TUGAS REFERAT
ATRESIA ATRESIA BILIER
Disusun oleh: dr. Disa Almira Yamin
Pembimbing: dr. Kamal Sumardin dr. Dian Arrishanty
PROGRAM INTERNSIP RUMAH SAKIT UMUM DAERAH CILEGON BANTEN !"#
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BAB I PENDAHULUAN
I.1. Latar Belakan
Pen$eb%b Pen$eb%b &oles'%sis &oles'%sis e&s'(%he)%'i e&s'(%he)%'i& & neon%'%l neon%'%l $%ng 'e(b%n$%& 'e(b%n$%& %*%l%h %'(esi% bilie(+ A'(esi% bilie( 'e(,%*i &%(en% )(oses in-l%m%si be(&e)%n,%ng%n $%ng men$eb men$eb%b& %b&%n %n &e(us%& &e(us%&%n %n )(og(e )(og(esisi- )%*% )%*% *u&'us *u&'us bilie( bilie( e&s'(%h e&s'(%he)% e)%'i& 'i& sehingg sehingg%% men$eb%b&%n h%mb%'%n %li(%n em)e*e+ .%*i/ %'(esi% bilie( %*%l%h 'i*%& %*%n$% %'%u &e0iln$% lumen )%*% seb%gi%n %'%u &eselu(uh%n '(%&'us bilie( e&s'(%he)%'i& $%ng men$eb%b&%n men$eb%b&%n h%mb%'%n %li(%n em)e*u+ A&ib%'n$ A&ib%'n$%% *i *%l%m h%'i *%n *%(%h 'e(,%*i 'e(,%*i )enum) )enum)u&% u&%n n g%(%m g%(%m em)e*u em)e*u *%n )ening )ening&%' &%'%n %n bili(u bili(ubin bin *i(e&+ *i(e&+ H%n$% H%n$% 'in*%&%n be*%h $%ng *%)%' meng%'%si %'(esi% bilie(+ Bil% 'in*%&%n be*%h *il%&u&%n )%*% usi% 1 minggu/ %ng&% &ebe(h%sil%nn$% %*%l%h 1#2/ 'e'%)i bil% )embe*%h%n *il%&u&%n )%*% usi% 3 1 minggu m%&% %ng&% &ebe(h%sil%nn$% h%n$% 4#2+ Oleh &%(en% i'u *i%gnosis %'(esi% bilie( h%ms *i'eg%&&%n se*ini mung&in/ sebelum usi% 1 minggu
I.!. E"idemi#l#i
A'(esi% bilie( *i'emu&%n )%*% " *%(i "5+!!! &el%hi(%n+ R%sio %'(esi% bilie( )%*% %n%& )e(em)u%n *%n %n%& l%&i6l%&i %*%l%h :"+ Mes&i ,%(%ng 'e'%)i .uml%h )en*e(i'% %'(esi% bilie( $%ng *i'%ng%ni Rum%h S%&i' Ci)'o M%ngun&usumo 7RSCM8 )%*% '%hun !!6!!4/ men0%)%i 49641 b%$i %'%u 4 )e(sen *%(i "# b%$i be()en$%&i' &uning %&ib%' &el%in%n -ungsi h%'i+ Se*%ng&%n Di Ins'%l%si R%%' In%) An%& RSU D(+ Su'omo Su(%b%$% %n'%(% '%hun ";;;6!!< *%(i ";9! )en*e(i'% (%%' in%)/ *i*%)%' ;# )en*e(i'% *eng%n )en$%&i' &uning g%nggu%n -ungsi h%'i *i *%)%'&%n %'(esi% bilie( ; 7;/<28+ D%(i ;!< &%sus %'(esi% bilie( $%ng 'e(*%-'%( *i lebih "!! ins'i'usi/ %'(esi% bilie( *i*%)%' )%*% (%s K%u&%si% 7#28/ be(&uli' hi'%m 7!28/ His)%ni& 7""28/ Asi% 728 *%n In*i%n Ame(i&% 7"/528
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K%sus A'(esi% Bilie( *il%)o(&%n seb%n$%& 5="!!+!!! &el%hi(%n hi*u) *i Bel%n*%/ 5/"="!!+!!! &el%hi(%n hi*u) *i Pe(%n0is/ #="!!+!!! &el%hi(%n hi*u) *i Ingg(is/ #/5="!!+!!! &el%hi(%n hi*u) *i Te>%s/ 9="!!+!!! &el%hi(%n hi*u) *i Aus'(%li%/ 9/<="!!+!!! &el%hi(%n hi*u) *i USA/ *%n "!/#="!!+!!! &el%hi(%n hi*u) *i *uni%
I.$ A%&ESIA BILIE&
P(oses in-l%m%si be(&e)%n,%ng%n $%ng men$eb%b&%n &e(us%&%n )(og(esi- )%*% *u&'us bilie( e&s'(%he)%'i& sehingg% men$eb%b&%n h%mb%'%n %li(%n em)e*u+ .%*i/ %'(esi% bilie( %*%l%h 'i*%& %*%n$% %'%u &e0iln$% lumen )%*% seb%gi%n %'%u &eselu(uh%n '(%&'us bilie( e&s'(%he)%'i& $%ng men$eb%b&%n h%mb%'%n %li(%n em)e*u+ A&ib%'n$% *i *%l%m h%'i *%n *%(%h 'e(,%*i )enum)u&%n g%(%m em)e*u *%n )ening&%'%n bili(ubin *i(e& 1/ '( ) P%sien *eng%n %'(esi% bilie( *%)%' *ib%gi men,%*i g(u)/ $%&ni : "+ Pe(in%'%l -o(m 7 Isol%'e* Bili%($ A'(esi%8 #5 ? ;! 2 Ben'u& ini *i'emu&%n )%*% neon%'%l *%n b%$i be(us i% 61 minggu+ In-lm%si %'%u )e(%*%ng%n $%ng )(og(esi@ )%*% s%lu(%n em)e*u e>'(%he)%'i& 'imbul se'el%h l%hi(+ Ben'u& ini 'i*%& mun0ul be(s%m% &el%in%n 0ongeni'%l l%inn$%+ + e'%l Emb(ioni0 -o(m "! ? 45 2 Ben'u& ini *i'%n*%i *eng%n 0holes'%'is $%ng mun0ul %m%' 0e)%'/ *%l%m minggu &ehi*u)%n )e('%m%+ P%*% ben'u& ini/ s%lu(%n em)e*u 'i*%& 'e(ben'u& )%*% s%%' l%hi( *%n bi%s%n$% *ise('%i *eng%n &el%in%n 0ongeni'%l l%inn$% se)e('i si'us in@e(sus/ )ol$s)leni%/ m%l(o'%si/ *%n l%in6l%in+
