkCMgWtaLasesmIukMritF¶n;.

eKenAminTan;yl;c,as;enAeLIynUvtYnaTIrbs;ExSRsLay Butyric acid

kñúgkareP£ac[mankarsMeyaK ExSháama:. eTaHya:gNak¾edayBYk butyrates

d¾manRbsiT§iPaBbMput KW Arginine butyrate EdleKeRbIedaycak;bBa©Últam

srésQam. GñkCMgWmYycMnYnmankareqøIytb)anl¥ cMeBaHkarBüa)alEbb

enH Etehtuplrbs;vaeKminTan;yl;c,as;las;enAeLIyeT. karvivtþn_naeBl

GnaKtGacBwgEp¥kelIkareRbIR)as;bBa©ÚlKñanUv Arginine butyrate nig

140

Hydroxyurea b¤ Arginine butyrate nig Erythropoietin b¤eRbIfñaMTaMgbIcUlKña.

kñúgkrNICMgWtaLasesmIukMritF¶n;/ eKalbMNgénkareRbIfñaMTaMgenH KWedIm,IBnüa

cenøaHeBlbBa©ÚlQam ÉcMeBaHCMgWtaLasesmIukMritmFümvijKWbeBa¢ósb¤Bnüa

tMrUvkarbBa©ÚlQam ehIykat;bnßykarQWcukcab;q¥wgbNþalmkBIkarrIkénxYrq¥wg.

Büa)aledayBn§ú (Gene therapy)

karBüa)alcugeRkaycMeBaH CMgWtaLasesmIukMritF¶n; TamTarkarbBa¢ÚnBn§ú

rbs;GñkmansuxPaBl¥cUlkñúgekasikaedIm ¬Stem cells¦ énxYrq¥wg KWCaCMhan

mYyEdleKehAfa karBüa)aledayBn§ú. 10qñaMcugeRkayenHmankarvivtþn_

ya:gFMeFgkñúgkarbegáItRbB½n§CIvsaRsþ EdlmansmtßPaBGacnaMykBn§úGñk

mansuxPaBl¥cUleTAkñúgekasikaedImrbs;GñkCMgW. kñúgbNþaqñaMcugeRkay/ eK

)aneRbIGtisuxumR)aNCavIrus ¬Retrovirus¦ TTYl)anlT§plya:gRbesIr.

ya:gNak¾eday/ bBaðaecaTCamYyviFIEbbenH KWRtUvFana[)anfa ExSEbtta

RtUvekItecjBIBn§úmansuxPaBl¥EdleKbBa©ÚleTAenaHmanKuNPaB nig brimaN

x<s;RKb;RKan;kñúgry³eBlmYyEvg. elIsBIenH/ enAmankarRBYy)armÖN_BI

suvtßiPaBénkarbBa©ÚlvIrus nig karb:HBal;Cayfaehtudl;Bn§úepSg²eTot.

eraKvinicä½yrkPaBsIuKñaénHLA munkarbgákMeNIt

kñúgbNþalqñaMcugeRkayenH karRsavRCav)anepþatelIbec©keTsmYyeKehAfa

eraKvinicä½yBn§úmunkarbgákMeNIt b¤ PGD ¬Pre-implantation Genetic

Diagnosis¦ kñúgbec©keTsenH eKyk HLA rbs;kumarmanCMgWmkpÁÚrpÁgeGay

sIuKñaCamYy HLA rbs;emCIvitRsþIEdlKμanpÞúkCMgWtaLasesmIu bnÞab;mkeKyk

mkbgákMeNItkñúgs,ÚnRsþI. kumarEdlnwgcab;kMeNIt nwgmanlT§PaBpþl;[

bgbegáItrbs;va nUvPaBdUcKñaebHbiTénxYrq¥wgsMrab;eFVIkarbnSaM. viFIEbbenH/

va)anecaTCabBaðadl;EpñksIlFm’CaxøaMg Etva)anpþl;nUvkþIsgÇwmfμIsMrab;kar

Büa)al. elIsBIenHeTot/ viFITaMgenHRtUvkarcMNayya:geRcIn nig TamTarnUv

bec©keTsx<s;. eTaHya:gNak¾eday/ karvivtþn_eTAmuxya:gelOneFVIeGay

bec©keTsenHGaceRbIR)as;)anya:gTUlMTUlaykñúgeBlGnaKtxagmuxya:gxøI.

141

CMBUkTI 10

bBaðaecaTEpñkcitþsgÁm

cMeBaHGñkCMgW

minxusBICMgWraMuér:epSg²eToteLIy GñkCMgWtaLasesmIukMritF¶n;RtUvRbQmmux

nwgkarlM)akF¶n;F¶rmYycMnYn. tMrUvkarragÁkay k¾dUcCakgVHkaryl;dwgCa

saFarN³tambNþaRbeTsCaeRcInEdlmanekItCMgWenH )aneFVI[GñkCMgW

«Bukmþay nig RKYsarrbs;eK CYbkarlM)akpøÚvcitþkñúgkarxitxMedaHRsaybBaða

CMgWtaLasesmIuenH. kñúgeBlNamYykñúgCIvit/ GñkCMgWGacnwgCYbbBaðapøÚvcitþ

dUcCa³ karGs;Twkcitþ/ karxkbMNg/ PaBesakesA/ xwgcMeBaHxøÜnÉg/ karFøak;

Twkcitþ/ sPaBfb;GarmμN_/ karP½yxøacesckþIsøab;/ kar)at;bg;PaBeCOCak;/

PaBÉkeka/ mYrem:A/ Gs;sgÇwm/ manGarmμN_xVHesckþIRsLaj;BImnusSdéT/

karminTukcitþ/ manGarmμN_famanPaBxaXaMgcMeBaHxøÜnRCul/ minsUv[tMél

cMeBaHxøÜnÉg nig GarmμN_epSg²eTot.

mü:ageTot/ GñkCMgWmanGarmμN_rMCYlcitþCaviC¢manepSg²eTotEdr dUcCa

PaBkøahan nig karBuHBarGMNt; EdlCYyBYkeKeGayqab;manKMnitcas;TMu

ehIykøayCaGñkkøahanmanKMnitéqñRbDit nig eCOCak;.

cMeBaH«BukmþayGñkCMgW

eRkABIrMCYlcitþGviC¢man nig viC¢man dUcGñkCMgWEdr/ «BukmþayenAmanTTYl

GarmμN_faxøÜnCaGñkTTYlxusRtUv b¤ mankMhuscMeBaHsßanPaBsuxPaBrbs;

kUnxøÜnÉgeTot. «BukmþayPaKeRcInrn§t;citþCaxøaMg eBleKdwgfakUnrbs;eK

maneraKvinicä½yCaCMgWenH. enARbeTsEdlKμankaryl;dwg b¤ mankaryl;dwg

bnþicbnþÜcGMBICMgWenH nig karBüa)alrbs;va/ matabitaminGacpþl;karEfrTaM

edayRtwmRtUvenAÉpÞHeLIy EdlvaCacMENkd¾sMxan;cMeBaHeCaKvasna

142

¬buernimitþ¦ kñúgry³eBlEvgrbs;kumar. eraKvinicä½yCMgWtaLasesmIu Gacbgá

pgEdrdl;PaBtantwgF¶n;F¶rcMeBaHTMnak;TMngbþIRbBn§ CYnnaMeTAdl;karEbk)ak;

b¤ ElglHKña. müa:gvijeTot/ CMgWk¾GaceFVIeGaybþIRbBn§rbs;«Bukmþaykan;

EtCitsñiT§EfmeTotk¾man edaytaMgcitþfanwgkarBarKaMRTkUnrbs;BYkeK.

dUcKñaenHEdr matabitaGacykcitþTukdak;EfrkSaeRcIncMeBaHkUnmanCMgW

taLasesmIu bgáPaBtantwgdl;TMnak;TMngCamYysmaCikepSg²eTotkñúgRKYsar

CaBiesskUn²epSgeTot .

cMeBaHGñkCMgW nig «Bukmþay

GarmμN_GñkCMgW k¾dUcCa«Bukmþay EdlmancMeBaHkarBüa)alRbkbedaykar

QWcab;kñúgry³eBlyUr edaykarbBa©ÚlQam nig kMcat;CatiEdk rEmgGacnaM

[ekItmanbBaðapøÚvcitþ. vaekItecjBIPaB)at;muxmat;kñúgsgÁmedaysarCMgW

taLasesmIu nig CMgWBn§úbnþBUCepSgeTot/ «BukmþayPaKeRcInmincg;elIkyk

eraKvinicä½ymkBiPakSaCamYyGñkdéTeLIy edayxitxMlak;)aMg sUm,IEt

smaCikkñúgRKYsarCitditk¾min[dwgEdr. sUm,IEtbc©úb,nñenH enAbNþaRbeTs

EdlkMrit nig KuNPaByl;dwg rbs;saFarN³CnmankMritx<s; RBmTaMgman

kareCOnelOneTAmuxEpñkKøInikcMeBaHGayukal nig KuNPaBCIvitrbs;GñkCMgW

taLasesmIu/ k¾GñkCMgW nig «Bukmþay CYnkalRkumRKYsarTaMgmUl enAEtman

TTYlGarmμN_EbbxagelI rhUtTal;EteK)anyl;dwg TTYlykkarrs;enA

CamYytaLasesmIu ehIyyl;dwgBIviFITb;Tl;CamYyCMgW. dUcenH«Bukmþay

nig GñkCMgW nwgTTYlrgTukçRBYykñúgry³eBlmYy rhUtdl;bBaðapøÚvcitþGviC¢man

d¾xøaMgenH)anFUres,Iyvij ehIyCMgW nig karEfTaMCMgWsMrbsMrYlkñúgCIvPaBRbcaM

éf¶eK)an. karKaMRTBIRKUeBTü kñúgGMLúgeBlenHGacRtUvkarcaM)ac;CaTIbMput.

eKeXIjPaBesakesAénerOgEbbenH enAbNþaRbeTsEdlTTYlrgCMgW

taLasesmIu ehIyFn;FanmankMrit KwfaGñkCMgW nig «BukmþaysikSaEsVgyl;

GMBICMgW nig karBüa)al b:uEnþEbrCaman»kastictYckñúgkarbBa¢ak;GMBIbBaða

rbs;GñkCMgW. sPaBEbbenHnaM[ekItCaPaBmYhμg Gs;sgÇwm KμankMlaMg

143

kMEhg Føak;Twkcitþ nig TTYlPaBÉkekakñúgsgÁm. GñkCMgWkñúgRbeTsTaMgenH

søab;TaMgekμgv½y CYnedaymin)aneFVIeraKvinicä½y b¤ eFVIeraKvinicä½yxusehIy

Büa)alminRtwmRtUv b¤ min)anBüa)alesaHEtmþg.

TTYlBt’man nig karKaMRT

sMxan;matabitamankUnmanCMgW RtUvEsVgrkRbPBBt’man[)aneRcIn tamEdl

GaceFVIeTA)anGMBICMgWtaLasesmIu tambNÑaKar tamGIunFWENt nig BI«Buk

mþayepSgeTotEdlmankUnekItCMgWtaLasesmIu. smaKmn_CatiCMgW

taLasesmIu nig shB½n§GnþrCatiCMgWtaLasesmIu edIrtYnaTICaKnøwH kñúgkar

pþl;dl;matabitanUvRbPBBt’mansMxan; nig karKaMRT edayCYyBRgwgnUvCMenO

rbs;BYkKat;kñúgkarCYykUnecA[manCIvitrs;enA)anl¥ ebIeTaHbICamanCMgW.

«BukmþayEdlTTYlBt’man)anl¥ GMBICMgWtaLasesmIu GMBIkarBüa)al nig

karkarBar/ Kat;manlT§PaBya:gl¥ edIm,IKaMRTdl;kUnrbs;Kat; ehIyebIsinCa

Kat;manbMNg Kat;GackarBarmin[mankUnTI2manCMgWenH. evC¢bNÐit KWCa

RbPBBt’manbEnßmsMxan;mYy EdlCYypþl;karENnaM nig kareCOCak;. tYnaTI

rbs;Kilanubdæak-yika k¾sMxan;Edr. kñúgkrNImYycMnYn TMnak;TMng

Kilanubdæak-yika nig GñkCMgWxusKñaBITMnak;TMngrvagevC¢bNÐit nig GñkCMgW.

edaysarmaneBlevlaeRcIn nig eTogTat;CamYyGñkCMgW Kilanubdæak-yika

rEmgEtgEtCaGñkdMbUgEdlTTYl)anBt’mansþIBIbBaðaBiess² EdlGñkCMgWGac

CYbRbTH. elIsBIenHeTot/ edayTIkEnøgbBa©ÚlQammanPaBmindUckEnøg

déT/ Kilanubdæak-yikaRtUvEtmanTMnak;TMngl¥CamYyRkumRKYsar nig mitþPkþi

GñkCMgWEdlBYkKat;RtUvkarcg;dwgBIkarlM)akénkarBüa)al EdlGñkCMgW

GacCYbRbTH. CYnkalGñkTaMgenH nwgsμ½RKcitþCYypþl;Bt’manrYcCaeRsc

munGñkCMgWeTAeTot. eTaHbIya:gNak¾eday/ sMxan;KWfaBt’manTaMgenH RtUvTuk

CUneTAevC¢bNÐit edIm,IsMerceFVIkarBüa)al.

EpñkmYyd¾sMxan;bMputéntYnaTIrbs;evC¢bNÐit nig Kilanubdæak-yika KWTak;Tg

nwgkarGnuvtþn_tamkarBüa)al. «Bukmþay nig GñkCMgWPaKeRcIn)an[dwgfa

144

TidæPaBlM)akbMputénkarEfTaMCMgWtaLasesmIukMritFn; KWenAeBlcab;epþIm

karBüa)alkMcat;CatiEdk. eRkayBI)anRTaMRTnwgkarbBa©ÚlQamral;Exmk

GñkCMgW nig matabitaRtUvRbQmmuxnwg]bsKÁmYyepSgeTot. RKUeBTü

Büa)al nig buKÁlikEfTaMrEmgGacCYysMrYl)anya:geRcInkñúgdMNak;kalenH

edaypþl;[kmμviFIBüa)alTTYl)aneCaKC½y nig KμanPaBtwgEtgeRcIn.

GñkCMgWtaLasesmIukMritF¶n; Cakumar CaekμgCMTg; nig CamnusS

eBjv½y

GñkCMgWCakumar ³ kñúgbNþaqñaMdMbUg kumarTTYlGarmμN_Qwcab; nig karrMxanBI

karBüa)al ehIyBYkeKminyl;BIGVIEdlekIteLIgcMeBaHBYkeKeLIy.

ya:gNak¾eday/ enHk¾CaeBlevlaEdlkumarekItmannUvCMenO nig kareCOTuk

citþcMeBaHGñkEdlemIlEfTaMBYkeK. dUecñHsMxan;Nas;Edl«Bukmþay Rbjab;

TTYlyksßanPaBenH edIm,Icab;epþImksagTMnak;TMngl¥mYyrwgmaMCamYykumar

EdlCakarCYy[TTYlykkarBüa)al. karlM)akgayekIteLIgNas;cMeBaH

kumarGayu 2-3qñaM EdlCav½ykumarcab;epþImmanm©as;karelIxøÜnÉg KWvaecHeFVI

kargaredayxøÚnÉg ecHpþÜcepþImKMnit. KwenAeBlenaHÉgehIy Edlkumar

cab;epþImyl;faxøÜnvaTTYlnUvPaBxaXaMgGVIEdleKcg;eFVI )ann½yfaxøÚneKmin

GaceFVIGVImYy)andUcekμgdéTEdlmanGayuRs)al²Kña.

eBlkumarQandl;v½ycUlsalaeron sßanPaBrwtEtlM)akEfmeTot. eRkABI

karQWcab; nig karrMxanBIkarBüa)al/ karyl;dwgBIsPaBxøÜnvaxusBIeK

CaBiess sPaBrUbragÁxuseKeBlEdlmanbMErbMrYlq¥wg b¤ karlUtlas;

yWtya:v bNþalmkBIRkcab;epþImBüa)al b¤ Büa)almin)anRtwmRtUv TaMgenH

Gacmanplb:HBal;F¶n;F¶reTAelIsuxPaBpøÚvcitþrbs;eK. KwenAdMNak;kalenH

ehIy EdlkumarecHEtTTUcsYrnaM«BukmþayeGayBnül;va. enaHehIyEdl

«Bukmþay nig RKUeBTü edIrtYnaTIsMxan;kñúgkarBnül;GMBICMgW ehIysMxan;Nas;

eTAeTot tYnaTIkñúgkarBnül;[kumarmanCMenOcitþ. RbsinebI«Bukmþay nig

145

RKUeBTü sMerckic©karenH)anedayKμanbgáPaBTMeyI b¤ KMnitGviC¢mandl;kUn

enaH/ fñalenHnwgkøayCafñalbNþúHkumar eBjedayCMenOcitþ rhUtdl;v½yCMTg;

nig dl;eBjv½y.

GñkCMgWekμgCMTg; ³ enHGacfaCadMNak;kald¾lM)akbMput/ CaeBlEdlGñkCMgW

v½yekμggayb:HBal;GarmμN_ pøÚvcitþ dUceKniyayBIdMNak;kallM)akqøgBI

yuvPaBeTACaeBjv½y. varEmgCadMNak;kalmYyEdlTidæPaBénCIvitsMrab;

karRKb;RKgCMgWtaLasesmIumansPaBEhlhalbMput Etk¾CadMNak;kal

mYyEdlGñkCMgWcaM)ac;RtUvTTYlsÁal;xøÜnÉgfaxøÜneKman\T§iBlGacRKb;RKg

)annUvKuNPaBCIvitrbs;eK.

