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8/18/2019 Approach to Hepatomegaly, Splenomegaly and Hepatosplenomegaly
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Approach toHepatosplenomega
ly24/8/2015
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Case 1• MA, 2 years old boy
• NKMI, NKDA
• Presented with e!er or " wee#s asso$iated with$o%&h and r%nny nose or 1 wee#
• No abdo'inal distension noted
• (as bro%&ht to )P to see# treat'ent and$o'*leted one $o%rse o a'o+i$illin, howe!er
sy'*to's was not i'*ro!ed
• -%bse.%ently $hild was bro%&ht to Pri!ate os*ital
• nre'ar#able antenatal, birth and de!elo*'entalhistory
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Physi$al e+a'inationAlert, *er%sion &ood, not ta$hy*noei$, 'ild
*allor, not a%ndi$e
P%lse rate 1"0 3e'* "
P 68/50
7%n&s and C- %nre'ar#able
Abdo'en Liver 3cm and spleen 2cm *al*ablebelow $ostal 'ar&in
Shotty cervical and inguinal lymph nodepalpable
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Workingdiagnosis??
Investigation??
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In!esti&ation doneDate 2!"!2#$ 3!"!2#$ %!"!2#$e'o&lobin 52 5.4 5.0
(C &'2 34.35 34.63
Platelet 25 31 28
9C $o%nt showed ly'*ho$yte *redo'inant
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In!esti&ation doneDate 2!"!2#$ 3!"!2#$ %!"!2#$e'o&lobin 52 5.4 5.0
(C 2 34.35 34.63
Platelet 25 31 28
9C $o%nt showed ly'*ho$yte *redo'inant
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Date 2!"!2#$ 3!"!2#$ %!"!2#$
Urea 5.2 5.5 4.1
Creatinine 34 31 35
Na 139 136 421
K 4.6 4.6 4.4
Cl
S.Bilirubin 2.1
Albumin 45
Globulin 32
AL 10
AL! 88
otal !rotein ##
L$% 6#4
Uri& A&i' 335
C(! 15
Blood C&S NG NG
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Any other (urtherinvestigation?
8/18/2019 Approach to Hepatomegaly, Splenomegaly and Hepatosplenomegaly
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• e* , e* C, I :1"/8/15; N<
• )*+ urgent, 7e%#o$ytosis with
ly'*ho$ytosis Presen$e o 32- blasts cellswhi$h are s'all in si=e with s$anty $yto*las'and in$ons$*i$%o%s n%$leoli s%&&esti!e oA$%te 7e%#e'ia :'ost li#ely A77;
•)eripheral blood Immunophenotypingwas sent showed precursor *.ALL
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Case 2ID, 2 years old 'alay boys
NKMI, NKDA
*resented with 2 'onths history o abdo'inal *ain
asso$iated with ew e*isodes o *assin& loose stoolInter'ittent *ain whi$h *artially resol!ed ater abdo'inal
'assa&e
ro%&ht to Distri$t os* twi$e, was treated as &astritis
Mother $lai'ed she elt so'e 'ass while 'assa&in& theabdo'en
-%bse.%ently $hild was bro%&ht to Pri!ate )P
Child was reerred to ) as Pri!ate )P noti$e abdominalmass while *eror'in& *hysi$al e+a'ination
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Physi$al >ndin&Alert, *er%sion &ood, war' *eri*hery, 'ild
*allor
P 6?/5, P
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Workingdiagnosis??
Investigation??
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Date 2/!&!$
ae'o&lobin 101 3(C 1?6
Platelet ??
0rea %'#
Creatinine 4"
Na 1"4
K 42
-ilir%bin 1
Alb%'in 4"A73 1"
A7P 182
3Protein 8"
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Any (urtherinvestigation??
