Isi Sindrom Rubella Kongenital

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    Minireferat Fetomaternal 

    SINDROM RUBELLA KONGENITAL

    OLEHD. Irsat Syafardi

    PEMBIMBING

    dr. Risman F. Kaban M. K!d"OG# S$OG

    DEPARTEMEN OBSTETRI DAN GINEKOLOGI

    FAKULTAS KEDOKTERAN UNI%ERSITAS SUMATERA

    UTARA

    M E D A N & ' ( )

    1

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    DAFTAR ISI

    DAFTAR ISI....................................................................................................

    i

    DAFTAR GAMBAR........................................................................................

    ii

    DAFTAR TABEL.............................................................................................

    iii

    BAB I PENDAHULUAN..................................................................................

    1

    1.1. Latar Belakang....................................................................................

    11.2. Tujuan..................................................................................................

    2

    1.2.1. Tujuan Uu......................................................................................

    21.2.2. Tujuan !"u#u#....................................................................................

    21.$. Man%aat...............................................................................................

    2

    BAB II TIN&AUAN PUSTA!A.........................................................................

    $

    2.1. De%ini#i.................................................................................................

    $2.2. E'i(ei)l)gi........................................................................................

    $2.$. Pen*e+a+............................................................................................

    ,

    2

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    2.,. Pat)gene#i#.........................................................................................

    -2.. Mani%e#ta#i !lini#.................................................................................

    /2.-. Diagn)#i#.............................................................................................

    112.0. Penatalak#anaan................................................................................

    1$2./. Penega"an........................................................................................

    1,

    BAB $ PENUTUP...........................................................................................

    1,

    $.1. !e#i'ulan..........................................................................................

    1-

    DAFTAR PUSTA!A........................................................................................

    10

    DAFTAR GAMBAR

    Ga+ar 1.......................................................................................................

    3

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    DAFTAR TABEL

    Ta+el 1.........................................................................................

    1

    Ta+el 2.........................................................................................

    1,

    4

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    BAB IPENDAHULUAN

    (.(. Latar B!*a+an,

    Ber(a#arkan (ata (ari 3H45 'aling ti(ak 2$-. ka#u# Sin(r)

    Ru+ella !)ngenital 6SR!7 terja(i #etia' ta"un (i negara8negara

    +erke+ang (an (a'at eningkat 1 kali li'at 'a(a #aat terja(i

    e'i(ei. Se+elu 9ak#in Ru+ella (iteukan5 e'i(eik Ru+ella (i

    negara aju unul 'a(a inter9al $81 ta"un. Setela" 'eneuan

    9ak#in 'a(a ta"un 1:-:5 in#i(en#i Ru+ella enurun (ra#ti# enja(i

    "an*a , 'en(erita 'er 1. ')'ula#i 'er ta"un (i (unia (engan

    2$082, ka#u# 'er ta"un.152

    Sin(r) Ru+ella !)ngenital 6SR!7 a(ala" in%ek#i tran#'la#enta

    'a(a janin (engan Ru+ella5 +ia#an*a 'a(a ke"ailan trie#ter 

    'ertaa5 *ang (i#e+a+kan )le" in%ek#i aternal. Ma#ala" utaa

    a(ala" ri#ik) keaatan 'a(a +a*i (ala in#i(en#i *ang #angat

    tinggi. In%ek#i aternal 'a(a trie#ter 'ertaa /; akan

    5

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    en*e+a+kan in%ek#i %etu#5 +ila terja(i 'a(a trie#ter ke(ua5 "an*a

