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GLOMERULOSCL EROZAFOCAL A SI SEGMENTARA
Dr. Iulia-Tamara Tudosa
Prof.Dr. Dan Vladutiu
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APF: menarha- 1 ani!" #- $%fara probleme! & ' spontane
(la"-1$ #')APP : *T' primara grad II
IM gr.III+#itiaza renalaInfectii T # repetate
CVM: corespunzatoare %eaga consum - alcool, tigari,cafea
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TT la momentul ultimei internari
rednison -&tb/zi0meran mg/zi
2urosemid mg/zi3nap 1 mg/zi (daca T'41& /5 mm*g)h6siotens , mg/ziMetoprolol $7mg8$/zi
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Istoricul boliiDebut 1" 9ec.$ 1 cu:
sdr.edematos, cefalee, ameteli, dureri cu caracter de arsuratoracica, oligurie, valori tensionale crecute(T' 1; /1 mm*g)
#<= >istrita-9g: SN de cauza neprecizata, IRA,
pleurezie bazala bilaterala, IM grII, IVS
CORTICOTERAPIA din !Dec" !#$ cu Predni%&n'!(g)zi si tt diuretic cu 2urosemid mg/zi.
#e rec. internare in ?linica %efrologie ?luj-%apoca pt. precizare dg.
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Istoricul bolii!*"!#" !#* + # "!#" !#* #?<= %efrologie pt:
3deme generalizate, valori tens.@, Ascaune moi/zi, rectoragii(1episod anterior internarii cu o zi), inapetenta, astenie (de 7sapt)#e investigheaza: =#: ascita importanta, ambii rinichi %!
gastroenterologic: 39#, 39I 3B. lab:
retentie azotata moderata: creat A, 7/ ,$mg/dl! uree-1$ mg/dl!2C 3 I 1Aml/min/1,;Am$
*ipoalbuminemie $,Ag/dl! hipoproteinemie ,;g/dlroteinurie 11,$g/g creatinina3#=: proteine 7 mg/dl! er6 7 /ul! densitate 1 $7=rocultura -negativa
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Istoricul boliiImunologie: IgCD, IgM usor@, '%' negativ.
3lectroforeza proteine serice: aspect monoclonal -zona beta.*ipogammaglobulinemie9at.#% sever si hipoproteinemie tt. anticoagulant cu 2raBi-stop din 11.Ian! 2urosemid si %efriB!
3nalapril7mg, h6siotens , B$/zi,'spenter;7mg/zi3Bt. pacientei cu transfer la I.0.?.%. – *emato-logie suspiciune M.M. punctie sternala, 32 IC si 32compo prin I2! gr.calota infirmare MM
# "!#" !#*+ '"!#" !#* –Internare %efrologie%dr"ne-r&tic pri(ar biopsie "!#" !#* .S/S
Imunofluorescenta: %egativa pt: IgC, Ig',IgM, ?A,?1E, 8,F.
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Istoricul bolii-Imunofluorescenta: %egativa pt: IgC, Ig',IgM, ?A,?1E, 8, F.
#e reduce doza de rednison la jumatate &tb/zi din$ .Ian.$ 17.
#0"! " !#* – ?ontrol: 'lterarea starii generale, frison,astenie fizica marcata.3B.lab: DDD a proteinuriei la aproB.$g/g creatinina,ameliorarea functiei renale (creatinina 1,;mg/dl),hipopotasemie usoara si hiposodemie importanta, alcaloza
respiratorie (hiperventilatie).#-a adm. Tt. de 3*3 si anti*T'. #-a rec internare in
regim continuu.