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3%'o%r 'ar#er:26//2015;
A+) B1??00 ater dil%tion:I%/'7;
0S abdomen :"0//2015; showed lar&e 'ass at theri&ht ilia$ ossa re&ion e+tendin& to the e*i&astri$re&ion, li#ely li!er in ori&in
r&ent 11 thora4!abdomen:?/8/2015; showedlar&e li!er 'ass 'ost li#ely a he*atoblasto'a with
*%l'onary nod%les
-%bse.%ently $hild was reerred to P) or %rther'ana&e'ent
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Approach to Hepatomegaly5Splenomegaly andHepatosplenomegaly
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7i!er -it%ated at ri&ht hy*o$hondria$ re&ion
Mo!es down with ins*iration
nable to &et abo!enlar&ed to
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e*ato'e&aly• 3he *resen$e o a *al*able li!er does not
always indi$ate he*ato'e&aly
•
In &eneral, a li!er ed&e greater than 3'$ cmin ne6borns and greater than 2 cm inchildren below the ri&ht $ostal 'ar&ins%&&ests enlar&e'ent
•7ormal 8ange o( Liver span1 wee# o a&e is 45 to 5 $'
At 12 years, the nor'al !al%e or boys is to8 $' and or &irls is ? to ?5 $'
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Pitalls in he*ato'e&aly• 3he liver can be displaced in(eriorly by the
dia*hra&' or thora$i$ or&ans, &i!in& thei'*ression o he*ato'e&aly
• Children who ha!e orthopedic abnormalitiess%$h as narrow $hest walls or *e$t%s e+$a!at%'
$an erroneo%sly a**ear to ha!e he*ato'e&aly
• A nor'al !ariant o the ri&ht lobe o the li!er,$alled a 8iedel lobe, 'ay e+tend ar below theri&ht $ostal 'ar&in and be $on%sed as*atholo&i$ he*ati$ enlar&e'ent
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Ca%ses o he*ato'e&aly9echanism• Ina''ation
• In>ltrati!e
• Ina**ro*riate stora&e
• as$%lar $on&estion• illiary obstr%$tion
9neumonic :SHI8;
•Str%$t%ral, Stora&e/'etaboli$
• Hae'atolo&i$al, Heart
• Ine$tion, ina''atory, in>ltrati!e
• 8eti$%loendothelial, 8he%'atolo&i$al
• %'or, ra%'a
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9echanism Disease
Ina''ation •In(ections
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9echanism Disease
Ina**ro*riate -tora&e •LipidE &a%$herGs ds, Nie'annPi$#
•1arbohydrateE&ly$o&en stora&eds, hereditary r%$toseintoleran$e, &ala$tose'ia,M%$o*olysa$$haridoses
•)rotein!amino acidE
tyrosinae'ia, %rea $y$le dee$t
•9ineralE wilson ds,hae'o$hro'atosis
•lectrolyte transportE $ysti$
>brosis
•7utritionE total *arenteraln%trition
•)roteaseE al*haE1Eantitry*sin
de>$ien$y
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9echanism Disease
In>ltrati!e •)rimary neoplastic tumorse*atoblasto'a/e*ato$ell%lar$ar$ino'a
•)rimary non.neoplastictumorse'an&io'a,he'an&ioendothelio'a,terato'a, o$al nod%lar
hy*er*lasia
•9etastatic or disseminatedtumorsE7e%#e'ia, ly'*ho'a,ne%roblasto'a, histio$ytosis
•1ystsEParasiti$ $yst, $holedo$hal$yst, *oly$ysti$ li!er disease
•Hemophagocytic syndromes
•4tramedullaryhematopoiesisEthalase'ia,
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9echanism Disease
as$%lar Con&estion •SuprahepaticCon&esti!e heart ail%re
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8/18/2019 Approach to Hepatomegaly, Splenomegaly and Hepatosplenomegaly
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8/18/2019 Approach to Hepatomegaly, Splenomegaly and Hepatosplenomegaly
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8/18/2019 Approach to Hepatomegaly, Splenomegaly and Hepatosplenomegaly
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-*leen• 7o$ated at let hy*o$hondria$ re&ion