Gakb,kiriyarwgrUsrbs;ekμgCMTg;manCMgWtaLasesmIudUcKñanwgekμgFmμtaEdr.

b:uEnþ cMeBaHekμgmanCMgW/ PaBrwgrUsenaHmanrUbPaBCakarCMTas;nwgkarBüa)al

CaBiesskarBüa)alkMcat;CatiEdk. «Bukmþay bgb¥Ún evC¢bNÐit Kilanub,dæa-

kyika RKUeBTücitþsaRsþ TaMgGs;KñamantYnaTIsMxan;kñúgkarpþl;karKaMRTdl;ekμg

CMTg;manCMgWtaLasesmIu edIm,I[eKmanCMenOcitþehIyrIkray TTYlykkar

Büa)al edayrMlwkBYkeKBIeRKaHfñak;énkarminGnuvtþn_tamkarENnaMrbs;

RKUeBTü. kñúgkic©xitxMelIkTwkcitþ nig KaMRTdl;GñkCMgWekμgCMTg;/ RKUeBTüxøH

elIkCa]TahrN_GMBIGñkCMgWmun² EdlTTYl)aneCaKC½ymansuxPaBl¥ man

esckþIrIkray EdlTaMgenH)anmkBIkarGnuvtþn_tamkarBüa)al.

GñkCMgWCamnusSeBjv½y ³ eBlcUldl;eBjv½y GñkCMgWRtUvRbQmmuxnwg

karCMnHcitþfμI. CakarBit/ GñkCMgWrEmgmanCMenOcitþ nig kþIsgÇwmEfmeTot

EdlkumarPaBminTan;man. minxusBI«Bukmþay nig RKYsarrbs;eKeT GñkCMgW

manPaBxøaMgkøa nig v½yqøatCagmun xøÜneKeFVICaKMrUsMrab;GñkCMgWv½yekμg nig

RKYsarrbs;eK. Etya:gNak¾eday eBlEdlGñkCMgWcab;epþImeFVIkarsMercBI

ExSCIvit nig GaCIBrbs;Kat; dUcCaTMnak;TMngyUrGEgVg b¤ karsikSabnþeTot

karrkkargareFVI GaBah¾BiBah¾ nig lT§PaBénkarksagRKYsar/ eBlenaHtMrUv

karénkarBüa)alCMgWrbs;BYkeK )anbgánUvPaBmYhμgya:gxøaMgcMeBaHBYkeK.

146

dUecñHGñkCMgWenAEtRtUvkarkarelIkTwkcitþya:gxøaMg edIm,IrkSa[)annUvrebob

Büa)alsMrab;BYkeKpÞal; nig sMrab;GñkenACMuvijBYkeK. PaBtwgEtgmYyeTot

k¾GacnwgekItmaneLIgRbsinebIGñkCMgWdwgxøÜnfaKat;KμanlT§PaBeFVICamþay b¤

Ca«Bukrbs;eK)an.

karRBYy)armÖcMeBaHesckþIsøab; enAEtCamUlehtucMbgbgá[Føak;Twkcitþ

CaBiessGñkCMgWeBjv½yEdlmankaryl;dwgBIplvi)akevC¢saRsþrbs;CMgW

ehIyGacmankar)at;bg;mitþPkþiedaysarEtCMgW. ral;bBaðalM)aknImYy²Edl

ekItmaneLIg eTAtamv½y nig dMNak;kalepSg² TaMgenHsuT§EtTamTarnUvkar

KaMRT nig elIkTwkcitþCab;CaRbcaMBIRkumRKYsar mitþPkþi nig buKÁlikRKUeBTü

ehIysMxan;bMputenaHKW\riyabf nig karxitxMRbwgERbgCaviC¢manénGñkCMgWxøÜn

ÉgpÞal;. CMenOeCOCak;mutmaM PaBcas;TMu EdlGñkCMgWtaLasesmIukMritF¶n;

TTYl)an manTMnak;TMngeTAnwgkMritKaMRTEdlBYkeKTTYl)an ehIynwgKμan

lT§plGVITaMgGs; ebIKμankarcUlrYmedaypÞal;BIGñkCMgWxøÜnÉgenaHeT )ann½yfa

smrPUmienaHminGacQñH)aneLIy. [EtmankarBüa)aledayRtwmRtUv ehIy

GacéllkeTA)an rYmCamYynwgkarKaMRTRKb;RKan;/ GñkCMgWPaKeRcInGac

Tb;Tl;nwgTidæPaBGviC¢manrbs;CIvitCamYyCMgWraMuér:RbcaMkay ehIyGacbMeBj

)annUvral;bMNg RBmTaMgeFVIeGayescþIR)afña nig kþIsubinþkøayCakarBit)an.

CaGkusl/ RbeTsPaKeRcInEdlTTYlrgF¶n;F¶redayCMgWtaLasesmIukMritF¶n;

suT§EtCaRbeTskMBugGPivDÆn_ EdlenATIenaH GaC£aFrsuxaPi)alfñak;Cati

matabitaGñkCMgW RBmTaMgbuKÁlikeBTüGacpþl;karKaMRTdl;GñkCMgW)ansþÜcesþIg

Nas; edaysarEtFn;FanenAmankMrit rcnasm<n½§suxaPi)alTn;exSay nig

edaysarmanGaTiPaBsuxaPi)alepSg²eTot.

tYnaTId¾sMxan;mYyrbs; shB½n§GnþrCatiCMgWtaLasesmIu ¬s>G>t¦ Kwkar

elIkkMBs;bNþasmaKmCatiCMgWtaLasesmIu pþl;karKaMRT nig elIkTwkcitþCa

RbcaMdl;GaC£aFrsuxaPi)alfñak;Cati enAtambNþaRbeTsTTYlrgCMgW

taLasesmIu kñúgbMNgeFVI[RbeTsTaMgenaHTTYlsÁal;bBaða nig plb:HBal;

d¾F¶n;F¶rénCMgWeTAelIsuxPaBsaFarN³ RbsinebIminRKb;RKg b¤ RtYtRtaeGay

)anRtwmRtUveT.

147

CMBUkTI 11

Bt’bEnßm

karrs;ran nig KuNPaBCIvit

karGPivDÆn_ya:gsem,Im cMeBaHkarEfTaMBüa)alCMgWtaLasesmIukMritF¶n;kñúg

ry³eBl 10 -20qñaMcugeRkayenH )aneFVIeGayekInGayukal nig KuNPaB

CIvitKYr[kt;sMKal; cMeBaHGñkCMgwEdlGacTTYl)anesvaenaH nig Gnuvtþ)an

tamkarENnaMBüa)alrbs;RKUeBTü. eTaHCaenAbNþaRbeTsEdlmankar

EfTaMKøInikcMeBaHGñkCMgW)anRtwmRtUvk¾eday k¾plvi)akbNþalmkBIelIsCati

Edk enAEtCamUlehtucMbgbgá[GñkCMgWtaLasesmIukMritF¶n;søab;)anEdr.

TaMgRbeTsGPivDÆn_ nig kMBugGPivDÆn_ ktþakMNt;karrs;ranrbs;GñkCMgW Kwkar

Gnuvtþn_eGay)antamkarBüa)alkMcat;CatiEdkedayfñaM DFO. xN³Edl

GñkCMgWenARbeTskMBugGPivDÆn_ RbQmmuxnwglT§PaBTTYl)anfñaM DFO ehIy

GñkCMgWenARbeTsGPivDÆn_gayTTYl)anfñaMenH bBaðaecaTeLIgenaH KwRtUvFana

[)anfaGñkCMgW)anRbkan;P¢ab;nUvrbbBüa)al eBareBjedaykarlM)ak nig

PaBQwcab; ehIyFana[)anfaGñkCMgW)aneRbIfñaM DFO jwkjab;tamkar

ENnaM b¤ eT.

karRsavRCavecjpSayqñaM 1996 eday eG> BIka nig shkarI )anbgðajfa

GRtars;ranekIneLIgx<s;bMput cMeBaHGñkCMgWTTYlfñaM DFO cak;bBa©Últam

srésQam )aneRcInCag 225 dgkñúgmYyqñaM Kwfa ebIGñkCMgWcak;fñaM DFO

kan;Etticdg GayuKat;kan;EtxøI. sarsMxan; KwRtUvFana[)anfa GñkCMgW)an

Rbkan;P¢ab;nUvkarBüa)alkMcat;CatiEdk )ann½yfa RKUeBTü matabita nig

RKYsarGñkCMgWmantYnaTIsMxan;kñúgkarKaMRT nig elIkTwkcitþGñkCMgW.

rUbftGñkCMgWmñak;

148

GñkniBn§ qñaM cMnYnGñkCMgW GRtars;rankñúgcMeNamGñkCMgWbBa©ÚlQameRcIn

ehIyeRbIfñaM DFO kMcat;CatiEdk

Modell et al 1982 92 25° enAGayu 25qñaM kñúgcMeNamGñkCMgWenAcRkPBGg;eKøs

ekIteRkay qñaM 1963 ehIyTTYlkarBüa)alenAmnÞIreBTüCMnaj

nig minCMnaj KW karrs;ranmanCIvit)anRbesIrcMeNamGñkEdl

)anTTYl DFO eRbItamkMritmFüm elIsBI 4 Rkam¼s)aþh_.

Zurlo et al 1989 1087 84/2° enAGayu 15qñaM kñúgRkummnusSekItenAqñaM 1965 - 69

Tl;nwg 96/9° enAGayu 15qñaM kñúgRkummnusSekItenAqñaM

1970 - 74 . GñkCMgWTaMgenHTTYlkarBüa)alenAmCÄmNÐl

CMnajcMnYn 7 kEnøg.

Brittenham et al 1994 59 100° enAGayu 25qñaM cMeBaHGñkCMgWkMcat;CatiEdk)anl¥ Tl;nwg

35° enAGayu 25qñaM cMeBaHGñkCMgWkMcat;CatiEdkmin)anl¥Edl

ekItrvagqñaM 1963 - 87/ sßiteRkamkartamdanmYyry³kal

edayGñkCMnaj kñúgmCÄmNÐlEtmYy.

Olivieri et al 1994 97 91° kñúgRkumTTYlkarkMcat;CatiEdk)anl¥ Tl;nwg 18°

kñúgRkumkMcat;CatiEdkmin)anl¥ eRkayBIBüa)alry³ 15qñaM.

GñkCMgWTaMgGs;TTYlkarBüa)alenAmCÄmNÐlCMnaj 3 kEnøg.

Giardina et al 1996 88 rs;)anGayumFüm 29qñaM cMeBaHGñkCMgWekIteRkayqñaM 1963

ehIyTTYlkarBüa)alenAmCÄmNÐlEtmYy.

Piga et al 1996 257 66° enAGayu 25qñaM cMeBaHGñkCMgWekItrvagqñaM 1958 ;- 93

ehIyTTYlkarBüa)alkñúgmCÄmNÐlCMnajEtmYy.

Bargna-Pignatti 1998 1146 82° enAGayu 25qñaM cMeBaHGñkCMgWekItrvagqñaM 1970 - 74

ehIyTTYlkarBüa)alenAmCÄmNÐlCMnaj 7 kEnøg.

Modell et al 2000 796 GRtasøab; 50° munGayu 35qñaM kñúgcMeNamGñkCMgWenAcRkPB

Gg;eKøs EdlTTYlkarBüa)alenAmCÄmNÐlCMnajpg nig

minCMnajpg.

Dewis et al 2001 103 78° enAGayu 40qñaM cMeBaHRkummnusSsuT§EtekItenAqñaM 1957

- 97 ehIy 100° enAGayu 25qñaM cMeBaHGñkCMgWekIteRkayqñaM

1974 . GñkCMgWTaMgGs;TTYlkarBüa)alenAmCÄmNÐl

CMnajEtmYyenAcRkPBGg;eKøs.

(Advances in experimental medicine and biology, volume 509, p125)

149

taragxagelIenHbgðajlT§plénkarsikSaPaKeRcIn EdleFVIeLIgenAry³eBl

Cag20qñaMcugeRkay eTAelIkarrs;ranmanCIvitkñúgCMgWtaLasesmIukMritF¶n;F¶r .

Tinñn½yenHbBa¢ak;fa GñkEdlekItCMgWtaLasesmIukMritF¶n; ehIyTTYlkar

Büa)al)anl¥BIdMbUgTI PaKeRcInGñkekIteRkayqñaM 1974 eBlEdlcab;epþIm

mankaryl;dwgBIkareRbIR)as;)anl¥fñaM DFO ehIyGñkCMgWTTYl nig Gnuvtþn_

)antamkarBüa)alRtwmRtUvmk GñkCMgWTaMenaHman»kasrs;)anyUrehIyman

dMeNIrCIvit)anl¥. ktþaKnøwHeFVI[RbesIrcMeBaHkarpþl;esva nig dMeNIrCIvit

rbs;GñkCMgWtaLasesmIukMritF¶n; enAbNþaRbeTsCaeRcIn KwkarbegáIt[man

mCÄmNÐlevC¢saRsþbEg¥k CalkçN³saFarN³mYy EdlGñkCMgWGacTTYl

)ankarBüa)almanKuNPaBl¥bMput. ya:gNak¾eday enAmankargarCaeRcIn

eTotRtUveFVIenAtambNþaRbeTskMBugGPivDÆn_mYycMnYn edIm,IelIkkMBs;sþg;da

¬niyam¦ EfTaMKøInikGñkCMgWtaLasesmIu .

bBaðaepSg²eTot EdlGacCakarRBYy)armÖrbs;GñkCMgW k¾dUcCamatabita

GñkCMgW KWTak;TgnwgbBaðaGaBah¾BiBah¾/ RkumRKYsar/ karhUbcuk nig skmμPaB

ragkay.

rbbGahar nig taLasesmIu

CaTUeTAGñkCMgWtaLasesmIu mincaM)ac;jaMucMNIGaharBiessGVIeT. b:uEnþ GñkCMgW

RtUveCosvagGaharsMbUrCatiEdk. CakarRbesIrKYreCosvagsura b¤ pwkkñúg

kMrittictYc eRBaHeføImrbs;eKgayTTYlrgkarb:HBal; müa:gedaysareføIm

GñkCMgWtaLasesmIupÞúkCatiEdkeRcIn ehIymüa:geTotGacfamanCMgWrlakeføIm

¬sUmGanGMBIcMNIGaharkñúgCMBUkBiess¦.

kILa nig taLasesmIu

GñkCMgWtaLaesmIuGaccUlrYmkñúgkILaCaeRcInRbePT GaRs½yeTA

elIsPaBKøInikrbs;GñkCMgW nig karENnaMrbs;RKUeBTü .

150

visSmkal nig taLasesmIu

shB½n§GnþrCatiCMgWtaLasesmIu ¬s>G>t¦ )ancgRkgbBa¢IeQμaHGñkCMnaj

evC¢saRsþ nig mCÄmNÐlevC¢saRsþCMuvijBiPBelak ¬Gacrk)antameKhTMB½r

rbs; s>G>t¦ EdlGacpþl;esvaBüa)alCUnGñkCMgWtaLasesmIukMritF¶n;)an.

munnwgeFVIdMeNIreTANa/ GñkCMgWKYrdwgTItaMgmCÄmNÐlevC¢saRsþEdlenAEk,r²

nwgkEnøgEdlxøÜncg;eTA ehIyKYrTak;TgCamYyRKUeBTüBüa)alenAmCÄmNÐl

enaHmunnwgecjBIlMenAdæaneTA. GñkCMgWRtUvR)akdfa xøÜn)anTTYlkarcak;fñaM

bgáarcaM)ac;TaMgGs; ¬Canic©kal RtUvRbwkSaCamYyevC¢bNÐitrbs;xøÜn¦ munnwg

eTATsSnaRbeTsNamYy ehIyRtUvyl;dwgBIGRtaCMgWqøgBiessNamYy

enAtMbn;EdlxøÜneRKageTATsSna ¬sUmGankarENnaMGMBIkareFVIdMeNIr Edlman

pSayelIeKhTMB½rrbs;GgÁkarsuxPaBBiPBelak¦. ral;fñaMEdlcaM)ac; sMrab;

rbbBüa)alrbs;GñkCMgW rYmmanfñaMpSH nig sMPar³s¥atKμanemeraK RtUvevcx©b;

edayykcitþTukdak; ehIydak;kñúgkabUb b¤ va:lIkan;enAnwgéd.

cMeBaHGñkCMgWEdleRKageTAelgtMbn;PñMx<s;² RtUvman eGm:UkøÚb‘Ín kMritFmμta

ehIyGñkCMgWxøÜnÉgpÞal;RtUvman»kashVwkhat;pSaMunwgry³kMBs;x<s;Camun

sin. dUecñHGñkCMgWRtUvkMritxøÜnÉg kMueTAtMbn;NaEdlmankMBs;x<s;Cag

11000hVIteLIy b¤k¾RtUv[c,as;fa xøÜnRtUvbBa©ÚlQamPøam munnwgecj

dMeNIreTAry³kMBs;x<s;. eRkABIenH KμankarhamXat;BITIkEnøgeFVIdMeNIreT.

karGaBah¾BiBah¾ nig RKYsar

BitNas;GñkCMgWtaLasesmIukMritF¶n; GacerobkarehIymankUn)an. kUnrbs;

eKmansuxPaBya:gNaenaH sßitenAelIsßanPaBsuxPaBénédKUrbs;eK )an

esckþIfa édKUmansuxPaBl¥Fmμta/ manpÞúkCMgW b¤ manCMgWtaLasesmIuEtmþg.