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G%mb%( "+ A'(esi% Bilie(
G%mb%( + Sis'em He)%'obile(
K%s%i meng%,u&%n &l%si-i&%si %'(esi% bilie( seb%g%i be(i&u' : I+ A'(esi% 7seb%gi%n %'%u 'o'%l8 *u&'us bilie( &omunis/ segmen )(o&sim%l )%'en+ II%+ Obli'e(%si *u&'us he)%'i&us &omunis 7*u&'us bilie( &omunis/ *u&'us sis'i&us/ *%n &%n*ung em)e*u semu%n$%no(m%l8+ IIb+ Obli'e(%si *u&'us bilie(&omunis/ *u&'us he)%'i&us &omunis/ *u&'us sis'i&us+ K%n*ung em)e*u no(m%l+ III+ Semu% sis'em *u&'us bilie( e&s'(%he)%'i& meng%l%mi obli'e(%si/ s%m)%i &e hilus+
Ti)e I *%n II me(u)%&%n ,enis %'(esi% bilie( $%ng *%)%' *io)e(%si (correctable), se*%ng&%n 'i)e III %*%l%h ben'u& $%ng 'i*%& *%)%' *io)e(%si (noncorrectable). S%$%ngn$% *%(i semu% &%sus %'(esi% bilie(/ h%n$% "!2 $%ng 'e(golong 'i)e I *%n II 1
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G%mb%( 4+ Kl%si-i&%s A'(esi% Bilie(
III.1.a. Eti#l#i
E'iologi %'(esi% bilie( m%sih belum *i&e'%hui *eng%n )%s'i+ Seb%gi%n %hli men$%'%&%n b%h% -%&'o( gene'i& i&u' be()e(%n/ $%ng *i&%i'&%n *eng%n %*%n$% &el%in%n &(omosom '(isomi "9/"1 *%n " se('% 'e(*%)%'n$% %nom%li o(g%n )%*% 4!2 &%sus %'(esi% bilie(+ N%mun/ seb%gi%n bes%( )enulis be()en*%)%' b%h% %'(esi% bilie( %*%l%h %&ib%' )(oses in-l%m%si $%ng me(us%& *u&'us bilie(/ bis% &%(en% in-e&si %'%u is&emi 1
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H%l )en'ing $%ng h%(us *i&e'%hui %*%l%h b%h% %'(esi% bilie( bu&%nl%h )en$%&i' $%ng *i'u(un&%n+ K%sus *%(i %'(esi% bilie( )e(n%h 'e(,%*i )%*% b%$i &emb%( i*en'i&/ *im%n% h%n$% " %n%& $%ng men*e(i'% )en$%&i' 'e(sebu'
,
III.1.-. Pat#isi#l#i
Mes&i)un his'o)%'ologi %'(esi% bilie( 'el%h *i)el%,%(i se0%(% e&s#si- *%l%m be*%h s)esimen *%(i sis'em bilie( e>'(%he)%'i0 $%ng *i*%)%' *%(i b%$i $%ng meng%l%mi )o('oen'e(os'om$/ )%'ogenesis &el%in%n ini m%sih &u(%ng *i)%h%mi+ M%s%l%h A'(esi% Bilie( $%ng mun0ul )%*% ben'u& -e'%l be(hubung%n *eng%n %nom%li &ongeni'%l l%in+
N%mun/ )%*% ben'u& $%ng lebih umum/ $%&ni 'i)e
neon%'%l *i'%n*%i oleh lesi in-l%m%si $%ng )(og(esi-/ $%ng *i%&ib%'&%n in-e&si %'%u (%0un $%ng men$eb%b&%n (us%&n$% s%lu(%n em)e*u+ Agen in-e&si $%ng 'el%h *i'eli'i oleh bebe(%)% s'u*i 'el%h mengi*en'i-i&%si )ening&%'%n 'i'e( un'u& (eo@i(us %n'ibo*i 'i)e 4 )%*% )%sien *eng%n %'(esi% bilie( bil% *ib%n*ing&%n *eng%n &on'(ol+ i(us l%inn$% $%ng 'e(i*en'i-i&%si/ 'e(m%su& (o'%@i(us *%n si'omeg%lo@i(us 7CM8/+ 1( *
G%mb%( <+ His'o)%'ologi A'(esi% Bilie(
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III.1./. eala Klinik