• Mo!e down with ins*iration
•
nable to &et abo!e• s%ally not *al*able
• -'ooth, re&%lar 'ar&in
• Presen$e o s*leni$ not$h
• 3ra%beGs s*a$e H 6, 10 and 11th rib s*a$es
• nlar&ed towards
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-*leno'e&alyNor'al -*leen is *al*able below the let
$ostal 'ar&in in nearly oneEthird o neonates
owe!er, a splenic edge (elt more than 2cm below the ribs de>nitely is an abnor'al>ndin&
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Ca%ses o s*leno'e&aly• 9oderate or massive s*leno'e&aly:8$' or
'ore below let $ostal 'ar∈
• Mne%'oni$ 1HI9)S
1, 1ardia$ and conne$ti!e tiss%e diseases
H Hae'atolo&i$al
I, Ine$tion, In%ry
9 9ali&nan$y) )ortal y*ertension
S stora&es diseases
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Ca%ses o -*leno'e&alyModerate s*leno'e&aly9echanism Diseases
Ine$tion •*acterialE-%ba$%te ba$terialendo$arditis, -e*ti$ae'ia,-*leni$ abs$ess, 3y*hoid e!er,
r%$ellosis, 7e*tos*irosis, 3%ber$%losis, Cat s$rat$hdisease
•
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9echanism 1auses
Ina''ation/disordered
i''%nore&%lation
-7, brosis•SuprahepaticE %dd Chiarisyndro'e
-*leni$ !ein thro'bosis
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9echanism Disease
Mali&nan$y 7e%#e'ia, odinGs ly'*ho'a,Non odinGs ly'*ho'a
-tora&e disease 7i*idosesM%$o*olysa$$haridosesA'yloidoses
-tr%$t%ral e'ato'a-*leni$ $yst/*se%do$yste'an&io'a7y'*han&io'a
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Massi!e -*leno'e&aly
9echanisms DiseasesIne$tions Chroni$ 'alaria
-$histoso'iasisis$eral lesh'ananiasis:#alaEa=ar;
+tra'ed%lary hae'ato*oiesis ereditary s*hero$ytosis 3halasae'ia Maor
Con&esti!e Non $irrhoti$ *ortal >brosis
Mali&nan$y NonEhodinGs ly'*ho'aMetastati$ diseaseCM7
-tora&e disease 7i*idoses disease
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e*atos*leno'e&aly9echanism Diseasee'atolo&i$al 3halasae'ia,'yelo*rolierati!e
ds and ly'*ho*rolierati!e ds
Ine$tion , 3@brosis
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I'*ortant historyNeonatesImportant history Associated diseases!problems
Prolon&ed a%ndi$e +trahe*ati$ biliary atresiaAl*haE1 antitry*sin de>$ien$y)ala$tose'ia
o'ittin& and diarhhea Metaboli$/stora&e disorderse*ti$ae'ia
9a'ily history o early inantdeath or he*ati$,ne%rode&enerati!e, or *sy$hiatri$
disease
Metaboli$ diseases
Antenatal history 3@brosis
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Child
Important history )ossible diseases!problems9e!er Ine$ti!e $a%ses
9orei&n tra!ellin&, in&estion oshell>sh or dr%&s
iral he*atitisParasiti$ ine$tion
history o *oor wei&ht &ain,
!o'itin&, diarrhea, distin$ti!eodors, loss o de!elo*'ental'ilestones, $o'*le+ sei=%redisorder, or hy*otonia
Metaboli$ disorder
A$holi$ stool or dar# $olor %rine e*ati$ dys%n$tion
istory o blood trans%sion iral e*atitis or C, IDr%& history & N-AID-, Isonia=id,
*ro*ylthio%ra$il, ands%lona'ides
history o ina''atorybowel disease ori''%node>$ien$y
Pri'ary s$lerosin& $holan&itis
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Asso$iated *hysi$al
>ndin&sAssociated physical =ndings )ossible problem!diseasesAne'ia •e'atolo&i$al
disorder/'ali&nan$y•Ine$tion
Fa%ndi$e •e'olyti$ ane'ia•e*ati$ dys%n$tion•illiary tree dys%n$tion
ye 9indin&sCatara$ts
KayserE9leis$her rin&sChorioretinitisPosterior e'bryoto+on
• 3@
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In!esti&ation
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hank you
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