RbsinebIGñkCMgWmñak;erobkarCamYyGñkCMgWtaLasesmIumñak;epSgeTot kUnTaMg

Gs;rbs;BYkeKnwgmanCMgWEdr. RbsinebIGñkCMgWmñak;erobkarCamYyGñkpÞúk

CMgWEbtta taLasesmIu 50° énkUnekItmkmanCMgW ehIy 50° eTotCa

rUbftGñkCMgWmñak;CamYykUn

rbs;nag EdlmansuxPaBl¥

151

pÞúkCMgW . müa:geTotRbsinebIGñkCMgWmñak;erobkarCamYyGñkFmμtaKμanpÞúkCMgW

enaH 100° kUnekItmkRKan;EtCaGñkpÞúkCMgWb:ueNÑaH.

segçbmk GñkCMgWEdlGnuvtþ)antamkarENnaMrbbBüa)al GacmanCIvitrs;

enAsuxsb,ayesÞIrEtFmμta. ]TahrN_ enARbeTs sIuRBUs GñkCMgW

taLasesmIu 83°)anbBa©b;karsikSaCan;x<s; ehIy 25° eTot)anTTYl

sBaØabR½tsalkviTüal½y. GñkCMgW 22° )anerobkar kñúgenaH 73° man

kUn xøHmankUn 3 b¤ 4nak;. GñkCMgW 79° enA sIuRBUs Rbkbkic©karkñúgEpñk

sib,kmμ nig ksikmμ/ CaelxaFikar/ CaRKUbeRgon/ CaKilanub,dæak-yika/

CabuKÁlikevC¢saRsþ b¤ GmevC¢saRsþ.

152

CMBUkTI 12

eGBIedmIsaRsþ nig karkarBarCMgWtaLasesmIu

eGBIedmIsaRsþ

BImuneKKitfa taLasesmIuCaCMgWmanedImkMeNItenAEttMbn;smuRTemDIETra:eN

eT/ b:uEnþbc©úb,nñenH eKdwgfa vamanenABaseBjRKb;tMbn;CaeRcInén

BiPBelak. eKeXIjmantaLasesmIuenABaseBjGWr:ubxagt,Úg cab;BI

RbeTs B½rTuykal; eTAdl;RbeTseGs,a:j/ GIutalI nig Rkic nig

RbeTsGWr:ubkNþalmYycMnYn RBmTaMgEpñkxøHénGtItshPaBsUevot.

taLasesmIuekItmanpgEdrenAtMbn;mCÄwmbUBa’rhUtdl;GIur:g; )a:KIsßan/

\NÐa/ bg;køaeds/ éf/ ma:eLsIu/ \NÐÚeNsIu nig RbeTscinPaKxagt,Úg

RBmTaMgRbeTsenAtameqñrPaKxageCIgénTVIbGaRhVik nig enAGaemrik

xagt,Úg.

taLasesmIumanGRtaQWCaBiess enAtamtMbn;Edlman b¤ Føab;manCMgW

RKuncaj; ¬emIlrUb 12a¦. )a:ra:sIutCMgWRKuncaj; KWCaPñak;garbgáCMgWmYyEdl

cMlgeTAmnusStamry³mUsEdkeKalxaM ehIybgáCaCMgW edaybMpøajeKalika

QamRkhm ¬emIlrUb 12b¦. eKKitfa enAtMbn;EdlFøab;manCMgWRKuncaj;

srIragÁkayrbs;mnusSmanbMErbMrYltUcmYyelIBn§ú EdlCaKuNRbeyaCn_d¾

RbesIrCagGñkEdlKμanbMErbMrYlBn§ú. karenHeFVI[mankarpøas;bþÚrdl;

briyakaseKalikaQamRkhmEdleFVI[)a:ra:sIutCMgWRKuncaj;minGacmanCIvit

nig ekItkUnekItecAteTAeTot)an. sPaBpøas;bþÚrenHnaMeGayekItCMgW Ebtta

taLasesmIukMritRsal. eKeCOfa minxusBICMgW Gal;hVa taLasesmIu nig CMgW

Sickle cell Edr/ GñkCMgW Ebtta taLasesmIukMritRsal suT§EtmanlT§PaB

Tb;Tl;CMgWRKuncaj; RbesIrCagbuKÁlsuxPaBFmμta KWfa kñúgry³eBlCaeRcIn

qñaMmkenH kñúgbNþatMbn;enATUTaMgBiPBelakEdlmanCMgWRKuncaj; cMnYnGñkCMgW

Countries affected by

malaria before

establishment of control

programmes

12a

Malaria parasites

(trophozoites of

P. falciparum in red cells)

12b

153

Ebtta taLasesmIukMritRsal )anekIneLIgya:gxøaMg xN³EdlbuKÁlsuxPaB

FmμtaCaeRcIn)ansøab;edaysarCMgWRKuncaj;F¶n;F¶r. eTaHbIkñúgbNþaqñaM

cugeRkayenH kmμviFIbM)at;CMgWRKuncaj; )aneFVIeGayFøak;cuHya:gKMhuk énkrNI

CMgWRKuncaj;kñúgtMbn;CaeRcInénBiPBelakk¾eday k¾dMeNaHRsayCMgW

taLasesmIu nig CMgWeGm:UkøÚb‘ÍnkMritF¶n;epSg²eTot enAEtCabBaðaRbQmKYr[

kt;sMKal;dEdl.

kareFVIcMNakRsukrbs;RbCaBlrdæ nig GaBah¾BiBah¾rvag

Catisasn_xus²Kña )annaMykCMgWtaLasesmIu eTAesÞIrEtRKb;

RbeTsTaMgGs;kñúgBiPBelak dUcCa GWr:ub xageCIgEdlBImun

mkminEdlmanCMgWtaLasesmIuesaH Etbc©úb,nñenH )ankøay

eTACabBaðasuxPaBsaFarN³d¾cMbgmYy.

tamRbPBKYreGayTukcitþ)an)a:n;RbmaNfa RbEhl 1/5°

énRbCaBlrdæTUTaMgBiPBelak KW 80 - 90 lannak;CaGñkpÞúkCMgW Ebtta

taLasesmIu ehIyCaerogral;qñaM mankumarRbmaN 60>000nak; ekItBImþay

mkmanCMgWenH/ mYyPaKFMsßitenARbeTskMBugGPivDÆn_. eKdwgc,as;fa tYelx

enHCakarvaytMélTab edaysarfamanTinñn½yBitR)akdtictYcNas;EdleK

Gacrk)ansþIBIGRtapÞúkCMgW Ebtta taLasesmIu ¬edaykarBinitüBn§ú¦ kñúg

RkumRbCaBlrdæCaeRcIn CaBiesskñúgtMbn;énBiPBelakEdleKsÁal; b¤ rMBwgfa

nwgTTYlkarb:HBal;ya:gF¶n;F¶r. enATUTaMgBiPBelak/ tamÉksarrbs; s>G>t

)an[dwgfamanEtGñkCMgW taLasesmIukMritF¶n; RbmaNCa 20>000nak;

b:ueNÑaH enAmanCIvitehIy)ancuHbBa¢IreQμaHfa)anTTYlkarBüa)al karenHKUs

bBa¢ak;nUvkarBitd¾CUct;fa mYyPaKFMénkumarEdlTTYlrgCMgWenHekItenARbeTs

kMBugGPivDÆn_ søab;edayBMu)anTTYlkareFVIeraKvinicä½y b¤ eFVIeraKvinicä½ymin)an

RtwmRtUv TTYlkarBüa)almin)anl¥ b¤ min)anTTYlkarBüa)al.

EpnTIenH ¬emIlrUb 12c¦ bgðajBIRbeTsEdlb:HBal;eday CMgW Ebtta

taLasesmIu. ebIeKKitbBa©ÚlCamYyCMgWeGm:UkøÚb‘ÍnF¶n;F¶repSgeTot dUcCaCMgW

12c

(From TIF’s “Guidelines for

the Clinical Management

of Thalassaemia”

154

Sickle cell nig HbE/β - taLasesmIu/ enaHmanRbmaN 5° énRbCaCn

TUTaMgBiPBelakTTYlrgCMgWEbbenH.

karkarBar

taLasesmIu KWCaCMgWmYyGackarBar)an. manRbeTsmYycMnYn

)anbgðajPaBeCaKC½yya:gc,as; dUcCa GIutalI/ Rkik nig sIuRBYs

EdlknøgmksuT§EtsßitkñúgcMeNamRbeTsTImYyEdlbegáIt)an

kmμviFICatiRbkbedayeCaKC½y edaykat;bnßy)anKYr[kt;sMKal;

nUvkMeNItkumarmanCMgWtaLasesmIu ehIykrNIxøHRtUv)anbnßy

dl;cMNucesÞIrEtKμan. pÞúyeTAvijenAcRkPBGg;eKøs knøgmkFøab;

manGnuvtþkmμviFIkarBar EtKaμneKalneya)ayfñak;CatisMedAkarBar

eT GRtakMeNItkumarmanCMgW)anFøak;cuHEt 50° b:ueNÑaH ¬emIlrUb 12d¦ .

bc©úb,nñenH TidæPaBKnøwHénkmμviFIkarBarRbkbedayeCaKC½ybMput)anbegáIt

CamUldæansMrab;kmμviFIenAbNþaRbeTsepSg²eTot EdlTTYlrgCMgW.

TaMgenHman ³

� Tak;TajqnÞH nig karebþC£acitþrbs;rdæaPi)al

� begáItnUvyuT§nakarGb;rMsuxPaBRbkbedayfamBleday

pþl;karyl;dwgdl;mRnþIsuxaPi)al nig saFarN³CnTUeTA.

� begáItmnÞIrBiesaFn_RbkbedayKuNPaBedIm,I ³

- BinitüQamedIm,IRsavRCav

- eFVIeraKvinicä½yelITarkñúgépÞ

� elIkkMBs;esvaEpñksmÖB nig Bn§ú

sarsMxan;énkarkarBar

tamGgÁkarsuxPaBBiPBelak)an[dwgfa karcMNayRbcaMqñaMénkmμviFIkarBar

fñak;CatimYyenAbNþaRbeTsCaeRcIn esÞIrEtesμInwgtMélBüa)alsMrab;ry³eBl

mYyqñaM cMeBaHGñkCMgWmYyRkumEdlcab;kMeNItkñúgmYyqñaM.

12d

(WHO/HGN/WG/00.1 2000)

(WHO/HGN/WG/00.1 2000)

155

tMélcMNaysMrab;karBarRbcaMqñaMenAefrCab;rhUt ÉtMélcMNaycMeBaH

karBüa)alvijekIneLIgBImYyqñaM eTAmYyqñaM ¬emIlrUb A¦ dUecñHplcMeNj

ecjBIkmμviFIkarBarekIneLIgCaerogral;qñaMeBlGnuvtþkmμviFI. GgÁkarsuxPaB

BiPBelak)anbgðajfa ebIKμankmμviFIkarBaredIm,IkMritcMnYnkMeNItkumarman

CMgWeTenaH RbeTsPaKeRcInnwgBMuGacmanlT§PaBéllkpþl;karBüa)al[

)anl¥ dl;RKb;GñkCMgWtaLasesmIu)aneLIy. dUecñH kmμviFIkarBarRbkbeday

RbsiT§iPaB mansarsMxan;Nas;edIm,ITuklT§PaBcMNayelIkarBüa)aleGay

)anl¥dl;GñkCMgWEdlmanRsab; ¬emIlrUb A¦.

rebobEdltaLasesmIucMlgCalkçN³tMNBUC

dUc)anCMrabkñúgCMBUkTImYy/ Ebtta taLasesmIukMritRsal cMlgBI

«BukmþayeTAkUntamrebob GUtUsUm:al er:esssIuv ¬Autosomal

recessive¦ )ann½yfa kumarTTYlBn§úbgáCMgW EtmYyBI«Buk b¤ BImþay

¬ebITTYlBn§úbgáCMgWTaMgBIr BImþaypg nig «Bukpg enaHbgáCasPaB

KøInikF¶n;F¶r ehAfa taLasesmIukMritF¶n;¦. eBlEdlTarkcab;kMeNIt

kñúgépÞ vaTTYlCalkçN³tMNBUC Bn§úEbttakøÚb‘Ín ¬β-globin gene¦ mYy

BI«Buk nig mYyeTotBImþay. eBlNaTaMg«BukTaMgmþaypÞúk Bn§úEbttakøÚb‘Ín

Fmμta kUnnwgTTYlCalkçN³ tMNBUC Bn§úEbttakøÚb‘Ín TaMgBIrFmμta dUcbgðaj

kñúgrUb.

eBlNamñak;kñúgcMeNam«Bukmþayman Bn§úEbttakøÚb‘Ín manCMgW )anesckþIfa

Kat;KWCaGñkpÞúk Ebtta taLasesmIu ehIymñak;eTotman Bn§úEbttakøÚb‘Ín

FmμtaKμanCMgW kUnnImYy²ekItmkmanmñak;kñúgBIrnak; b¤fa 50° man»kas

TTYltMNBUCBn§úCMgWtaLasesmIu BI«Buk b¤ mþay EdlmanpÞúkBn§ú

QW dUcbgðajkñúgrUb. kUn²TaMgenH GacCa ³

[i] GñkpÞúk Ebtta taLasesmIukMritRsal/ b¤

[ii] Ebtta taLasesmIu eGetr:UhSIúháÚt/ b¤

[iii] Ebtta taLasesmIukMritRsal

156

GñkpÞúktaLasesmIukMritRsal

GñkpÞúk taLasesmIukMritRsal minmanCMgWGVIeT. eKBMumaneraKsBaØapøÚvkay b¤

pøÚvcitþeLIy ehIymintMrUveGaymanrbbGaharBiess b¤ karBüa)al

evC¢saRsþeLIy. karvivtþn_eTAGnaKt/ sPaBenHminGackøayeTACaCMgWF¶n;F¶r

eLIy/ tamBiteTAebImin)aneFVIetsþBiesssMrab;CMgWenHeT PaKeRcInnwgmin)an

dwgfa BYkeKmanpÞúkCMgW. ya:gNak¾edayGñkpÞúkxøH GacmansPaBesøksøaMg

bnþicbnþÜc EdlGac[eKeFVIeraKvinicä½yedayPan;RcLMfaCaCMgWxVHCatiEdk.

kareFVIetsþmnÞIrBiesaFn_ Gac[eKeFVIkarEbgEckkrNITaMgBIr)anedaygay.

RsþICaGñkpÞúktaLasesmIu kalNamanépÞeBaH GacmansPaBesøksøaMgkMrit

mFümEdlGacFUres,Iyvij edaypþl;CatiEdkbEnßmkñúgGMLúgeBlmanépÞeBaH.

ehtuGVIGñkcaM)ac;RtUvdwgfa etIxøÜnGñkCaGñkpÞúkCMgW b¤ Gt;

fVIebIGñkpÞúktaLasesmIukMritRsal BMumanplb:HBal;suxPaBk¾eday EtebIGñk

pÞúkmñak;mankUnCamYyGñkpÞúkmñak;eTot enaHkñúgkUnbYnnak; manmñak; b¤ faman

»kas 25° nwgmanCMgWtaLasesmIukMritF¶n;.

dUcrUbPaBbgðajRsab;/ eBlEdl«BukmþayTaMgBIrCaGñkpÞúkCMgW

ral;eBlmanépÞeBaH kUnenaHman»kas 1¼4 ¬25°¦

nwgmanCMgWtaLasesmIukMritF¶n;/ man»kas 1¼2 ¬50°¦

CaGñkpÞúktaLasesmIukMritRsal ehIy 1¼2 ¬25°¦

man»kasminekItCMgWesaH.

eGm:UkøÚb‘ÍnxusFmμta nig CMgWeGm:UkøÚb‘ÍnepSg²eTot

eK)ansÁal;eGm:UkøÚb‘ÍnmnusSFMEdlCaRbePT {xusFmμta} mYycMnYnepSgeTot

Edlmanrcnasm<½n§xusFmμta nig sPaBKøInikEsþgecjxus²Kña. eKsÁal;

eGm:UkøÚb‘ÍnxusFmμta TaMgenHfaCaeGm:UkøÚb‘ÍnEdlmanrcnasm<½n§køay rYmman

HbS, HbE, HbC, HbH, HbD nig Hb Lepore. eGm:UkøÚb‘Ín TaMgenHcMlg

CalkçN³tBUCdUcKñanwg Ebtta taLasesmIuEdr. ya:gNak¾eday manEt

157

GñkTTYlcMlg Hb Lepore nig HbS BI«Buk nig mþayb:ueNÑaH EdlmansPaB

KøInikF¶n;F¶r eday Hb Lepore mansPaBdUcKñanwgCMgW Ebtta taLasesmIu

BIkMritmFüm eTAF¶n;F¶r/ ehIy HbS bgáCMgW Sickle cell EdlCaCMgWeGm:UkøÚb‘Ín

mYyF¶n;F¶r epSgBI Ebtta taLasesmIukMritF¶n; TaMgsPaBKøInikEsþgecj nig

TaMgkarEfTaMevC¢saRsþ. cMeBaH eGm:UkøÚb‘ÍnxusFmμtaepSgeTot HbC, HbD b¤

HbE KμanEsþgecjCaCMgWeT eTaHCacMlgtBUCBI«BukmþayTaMgBIrk¾eday.

ya:gNak¾eday eGm:UkøÚb‘ÍnkøayTaMgenHGacpÁúMKñaCamYy Ebtta taLasesmIu

kMritRsal begáItCaCMgWelahit EdlmanrUbPaBKøInikmankMritF¶n;F¶repSg²Kña.

HbE/ββ - taLasesmIu

HbE Ca eGm:UkøÚb‘ÍnxusFmμtamYyeXIjmanTUeTACageK CaBiesskñúgcMeNam

RbCaBlrdæEdlmanExSRsLayenAtMbn;GasIuGaeKñy_ dUcCa km<úCa/

evotNam/ éf.

ebIsinmñak;kñúgcMeNam«Bukmþay CaGñkpÞúkEbtta taLasesmIu

kMritRsal ehIymñak;eTotCaGñkpÞúk HbE eBlmanépÞeBaHmþg²

man»kas 25° kUnekItmkmanCMgW HbE/β - taLasesmIu.