T%n)% mem%n*%ng e'iologin$%/ ge,%l% *%n '%n*% &linis u'%m% &oles'%sis neon%'%l %*%l%h i&'0(us/ 'in,% %&oli&/ *%n u(in $%ng be(%(n% gel%)+ N%mun/ 'i*%& %*% s%'u )un ge,%l% %'%u '%n*% &linis $%ng )%'ognomoni& un'u& %'(esi% bilie(+ Ke%*%%n umum b%$i )%*% %&'u l%hi( bi%s%n$% b%i&+ I&'e(us bis% 'e(lih%' se,%& l%hi( %'%u '%m)%& ,el%s )%*% minggu &e 4+ Koles'%sis e&s'(%he)%'i& h%m)i( sel%lu men$eb%b&%n 'in,% $%ng %&oli&+ Sehubung%n *eng%n i'u seb%g%i u)%$% )en,%(ing &%s%( '%h%) )e('%m%/ *i%n,u(&%n mel%&u&%n )engum)ul%n 'in,% 4 )o(si+ Bil% sel%m% bebe(%)% h%(i &e'ig% )o(si 'in,% '0'%) %&oli&/ m%&% &emung&in%n bes%( *i%gnosisn
%$%*%l%h &oles'%sis
e&s'(%he)%'i&+ Se*%ng&%n
)%*% &oles'%sis
in'(%he)%'i&/ %(n% 'in,% *em)ul be(-lu&'u%si )%*% )0m0(i&s%%n 'in,% 4 )o(si Ikterus
I&'e(us 'imbul *i&%(en%&%n he)%( $%ng imm%'u( )%*% b%$i b%(u l%hi(+ No(m%ln$% i&'e(us %&%n menghil%ng )%*% 96"! h%(i se'el%h l%hi(+ Te'%)i b%$i *eng%n %'(esi% bile(/ i&'e(usn$% %&%n sem%&in n$%'% *%l%m 64 minggu Urin yan -er2arna ela"
H%l ini *iseb%b&%n &%(en% bili(ubin $%ng mening&%' *%l%m *%(%h/ &emu*i%n bili(ubin 'e(-il'(%si mel%lui gin,%l/ *%n *ibu%ng mel%lui u(in+ 3eses A/h#li/
eses %0holi0 'imbul *i&%(en%&%n 'i*%& %*%n$% bili(ubin $%ng m%su& &e *%l%m usus un'u& me%(n%i -eses+ Penurunan -erat -adan
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III.1.d. Pemeriksaan 3isik
P%*% )eme(i&s%%n -isi&/ 'i*%& %*% 'emu%n $%ng )%'hognomoni0 un'u& %'(esi% bilie( •
B%$i *eng%n %'(esi% bilie( bi%s%n$% meng%l%mi )e('umbuh%n no(m%l *%n )ening&%'%n be(%' b%*%n sel%m% minggu )e('%m% &ehi*u)%n+
•
He)%'omeg%li
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•
S)lenomeg%li menun,u&&%n si(osis $%ng )(og(esi- *eng%n hi)e('ensi )o('%l+
•
Mu(mu( ,%n'ung menun,u&&%n %*%n$% &el%in%n )%*% ,%n'ung
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III.1.e. Pemeriksaan Penunan
Belum %*% s%'u )un )eme(i&s%%n )enun,%ng $%ng *%)%' se)enuhn$% *i%n*%l&%n un'u& membe*%&%n %n'%(% &oles'%sis in'(%he)%'i& *%n e&s'(%he)%'i&+ Se0%(% g%(is bes%(/ )eme(i&s%%n *%)%' *ib%gi men,%*i 4 &elom)o&/ $%i'u )eme(i&s%%n : "8 L%bo(%'o(ium (u'in *%n &husus un'u& menen'u&%n e'iologi *%n menge'%hui -ungsi h%'i 7*%(%h/ u(in/ 'in,%8 8 Pen0i'(%%n/ un'u& menen'u&%n )%'ensi s%lu(%n em)e*u *%n menil%i )%(en&im h%'i 48 Bio)si h%'i/ 'e(u'%m% bil% )eme(i&s%%n l%in belum *%)%' menun,%ng *i%gnosis %'(esi% bilie(+
15 Pemeriksaan la-#rat#rium
a) Pemeriksaan rutin P%*% se'i%) &%sus &oles'%sis h%(us *il%&u&%n )eme(i&s%%n &%*%( &om)onen bili(ubin un'u& membe*%&%nn$% *%(i hi)e(bili(ubinemi% -isiologis+ Sel%in i'u *il%&u&%n )eme(i&s%%n *%(%h 'e)i leng&%)/ u,i -ungsi h%'i/ *%n g%mm%6GT+ K%*%( bili(ubin *i(e& < mg=*l 'i*%& sesu%i *eng%n obs'(u&si 'o'%l+ Pening&%'%n &%*%( SGOT=SGPT 3 "! &%li *eng%n )0ning&%'%n g%mm%6GT 5 &%li/ lebih meng%(%h &e su%'u &el%in%n he)%'oselule(+ Seb%li&n$%/ )ening&%'%n SGOT 5 &%li *eng%n )ening&%'%n g%mm%6GT 3 5 &%li/ lebih meng%(%h &e &oles'%sis e&s'(%he)%'i&+ Menu(u' i'ge(%l*/ &%*%( g%mm%6GT $%ng (en*%h 'i*%& men$ing&i(&%n &emung&in%n %'(esi% bilie(+ Kombin%si )ening&%'%n g%mm%6GT/ bili(ubin se(um 'o'%l %'%u bili(ubin *i(e&/ *%n %l&%li -os-%'%se mem)un$%i s)esi-isi'%s ;/;2 *%l%m menen'u&%n %'(esi% bilie(+
b) Pemeriksaan khusus
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Peme(i&s%%n %s)i(%si *uo*enum 7DAT8 me(u)%&%n u)%$% *i%gnos'i& $%ng 0u&u) sensi'i-/ 'e'%)i )enulis l%in men$%'%&%n b%h% )eme(i&s%%n ini 'i*%& lebih b%i& *%(i )eme(i&s%%n @isu%lis%si 'in,%+ P%l%s&% men$%'%&%n b%h% &%(en% &%*%( bili(ubin *%l%m em)e*u h%n$% "!2/ se*%ng&%n &%*%( %s%m em)e*u *i *%l%m em)e*u %*%l%h #!2/ m%&% 'i*%& %*%n$% %s%m em)e*u *i *%l%m 0%i(%n *uo*enum *%)%' menen'u&%n %*%n$% %'(esi% bilie(+