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&ia'nostic (e eta)a
#dr nefrotic in remisie>? #td II F90HI (C#2#)'nemie secundara renala*ipopotasemie*iponatremie severa*T' primara Crd.IIIIM gr.II! I+#
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E*"LA+:creat 1, $mg/dl! uree 71mg/dl! 2C 71ml/min/1,;Am$)
*iposodemie severa %a 11&,"mmol/*ipopotasemie usoara F A,&mmol/'#T = venos: alcaloza metabolica*ipoproteinemie usoara &,Ag/dl
'lbumina A,&g/dlroteinurie $,"g/g creatinina3#=: proteine1 mg/dl, er67 /ul, densitate 1 17Imunologie: IgM@ usor *iper?ho A1 mg/dl,*ipertrigliceridemie TC $77mg/dl2unctia hepatica %- ' 'T 1&=/ ! '#'T 1A=/
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ParaclinicRgr" T&racica 4 relatii n&r(ale
E5.: RS, AV6'*)(in, A7 8RS69'! grade, -ara(&di-icari i%c e(ice"
;S: RD+ u%&ara in&(&genitate %inu%ala, p&%ibiladi%creta dilatare piel&caliceala, pac etul 1a%culare%te %i el (ai bine reprezentat, V;+ n&r(ala "
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&ia'nostic ,nalSdr ne-r&tic in re(i%ie
<CR Std II 5DO8I =.S/S>Ane(ie %ecundara renalaHip&p&ta%e(ie
Hip&natre(ie %e1eraHTA pri(ara .rd"IIIIM gr"II2 IVS
Di%lipide(ie (i?taHe(&r&izi interni tr&(b&zatiDi1erticuli c&l&n tran%1er%
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Trata ent reco an(at
?ontinuarea tt medicamentos cu:3nalapril 1 mg/zi (daca T' 41& 1 mm*g)Tertensif # 1,7mg/zi (in caz de edeme)h6siotens , mg/ziMetoprolol $7mgB$/zi0meran mg/zi?onsult psihiatric 9ispensarizare nefrologica teritoriala, revine lacontrol in clinica peste cca & sapt si mai repede, lanevoie.
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E.olutie
e parcursul internarii prezinta stare generala buna,afebrila, fara edeme, diureza prezenta ($ ml), valoritensionale constante sub medicatie.
#-a instituit tratament de 3*3, cu corectarea lenta ahiposodemiei
acienta prezinta astenie marcata, cefalee difuza,tremor membre superioare, simptomatologieinterpretata ca fiind consecutiva tratamentului curednison, in conteBtul unei tulburari psihoemotionalecu somatizare.
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GSFS2orma cronic progresiva de b. renala, frecventa cauza deafectare glomerulara la adulti, caracterizata prin
proteinurie asimptomatica sau #%asociat sau nu cu I .
@eziune focala J 7 K din glomeruli sunt afectati segmentara J 7 K din ansele unui glomerul afectate
eziunea principala: leziune podocitaraL stergereaN pedicelelor (difuza in segmentele sclerotice,
partiala On lobulii %)Lcolaps capilare glomerulareLcicatrizare
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PATOGENEZA
GSFS i(io)atica1 )ri ara
GSFS secun(ara:
Medicamente - heroină, IFN, ciclos orina,amidronatVirusuri - !e atitic B, !IV, Par"o"irusfactori hemodinamici
cu reducerea masei renale# rinichi solitar,allo$refa renala, a$ene%ie renala unilaterala,re&u' "e%icoureteralo(e%itate im ortanta
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TT GSFS
#> Igien&+dieteticStatine, antic&agulantDiuretice 9)+ %upli(entare de p&ta%iu> Trat eti&l&gic
> Trata(ent pat&genetic 4 .S/S pri(ara:Predni%&n =# (g) g c&rp)zi> 4 re(i%iune in $!+0!B dincazuri2 cei ce ra%pund la c&rtic&terapie au pr&gn&%tic peter(en lung (ai bun
Cicl&%p&rina) Tacr&li(u% 9 Predni%&nMic&-en&lat (&-etilCicl&-&%-a(ida + a%&ciata in caz de lip%a de ra%pun% lagluc&c&rtic&izi
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E.olutie si )ro'nostic
.S/S idi&patice:- emisiune spontana (7-&7K)
.completa proteinurieJ ,Ag/$ h
.partiala Dp. JA,7g/$ h, si cu cel putin 7 K
fata de nivelul initial
I rapid progresiva (7-17K)
I lent progresiva ($ -& K)roteinurie persistenta (1 -A K)
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Factori (e )'" ne'ati.#eBul masculinasa neagra#% (suprav. renala la 1 ani este de numai A -7 lacazurile cu #%, fata de 5 K in formele non-
nefrotice)K de glomeruli afectati (4$7K)'fectarea tubulara
I la debut