CMgW HbE/β - taLasesmIu

CaCMgWkgVHeKalikaQamRkhmkMritFn;bgÁÜrmYy EdleraKsBaØa

EtgEtmansPaBRsedogKñanwgCMgW Ebtta taLasesmIukMrit

mFüm b:uEnþvaGacmansPaBF¶n;F¶rdUc CMgWtaLasesmIukMritF¶n;Edr. sPaB

KøInikEsþgecj nig karEfTaMevC¢saRsþénCMgW HbE/β - taLasesmIu

manEcgkñúgesovePAenH.

Hb Lepore/β - taLasesmIu

karpÁúMcUlKñaén Hb Lepore CamYy Ebtta taLasesmIu EsþgecjCasPaB

KøInikmYyF¶n;F¶r dUcKñanwg Ebtta taLasesmIukMritF¶n; ehIycMlgCalkçN³

tBUCdUckarerobrab;kñúgCMgW HbE/β - taLasesmIu xagelIEdr.

158

HbS/β - CMgWtaLasesmIu

ebIsinmñak;kñúgcMeNam«Bukmþay CaGñkpÞúk Ebtta taLasesmIu

kMritRsal ehIymñak;eTot CaGñkpÞúk HbS man»kas 25°

kUnekItmkmanCMgW Hbs/β - taLasesmIu.

HbS eXIjmanTUeTAelICnCatiGaRhVik b¤ GñkCab;ExSRsLay

BItMbn;emDIETra:eN. sPaBF¶n;F¶rénCMgW ERbRbYleTAtam

brimaN EbttakøÚb‘ÍnFmμta ¬normal β-globin¦ Edlpliteday Bn§úEbtta

¬β-gene¦. eBlNa Bn§úEbtta minplit EbttakøÚb‘Ín )aneT sPaBCMgW

esÞIrEtdUcKñaebHbiTnwgCMgW Sickle cell. eBlNa Bn§úEbtta plit

EbttakøÚb‘Ín )anxøH sPaBCMgWmanlkçN³minsUvF¶n;F¶reT. Bt’mankñúg

esovePAenH minniyayGMBICMgWenHeT Bt’manbEnßmmanelIeKhTMB½r

rbs;GgÁkarsuxPaBBiPBelak.

karpÁúMeGm:UkøÚb‘ÍnkøayepSgeTot GaccMlgCalkçN³tBUCEdr mandUcCa β/Hb

Lepore nig HbO Arab/β ¬TaMgBIrenHRsedogKñanwgCMgWtaLasesmIukMrit

mFüm¦ehIynig HbS/HbC, HbS/HbD Punjab nig HbS/HbO Arab TaMg

Gs;enHmansPaBRsedogKñanwgCMgW Sickle cell EdlmankMritF¶n;F¶repSg²Kña.

Gal;hVa taLasesmIu

Normal alpha

globin genes

chromosomes 16

Chromosome

a-thalassaemia

minor

on the same

chromosome

Chromosome

a-thalassaemia

minor

on opposite

chromosomes

slient carrier

159

CMgW Gal;hVa taLasesmIu ¬a - thalassemia¦ xusEbøky:ag

xøaMgBICMgW Ebtta taLasesmIu ¬β - thalassemia¦. Bt’man

kñúgesovePAenH BMuniyayGMBIbuKÁlpÞúk Gal;hVa taLasesmIueT.

Bt’manbEnßmsþIGMBICMgWGal;hVa taLasesmIu manenAelIeKhTMB½r

rbs;GgÁkarsuxPaBBiPBelak. ya:gNak¾eday Bt’manCa

mUldæanxøHsþIBIeGBIedmIusaRsþ nig lkçN³cMlgbnþBUCénCMgWenH

manCUnenATMB½rbnÞab;.

enAkñúgBiPBelakmanGñkpÞúk Gal;hVa taLasesmIu cMnYn

260lanGñk krNIx<s;bMputmanenA \Nða/ GasIuGaeKñy_ nig

GaRhVik ehIymanEtbnþicbnþÜcenAtMbn;emDIETra:eN ehIyman

edaykMrenAGWr:ubxageCIg.

pÞúyBI EbttakøÚb‘Ín/ Gal;hVakøÚb‘ÍnekIteLIgBIBn§úcMnYnbYn EdlenA

mçagBIrelIExSTaMgsgxagénRkUm:UsUmTI 16.

Gal;hVataLasesmIukMritRsal pÁúMtamlkçN³epSg² edIm,I

[ekItCaCMgWelahitBIkMritRsaleTAF¶n;F¶r.

bNþMúeraKsBaØa Hydrops foetalis én Hb Bart ¬emIlrUb ii¦

CaCMgW Gal;hVa taLasesmIukMritF¶n;CageKbMput KWCasPaB

GUm:UhSIúháÚt ¬Homozygous state¦ sMrab;CMgW ao -

taLasesmIu )anesckþIfa Bn§úGal;hVakøÚb‘Ín ¬a-globin

genes¦TaMgbYnmindMeNIrkar ehIyKμanplitExSRbUetGIun

Gal;hVa ¬a-chains¦ eT. lkçN³enH

bgá[mankgVHRKab;QamRkhmF¶n;F¶r naM[TarkkñúgépÞsøab;.

eTaHbIsPaBKøInikEsþgecjmkmansPaBxus²KñaeRcInk¾eday k¾buKÁlmanCMgW

HbH ¬emIlrUb iii¦ mYyPaKFM mansuxPaBl¥Fmμta. TMrg;EtmYyKt;énCMgW

HbH EdlekItmanedaykMr ehIymansPaBF¶n;F¶r KW Hydrops foetalis.

[i]

[ii]

[iii]

160

sarsMxan;én Gal;hVa taLasesmIu kñúgdMeNIrKøInikén Ebtta

taLasesmIu

vtþmanCMgW Gal;hVa taLasesmIukMritRsal mansar³sMxan;cMeBaHGñkCMgW

Ebtta taLasesmIu GUm:UhSIúháÚt edaysar Gal;hVa taLasesmIuenH

GacCYy bnßyGtulüPaBrvagExSRbUetGIun Gal;haV nig Ebtta

EdlCakareFVI[sPaBKøInikCMgW Ebtta taLasesmIueTACaRsal.

EsVgyl;fa etIrUbGñkCaGñkpÞúk b¤ Gt;eT

karpþl;RbwkSaGMBIkarBinitüBn§ú

krNIPaKeRcIn etsþmnÞIrBiesaFn_samBaØGac[dwg)anfabuKÁlmñak;CaGñkpÞúk

taLasesmIukMritRsal b¤ ya:gNa. ya:gNak¾eday munGnuvtþetsþmnÞIr

BiesaFn_NamYy sMxan;buKÁlenaHRtUvTTYlkarpþl;RbwkSaGMBIkarBinitüBn§ú

ebIGaceFVIeTA)an edaypþl;CUnKat;nUvBt’man dMbUnμan karENnaMsþIBImUlehtu

EdlRtUveFVIetsþ ehIyniglT§pletsþmann½yya:gNacMeBaHKat;.

müa:gvijeTot karpþl;Bt’manTaMgenHRtUvEp¥kelIkmμviFIGb;rMsuxPaBeGayl¥.

Gñkpþl;RbwkSanwgTTYlkarhVwkhat;Biess edIm,ImanlT§PaBBiPakSaTidæPaB

sMxan;² GMBIkarkarBar nig GMBICMgWpgEdr rYmman ³

� TIkEnøgRtUveFVIetsþQam

� rebobbkRsaylT§pletsþQam

� Gtßn½yBaküfa GñkpÞúk/ rYmmandak;CMerIsCUndl;GñkpÞúkBIrnak; Edlman

KMeragmankUnCamYyKña b¤ dak;CUndl;GñkmanépÞeBaHrYceTAehIy

)anesckþIfa KUrRskrRbQmmuxnwgeRKaHfñak;.

� FmμCati nig karBüa)alCMgWtaLasesmIukMritF¶n; b¤ CMgWeGmU:køÚb‘ÍnepSg

NamYyeTot b¤ CMgWBn§ú.

� Gñkpþl;RbwkSaRtUvpþl;Bt’mandl;buKÁl nig KUrRskr eday[BYkeKeFVIkar

sMerccitþedayÉkÉg BIviFIEdlBYkeKR)afñaGnuvtþn_. ya:gNak¾edaydMbUnμan

pþl;edayGñkRbwkSa nig karsMerccitþrbs;BYkeK rEmgTTYl\T§iBlBICMenO

161

sasna nig vb,Fm’. karecjpSayrbs; s>G>t {karkarBarCMgW

taLasesmIu nig CMgWeGm:UkøÚb‘ÍnepSg²} PaKTI1 Gacpþl;dl;GñkGan

nUvBt’manCMnajsþIBIbBaðaTaMgenH.

kareFVIetsþmnÞIrBiesaFn_edIm,IkMNt;GñkpÞúk Ebtta

taLasesmIukMritRsal

etsþmnÞIrBiesaFn_sMrab;CMgWtaLasesmIu rYmmanetsþQamEdleKeFVICaRbcaM

eKehAfa karrab;ya:geBjeljénRKab;Qam ¬CBC = Complete Blood

Count¦ Edlmankarvas;kMriteGm:UkøÚb‘Ín nig rgVas;epSg²eTot Tak;TgnwgcMnYn

nig maDeKalikaQamRkhmEdleK[eQμaHfa {TMhMmFümrbs;eKalika

QamRkhm} ¬MCV = Mean Corpuscular Volume¦ nig { TMgn;mFümén

eGm:UkøÚb‘ÍnkñúgeKalikaQamRkhmnImYy²} ¬MCH = Mean Corpuscular

Haemoglobin¦. ]TahrN_ mnusSeBjv½y ebI MCV ticCag 75 GacbBa¢ak;

)anfa CaGñkpÞúkCMgW ¬Etvak¾GacbBa¢ak;BIsPaBxVHCatiEdkEdr EdletsþepSg

eTotnwgEjkkrNInImYy²)an¦. MCV GacmankMritTabcMeBaHkUnekμg ehIy

ERbRbYleTAtamGayu. eKBinitüeKalikaQamRkhm edaymIRkUTsSn_pgEdr

edIm,IdwgTMhM nig rUbragrbs;va. eKalikaQamRkhmrbs;GñkpÞúktaLas-

esmIu manragRsemalRkhmesøk ehIymanrUbragreqtrqUt ¬Poikilocy-

tosis¦¬emIlrUb 12f¦ xusBIeKalikaQamRkhmFmμta manBN’RkhmRkem:A

ragmUlptkNþal ¬emIlrUb 12e¦.

rgVas;RKab;Qam rbs;mnusSFmμtaTUeTA nig GñkpÞúkEbtta taLasesmIukMritRsal

mnusSFmμta GñkpÞúkEbtta taLasesmIu

Hb 135 g/dl Hb 142 g/dl

HCT 42% HCT 42,5%

MCV 88,8 fl MCV 61,2 fl

MCH 28,5 pg MCH 20,5 pg

MCHC 32,1 g/dl MCHC 33,4 g/dl

RDW-CV 13,1% RDW-CV 15,9%

A2 5,3%

162

ebIetsþmnÞIrBiesaFn_t²eTAeTot [dUcCa lT§PaBP¢ab;CatiEdksrub ¬TIBC =

Total Iron Binding Capacity¦ nig Ferritin] Gac[eKTat;ecalnUvkgVHCati

EdkEdlbgá[ MCV fycuH enaHeKGnuvtþetsþbEnßmeTot edIm,IbBa¢ak;

taLasesmIukMritRsal nig kMNt;RbePTrbs;va. etsþedIm,IkMNt; Ebtta

taLasesmuIkMritRsal KWCadMeNIrmYyEdleKehAfa karEjkRbePT

eGm:UkøÚb‘ÍnedaycrnþGKÁisnI ¬Hemoglobin Electrophoresis¦ EdlGac[

eKvas;brimaN HbA EdlsarFatupSMcMbgrbs;eGm:UkøÚb‘ÍnmnusSFM nig brimaN

HbA2 EdlmanEtbnþicbnþÜc. eGm:UkøÚb‘ÍnepSgeTotEdlmanCaFmμtakñúg

RKab;QamRkhmmnusSFM dUcCa eGm:UkøÚb‘ÍnTarkkñúgépÞ (Foetal hemoglobin

= HbF) Gacvas;)anEdreday viFIEjkedaycrnþGKÁisnI

¬Electrophoresis¦. krNIPaKeRcInetsþxagelIvaRKb;RKan;edIm,IkMNt;buKÁl

CaGñkpÞúkCMgW.

cMeBaH Gal;hVa taLasesmIukMritRsal eKEtgEtdwg)aneRkayBITat;ecal

bBaðaepSg²EdleFVIeGay MCV fycuH )anesckþIfa KμankgVHCatiEdk/

kgVHeKalika

QamRkhm

TMhMeKalikaQamRkhmtUc²

(Microcytosis) (MCV < 75fl)

HbA2 HbF epSg²

TMrg;c,as; 0 - + + > 3,5% 1 - 5%

βδ 0 - + + < 3,5% 5 - 10%

Lepore 0 - + + < 3,5% 1 - 5% 5 - 10%

Hb Lepore

GñkpÞúkCMgWkñúg

sPaBes¶oms¶at;0 0 < 3,5% < 2%

T M rg ; epSg² énEbtta taLasesm I u

Blood film from a

non-carrier (normal)12e

Blood film from a

ββ-thalassaemia carrier12f

163

lT§plFmμtacMeBaHkarEjkRbePTeGm:UkøÚb‘ÍnedaycrnþGKÁisnI/ ehIyKat;man

edImkMeNItBICnCatiEdlsMbUrGñkekItCMgWenH/ TaMgenHGacsnμt;)anfa Kat;Ca

GñkpÞúk Gal;hVa taLasesmIu. kñúgkal³eTs³xøH eKRtUvGnuvtþetsþ DNA

edIm,IkMNt;vtþman b¤ Gvtþman Ebtta b¤ Gal;hVa taLasesmIukMritRsal.

etsþBn§úEbbenH kMBug)anRtUveKeRbIR)as;ya:gTUlMTUlayeLIgsMrab;kMNt;

taLasesmIukMritRsal.

nrNaxøHKYreFVIetsþQam ?

edaysarkarkarBarCMgWtaLasesmIu vamansar³sMxan; ehIysPaBBitEdl

faCMgWenHmanekItesÞIrEtRKb;tMbn;TaMgGs;TUTaMgBiPBelak karBinitüQamrk

CMgWtaLasesmIukMritRsal KYrEtbBa©ÚlkñúgkmμviFIkarBarCMgWfñak;Catiedaycab;

epþImeFVIelIkumartUc² b:uEnþvacaM)ac;Nas;munerobGaBah¾BiBah¾ b¤ munman

épÞeBaH edIm,Ipþl;CMerIsRbesIrbMputdl;BYkeK. ya:gehacNas;eKKYr

BicarNaBinitüQam ³

k> eBldwgfamanbgb¥ÚnCaGñkpÞúkCMgW b¤ manCMgW

x> b¤k¾dwgfamanbgb¥Únrs;enA b¤ mkBIRbeTsEdlmanGRtaCMgWtaLasesmIux<s;

PaBrYmExSelahitCamYyKña

PaBrYmExSelahitCamYyKña mann½yfa manelahitRbhak;RbEhlKña dUcenH

karerobkarrvagbgb¥Únsac;Cit eKrab;fa GaBah¾BiBah¾ExSelahitCamYyKña

¬Consanguineous marriage¦. enAkEnøgCaeRcInkñúgBiPBelakenH eKelIk

Twkcitþ nig GnuvtþGaBah¾BiBah¾EbbdUecñH. ya:gNak¾eday TMnak;TMngsac;

Qamrvag«Buk nig mþaykan;EtCit eRKaHmankUnekItCMgWtaMgBIkMeNItk¾man

kan;EteRcIn dUcCaCMgWtaLasesmIu.

KUrRskrEdlKμanCab;sac;QamnwgKañ »kasRbEhl 98° mankUnsuxPaB

l¥Fmμta. KUrRskrEdlCabgb¥ÚnCIdUnmYYynwgKña »kasRbEhl 96° mankUn

suxPaBl¥. KUrRskrEdlCabgb¥ÚnCIdUnmYYynwgKña ehIyman«Bukmþay nig¼b¤

164

CIdUnCItak¾CaKUrRskrsac;QamCitEdrenaH »kasRbEhl 94° mankUn

suxPaBl¥.

dUcKñaenHEdr eRKaHmankUnekItCMgWfycuHya:gxøaMgeTAvij ebI«Bukmþayman

TMnak;TMngsac;Qamq¶ayBIKña. kñúgcMeNamkumarekItmk 1000nak; BI«Buk

mþayKμanCab;sac;QamnwgKña mankumarRbEhlEt 2 - 3 nak; b:ueNÑaHTTYl

Bn§úbgáCMgW/ EtebI«Buk nig mþaymanTMnak;TMngsac;QamnwgKña 2 - 20 nak;

nwgmanCMgWEbbenH KwvaGaRs½yelITMnak;TMngsac;QamCit b¤ q¶ay.