!5 Pen/itraan
a) Pemeriksaan ultrasonografi Theoni mengemu&%&%n b%h% %&u(%si *i%gnos'i0 USG 992 *%n *%)%' *i'ing&%'&%n bil% )eme(i&s%%n *il%&u&%n *%l%m 4 -%se/ $%i'u )%*% &e%*%%n )u%s%/ s%%' minum *%n sesu*%h minum+ Bil% )%*% s%%' %'%u sesu*%h minum &%n*ung em)e*u be(&on'(%&si/ m%&% %'(esi% bilie( &emung&in%n bes%( 7;!28 *%)%' *ising&i(&%n+ Dil%'%si %bno(m%l *u&'us bilie(/ 'i*%& *i'emu&%nn$% &%n*ung em)e*u/ *%n mening&%'n$% e&ogeni'%s h%'i/ s%ng%' men*u&ung *i%gnosis %'(esi% bilie(+ N%mun *emi&i%n/ %*%n$% &%n*ung em)e*u 'i*%& men$ing&i(&%n &emung&in%n %'(esi% bilie(/ $%i'u %'(esi% bilie( 'i)e I = *is'%l+
b) Sintigrafi hati Peme(i&s%%n sin'ig(%-i sis'em he)%'obilie( *eng%n iso'o) Te0hne'ium ;;m mem)un$%i %&u(%si *i%gnos'i& sebes%( ;1/<2+ Sebelum )eme(i&s%%n *il%&u&%n/ &e)%*% )%sien *ibe(i&%n -enob%(bi'%l 5 mg=&gBB=h%(i )e( o(%l/ *ib%gi *%l%m *osis sel%m% 5 h%(i+ P%*% &oles'%sis in'(%he)%'i& )eng%mbil%n iso'o) oleh he)%'osi' be(l%ngsung l%mb%' 'e'%)i e&s&(esin$% &e usus no(m%l/ se*%ng&%n )%*% %'(esi% bilie( )(oses )eng%mbil%n iso'o) no(m%l 'e'%)i e&s&(esin$% &e usus l%mb%' %'%u 'i*%& 'e(,%*i s%m% se&%li+ Di l%in )ih%&/ )%*% &oles'%sis in'(%he)%'i& $%ng be(%' ,ug% 'i*%& %&%n *i'emu&%n e&s&(esi iso'o) &e *uo*enum+ Un'u& mening&%'&%n sensi'i@i'%s *%n s)esi-isi'%s )eme(i&s%%n sin'ig(%-i/ *il%&u&%n )enghi'ung%n in*e&s he)%'i& 7)en$eb%(%n iso'o) *i h%'i *%n ,%n'ung8/ )%*% meni' &e6"!+ In*e&s he)%'i& 3 5 *%)%' men$ing&i(&%n &emung&in%n %'(esi% bilie(/ se*%ng&%n in*e&s he)%'i& 4 me(u)%&%n )e'un,u& &u%' %*%n$% %'(esi% bilie(+ Te&ni& sin'ig(%-i *%)%' *ig%bung *eng%n )eme(i&s%%n DAT/ *eng%n %&u(%si
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*i%gnosis sebes%( ;1/<2+ To((isi mengemu&%&%n b%h% *%l%m men*e'0&si %'(esi% bilie(/ $%ng 'e(b%i& %*%l%h mengg%bung&%n b%sil )eme(i&s%%n USG *%n sin'ig(%-i+
c) Liver Scan S0%n )%*% li@e( *eng%n menggun%&%n me'o*e HIDA 7He)%'obili%($ Imino*e%0e'i0 A0i*8+ Hi*% mel%&u&%n )emo'(e'%n )%*% ,%lu( *%(i em)e*u *%l%m 'ubuh/ sehingg% *%)%' menun,u&%n bil%m%n% %*% blo&%*e )%*% %li(%n em)e*u+
d) Pemeriksaan kolangiografi Peme(i&s%%n
ERCP
(Endoscopic
Retrograde
holangio
Pancreaticograph!) m0(u)%&%n u)%$% *i%gnos'i& *ini $%ng be(gun% un'u& membe*%&%n %n'%(% %'(esi% bilie( *eng%n &oles'%sis in'(%he)%'i&+ Bil% *i%gnosis %'(esi% bilie( m%sih me(%gu&%n/ *%)%' *il%&u&%n )eme(i&s%%n &ol%ngiog(%-i durante operasionam. S%m)%i s%%' ini )eme(i&s%%n &ol%ngiog(%-i *i%ngg%) seb%g%i b%&u em%s un'u& membe*%&%n &oles'%sis in'(%he)%'i& *eng%n %'(esi% bilie(+
$5 Bi#"si hati
G%mb%(%n his'o)%'ologi& h%'i %*%l%h %l%' *i%gnos'i& $%ng )%ling *%)%' *i%n*%l&%n+ Di '%ng%n seo(%ng %hli )%'ologi $%ng be()eng%l%m%n/ %&u(%si *i%gnos'i&n$% men0%)%i ;52/ sehingg% *%)%' memb%n'u )eng%mbil%n &e)u'us%n un'u& mel%&u&%n l%)%(%'omi e&s)lo(%si/ *%n b%h&%n be()e(%n un'u& )enen'u%n o)e(%si K%s%i+ Kebe(h%sil%n %li(%n em)e*u )%s0% o)e(%si K%s%i *i#'u&%n oleh *i%me'e( *u&'us bilie( $%ng )%'en *i *%e(%h hilus h%'i+ Bil% *i%me'e( *u&'us "!! !! u %'%u "5!