CMerIssMrab;KUrRskrRbQmmuxnwgeRKaHfñak;

kareFVIetsþTarkkñúgépÞ (Prenatal testing)

krNIRsþImñak;CaGñkpÞúkEbtta taLasesmIukMritRsal kMBugKitcg;mankUn b¤

manépÞeBaHrYceTAehIy édKUrrbs;nag ¬ebIsinminTan;)andwgBIsPaBpÞúkCMgW

xøÜnÉgeT¦ KYreFVIetsþQamPøam edIm,Idwgfa Kat;manpÞúktaLasesmIukMrit

RsalEdr b¤ Gt;. RbsinebIGñkTaMgBIrCaGñkpÞúk KUrRskrenHGaceFVIkarsMerc

citþ bnþEpnkarRKYsar b¤ ebIsinmanépÞeBaHrYceTAehIy GacBicarNabnþ

épÞeBaHeTAeTot b¤ y:agNa ehIyebIGaceFVI)an eyIgbnþeFVIetsþelITark

kñúgépÞrkemIlCMgWtaLasesmIu ebIeXIjfaTarkmanCMgW eKGacsMerccitþbBaÄb;

KP’. CMerIsepSg²eTotEdlKUrRskrRtUvBicarNa mandUcCa karElglHKña/

sMukUneKmkciBa©wm/ gakeTArkbgákMeNItkñúgmnÞIrBiesaFn_CamYyemCIvitburs b¤

RsþIrbs;Gñkd¾éTEdlmansuxPaBl¥. ebImindUecñaHeT/ «BukmþayEdlRbkan;

nUvCMenOsasnamutmaM GacsMerccitþmineFVIetsþelITark edIm,IdwgsPaBCMgW

rbs;TarkkñúgépÞ ehIybnþkarmanépÞeBaHteTAeTot.

eFVIetsþTarkkñúgépÞrkCMgWtaLasesmIu

manetsþ bI ya:gEdlGackMNt;faetITarkkñúgépÞmanCMgWtaLasesmIu b¤ Kμan ³

165

[i] karbUmykTwkePøaH ¬Amniocentesis¦

eKGnuvtþkarbUmykTwkePøaHmkBinitüenARtImasTI2 énépÞeBaH KWeRkay

BIépÞeBaH)anRbEhl 15 ¬18 -22¦ s)aþh_. edayeRbI eGkU

¬Ultrasound¦ CaCMnYy/ RKUeBTüCMnajEpñksmÖBcak;m¢últUcqμarcUlkñúg

épÞmþay bWtykTwkePøaH 2 - 3 søabRBa)ay. ekasikaTarkEdlman

kñúgTwkePøaHRtUvykeTAviPaKkñúgmnÞIrBiesaFn_ edIm,IkMNt;rkCMgW

taLasesmIurbs;TarkkñúgépÞ.

eKeRbIetsþenHeBlépÞeBaHvivtþ)anGayueRcIn. vaminbgáeRKaHfñak;dl;mþay

eT. ya:gNak¾eday vaGacbgá[TarkrlUtrvag 2 - 3 éf¶ eTA 2 - 3

s)aþh_eRkayetsþ.

[ii] karbUmykQamTgsuk ¬Cordocentesis¦

edaymanCMnYyBI eGkU eKcak;m¢últUcqμarmYycUlkñúgeBaH eq<aHeTArk

TgsukTark. eKbWtQamRbEhl 2 - 3 m>l ehIyQamTarkRtUveK

ykeTAEjkkñúgmnÞIrBiesaFn_. kñúgkrNIPaKeRcIn eRkamTwkédGñkCMnaj

eKTTYl)anekasikaTarksuT§ 100°. mUlehtubraC½yminTTYl)an

QamTarksuT§ bNþalmkBIépÞeBaHmanGayutic KwticCag 18 s)aþh_/

mþayFat;duHk,aleBaH nig sukenAEpñkxageRkayéns,Ún. épÞeBaHman

Gayutic k¾CamUlehtusMxan;bMputEdlbgá[manplvi)akF¶n;F¶rkñúgkar

bUmykQamTgsuk.

karEjkExSkøÚb‘Ín eday viFIEjkedaycrnþGKÁisnIkñúgsarFatuGn§il

¬Gel electrophoresis¦ EdlCaviFIFmμtarbs;mnÞIrBiesaFn_kñúgkarBinitü

rkCMgW. kareFVIeraKvinicä½y)anTan;eBl nig c,as;las; edayeRbI

viFIsikSam:UelKul ¬Molecular methods¦/ bc©úb,nñenH vaCMnYsesÞIrEt

TaMgRsugviFIbUmykQamTgsuk EdleKGnuvtþEtkalNaépÞeBaHmanGayu

eRcIn ehIyviFIRcwbsac;sukmkBinitü (CVS) minGac[eKsnñidæan)an nig

kalNaKUrRskrRbQmeRKaHfñak; BMuEdl)anBinitüBImun²mk.

166

[iii]karRcwbsac;sukmkBinitü (Chorionic Villus Sampling = CVS)

karRcwbsac;sukmkBinitü ¬CVS¦ GacGnuvtþ)anmun karbUmykTwk

ePøaHmkBinitü KWtaMgBIépÞeBaHmanGayu RbEhl 10 - 11 s)aþh_.

edaymanCMnYyBI eGkU GñkCMnajEpñksmÖBRcwbyksMNaksac;suk

¬Chorionic villi¦ EdlCaekasikapÞúkBt’manBn§údUcKñanwgTarkkñúgépÞ

ehIyEdleRkaymkeTotva[kMeNItCasuk. ekasikaenHRtUveKbWtyk

tamry³m¢últUcqμarmYy EdleKcak;cUleTAkñúgeBaHmþay

¬Transabdiminal¦ b¤k¾tamry³ kaetETr ¬Catheter¦ tUcqμarEdlcak;

tammat;s,Ún ¬Transcervical¦. bnÞab;mkeKeFVIkarviPaKekasikaenaH

rYckMNt;eraKvinicä½y.

dUcviFIbUmykTwkePøaHmkBinitüEdr karRcwbsac;sukmkBinitüBMupþl;eRKaH

fñak;dl;mþayeT. EtvamaneRKaHfñak;tictYcbgá[rlUtEdr. ebIkarrlUt

ekItman vamankarlM)aknwgdwgfaetIvabNþalmkBIkarRcwbsac;sukmk

Binitü b¤ ya:gNa eRBaHmankarrlUtCaeRcIn)anekItedayxøÜnÉgenArgVg;

s)aþh_TI12 énKP’.

GacmaneRKaHfñak;ekIneLIgedayeFVI[Gv³yv³TarkxusFmμta ebIeKqab;

GnuvtþkarRcwbsac;sukmkBinitüeBk )anesckþIfa muns)aþh_TI8 énKP’

eRkayrdUvcugeRkay. ya:gNak¾eday BMumanPsþútagbgðajBIeRKaHekIn

eLIgénPaBxusFmμtaNamYy eBlNaeKGnuvtþkarRcwbsac;sukmkBinitü

eRkayedIms)aþh_TI9 bnÞab;BIrdUvcugeRkay . ehtuenHehIy)anCaeK

GnuvtþviFIenHCaTUeTAenAeRkayedIm s)aþh_TI10 bnÞab;BIrdUvcugeRkay .

etIdMeNIrkarBinitüBn§úRbRBwtþeTAya:gdUcemþc ?

karbUmykTwkePøaH nig karRcwbsac;sukmkBinitü TaMgBIrenHEp¥kelImUldæan

Binitü DNA ehIykMNt;[sÁal; etImanPaBxusRbRktIénBn§ú b¤eT

167

¬karERbRbYlpøas;bþÚr = Mutation¦ EdlmanelI«Bukmþay EdlCameFüa)ay

d¾suRkwtkñúgkareFVIeraKvinicä½yCMgWtBUC. Etya:gNak¾eday dUcKñanwgetsþ

epSg²eTotEdr eTaHbImantictYck¾eday vaGacmanPaBel¥ógxusEdr.

Bn§úkMNt;lkçN³PinPaKEdleyIgTTYlCalkçN³tBUC kñúgenaHmaneGm:UkøÚb‘Ín

vaekIteLIgedaysar DNA. ral;CalikakñúgsarBagÁkay edayrYmTaMgsuk

rbs;TarkpgsuT§EtmanKMrU DNA edaypÞal;xøÜnTaMgGs;. ]TahrN_ kñúgkar

Rcwbsac;sukmkBinitü GñkviTüasaRsþmnÞIrBiesaFn_sikSaBn§úEdlkMNt;

sMeyaKeGm:UkøÚb‘Ín EdlBn§úenaHpÞúkenAkñúg DNA rbs;ekasikasuk edIm,I[dwg

fa eTAGnaKtetITarknwgmansuxPaBFmμta/ CaGñkpÞúkCMgWtaLasesmIu b¤ nwg

manCMgWtaLasesmIukMritF¶n; b¤ya:gNa. kareFVIviPaKenH CaFmμtaeRbIeBl

RbEhlmYys)aþh_.

karbBaÄb;KP’

ebIsinCaetsþbgðajfaTarkmanCMgW KUrRskrGacsMerccitþbBaÄb;KP’. tYnaTI

rbs;Gñkpþl;RbwkSaGMBIkarBinitüBn§ú nig RKUeBTüEp¥ksmÖB eBlenHmansar³

sMxan;xøaMgNas;. enAdMNak;kalenH ebIKUrRskrsMerccitþbnþKP’ enaHRtUv

suxcitþTTYlykkarBüa)alCMgWkUnGs;mYyCivit. ebIsineRCIserIsykkar

bBaÄb;KP’ ya:gNak¾eday karbBaÄb;KP’RtUveFVItamviFINamYykñúgcMeNamviFI

2 ya:g edayGaRs½yelIdMNak;kalénKP’.

karqab;bBaÄb;KP’

eKGnuvtþkarbBaÄb;KP’enH eBlKP’manGayuticCag 14 s)aþh_. eKKYrCMrab

R)ab;KUrRskrfa karbBaÄb;KP’enHmineFVI[)at;bg;»kasmankUnelIkeRkay

eT. eKKYrBnül;pgEdrfa eBlKUrRskrRbQmeRKaHfñak;manKP’mþg²GacekIt

TarkmkmanCMgW. RbsinebIbþIRbBn§manbMNgcg;dwgfa épÞeBaHkUnelIkeRkay

eTotmanCMgWtaLasesmIu b¤ ya:gNa eKRtUvGnuvtþkarrkeraKvinicä½yTarkkñúg

épÞmþgeTot.

168

karBnüabBaÄb;KP’

viFIbBaÄb;KP’EdlmanGayuelIsBI 14s)aþh_ KWRtUveFVI[mankarQWeBaHqøg

Tenøedaycak;G½rm:Un ¬Prostaglandin¦ eTAkñúgs,Ún. karQWeBaHGacRbRBwtþ

eTACaeRcInema:g ehIyviFIenHeFVI[RsþIb:HTgÁicGarmμN_ Cagkarqab;bBaÄb;KP’.

dUcKñaEdr vaminb:HBal;lT§PaBmanKP’mþgeTotrbs;RsþIeT.

CMhanepSg²eTot

kareFVIeraKvinicä½yTarkkñúgépÞ nig karbBaÄb;KP’ minTan;yl;RsbTaMgGs;Kña

faRtUvEteFVIenAeLIyeT. ya:gNak¾eday ebIKμankareFVIeraKvinicä½yTarkkñúgépÞ

nig karbBaÄb;KP’eT karkarBarminGacBwgEp¥kEteTAelIkMNt;sMKal;GñkpÞúk

CMgWenaHeT ehIykarBinitüQamedIm,IRsavRCavCMgW minGacmanRbsiT§iPaB nig

TTYleCaKC½yeLIy. viFIkarBarepSg²eTotkMBugRtUvbegáIteLIg dUcCa kar

viPaKekasikaTarkkñúgQamrbs;mþay. ya:gNak¾eday viFIenHmankMNt;

ehIymkTl;eBlenHminGacpþl;CMerIsKYr[Tukcitþ)an ykmkCMnYsviFIetsþ

TarkkñúgépÞ. bec©keTsepSgeTot Kw karkMNt;eraKvinicä½yBn§úmunkarbgá

kMeNIt ¬Pre-implantation Genetic Diagnosis = PDG¦ EdlCakareRbI

bec©kviC¢a DNA edIm,IeRCIserIsrkemCIvitmansuxPaBl¥rbs;RsþIpÞúkCMgWyk

eTAbgákMeNItkñúgmnÞIrBiesaFn_ rYcykeTAdak;kñúgs,Ún. karkMNt;eraKvinicä½y

Bn§úmunkarbgákMeNIt GacTTYlyk)anBIsMNak;saFarN³CnPaKeRcInEdl

CMTas;nwgkarbBaÄb;KP’ ehIyGacRtUv)aneKeRbIya:gTUlMTUlayeRcIneLIg

eBlNabec©keTsenHmantMélminsUvéfø ehIyminTamTarbec©kviC¢aeRcIn.

169

sTÞanuRkm

Agranulocyte RbePTekasikaQam>s EdlKμanKulikakñúgsuItU)øas.

AIDS mkBIBakü Aquired Immuno-Deficiency Syndrome

¬bNþMúeraKsBaØa bNþalmkBIkarcuHexSayRbB½n§karBar¦ Ca

CMgWbgáedayvIrus HIV CaTUeTAeRcInEtqøgtamQam b¤

karrYmsgVas.

Albumin CaRbUetGIunsamBaØmYy EdlmankñúgpøasμaQam sac;duM Epñks

éns‘ut nig TwkedaHeKa.

Aldosterone CaG½rm:UnesþrUGIutmYyEdlbMeBjmuxgarsMrbsMrYltulüPaBTwk

nig GMbil kñúgsarBagÁkay.

Alloimmune karbegáItGgÁbdibkçTb;Tl;sarFatuBIxageRkA ]TarhN_

QamEdlbBa©ÚleTA.

Anaphylaxis PaBgayRbtikmμxøaMg cMeBaHsarFatuGIVmYy ]TarhN_

RbUetGIun b¤ fñaMeBTü edaybgáeGaysðúk ¬Shock¦.

Antibody CaRbUetGIunEdlskmμPaBTb;Tl;nwgGg;TIEhSn ¬Antigen¦

CaEpñkénkareqøIytbrbs;RbB½n§karBarragkay.

Antigen CasarFatuEdlGacePJaCRbB½n§karBarragkayeGayeqøIytb.

Antihistamine smasFatu EdleKeRbIsMrab;Büa)alRbtikmμ Gal;ElhSIú

¬Allergy¦.

Antioxidant sarFatuepSg² dUcCa Beta-carotene, Vitamin C nig

Alpha-tocopherol EdlTb;;mineGaymanRbtikmμGuksIutkmμ

b¤ RbtikmμEdlCMrujeday GuksIuEsn nig EBruksIut EdleK

eCOfa vakarBarragkayeGaycakputBI\T§iBlrbs;

ra:DIkal;esrI ¬Free radical¦.

Arrhythmia cgVak;ebHdUgmineTogTat; nig exSay

170

Autosome RkUm:UhSÚmEdlminEmnCaRkUmU:hSÚmePT.

Blood plasma FaturavBN’elOgRBEltkñúgQam EdlekItBIFatuTwk nig

sarFaturlaykñúgTwkdUcCa RbUetGIun eGLicRtUlIt sár xøaj;

plitplkaksMNl;rbs;emtabUlIs GamINUGasIud G½rm:Un

nig vItamIn.

Bovine spongiform

encephalopathy CMgWRbB½n§sésRbsaTkNþalvivtþbnþicmþg²bNþaleGaysøab;

EdlekItelIstVeKaRkbI RsedogCMgW Srapie EdlekItelIecom

nig BEB ehIyRbEhlCabgáedayRbUetGIunbgáCMgW vaqøgtam

cMNIGahar ¬GacehAmüa:geTotfa CMgWeKaqáÜt¦.

Carbohydrate smasFatuepSg²EdlmanpÞúk kae)an GIuRdUEsn nig

GuksIuEsn dUcCa sár GamIdug nig EsluyLÚs PaKeRcInekIt

edayrukçCatiébtg.

Carbone dioxide ]sμ½nF¶n;KμanBN’ ekItkñúgkardkdegáIm nig karpuyrlYy b¤

cMehHénstV nig rukçCati.

Chromosome rcnasm<½n§dUcsésGMe)aHenAkñúgsñÚlrbs;ekasika. vapÞúk

Bt’manbnþBUC kñúgTMrg;CaBn§ú EdlRKb;RKgskmμPaB nig

muxgarTaMgGs;rbs;ekasika.

Citrate GMbilekItBIGasIud sIuRTic ¬Citric Acid¦.

Corticosteroid bNþa Steroids mYycMnYnrbs;RkeBj Adrenal-Cortex

eKeRbICaPñak;garRbqaMgnwgkarrlak.

Creutzfeldt-Jakob

disease CMgWxYrk,ald¾kMrman vavivtþbnþicmþg²bNþaleGaysøab;

bgáedayRbUetGIunbgáCMgW ehIysMKal;edayCalikaxYrk,al

manRbehagtUc² GñkCMgWmanPaBePøcPøaMgmunGayu

ehIyclnasac;dMubnþicmþg²)at;bg;PaBsIusgVak;Kña.

Deferiprone eQμaHBaNiC¢kmμénfñaMkMcat;CatiEdk eRbIelbtammat;

plitedayRkumh‘un»sf Apotex .

171

Deoxyribonucleic

acid (DNA) GasIudnuyekøGiucepSg²EdlCamU:elKulmUldæanéntMNBUC

ksageLIgrebobCaCeNþIrvil.

Desferal eQμaHBaNiC¢kmμsMrab; Desferrioxamine plitedayRkumh‘un

»sf Novartis-Pharma.

Desferrioxamine fñaMkMcat;CatiEdk

DIC Disseminated Intravascular Coagulopathy

¬kMNkQamkñúgsésQamBaseBjRKb;TIkEnøg¦.

DNA sUmemIBakü Deoxyribonucleic acid

Electrophoresis clnaEjkPaKl¥ittUc²tamry³sarFaturav b¤ xab;eRkam

skmμPaBkMlaMgTMnajGKÁisnI rbs;eGLicRtUd EdleKdak;

cUlkñúgsarFaturav b¤ xab;enaH.

Embolism karsÞHPøam²énsésQameday Embolus ¬PaKl¥itxus

Fmμta ]TahrN_ dUcCa BBuHxül; EdlGENþtkñúgQam¦

cUreRbobeFobeTABakü Thrombus.

Enzyme bNþMúRbUetGIunEdlCYyCMrujRbtikmμCIvKImIBiess enAkMritsI

tuNðPaBrbs;sarBagÁkay.

Erythroblast ekasikarbs;xYrq¥wgRkhm EdlsMeyaKeGmU:køÚb‘Ín ehIyCa

ekasikasßitkñúgdMNak;kalkNþalkñúgvdþdMbUg énkarbegáIt

eKalikaQamRkhm ebIniyay[TMlMTUlayCagenHKWCa

ekasikaedImrbs;eKalikaQamRkhm.