*il%&u&%n fro"en section )%*% s%%' l%)%(%'omi e&s)lo(%si/
un'u& menen'u&%n %)%&%h )o('oen'e(os'omi
*%)%' *i&e(,%&%n+ G%mb%(%n
his'o)%'ologi& h%'i $%ng meng%(%h &e %'(esi% bilie( mengh%(us&%n in'e(@ensi be*%h se0%(% *ini+ F%ng men,%*i )e('%n$%%n %*%l%h %&'u $%ng )%ling o)'im%l un'u& mel%&u&%n bio)si h%'i+ H%(us *is%*%(i/ 'e(,%*in$% )(oli-e(%si *u&'ule( 7g%mb%(%n his'o)%'ologi& $%ng men$o&ong *i%gnosis %'(esi% bilie( 'e'%)i 'i*%&
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)%'ognomoni&8 meme(lu&%n %&'u+ Oleh &%(en% i'u 'i*%& *i%n,u(&%n un'u& mel%&u&%n bio)si )%*% usi% # minggu
III.1.. Dian#sa
Di%gnosis %'(esi% bilie( *i'eg%&&%n be(*%s%(&%n %n%mnesis/ )eme(i&s%%n -isi& *%n )eme(i&s%%n )enun,%ng+ M%ni-es'%si &linis u'%m% %'(esi% bilie( %*%l%h 'in,% %&oli&/ %i( &emih se)e('i %i( 'eh/ *%n i&'e(us+ A*% em)%' &e%*%%n &linis $%ng *%)%' *i)%&%i seb%g%i )%'o&%n un'u& membe*%&%n %n'%(% &oles'%sis in'(%he)%'i& *%n e&s'(%he)%'i&/ $%i'u: be(%' b%*%n l%hi(/ %(n% 'in,%/ umu( )en*e(i'% s%%' 'in,% mul%i %&oli&/ *%n &e%*%%n he)%(+ K(i'e(i% ini 7T%bel "8 mem)un$%i %&u(%si *i%gnos'i& s%m)%i 12+ Mo$e( *&&+ men%mb%h&%n s%'u &(i'e(i% l%gi/ $%i'u g%mb%(%n his'o)%'ologi& h%'i
T%bel "+ Em)%' &(i'e(i% &linis 'e()en'ing un'u& membe*%&%n Koles'%sis In'(%he)%'i& *%n E&s'(%he)%'i&
III.1.. Dian#sa Dierential • • • •
Hi)o)l%si% bilie(/ s'enosis *u&'us bilie( Pe(-o(%si s)on'%n *u&'us bilie( M%ss% 7neo)l%sm%/ b%'u8 #nspissated bile s!ndrome
10
11
• • • •
He)%'i'is neon%'%l i*io)%'i& Dis)l%si% %('e(iohe)%'i& 7sin*(om Al%gille8 Pen$%&i' C%(oli 7)eleb%(%n &is'i& )%*% *u&'us in'(%he)%'i&8+ He)%'i'is
III.1.h. Penatalaksanaan %era"i medikament#sa
"8 Mem)e(b%i&i %li(%n b%h%n6b%h%n $%ng *ih%sil&%n oleh h%'i 'e(u'%m% %s%m em)e*u 7%s%m li'o&ol%'8/ *eng%n membe(i&%n :
enob%(bi'%l 5 mg=&gBB=h%(i *ib%gi *osis/ )e( o(%l+ enob%(bi'%l %&%n me(%ngs%ng enim glu&u(onil '(%ns-e(%se 7un'u& mengub%h bili(ubin in*i(e& men,%*i bili(ubin *i(e&8 enim si'o&(om P6<5! 7un'u& o&sigenis%si 'o&sin8/ enim N% K ATP%se 7mengin*u&si %li(%n em)e*u8+
Koles'i(%min " g(%m=&gBB=h%(i *ib%gi # *osis %'%u sesu%i ,%*%l )embe(i%n susu+ Koles'i(%min memo'ong si&lus en'e(ohe)%'i& %s%m em)e*u se&un*e(+
8 Melin*ungi h%'i *%(i %' 'o&si&/ *eng%n membe(i&%n :
As%m u(so*eo&si&ol%'/ 4"! mg=&gBB=h%(i/ *ib%gi 4 *osis/ )e( o(%l+ As%m u(so*eo&si&ol%' mem)un$%i *%$% i&%' &om)e'i'i- 'e(h%*%) %s%m li'o&ol%' $%ng he)%'o'o&si&+
%era"i nutrisi/ $%ng be('u,u%n un'u& memung&in&%n %n%&
'umbuh *%n be(&emb%ng seo)'im%l mung&in/ $%i'u : "8 Pembe(i%n m%&%n%n $%ng meng%n*ung medium chain trigl!cerides 7MCT8 un'u& meng%'%si m%l%bso()si lem%&+ 8 Pen%'%l%&s%n%%n *e-isiensi @i'%min $%ng l%(u'*%l%m lem%&+
%era"i -edah
11
12
Kasai Pr#sedur
P(ose*u(
$%ng
'e(b%i&
%*%l%h
mengg%n'i
s%lu(%n
em)e*u
$%ng
meng%li(&%n em)e*u &e usus+ Te'%)i )(ose*u( ini h%n$% mung&in *il%&u&%n )%*% 56"!2 )en*e(i'%+ Un'u& melom)%'i %'(esi% bilie( *%n l%ngsung menghubung&%n h%'i *eng%n usus h%lus/ *il%&u&%n )embe*%h%n $%ng *isebu' )(ose*u( K%s%i+ Pembe*%h%n %&%n be(h%sil ,i&% *il%&u&%n sebelum b%$i be(usi% 1 minggu+ Bi%s%n$% )embe*%h%n ini h%n$% me(u)%&%n )engob%'%n semen'%(% *%n )%*% %&hi(n$% )e(lu *il%&u&%n )en0%ng&o&%n h%'i+
G%mb%( 5+ K%s%i P(ose*u(e
P(ose*u( &%s%i bis% membu%' seb%gi%n )%sien be(umu( )%n,%ng+ N%mun/ -ungsi h%'i )%*% seb%gi%n )%sien l%inn$% sem%&in membu(u&+ Umumn$%/ )%sien *%'%ng &e (um%h s%&i' *%l%m &on*isi $%ng su*%h bu(u&/ $%&ni s%%' b%$i be(usi% lebih *%(i *u% bul%n+ Pen*e(i'% )en$%&i' gin,%l memili&i %l'e(n%'i- )engob%'%n *i%lis%/ 'e'%)i 'i*%& *emi&i%n h%ln$% *eng%n )en*e(i'% )en$%&i' h%'i $%ng be(%'+ .i&% h%'i su*%h 'i*%& be(-ungsi l%gi/ m%&% s%'u6s%'un$% )ilih%n )engob%'%n %*%l%h )en0%ng&o&&%n h%'i+
1!