Erythroblastosis

foetalis (Rhesus or

Rh disease) CMgWEbkeKalikaQamRkhm ekItelITarkkñúgépÞ nig TarkeTIb

ekItmanlkçN³sMKal;edaykarekIneLIg Erythroblasts

kñúgQam ehIymanxan;elOg. TaMgenHekItmaneBlRbB½n§

Rh GviC¢manrbs;mþayplitGgÁbdibkç RbqaMgnwgGg;TIEhSn

kñúgQamrbs;TarkEdlman Rh viC¢man. GgÁbdibkçqøgkat;

172

sukeTAbMpøajeKalikaQamRkhmrbs;TarkkñúgépÞ.

Erythropoietin sarFatuG½rm:UnEdlbegáIteLIgCaBiessedaytMrgenam

EdlePJaCeGaymankarpliteKalikaQamRkhm.

Ferritin CaRbUetGIunpÞúkCatiEdkkñúgPaBRkam EdlbMeBjmuxgarkñúg

karsþúkCatiEdk ehIyBiessenAnwgeføIm nig GeNIþk.

Fibrinogen RbUetGIunmankñúgQam plitenAÉeføIm ehIyRtUv)anbþÚr

CaRbUetGIunmanlkçN³CasésEdleKehAfa Fibrin

kñúgeBlbegáItqñúkQamTb;karhUrQam.

Fibrosis CasPaBmYyEdlsMKal;edaykarekInCalikaman

sésevoveRcIn.

Foetal hemoglobin eGm:UkøÚb‘Ín EdlmanExSGal;hVaBIr nig ExSEbttaBIr ehIyCa

eGmU:køÚb‘Ín EdlmaneRcInCageKkñúgQamTarkeTIbnwgekIt

ehIysßitenAkñúgsmmaRtmYyekIneLIgkñúgsPaBCMgW

esøksøaMgmYycMnYn ¬]TahrN_ taLasesmIu¦.

Folic acid RkumvitamIneb ¬Vitamin B complex¦ manlkçN³CaRkam

eRbIkñúgkarBüa)alCMgWesøksøaMg bNþalmkBIxVHGaharUbtßmÖ.

Free radical bMENkm:UelKul b¤ GatUmEdlRbtikmμxøaMg.

Gamma globulin EpñkRbUetGIunkñúgQamEdlsMbUredayGgÁbdibkç.

Genotype bNþúMBn§úTaMgGs; b¤ mYyEpñkénbuKÁlmñak; b¤ RkummYy.

Globin RbUetGIunmYyKμanBN’ TTYl)anedaykardk Heme ecjBI

RbUetGIunmYy dUcCa eGm:UkøÚb‘Ín ¬haemoglobin¦.

Gram negative minCab;BN’RsKaM eBleKbMBak;BN’ eKeRbIsMxan;sMrab;

)ak;etrI.

Gram positive Cab;BN’RsKaM eBlbMBak;BN’ eKeRbIsMxan;bMputsMrab;

)ak;etrI.

Heme sarFatupÞúkCatiEdkmanBN’Rkhmcas; mankñúgeGm:UkøÚb‘Ín nig

mIjÚkøÚb‘Ín ¬RbUetGIunenAkñúgsréssac;dMu¦.

173

Hematopoiesis karbegáItQam b¤ RKab;Qam.

Hemoglobin (Hb) eGm:UkøÚb‘Ín CaFatuEdlmanpÞúkCatiEdk ehIysßitkñúg

eKalikaQamRkhm. FatuenHdwknaMGuksIuEhSnBIsYteTA

Calika ehIymanExSRbUetGIunbYn KW Gal;hVa/ Ebtta/ háam:a

nig Edlta.

HIV Human Immunodeficiency Virus CaRkumrbs; Retrovirus

EdlbMpøajRbB½n§karBarragkay ehIybNþaleGay

ekItCMgWeGds¾.

Hormone plitplrbs;ekasikamanCIvitEdleFIVdMeNIrkñúgvtßúrav

rbs;sarBagÁkay ehIyeFIVeGayman\T§iBlBiessmYy.

Hydrops foeralis karehImya:gFM manlkçN³F¶n;F¶rénTarkkñúgépÞ.

Hypogonadism karcuHexSaymuxgarénRkeBjePT Gonads .

Hypoparathyroidism karcuHfyénG½rm:UnRkeBj Parathyroid kñúgsarBagÁkay .

Hypothalamus Epñk)aténxYrk,al Diencephalon EdlCamCÄmNÐl

sMrbsMrYlRbB½n§RbsaTsVy½t ¬]TahrN_ sMrab;RKb;RKg

karjaMuGahar ¦.

Hypothyroidism skmμPaBcuHexSayrbs;RkeBj Thyroid.

Immune system RbB½n§karBarragkay.

Immunoglobulin GgÁbdibkç¬GkSrkat; Ig ¦

Interferon Cam:UelKulTb;Tl;nwgvIrus )anmkedayekasikaeBIbRbTHnwg

vIrus )ak;etrI b¤ fñaMRbqaMgvIrus EdleKplitedaykarsMeyaK.

In Vitro enAeRkAsarBagÁkaymanCIvit Etkñúgbrikassib,nimμit

Leukocyte ekasikaQam>s

Lymphocyte ekasikaQam>s KμanBN’ manedImkMeNItecjBIekasikaedIm

¬Stem cell¦ EdlbegáItCaekasikakñúgTwgrég nig

RbB½n§karBarsarBagÁkay. vamanBI 20° - 30°

énekasikaQam>skñúgxøÜnmnusSFmμta.

174

Magnetic Resonance

Imaging (MRI) bec©keTseFIVeraKvinicä½y EdlminbgákarQWcukcab;dl;GñkCMgW

edaybegáIt)anCarUbPaBedaykMuBüÚT½rénCalikarsarBagÁkay

EpñkxagkñúgedayeRbIrlkviTüú edIm,Ivas;rMj½rma:ejTikrbs;

GatUmkñúgsarBagÁkay.

Mitochondrion

¬Bhuvcn³

Mitochondia¦ rcnasm<½n§TTYlxusRtUvkñúgkarplitfamBlsMrab;ekasika

tamry³kardkdegðImrbs;ekasika.

Mutation bMErbMrYlvtßúFatutMNBUC

Myocardium RsTab;sac;dMukNþalrbs;ebHdUg

Oestrogen sarFatu ¬Ca G½rm:UnePT¦ gaynwgCMrujeGaycMNg;ePT

ehIyePJaCeGaymankarvivtþn_criklkçN³CaePT®sþI.

Organomegaly karrIkFMxusFmμtaénsrIragÁ ¬]TahrN_ eføIm GeNþIk¦

Osteoporosis sßanPaBEdlb:HBal;q¥wg sMKal;edayfycuHPaBtan;rbs;q¥wg

bgáeGayq¥wgeTACaes<at nig puygay)ak;.

Ovary RkeBjbnþBUCrbs;RsþI CaFmμtavamanmYyKU.

Pancreas RkeBjFMmYy sßitenABIeRkayRkBH P¢ab;CamYyKl;rbs;

eBaHevontUc ¬Duodenum¦ vabeBa©jGg;hSIúmrMlayGahar

nig G½rm:Un RbePT Insulin nig Glucagon.

Pancytopenia karfycuHxusFmμtanUvcMnYneKalikaQamRkhm

eKalikaQam>s nig pøaEktkñúgQam.

Parathyroid gland RkeBjbeBa©jkñúgtUc²TaMgbYn EdlCab; b¤ p¥wbeTAnwg

RkeBjTIrUGIut ¬Thyroid¦. RkeBjenH plitG½rm:Un

)a:r:aTIrUGIut ¬Parathyroid hormone¦ .

Phenotype lkçN³sm,tþi EdlGacemIleXIjnwgEPñkénsrIragÁmanCIvit

)anmkedayRbtiskmμrvag Genotype nig brisßanxageRkA.

Pituitary gland RkeBjbeBa©jkñúg ragBgRkeBItUc BN’RkhmRbepH

175

mansésQameRcIn vaP¢ab;eTAnwgEpñksþÜcxageRkam

énxYrk,al.

Plasma FaturavénQam Twkrég b¤ EbbCaTwkedaHeKa Edl)anmk

eRkayBIykFatuGENþtkñúgenaHecjGs;.

Platelet ekasikaragCafastUc² beBa©jBIxYrq¥wgcUleTAkñúgQam.

Polymorphism EdlmanlT§PaBkMNt;TMrg;xus²Kña.

Prion RbUetGIun EdleKrEmgKitfabgáeGaymanCMgWqøgepSg²

enARbB½n§RbsaT.

Progesterone G½rm:Un Steroid ePT®sþIbeBa©jeday Corpus luteum edIm,I

erobcMsac;épÞs,Ún eGayTarkEdlcab;kMeNItGaclUtlas;.

kñúgeBlKP’kMBugvivtþ G½rm:UnenHbeBa©jedaysuk sMrab;karBar

TarkkñúgépÞkMBugvivtþkMueGayrlUt.

Prophylactic manTisedAkarBarCMgW.

Refractory saMueTAnwgkarBüa)al b¤ mineqøIytbcMeBaHrMejac.

Restriction enzyme Gg;hSIúmepSg²Edlkat;pþac; DNA eTACabMENk²Rtg;

TItaMgBiess² enAxagkñúgm:UelKul ehIyeKrEmgeRbIvaCa

sMPar³sMrab;eFIVkarviPaKm:UelKul.

Retrovirus RkumvIrusEdlman RNA ¬dUcCa HIV ¦ EdlqøgeTAekasika

edayeFIVeGayekasikaenaH cMlgyk RNA rbs;vIrusenaH

edIm,IbegáItCa DNA.

Ribavirin fñaMRbqaMgvIrus )anmkedaykarsMeyaK.

Ribonucleic acid (RNA)

Saline Edlman b¤ pÞúkCatiGMbil.

Secondary

amenorrhoea kar)at;rdUvelI®sþI EdlBImunmkFøab;manrdUvFmμta.

Sepsis sßanPaBBul EdlCalT§plénkarratt,atrbs;)ak;etrI

Serum Epñkénvtßúravrbs;QamstV bnÞab;BIQamenaHkk.

176

vamanBN’ragelOg ekIteLIgbnÞab;BIktþaQamkk RtUv)aneRbI

Gs;edaykarbegáItkMNkQam. eKehAfa Blood serum.

Stem cell ekasikaedImdMbUg BMumanlkçN³kMNt;Biess Edlpþl;kMeNIt

eTACaekasikaepSg² xusKña ¬haemotopoietic stem cell

kñúgxYrq¥wg ¦ .

Streptococcus BYk)ak;etrIEdlCaPñak;garbgáeraKdl;mnusS nig stVciBa©wm

tampÞH.

Testosterone G½rm:UnbursbegáItedayBgsVas EdlTTYlxusRtUvkñúgkareFIV

eGayekItman nig EfrkSalkçN³sMKas;man

criklkçN³CaePTburs.

Thrombocyte emIl Platelet

Thrombophilia karkMNt;TukCamunEdlmanCatMNBUC b¤ eTIbnwgekItman

edaybegáItCadMukMNkQamkñúgsésQam ¬Thrombosis¦.

Thrombus dMukMNkQamEdlekItkñúgsésQam ehIys¥itCab;enAnwg

kEnøgEdlvaekIt ¬cUreRbobeFobnwgBakü Embolus¦.

Thyroid gland RkeBjbeBa©jkñúgenAEpñk)atxagmuxénk EdlbegáItG½rm:Un

Thyroxine nig Triiodothyronine.

TRALI Transfusion Related Acute Lung Injury

¬bBaðasYtekItmanPøambNþalmkBIkarbBa©ÚlQam¦.

Urea smasFaturwgd¾cMbgEdlmankñúgTwkenaménBYkstVciBa©wm

kUnedayTwkedaH ehIyvaCalT§plcugeRkayénkar

EbkFatuRbUetGIun.

Uric acid kaksMNl;énkarkat;pþac; Nucleic acids kñúgekasika

sarBagÁkay. mYycMENktUcrbs; Uric acids )anmkeday

karrMlayGaharsMbUr Nucleic acid dUcCa eføIm tMrgenam.

Uric acids PaKeRcInRtUvqøgkat;tMrgenam ehIybeBa©jecal

eTAtamTwkenam.

177

Vitamin D CavItamInrlaykñúgxøaj;/ tamlkçN³KImIsaRsþ vaCaRbePT

Steroid ehIyvamansarsMxan;cMeBaHrcnasm<½n§Fmμta

rbs;eFμj nig q¥wg.

Zinc (Zn) elahFatu BN’sragexovbnþic manv:aLg;BIr. vaCamIRkU

sarCaticMruHd¾caM)ac;rbs;rukçCati nig stV.

Zygote ekasikaEdlRtUv)anbdisn§ieLIgeBlemCIvitbursPBRbsBV

nwgemCIvit®sþI.

¬sTÞanuRkmenH Ep¥keTAtamniymn½ykñúgeKhTMB½r Merriam-Webmaster’s Medical Desk

Dictionary, Merriam-Webmaster, Springfield MA, 1996¦

178

Bt’mansMxan;²

cMNIGahar nig CMgWtaLasesmIu

kat;bnßyCatiEdkRsUbBIcMNIGahar

kñúgCMgWtaLasesmIu eTaHCaPaBelIsbnÞúkCatiEdkPaKeRcInbNþalmkBIkar

bBa©ÚlQamk¾eday karekIneLIgénkarRsUbCatiEdkBIcMNIGahar k¾CabBaða

sMxan;mYyEdr. CaFmμta mankarRsUbCatiEdkBIcMNIGahareTAkñúgxøÜnrbs;

eyIgkñúgbrimaNtictYcb:ueNÑaH. karRsUbCatiEdkekIneLIgeBlNaeGmU:køÚb‘Ín

¬Hb¦ kñúgQamcuHfy. dUecñHGñkmaneGmU:køÚb‘ÍncuHfy dUcCa GñkCMgW

taLasesmIukMritmFüm b¤ GñkCMgW taLasesmIukMritF¶n;EdlbBa©ÚlQam

min)aneTogTat; KYrsMrbsMrYlcMNIGahar[smRsb edIm,I[brimaNCatiEdk

srubkñúgcMNIGahar nig kñúgsarBagÁkayrbs;xøÜnmantictYc. CatiEdk)an

BIcMNIGaharmanBIrEbb KW CatiEdkEdlmankñúgsac;BN’Rkhm

¬CatiEdkkñúgGaharsac;¦ nig CatiEdkEdlmankñúgcMNIGaharepSg²

¬CatiEdkeRkABIGaharsac;¦.

CatiEdkkñúgGaharsac;

CatiEdkmankñúgsac;RkhmmandUcCa sac;eKa sac;ecom sac;RCUk nig

sac;man;exμA k¾dUcCacMNIGaharsmuRT dUcCa RtIsaDIn xüg RKM. eføImKWCa

RbPBsMbUrCatiEdk. Büayamkat;bnßycMNIGaharTaMgenH ehIyCMnYsGahar

sac;TaMgenHedayRbUetGIun)anBIsENþkesog. ya:gNak¾eday k¾minCakarl¥

EdrEdleCosvagTaMgRsugnUvsac;man; nig sac;RtI BIcMNIGaharrbs;Gñk

eRBaHGaharTaMgenHvapÞúknUvGaharUbtßmÖcaM)ac;epSg²eTot CaBiesssMrab;

kumar. cUreRCIserIsjaMusac;man;Epñks CaCagsac;Rkhm eRBaHvaman

CatiEdktic.

179

CamFümCatiEdkRbmaN 35° )anRsUbcUlkñúgsarBagÁkayrbs;eyIgeRkay

BIjaMuGaharmansac;Rkhm. EtcMnYnenHGacERbRbYlcenøaH 10 - 40°

sMxan;vaGaRs½yeTAelIGaharmanpÞúkTwkedaHeKa b¤ plitplBITwkedaHeKa.

Catikal;süÚmEdlmankñúgTwkedaHeKa/ QIs_ ¬Cheese = RhVÚm:as;¦/ y:aGY

¬Yoghurt¦ nig kñúgTWkedaHeKaxab; vabnßykarRsUbCatiEdk Edlmankñúg

Gaharsac;. BüayamjaMuTwkedaHeKamYyEkv eBljaMuGaharmanCatisac;

ehIyeRbITwkedaHeKakñúgkardaMsø. ]TahrN_l¥² mandUcCa TwkRClk;QIs_

kñúgmðÚbEbb)araMgeQμaHfa Lasagne, Pasticcio, Mousaka nig Cannelloni

edaydak;QIs_[eRcInkñúg Spaghetti bolognaise ehIyeRbIy:aGY nig TwkedaH

eKaedIm,IeFVIsmøeRKOgkarIu.

kñúgmYyéf¶jaMuTwkedaHeKaya:gehacNas;[)anRbEhlknøHlIRt eRBaHfava

CYykarBareraKBuKq¥wgpgEdr. ebIsin)armÖxøacFat; eRbITwkedaHeKaKμanCati

ERkm b¤ manCatiERkmtictYc EtvasMbUrCatikal;süÚm dUcTwkedaHeKa

FmμtaEdr.

CatiEdkeRkABIGaharsac;

eRkABIGaharsac; CatiEdkmankñúgcMNIGaharepSg² dUcCa s‘ut sUkULa

RKab;FBaØCati bEnø EpøeQI emIm ¬dMLÚg/ éqfav¦ nig sENþk. enAcRkPB

Gg;eKøs manmðÚbGaharmYycMnYneKbEnßmCatiEdk dUcCa RKab;FBaØCatijaMuCa

GahareBlRBwk emSARsUvsalI nig nMb½ug. EtRbeTsepSg²eTotRbEhl

mineFVIEbbenHeT.

GaRs½yeTAelIsmasPaBGahar karRsUbykCatiEdkEdlmanRbPBeRkABI

Gaharsac;cUlkñúgsarBagÁkay manbrimaNticCagRbPBBIGaharsac;

RbEhlCag 20dg. cMNIGaharEdlbnßykarRsUbcUl mandUcCa ³ (i) RKab;

FBaØCati nig (ii) plitplBITwkedaHeKa/ ÉGaharEdlbegáInkarRsUbCatiEdk

vij mandUcCa ³ (i) EpøeQI nig bEnøEdlsMbUrvItamInes/ (ii) sac;stV RtI ex©A

xüg egov RKM nig bkSI/ nig (iii) RCk; ¬dUcCaRCk;Rtsk; RCk;és<ekþab>>>¦

180

TwksIuGIuveFVIBIsENþkesog TwkexμH nig sura.

karecos[putBICatiEdkEdlmanRbPBeRkABIGaharsac; CakarBi)ak eRBaHfa

EdkmanenAkñúgesÞIrRKb;GaharTaMgGs;. b:uEnþeKGacpøas;bþÚrrebobhUbcuk

edayjaMu[)aneRcInnUvcMNIGaharNaEdlbnßykarRsUbCatiEdk ehIykat;

bnßyGaharNaEdlbegáInkarRsUbCatiEdk.

GaharbnßykarRsUbCatiEdkEdlmanRbPBeRkABIGaharsac;

1> RKab;FBaØCati

emSARsUvsalI eBat Ggár sENþkdI sENþkesog suT§EtbnßykarRsUb

CatiEdkcUlkñúgsarBagÁkayrbs;eyIg ehIyRbqaMgnwg\T§iBlvItamInes.

GaharsMbUrvItamInes begáInkarRsUbCatiEdk. ykl¥RtUvjaMuGaharEdlCa

RKab;FBaØCati[)aneRcIn ehIyRtUvecosvagGaharsMbUredayvItamInes dUcCa

TwkRkUcCaedIm. BüayamjaMuTwkedaHeKaCamYyRKab;FBaØCati ¬dUcCa nMb½ugdak;

QIs_ nMb½ugecondak;bWr QIs_ma:karUnI RKab;FBaØCati nig TwkedaHeKa¦ .

enAcRkPBGg;eKøs eRkABIemSARsUvsalIsuT§ RKb;emSARsUvsalIEkéqñTaMgGs;

RtUvc,ab;tMrUveGaybEnßmCatiEdk. cMeBaHemSAFBaØCatisMrab;GahareBlRBwk

vij manxøHeKbEnßmCatiEdk ehIyxøHeTotKμanbEnßmeT. dUecñH CakarRbesIr

RtUveRCIserIsykemSARsUvsalIsuT§ b¤ nMb½ugeFVIBIRsUvsalIsuT§KμanbEnßm

CatiEdk ehIyRtUvGansøaksMKal;sresrelIkBa©b;edayykcitþTukdak;

munnwgTijmYyNaykmkeFVICaGahareBlRBwk. GaharEbbenHmandUcCa

bbrRsUvsalI nig RKab;FBaØCatikBa©b;mYycMnYn Edllk;tamhagTMnijEpñk

suxPaB EtRtUvemIlsøaksMKal;[c,as;edIm,IeRCIserIsykRbePTKμanbEnßm

CatiEdk.

enARbeTsepSg²eTot emSA nig RKab;FBaØCatijaMuCaGahareBlRBwk GacKμan

bEnßmCatiEdkeT.

181

RbUetGIunsENþkesog GacCYybnßybrimaNCatiEdkRsUbcUlkñúg

sarBagÁkay. RbUetGIunsENþkesog GacCYydl;rsCatimðÚbGaharCaeRcIn

¬]TahrN_ Spaghetti bolognaise, smø/ econbMBg¦ ehIyrsCatiGac

kan;EtRbesIr ebImanbEnßmeRKOgeTspg.

2> TwkEt kaehV nig eRKOgeTs

TwkEt kaehV nig eRKOgeTsmYycMnYn ¬]TahrN_ CIrnagvg CIv:an;s‘uy

søwkéRK>>>¦ CYybnßykarRsUbCatiEdk. cUrjaMuTwkEt nigkaehV[)anerogral;

éf¶ CaBiesseBljaMu)ay. ritEtRbesIrEfmeTot ebIjaMuCamYyTwkedaHeKa.

TwkEt KWCaRbPBd¾sMbUrsarFatukMcat;CatiBulGuksIut ehAfa Gg;TIGuksIudg;

¬Antioxidants¦ EdlelIkmkBiPakSaxageRkam.

3> plitpleFVIBITwkedaHeKa

TwkedaH QIs_ y:aGY bnßykarRsUbCatiEdkcUlkñúgsarBagÁkay. kal;süÚm

k¾sMxan;Nas;EdrsMrab;karBareraKBukq¥wg dUecñHCakarRbesIrGñkRtUvbBa©Úl

plitpleFVIBITwkedaHeKa[)aneRcIneTAkñúgcMNIGaharjaMu tamlT§PaBEdl

GaceFVI)an. RbePTTwkedaHeKamanxøaj;tic ¬TwkedaHeKaKμanCatiERkm b¤ man

CatiERkmbnþicbnþÜc¦ nig QIs_ suT§EtsMbUrkal;süÚm ehIyGacCakareBjcitþ

sMrab;Gñkmincg;eLIgTMgn;. Caerogral;éf¶ ya:gehacNas;jaMuTwkedaHeKa

[)anRbEhlknøHlIRt.

GaharbegáInkarRsUbCatiEdkEdlmanRbPBeRkABIGaharsac;

1> vItamInes ¬Vitamin C¦

vItamInes mankñúgEpøeQI/ TwkEpøeQI nig bEnø. CakarRbesIrEdlRtUv

ecosvagBisarTwkEpøeQI dUcCa TwkRkUcRc)ac; eBljaMuGahar b¤ eBljaMu

ePsC¢³eBlRBwk. cUrjaMuTwkEt b¤ kaehV CMnYsvijeRBaHvaGacTb;karRsUb

CatiEdk. b¤k¾jaMuTwkedaHeKamYyEkvvij ¡ RsaebobegáInkarRsUbCatiEdk

dUecñHeBlBisaGahar ecosvagjaMuvajwkjyeBk b:uEnþGñkGacjaMuvaEtÉg

182

CamYyRKab;FBaØCatiepSg ]TahrN_ sENþkdIlIg. ya:gNak¾eday EpøeQI

nigTwkEpøeQI KWRbPBsMbUrsarFatukMcat;CatiBul ehIyKYrjaMuvaEtÉg kMulay

LMCamYyGaharepSg. bEnøRsuH b¤ es¶armanCativItamInestictYc eRBaHva

RCabecjBIbEnøeTAkñúgTwk.

2> sac;stV sac;bkSI RtI nig GaharsmuRT

sac;stV sac;stVsøab RtI nig GaharsmuRT minRKan;EtsMbUrCatiEdkb:ueNÑaH

eT EfmTaMgCYyCMrujkarRsUbCatiEdkBIcMNIGaharEfmeTot. y:agNak¾

eday vaminCakarl¥eTEdlGñkRtUvtmGaharTaMgenHTaMgRsugEtmþg eRBaHva

manpÞúkGaharrUbtßmÖcaM)ac;epSg²eTot BiesssMrab;kumar nig ekμgCMTg;.

3> RCk; ¬RCk;Rtsk; RCk;és<ekþab>>>¦ TwkexμHeFVIBIsENþkesog

TwkRClk;manCatisura

RCk;és<ekþab RCk;xÞwm)araMg éqfav nig kar:ut RBmTaMgplitplsENþkesog

p¥ab; ¬]TahrN_ TwksIuGIuv¦ CYybegáInkarRsUbCatiEdk. brimaNCatiEdk

RsUbrwtEtx<s; eBleKjaMu)ay b¤ nMb½ugCamYyRCk;bEnøTaMgenH.

CaTUeTAGaharmanCatiEdktic CaGahareFVIBIRKab;FBaØCati ¬eBat emSARsUv

salIsuT§ sENþk¦ nig emImbEnø ehIyminsUvmansac;stV RtI b¤ minsMbUr

vItamInes. GaharmanCatiEdkmFüm CaGahareFVIBIRKab;FBaØCati emImbEnø

Etmansac;eRcIn nig sMbUrvItamInes. GaharmanCatiEdkx<s; CaGahar

sMbUrsac;stV nig RtI kñúgbrimaNeRcIn. GaharmanCatiEdkx<s; k¾manpÞúk

pgnUvkMritvItamInesx<s; dUcCa EpøRkUcqμar nig bEnømYycMnYn. Gaharman

CatiEdkx<s; GacbnßymkCaGaharmanCatiEdkmFüm)an edayjaMu[)an

eTogTat;nUvGaharEdlbnßykarRsUbCatiEdkcUlkñúgsarBagÁkay dUcCa

plitpleFVIBITwkedaHeKa RKab;FBaØCati sENþk Et nig kaehV.

Gg;TIGuksIudg; kñúgGahar

Gg;TIGuksIudg; ¬Antioxidants¦ kñúgGaharmansar³sMxan;Nas; dUcGtßn½y

183

énBaküdUecñHEdr. vakarBarkarxUcxatedayCatiBul RbePTGuksIutkñúg

sarBagÁkay. kñúgn½yenHvaedIrtYnaTIsMxan;kñúgkarBarCMgW dUcCa CMgWsrés

QamebHdUg nig CMgWmharIk.

kñúgCMgWtaLasesmIu edaysarmanCatiEdkeRcInelIslb;kñúgsarBagÁkay

vamaneRKaHfñak;x<s; bgá[mankarxUcxatedayGuksIut. kñúgGtßbTenH

GñkniBn§nwgepþateTAelI Gg;TIGuksIudg; sMxan;² 4 RbePT KW vItamInGW

¬Vitamin E¦/ vItamInes ¬Vitamin C¦/ sarFatuBN’elOg ¬Carotenoids¦

nig sarFatukøin ¬Flavonoids¦.

1> vItamInGW ¬Vitamin E¦

vItamInGW CaGg;TIGuksIudg;kñúgcMNIGahard¾sMxan;CageKbMput. karsikSamYy

cMnYn)anrkeXIjfa manGñkCMgWtaLasesmIuCaeRcInmankMrit vItamInGW kñúg

QamticCagGñkKμanCMgW. enHGacmkBIGñkCMgWTaMgenH min)ancMNIGaharman

vItamInGWeRcIn b¤ k¾mkBIGñkCMgWmantMrUvkarvItamInGWx<s;. kñúgkarsikSaCaeRcIn

)anbgðajfa eBleKpþl;vItamInGWbEnßm enaHkMritvItamInGW kñúgQam)an

RbesIreLIg. fVIebI evC¢bNÐit b¤ GñkCMnajGaharUbtßmÖ ENnaM[Bisar

vItamInGWbEnßmk¾eday RtUvcaMfavItamInNak¾eday vacUlkñúgsarBagÁkay

rbs;elakGñk Kwtamry³cMNIGahar.

vItamInGW varlaykñúgxøaj; )anesckþIfavamanvtþmankñúgGaharEdlman

xøaj;eRcIn. RbPBvItamInGWl¥bMput KweRbgrukçCati ¬eRbgGUlIv eRbgQUkr½tñ

eRbgdUg nig eRbgsENþk¦. l¥CageK KW eRbgGUlIv eRBaHRbePTxøaj;kñúg

eRbgenHGacCYykarBarCMgWebHdUg. enAbNþaRbeTstMbn;emDIETra:eN

¬RbeTsRkic B½rTuyhÁal; eGs,aj nig GIutalI¦ EdleRbIR)as;eRbgGUlIveRcIn

CMgWebHdUgekItmanticCagenAGWr:ubxageCIg. b:uEnþcUrcgcaMfaBYkvItamIn RtUv

bMpøajbnþicmþg²edaykarqa b¤ econ. dUcenHviFIl¥bMput edIm,ITTYl)anvItamIn

eRcInbMputBIeRbgGUlIv KwbEnßmeRbgenHeTAelImðÚbenAcugbBa©b;karcMGin b¤

184

eRkayBIcMGinrYcral;. eRbgGUlIvlayCamYyRkUcqμar CaeRKOgbEnßmelI

sac;RtI sac;man; bEnøRsuH nig jaMPøa eGaykan;EtmanrsCatiq¶aj;.

cUreRCIserIsykeRbgGUlIvsuT§ ebIsincg;)anxøinl¥ ehIyeRbIvaCaTwkjaMPøa

b¤k¾sakl,gykeRbgGUlIvRbePTcMraj; ebIsincg;eRbIvasMrab;cMGinmðÚb b¤

eFVInMCaedIm.l. b½r\NðaeQμaHfa hÁI ¬Ghee¦ k¾manpÞúkvItamInGW Edr

EtedayeRbgGUlIvmanRbeyaCn_dl;suxPaBl¥Cag GñkGaceBjcitþsakl,g

eRbIva kñúgkarcMGinGahar.

RbPBepSgeTotrbs;vItamInGW manplitplBITwkedaHeKa RKab;FBaØCati s‘ut

nig sac;. plitplBITwkedaHeKamanPaBl¥viesskñúgkarykmkeFVICacMNI

Gahar eRBaHfaminRKan;EtvamanvItamInGW b:ueNÑaHeT EtvaTb;karRsUbCatiEdk

BIGaharcUlkñúgsarBagÁkay ehIyvamanCatikal;süÚmeRcIn EdlCYykarBar

eraKBukq¥wgEfmeTotpg. GñkGacsakl,geRbITwkedaHeKakñúgkareFVIGahar b¤

k¾jaMuTwkedaHeKamYyEkv eBljaMuGahar. TwkedaHeKaKμanCatiERkm mankMrit

vItamInGW ticCagTwkedaHeKamanCatiERkm EtbrimaNkal;süÚmmandUcKña.

2> vItamInes ¬Vitamin C¦

vItamInes begáInkarRsUbCatiEdkEdlmanRbPBeRkABIGaharsac; dUecñH

vItamInes KWCaGg;TIGuksIudg;d¾manfamBlmYy kareRbIGaharEdlman

pÞúkvItamInes layCamYyGaharmanCatiEdkeRcIn RtUvmankMrit. EbbenH

vamansar³sMxan;BiessedayELksMrab;GñkCMgWtaLasesmIukMritmFüm

EdlbBa©ÚlQammin)aneTogTat;.

cUrcgcaMfa CatiEdkmanRbPBBIcMNIGaharCaeRcIn kñúgs‘ut sUkULa

RKab;FBaØCati bEnø EpøeQI emIm ¬dMLÚg/ éqfav¦ nig sENþk. enAcRkPB

Gg;eKøs manmðÚbGaharmYycMnYneKbEnßmCatiEdk dUcCa RKab;FBaØCatijaMuCa

GahareBlRBwk emSARsUvsalI nig nMb½ug. EtRbeTsepSg²eTot RbEhlmin

eFVIEbbenHeT.

eKeXIjvItamInes eRcInmankñúgEpøeQI TwkEpøeQI nig bEnø. vaGacCakar

185

RbesIrEdlRtUvjaMuEpøeQI b¤ TwkEpøeQImYyEkvdac;EtÉg KwcenøaHeBl)ay

ehIyminGMLúgeBljaMu b¤ eRkayBIjaMu)ayrYcPøameT. GñkCMnajEpñksuxPaB

ENnaMRbCaBlrdæ[hUbbEnø nig EpøeQI[)an 5 dgerogral;éf¶.

]TahrN_ jauMmþgmandUcCa TwkEpøeQImYyEkv EpøeQImYycMNit

¬dUcCaEpøe)a:m EpøB½r eck RkUceBaF×sat; RkUcføúgknøHExøb b¤ eb:ge)a:HmYyEpø¦

bEnø ¬dUcCa kar:ut Rtsk; sENþk)araMg b¤ saLat;¦. vItamInes rlaykñúg

Twk dUecñHebIykbEnøeTAes¶ar b¤ RsuH vItamInesnwgrlaycUlkñúgTwkGs;.

karcMhuybEnøkñúgkMritlμm nwgrkSa)anRbesIrnUvvItamInkñúgbEnø. bEnøcMGin

CamYyeRbgGUlIv nig RkUcqμar GaceFVIGahard¾manrs;Cati. vItamInGW nig

vItamInes eBlBYkvaenACamYyKña skmμPaBBYkvaritEtl¥ dUecñHsUmkMuePøc

bEnßmfamBldl;bEnø rbs;elakGñkedayeRbIeRbgGUlIv ¡

3> sarFatuBN’elOg ¬Carotenoids¦

CaTUeTA RbPBcMNIGaharEdlmansarFatuBN’elOg mandUcCa kar:ut el<A

sac;elOg eBatelOg eb:ge)a:H lðúg RkUceBaFisat; nig bEnøsøwkébtgcas;.

GaharTaMgenHPaKeRcInmanvItamInesx<s; dUecñHeyIgRtUvRbugRby½tñ dUcbBa¢ak;

xagelI. eyIgsUmbBa¢ak;CUnfa karRsUbyksarFatuBN’elOgBIcMNIGahar

mankMritekInx<s; eBlcMNIGaharenaHmanCatixøaj; b¤ eRbg dUecñHkMuePøcbEnßm

eRbgGUlIv. sarFatuBN’elOgGacRtUvbMpøajenAsItuNðPaBx<s; dUecñHsUm

daMsø enAsItuNðPaBTab nig ry³eBlxøIRbsinebIGaceFVIeTA)an.