13
Pen/ank#kan atau %rans"lantasi Hati
T(%ns)l%n'%si h%'i memili&i 'ing&%' &ebe(h%sil%n $%ng 'inggi un'u& %'(esi% bilie( *%n &em%m)u%n hi*u) se'el%h o)e(%si mening&%' se0%(% *(%m%'is *%l%m bebe(%)% '%hun 'e(%&hi(+ An%&6%n%& *eng%n %'(esi% bilie( se&%(%ng *%)%' hi*u) hingg% *e%s%/ bebe(%)% b%h&%n 'el%h mem)un$%i %n%&+ Kem%,u%n
*%l%m
o)e(%si
'(%ns)l%n'%si
'el%h
,ug%
mening&%'&%n
&emung&i%n%n un'u& *il%&u&%nn$% '(%ns)l%n'%si )%*% %n%&6%n%& *eng%n %'(esi% bilie(+ Di m%s% l%lu/ h%n$% h%'i *%(i %n%& &e0il $%ng *%)%' *igun%&%n un'u& '(%ns)l%'%si &%(en% u&u(%n h%'i h%(us 0o0o&+ B%(u6b%(u ini/ 'el%h *i&emb%ng&%n un'u& menggun%&%n b%gi%n *%(i h%'i o(%ng *e%s%/ $%ng *isebu' (e*u0e* sie %'%u s)li' li@e( '(%ns)l%n'%si/ un'u& '(%ns)l%n'%si )%*% %n%& *eng%n %'(esi% bilie(+
III.1.i. K#m"likasi Kolangitis: &omuni&%si l%ngsung *%(i s%lu(%n em)e*u in'(%he)%'i0 &e usus/
*eng%n %li(%n em)e*u $%ng 'i*%& b%i&/ *%)%' men$eb%b&%n %s0en*ing 0hol%ngi'is+ H%l ini 'e(,%*i 'e(u'%m% *%l%m minggu6minggu )e('%m% %'%u bul%n se'el%h )(ose*u( K%s%i seb%n$%& 4!6#!2 &%sus+ In-e&si ini bis% be(%' *%n &%*%ng6&%*%ng -ulmin%n+
A*% '%n*%6'%n*% se)sis 7*em%m/ hi)o'e(mi%/ s'%'us hemo*in%mi&
'e(g%nggu8/ i&'e(us $%ng be(ul%ng/ -eses %0holi0 *%n mung&in 'imbul s%&i' )e(u'+ Di%gnosis *%)%' *i)%s'i&%n *eng%n &ul'u( *%(%h *%n = %'%u bio)si h%'i+ Hipetensi portal: Po('%l hi)e('ensi 'e(,%*i se'i*%&n$% )%*% *u% )e('ig% *%(i %n%&6
%n%& se'el%h )o('oen'e(os'om$+ H%l )%ling umum $%ng 'e(,%*i %*%l%h @%(ises eso-%gus+ Hepatopulmonary syndrome dan hipertensi pulmonal: Se)e('i )%*% )%sien
*eng%n )en$eb%b l%in se0%(% s)on'%n 7si(osis %'%u )(ehe)%'i0 hi)e('ensi )o('%l8 %'%u *i)e(oleh 7be*%h8 )o('os$s'emi0 shun's/ shun's )%*% %('e(i@enosus )ulmo mung&in 'e(,%*i+ Bi%s%n$%/ h%l ini men$eb%b&%n hi)o&si%/ si%nosis/ *%n *$s)neu+ Di%gnosis *%)%' *i'eg%&%n *eng%n s0in'ig(%)h$ )%(u+ Sel%in i'u/ hi)e( #si )ulmon%l *%)%' 'e(,%*i )%*% %n%&6%n%& *eng%n si(osis $%ng men,%*i )en$eb%b &elesu%n *%n b%h&%n &em%'i%n men*%*%&+ Di%gnosis *%l%m &%sus ini *%)%' *i'eg%&%n oleh
1$
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e0ho0%(*iog(%)h$+ T(%ns)l%n'%si li@e( *%)%' memb%li&%n shun's/ *%n *%)%' memb%li&&%n hi)e('ensi )ulmon%l &e '%h%) semul%+ Keganasan: He)%'o0%(0inom%s/ he)%'obl%s'om%s/ *%n 0hol%ngio0%(0inom%s *%)%'
'imbul )%*% )%sien *eng%n %'(esi% bilie( $%ng 'el%h meng%l%mi si(osis+ S&(ining un'u& &eg%n%s%n h%(us *il%&u&%n se0%(% 'e(%'u( *%l%m 'in*%& l%n,u' )%sien *eng%n o)e(%si K%s%i $%ng be(h%sil+
Hasil setelah aal #"erasi Kasai
Si(osis bilie( be(si-%' )(og(esi- ,i&% o)e(%si K%s%i g%g%l un'u& memulih&%n %li(%n em)e*u/ *%n )%*% &e%*%%n ini h%(us *il%&u&%n '(%ns)l%n'%si h%'i+ H%l ini bi%s%n$% *il%&u&%n *i '%hun &e*u% &ehi*u)%n/ n%mun *%)%' *il%&u&%n lebih %%l 7*%(i # bul%n hi*u)8 un'u& mengu(%ngi &e(us%&%n *%(i
h%'i+
A'(esi% bilie(
me%&ili lebih *%(i se'eng%h *%(i in*i&%si un'u& '(%ns)l%n'%si h%'i *i m%s% &%n%&6 &%n%&+ H%l ini ,ug% mung&in *i)e(lu&%n *%l%m &%sus6&%sus *im%n% )%*% %%ln$% su&ses se'el%h o)e(%si K%s%i 'e'%)i 'imbul i&'e(us $%ng (e&u(en 7&eg%g%l%n se&un*e(
o)e(%si
K%s%i8/
%'%u
un'u&
be(b%g%i
&om)li&%si
*%(i
si(osis
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III.1.. Pr#n#sis