4> sarFatukøin ¬Flavonoids¦

eXIjmankñúg TwkEt RsaRkhm EpøeQI nig bEnø. enHCaelsmYyGac[

eyIgjaMuRsaRkhmmYyEkv eBlBisarGahar ¡ EtebI»kasminGnuBaØatieT

cUrjaMuTwkEtmYyEBgvij. TwkEtminRtwmEtpþl;Gg;TIGuksIudg; CaeRcInb:ueNÑaH

eT vaEfmTaMgTb;karRsUbCatiEdkBIcMNIGahar CaBiesseBljaMuvaCamYy

TwkedaHeKa. cUrsakl,gBisaTwkEt CaRbcaM[)anbIeTAbYnEkvCaerogral;éf¶.

cUrcgcaMfa mnusSeyIgRtUvkarTwkRbEhl 8 EkvkñúgmYyéf¶ edIm,I[manCati

TwkRKb;RKan;kñúgxøÜn.

186

esckþIsegçb

� vItamInGW ¬Vitamin E¦sMbUrmankñúgeRbgrukçCati dUcCaeRbgGUlIv nig

eRbgpáaQUkrt½ñ. l¥CageK KweRbgGUlIv eRBaHvaGacCYykarBarCMgW

ebHdUg. cUrbEnßmeRbgGUlIvenAcugbBa©b;énkarcMGinGahar eRkayeBl

Gaharq¥in b¤ EfmeTAelIbEnøeqA eRBaHkMedAGacbMpøajvItamIn.

� vItamInes ¬Vitamin C¦ mankñúgEpøeQI nig bEnø . RbesIrbMputkMujauM

EpøeQI nig bEnøTaMgenaHCamYyGaharEdlsMbUrCatiEdk RbsinebIelak

GñkmanCMgWtaLasesmIukMritmFüm ehIyKμankarbBa©ÚlQam. cUrGñk

hUmEpøeQI nig bEnøenAcenøaHeBljaMuGahar. cUrbEnßmeRbgGUlIv

dl;bEnørbs;Gñk eRBaHvItamInGW nig vItamInes eBlBYkvaenACamYyKña

skmμPaBBYkvaritEtl¥.

� sarFatuBN’elOg ¬Carotenoids¦ mankñúgkar:ut el<Asac;elOg

eBatelOg eb:ge)a:H lúðg RkUceBaF×sat; nig bEnøsøwkébtgcas;.

edaysarGaharTaMgenHsMbUrvItamInes dUcenHRtUvRbugRby½tñdUc

)anerobrab;xagelI. CafμImþgeTot cUrkMuePøceRbgGUlIv.

� Et nig RsaRkhm mansarFatukøin ¬Flavonoids¦ ehIyk¾Ca

Gg;TIGuksIudg;Edr. elIsBIenH TwkEtTb;karRsUbCatiEdk.

GtßbTenH)anykbBa©ÚlkñúgesovePAenHedaymankarGnuBaØatiBIGñkniBn§ ³

Dr. Dona Hileti-Telfer

Senior Dietician

Great Ormond St. Hospital for Sick Children

London

187

eRbI T2* sMrab;vas;kMritbnÞúkCatiEdkkñúgebHdUg

T2* KWCakarvas;EvgefrevlamYyEdlGacqøúHbBa©aMgbnÞúkCatiEdkkñúgCalika.

karvas;Evg T2* enHTTYlrUbPaBbgðajelIma:sIun MRI sþg;dar ¬Standard

Magnetic Resonance Image¦. ma:sIun MRI eRbIrlkviTüú nigma:ej:Tik

d¾xøaMg edIm,IbgðajrUbPaBCalikasarBagÁkay. ma:sIun MRI kMNt;PaBxus²Kña

énKuNPaBma:ej:TikkñúgsarBagÁkay ehIyERbkøayPaBxus²KñaTaMgenHeTA

CarUbPaBEdlRKUeBTüGaceRbIedIm,IeFVIeraKvinicä½yCMgWepSg². karelIsbnÞúk

CatiEdkbgá[mankarERbRbYllkçN³ma:ej:TikkñúgCalika KWeFVI[ T2* ¬T2*

value¦ mantMéltUcCagFmμta. karvas;Evg T2* énebHdUg mansar³sMxan;

Nas; eRBaHeK)anrkeXIjfa ]TahrN_ebIGñkCMgWman T2* énebHdUgman

tMélticCag 20 milliseconds ¬1 millisecond = 1¼1000 énvinaTI¦

enaHeRKaHfñak;cMeBaHebHdUgmankMritx<s; edaysarelIsbnÞúkCatiEdkCag T2*

elIsBI 20 milliseconds.

kareFVIetsþ T2* CakarBinitümYymanefrevla 45 naTIkñúgma:sIun MRI

biTCit. eK[GñkCMgWedkelIturMkilmYy ehIyeKdak;G½kSGKÁisnIBiess

mYycMnYnP¢ab;nwgRTUgGñkCMgWedIm,ItamdancgVak;ebHdUg. GñkCMgWRtUvBak;

nwgk,alnUv]bkrN_mü:ag manmIRkUhVÚnnwgRtecok (Headset with a

microphone) edIm,IeFVITMnak;TMngCamYyGñkbec©keTs. tuGñkCMgWRtUvruj

cUlyWt² eTAkñúgma:sIun MRI edIm,I[xøÜnGñkCMgWTaMgmUlsßitkñúgRbehagma:sIun

¬ma:sIun MRI manrUbragÁRsedognMkgdUNat;d¾FMmYy manRbehageRCA¦.

kñúgGMLúgeBlkMBugft/ GñkCMgWRtUvedk[es¶óm sþab;karENnaM[Gt;degðImmYy

ry³eBlxøI edIm,IeKvas; T2*. kñúgxN³eBlkMBugRbEmRbmUlkarvas;Evg/

ma:sIun MRI beBa©jsMelgxÞ½r. ]bkrN_EdlGñkCMgWBak;nwgk,al nwgkarBar

RtecokBIsMelgxÞ½renH. etsþenHmanlkçN³minb:HBal; ehIyminbgákar

QWcukcab;eLIy.

188

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eFVIkarvas; T2* eKGacvaytMél)anpgEdr BImuxgarrbs;ebHdUg. karvas;

muxgarrbs;ebHdUgeFVIeLIgtamry³ma:sIun MRI eKKitfamanlkçN³BitR)akd

Cagma:sIuneGkU ¬Ultrasound¦ vas;ebHdUgFmμtaeTAeTot. MRI GaceFVIkar

vas;EvgpgEdrnUvTMhM nig dMeNIrRbRBwtþieTAénftebHdUgeRkamxagsþaM EdlCa

ftrujQamcUleTAsYt. GñkCMgWtaLasesmIu gaymaneRKaHfñak;ekItCMgW

sMBaFQamekInkñúgsYt EdlGacbgá[ftebHdUgeRkamxagsþaMrIk. sPaBenH

GacbnßyRbsiT§iPaBkñúgkarrujRcanQamrbs;ftebHdUgenH.

bNþaGñkviTüasaRsþEdleK[Gnuvtþkic©karenH bc©úb,nñkMBugEteFVIkarbBa¢ak;

GHGagnUvBt’manxagelIenHEdlTTYl)anBIkarBinitüGegátEpñkKøInik ehIy

TIF RBmTaMg NIH enAshrdæGaemrikkMBugKaMRTkic©RbwgERbgenH.

189

taragvaytMél nig karBüa)al

190

191

192

193

194

195

196

197

198

BakübMRbYj/ Ékta nig rgVas; Edl)aneRbIkñúgesovePAenH

Ékta Edl)aneRbI BakübMRbYjCaskl

brimaNQam ³ mIlIlIRt ¬m>l = ml¦ 1¼1000

énmYylIRt

eGm:UkøÚb‘Ín ³ Rkam kñúgmYyedsIulIRt ¬Rkam¼d>l = g/dl¦

edsIulIRt = 1¼100 énmYylIRt

TMgn; ³ KILÚRkam ¬KRk = kg¦

kMriteRbI»sf ³ mIlIRkam ¬mRk = mg¦ ¬DFO, L1¦

1¼1000 énRkam

Ferritin ³ mIRkURkam¼l ¬μg/L¦

1¼10 000 énRkam kñúgmYylIRt

Parvovirus B-19 : B19

Epstein-Barr Virus : EBV

Deferioxamine : DFO

Iron ¬elahFatuEdk¦ : Fe

Hepatitis B Virus : HBV

Hepatitis C Virus : HCV

Human Immunodeficiency

Virus : HIV

Acquired Iimmunodeficiency

Syndrome : AIDS

Serum Alanine

Aminotransferase : sALT-Liver Function

Test

Serum Asparate

Aminotransferase : sAST-Liver Function

Test

Hemoglobin ¬eGm:UkøÚb‘Ín¦ : Hb

HAV ¬vIrusrlakeføImRbePT Ga¦ : Hepatitis A Virus

Magnetic Resonance Image : MRI

199

eKhTMB½rsMxan;²

� http://www.thalassaemia.org.cy

kñúgenH rYmman ³

1> Gñkpþl;RbwkSaviTüasaRsþ

2> shkarI-rinI viTüasaRsþ

3> smaKmCatiCMgWtaLasesmuInana

4> sMPar³sMrab;Gb;rM

5> RBwtþikarN_epSg²

Bt’manTak;TgnwgkareFVIdMeNIr

sUmGanesovePA {kareFVIdMeNIreTAbreTs nig suxPaB 2002}

erobcMeday GgÁkarsuxPaBBiPBelak (WHO)

� http://www.who.int/ith

� email: [email protected]

WHO ³ GgÁkarsuxPaBBiPBelak

WHO Geneva headquarters

� http:/www.who.ch

WHO Africa Regional Office

� www.whoafr.org

WHO Europe Regional Office

� www.who.dk

WHO Southeast Asia Regional Office

� www.paho.org

200

WHO Eastern Mediterranean Regional Office

� www.who.sci.eg

WHO Western Pacific Regional Office

� www.who.org.ph

HIV nig karrlakefIøm� www.hivandhepatitis.com

HIV

� www.who.int/hiv_aids/first.html

ICBS : International Consortium of Blood Safety

� www.icbs.com/about/htm

ISBT : International Society for Blood Transfusion

� [email protected]

Blood Safety

� www.int/health_topics/blood_safety/en

Interactive Groups for Discussions

� http:/groups.msn.com/thalassemiapatientsandfriends/home.htm

Product Information prepared by WHO

� www.inf/vaccines_documents/

201

Gas½ydæanTMnak;TMngsMxan;² nig Bt’man

WHO Headquarter

World Health Organisation Headquarters

Avenue Appia 20, 1211 Geneva 27, Switzerland

Telephone : (41 22) 791-21-11 / Fax : (41 22) 791-0746

Cable : UNISANTE GENEVA / Telex : 415 416

Email : [email protected] / Website : http:/www.who.ch/

AFRICA

WHO Regional Office

Dr. Naomi Nhwathiva, Chief DCP, Regional Office

for Africa, World Health Organisation,

Parirenyatwa Hospital, P.O Box BE 773, Harare, Zimbabwe

Telephone : (263) 407-69-51 or (263) 470-74-93

Fax : (263) 479-01-46 or (263) 479-12-14

Telex : 5217 or 5364 UNISANTE / Cable : UNISANTE

BRAZZAVILLE

Email : [email protected]

Website : http:/www.whoafra.org/

EUROPE

WHO Regional Office

J.E. Asvall, Regional Director, Regional Office

for Europe Accident Prevention Programme,

World Health Organisation, 8, Scherfigsvej, DK-2100

Copenhagen 0, Denmark

Telephone : (45) 39-17-17-17 / Fax : (45) 39-17-18-18

Telex : 15348 or 15390 / Cable : UNISANTE COPENHAGEN


Website : http:/www.who.dk/

202

AMERICAS

WHO Regional Office

George A.O. Alleyne, Regional Director, Regional Office

for the Americas, Emergency Preparedness & Disaster

Relief Coordination, World Health Organisation,

Pan American Sanitary Bureau, 525 3rd Street NW, Washington, DC

20037, USA

Telephone : (202) 974-3000 / Fax : (202) 974-3663

Telex : 248338-440057-64152-892744

Cable : OFSANPAN WASHINGTON

Email : [email protected] / Website : http:/www.paho.org/

SOUTHEAST ASIA

WHO Regional Office

Mrs Harsaran Bir Kaur Pandey, IO Regional Office

for South East Asia World Health Organisation,

World Health House Indraprastha Estate,

Mahatma Gandhi Rd, New Delhi, 110002 India

Telephone : (91) 11-331-7804 or (91) 11-331-7823

Fax : (91) 11-331-8607 or (91) 11-332-7972

Telex : 3165095 or 3165031 / Cable : WHO NEW DELHI


Website : http:/www.tron.um.u-tokyo.ac.jp/

EASTERN MEDITERRANEAN

WHO Regional Office

Hussen A.Gezairy, MD., F.R.C.S., Regional Director, Regional Office

for the Eastern Mediterranean World Health Organisation,

PO Box 1517, Alexandria, 21511 Egypt

Telephone : (203) 48-202-23 or (203) 48-202-24 or (203) 48-300-90

Fax : (203) 48-389-16 or (203) 48-243-29

Telex : 54028 or 54684 / Cable : UNISANTE ALEXANDRIA

Email : [email protected] / Website : http:/www.who.sci.eg

203

WESTERN PACIFIC

WHO Regional Office

Dr. Shigeru Omi, Regional Director, Regional Office

for the Western Pacific Health Services,

Development & Planning World Health Organisation

PO Box 2932, 1099 Manila, Philippines

Telephone : (632) 538-80-01 / Fax : (632) 521-10-36 or

(603) 536-02-79

Telex : 27652-63260-40365 / Cable : UNISANTE MANILA


Website : http:/www.who.org.ph/

Council of Europe (CoE)

Mr. Karl – Friedrich Bopp,

Administrative Officer, Health & Social Affairs

Directorate General III Social Cohesion 67075 Strasbourg, France

Tel : (33) 388412214 / Fax : (33) 388412726


European Commission

Dr. Lieve Fransen, Principal Administrator, Health & Family Planning,

AIDS Unit, Rue de Geneve/Genevestraat 12, B1140, Bruxelles

Tel : +32-2 2963698/2969117

Fax : 2963697

Food and Drug Administration (FDA), USA

Dr Jay Epstein, Director, Office of Blood Research and Review,

Center for Biologics Evaluation and Research, Food and Drug

Administration,

HFM-300,1401 Rockville Pike, Rockville, MD 20852

Tel : +13018273518 / Fax : +13018273533


204

International Consortium for Blood Safety (ICBS)

Dr Mohamed El-Nageh, Executive Director, New York Blood Center,

310E 67th Street, New York, NY 10021, USA

Tel : +12125703319 / Fax : +12125703320


International Federation of Blood Donor

Organisations (FIODS)

Mr Niels Mikkelsen, Secretary General, Bloddonorerne,

Denmark, Vesterbrogade 191, 1800 Frederiksberg, Denmark

Tel : +4570137014 / Fax : +4570127010


International Federation of Red Cross and Red

Crescent Societies (IFRCRCS)

Mr Peter Carolan, Senior Officer,

Case Postale 372, 1211 Geneva 19, Switzerland

Tel : +41227304222 / Fax : +41227330395


International Society of Blood Transfusion (ISBT)

Dr Paul Strengers, Secretary General,

PO Box 9190, NL 1006 AD Amsterdam, The Netherlands

Tel : 31205123212 / Fax : 31205123560


205

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ral;kic©xitxMGrKuNcMeBaHRbPBrcnarUbPaBTaMgGs;EdlRbIkñúgesovePAenH

eyIgk¾sUmGrKuNpgEdr cMeBaHkarpþl;viPaKTanBIGñkTaMgGs;enaH Edl)an

CYy nig pþl;rUbft taragnana nig rUbPaB. RbsinebImankarePøccenøaH

shB½n§GnþrCatiCMgWtaLasesmIu nig GñkniBnæ eBjcitþnwgbEnßmesckþIEføg

GMNrKuNkñúgkare)aHBumÖesovePAenH enAelIkeRkay²eTot.

esovePAenH epþateTAelItMrUvkarrbs;GñkCMgWtaLasesmIu. ral;kic©xitxMBiBN’naGMBICMgW nig

lkçN³rbs;va RtUv)aneFVItamrebobgay² ehIy)anbBa©úÚlbEnßmnUvkareCOnelOnEpñkKøInik nig

viTüasaRsþfμI² Edl)anrkeXIjkñúgb:unμanqñaMcugeRkayenH kñúgkarBüa)al nig

karBarCMgWtaLasesmIu. karpþl;nUvBt’manfμI² nig RtwmRtUvdl;GñkCMgWtaLasesmIuCaTUeTA

kñúgsklelak CakmμvtßúmYykñúgcMeNamkmμvtßúcMbg² nig CaehtuplnaMeGaybegáItCa

shB½n§GnþrCatiCMgWtaLasesmIu enHeLIg.

esovePAenHRtUv)anERbsMrYlCaexmrPasa nig e)aHBum<pSayeday

mnÞIreBTükumarCati

100/ mhavifI shB½n§rusSIú/ xN½ÐTYleKak/ PñMeBj/ km<úCa

GnuBaØatisiT§ibkERbBIÉksaredIm nig pþl;mUlniFieday

shB½n§GnþrCatiCMgWtaLasesmIu ¬s>G>t¦

Thalassaemia International Federation (TIF)

P.O. Box 28807, 2083 Nicosia, 31 Ifigenias Str., 2007 Nicosia, Cyprus

Website: http://www.thalassaemia.org.cy

ERbsMrYlCaexmrPasa

evC¢bNÐit Can supl

RKUeBTüÉkeTseraKkumar nig elahitsaRsþ

mnÞIeBTükumarCati PñMeBj

evC¢bNÐit tU eBCra

naykrgTTYlbnÞúkbec©keTs

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ISBN : 9963-623-40-9 ERbsMrYlCaexmrPasa nig e)aHBum<enAPñMeBj km<úCa qñaM 2008


Documents

Can supl tU eBCra - thalassaemia.org.cy · taLasesmIubNþalmkBI {eGm:UkøÚb‘ÍnxusFmμta} b¤ eGm:UkøÚb‘ÍnEdlmanrcnasm