Kebe(h%sil%n )o('oen'e(os'omi *i'en'u&%n oleh usi% %n%& s%%' *io)e(%si/ g%mb%(%n his'ologi& )o('% he)%'is/ &e,%*i%n )en$uli' &ol%ngi'is/ *%n )eng%l%m%n %hli be*%hn$% sen*i(i+ Bil% o)e(%si *il%&u&%n )%*% usi% 1 minggu m%&% %ng&% &ebe(h%sil%nn$% 9"/1#2/ se*%ng&%n bil% o)e(%si *il%&u&%n )%*% usi% 3 1 minggu m%&% %ng&% &ebe(h%sil%nn$% h%n$% 4<42+ Se*%ng&%n bil% o)e(%si 'i*%& *il%&u&%n/ m%&% %ng&% &ebe(h%sil%n hi*u) 4 '%hun h%n$% "!2 *%n meningg%l (%'%6(%'% )%*% usi% " bul%n+ An%& 'e(mu*% $%ng meng%l%mi o)e(%si K%s%i be(usi% 9# ,%m+ .%*i/ -%&'o(6-%&'o( $%ng mem)eng%(uhi &eg%g%l%n o)e(%si %*%l%h usi% s%%' *il%&u&%n o)e(%si 3 #! h%(i/ %*%n$% g%mb%(%n si(osis )%*% se*i%%n his'ologi& h%*/
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'i*%& %*%n$% *u&'us bilie( e&s'(%he)%'i& $%ng )%'en/ *%n bil% 'e(,%*i )en$uli' hi)e('ensi )o('%l+
III.!. Ineksi Saluran Kemih
In-e&si s%lu(%n %i( &emih %*%l%h in-e&si $%ng 'e(,%*i )%*% s%lu(%n %i( &emih/ mul%i *%(i u(e'(%/ buli6buli/ u(e'e(/ )i%l% gin,%l s%m)%i ,%(ing%n gin,%l+ In-e&si ini *%)%' be(u)% : 6
Pielone-(i'is %&u'
6
Pielone-(i'is &(oni&
6
In-e&si s%lu(%n %i( &emih be(ul%ng
6
B%&'e(iu(i% be(m%&n%
6
B%&'e(iu(i% %sim'om%'is
ETILGI
Kum%n )en$eb%b in-e&si s%lu(%n %i( &emih : 6
Kum%n g(%m neg%'i- : E+Coli 71528/ Klebsiel%/ En'e(o6b%&'e(/ P(o'eus/ *%n Pseu*omon%s+
6
S'%-ilo&o&us Au(eus/ S'(e)'o&o&us -e0%lis/ &um%n %n%e(ob/ TBC/ ,%mu(/ @i(us *%n ben'u& L b%&'e(i )(o'o)l%s+
!ATFISILGI
In-e&si *%)%' 'e(,%*i mel%lui )en$eb%(%n hem%'ogen 7neon%'us8 %'%u se0%(% %sen*ing 7%n%&6%n%&8+ %&'o( )(e*is)osisi in-e&si %*%l%h -imosis/ %li(6b%li& @esi&ou(e'e( 7(e-lu&s @esi&ou(e'e(8/ u(o)%'i obs'(u&'i-/ &el%in%n &ongeni'%l buli6 buli %'%u gin,%l/ *%n *i%)e( (%sh+ P%'ogenesis in-e&si s%lu(%n &emih s%ng%' &om)le&s/ &%(en% 'e(g%n'ung *%(i b%n$%& -%&'o( se)e('i -%&'o( )e,%mu 7 host 8 *%n -%&'o( o(g%nismen$%+ B%&'e(i *%l%m u(in *%)%' be(%s%l *%(i gin,%l/ )ielum/ u(e'e(/ @esi&% u(in%(i% %'%u *%(i u(e'(%+ Bebe(%)% -%&'o( )(e*is)osisi ISK %*%l%h obs'(u&si u(in/ &el%in%n s'(u&'u(/ u(oli'i%sis/ ben*% %sing/ (e-lu&s %'%u &ons'i)%si $%ng l%m%+ P%*% b%$i *%n %n%&
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%n%& bi%s%n$% b%&'e(i be(%s%l *%(i 'in,%n$% sen*i(i $%ng men,%l%( se0%(% %sen*ing+ B%&'e(i
u(o)%'ogeni&
$%ng
mele&%'
)%*%
)%*%
sel
u(oe)i'eli%l/
*%)%'
mem)eng%(uhi &on'(%&'ili'%s o'o' )olos *in*ing u(e'e(/ *%n men$eb%b&%n g%nggu%n )e(is'%l'i& u(e'e(+ Mele&%'n$% b%&'e(i &e sel u(oe)i'eli%l/ *%)%' mening&%'&%n @i(ulensi b%&'e(i 'e(sebu'+ Mu&os% &%n*ung &emih *il%)isi oleh gl!coprotein mucin la!er $%ng be(-ungsi seb%g%i %n'i b%&'e(i+ Robe&n$% l%)is%n ini *%)%' men$eb%b&%n b%&'e(i *%)%' mele&%'/ memben'u& &oloni )%*% )e(mu&%%n mu&os%/ m%su& menembus e)i'el *%n sel%n,u'n$% 'e(,%*i )e(%*%ng%n+ B%&'e(i *%(i &%n*ung &emih *%)%' n%i& &e u(e'e( *%n s%m)%i &e gin,%l mel%lui l%)is%n 'i)is 0%i(%n 7 films of fluid 8/ %)%l%gi bil% %*% (e-lu&s @esi&ou(e'e( m%u)un (e-lu&s in'(%(en%l+ Bil% h%n$% buli buli $%ng 'e(in-e&si/ *%)%' meng%&ib%'&%n i(i'%si *%n s)%sme o'o' )olos @esi&% u(in%(i%/ %&ib%'n$% (%s% ingin mi&si 'e(us mene(us 7urgenc!8 %'%u mi&si be(ul%ng &%li 7 fre$uenc!8/ s%&i' %&'u mi&si 7d!suri8+ Mu&os% @esi&% u(in%(i% men,%*i e*em%/ me(%*%ng *%n )e(*%(%h%n 7hem%'u(i%8+ In-e&si gin,%l *%)%' 'e(,%*i mel%lui collecting s!stem+ Pel@is *%n me*ul% gin,%l *%)%' (us%&/ b%i& %&ib%' in-e&si m%u)un oleh 'e&%n%n u(in %&ib%' (e-lu&s be(u)% %'(o-i gin,%l+ P%*% )ielone-(i'is %&u' *%)%' *i'emu&%n -o&us in-e&si *%l%m )%(en&im gin,%l/ gin,%l *%)%' membeng&%&/ in-il'(%si le&osi' )olimo(-onu&le%( *%l%m ,%(ing%n in'e(s'i'i%l/ %&ib%'n$% -ungsi gin,%l *%)%' 'e(g%nggu+ P%*% )ielone-(i'is &(oni& %&ib%' in-e&si/ %*%n$% )(o*u& b%&'e(i %'%u %' me*i%'o( 'o&si& $%ng *ih%sil&%n oleh sel $%ng (us%&/ meng%&ib%'&%n )%(u' gin,%l 7 renal scarring 8+
E6ALA KLINIS
Ge,%l% &linis in-e&si s%lu(%n %i( &emih b%gi%n b%%h se0%(% &l%si& $%i'u n$e(i bil% bu%ng %i( &e0il 7*$su(i%8/ se(ing bu%ng %i( &e0il 7-(euen0$8/ *%n ngom)ol+ Ge,%l% in-e&si s%lu(%n &emih b%gi%n b%%h bi%s%n$% )%n%s 'inggi/ ge,%l% ge,%l% sis'emi&/ n$e(i *i *%e(%h )ingg%ng bel%&%ng+ N%mun *emi&i%n suli' membe*%&%n in-e&si s%lu(%n &emih b%gi%n %'%s *%n b%gi%n b%%h be(*%s%(&%n ge,%l% &linis s%,%+ Ge,%l% in-e&si s%lu(%n &emih be(*%s%(&%n umu( )en*e(i'% %*%l%h seb%g%i be(i&u' :
1,
17
!+" Bul%n
: G%nggu%n )e('umbuh%n/ %no(e&si%/ mun'%h *%n *i%(e/ &e,%ng/ &om%/ )%n%s=hi)o'e(mi% '%n)% *i&e'%hui seb%bn$%/ i&'e(us 7se)sis8+
" bln6 'hn : P%n%s=hi)o'e(mi% '%n)% *i&e'%hui seb%bn$%/ g%nggu%n )e('umbuh%n/ %no(e&si%/ mun'%h/ *i%(e/ &e,%ng/ &om%/ &oli& 7%n%& men,e(i' &e(%s8/ %i( &emih be(b%u=be(ub%h %(n%/ &%*%ng6&%*%ng *ise('%i n$e(i )e(u'=)ingg%ng+ 6# 'hn
: P%n%s=hi)o'e(mi% '%n)% *i&e'%hui seb%bn$%/ 'i*%& *%)%' men%h%n &en0ing/ be(b%u
)ol%&isu(i%/ *isu(i%/ enu(esis/ %i( &emih *%n
be(ub%h
%(n%/
*i%(e/
mun'%h/
g%nggu%n
)e('umbuh%n se('% %no(e&si%+ #6"1 'hn
: N$e(i )e(u'=)ingg%ng/ )%n%s '%n)% *i&e'%hui seb%bn$%/ '%& *%)%' men%h%n
&en0ing/ )ol%&isu(i%/ *isu(i%/ enu(esis/ %i(
&emih be(b%u *%n be(ub%h %(n%+
DIAN7SIS Biakan air kemih :
Di&%'%&%n in-e&si )osi'i- %)%bil% : 6
Ai( &emih '%m)ung )o(si 'eng%h : bi%&%n &um%n )osi'i- *eng%n ,uml%h &um%n J"!5=ml/ &%li be('u(u'6'u(u'+
6
Ai( &emih '%m)ung *eng%n )ungsi buli6buli su)(%)ubi& : se'i%) &um%n )%'ogen $%ng 'umbuh )%s'i in-e&si+ Pembi%&%n u(in mel%lui )ungsi su)(%)ubi& *igun%&%n seb%g%i gold standar.
Dug%%n in-e&si : 6 Peme(i&s%%n %i( &emih : %*% &um%n/ )iu(i%/ 'o(%& leu&osi' 6 U,i &imi% : TTC/ &%'%l%se/ glu&osu(i%/ le&osi' es'e(%se 'es'/ ni'(i' 'es'+ 8en/ari akt#r resik# ineksi saluran kemih 9
6 Peme(i&s%%n ul'(%sonog(%-i gin,%l un'u& menge'%hui &el%in%n s'(u&'u( gin,%l *%n &%n*ung &emih+ 6
Peme(i&s%%n Mi&sio Sis'o U(e'(og(%-i=MSU un'u& menge'%hui %*%n$% (e-lu&s+
1*
18
6 Peme(i&s%%n )ielog(%-i in'(% @en% 7PI8 un'u& men0%(i l%'%( bel%&%ng in-e&si s%lu(%n &emih *%n menge'%hui s'(u&'u( gin,%l se('% s%lu(%n &emih+
"IAG#SA BA#"I#G
F%ng )en'ing %*%l%h membe*%&%n %n'%(% )ielone-(i'is *%n sis'i'is+ Ing%' %&%n )ielone-(i'is %)%bil% *i*%)%'&%n in-e&si *eng%n hi)e('ensi/ *ise('%i ge,%l%6ge,%l% umum/ %*%n$% -%&'o( )(e*is)osisi/ -ungsi &onsen'(%si gin,%l menu(un/ (es)ons 'e(h%*%) %n'ibio'i& &u(%ng b%i&+
PENATALAKSANAAN A*% 4 )(insi) )en%'%l%&s%n%%n in-e&si s%lu(%n %i( &emih : 6
Membe(%n'%s in-e&si
6
Menghil%ng&%n -%&'o( )(e*is)osisi
6
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