    2; %etu# *ang akan terin%ek#i5 (an +ila terja(i 'a(a trie#ter $5

    $81;.$

    Diagn)#i# SR! #ulit (ik)n%ira#i aki+at gejalan*a *ang

    a#i't)atik atau #u+klini# 'a(a aternal #e"ingga ti(ak a(a

    alar untuk e'er"atikan k"u#u# janin *ang (ikan(ung. Di

    #a'ing itu5 tera'i SR! juga "an*a +er#i%at #u')rti%. 4+at8)+atan

    *ang (ike+angkan a#i" (i'ertan*akan keaanann*a 'a(a

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    BAB IITIN1AUAN PUSTAKA

    &.(. D!finisi

    Sin(r) Ru+ella !)ngenital 6SR!7 a(ala" in%ek#i tran#'la#enta

    'a(a janin (engan Ru+ella5 +ia#an*a 'a(a ke"ailan trie#ter 

    'ertaa5 *ang (i#e+a+kan )le" in%ek#i aternal. SR! ketika +a*i

    +era(a (ala kan(ungan *ang (a'at en*e+a+kan in%ek#i kr)nik

    intrauterin (an engganggu 'ertu+u"an (an 'erke+angan janin

    #a'ai e*i#akan aat +a

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    In%ek#i aternal 'a(a trie#ter 'ertaa /; akan en*e+a+kan

    in%ek#i %etu#5 +ila terja(i 'a(a trie#ter ke(ua5 "an*a 2; %etu#

    *ang akan terin%ek#i5 (an +ila terja(i 'a(a trie#ter $5 $81;.152

    &anin *ang tertular +eri#ik) engalai SR!5 terutaa +ila in%ek#i

    terja(i 'a(a u#ia janin ?, +ulan. SR! +ia#an*a terja(i "an*a +ila

    i+u terin%ek#i 'a(a #aat uur ke"ailan a#i" kurang (ari , +ulan.

    Bila #u(a" le

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    #er)l)gi# *ang e'at (an 'rakti#. Ber+agai jeni# jaringan5

    k"u#u#n*a ginjal kera 'aling +aik (igunakan untuk enga#ingkan

    9iru#5 karena (a'at eng"a#ilkan 'ara# 6le9el7 9iru# *ang le+i"

    tinggi (an #eara uu le+i" +aik untuk eng"a#ilkan antigen.

    Pertu+u"an 9iru# ti(ak (a'at (ilakukan 'a(a telur5 tiku# (an

    kelini (e

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    +i)#inte#i#5 atura#i5 (an 'ele'a#an. Setela" a("e#i (an 'enetra#i5

    a#a nukleat 9iru# "aru# #u(a" uncoating  (i 'erukaan #el (ala

    9iru# )le" enCi li#)#). !eu(ian5 akan terja(i re'lika#i (an

    atura#i (an 9iru# +aru #ia' (ile'a#kan.1

    @iru# Ru+ella (itran#i#ikan elalui 'erna'a#an (an engalai

    re'lika#i (i na#)%aring (an kelenjar geta" +ening. @ireia terja(i

    antara "ari ke8 #a'ai "ari ke80 #etela" ter'ajan 9iru# Ru+ella.

    Ma#a inku+a#i 9iru# Ru+ella +erki#ar antara 1,821 "ari. Ma#a

    'enularan 1 inggu #e+elu , "ari unuln*a rua./81

    &.). Pat2,!n!sis

    Su+er in%ek#i Ru+ella janin a(ala" (ari 'la#enta

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    in%ek#i kr)ni# *ang eru'akan k"a# Ru+ella intrauterin (ala

    inggu8inggu a

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    Pa(a (aera" *ang +aru akan terja(i 'ele'a#an (an 'erang#ang

    ke)kin IL82 (an IFN8gaa. Sel T"e akan e'r)(uk#i IL8, (an

    IL81 untuk akti9a#i re#')n iun "u)ral #el B. IFN (iteukan

    +er'eran (ala akti9a#i #el N!5 eningkatkan )lekul MH15 (an

    IFN8al%a elalui kina#e (an 'r)tein STAT.1

    &.3. Manif!stasi K*inis

    SR! (a'at en*e+a+kan gangguan 'a(a aternal (an %etal. Ma#a

    inku+a#i Ru+ella a(ala" 1282$ "ari. Peri)(e in%ek#iu# a(ala" 80

    "ari #e+elu )n#et unuln*a rua. Pa(a

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    Gejala 'r)(r)al ini akan +erlang#ung 18 "ari #e+elu )n#et

    rua #karletini%)r *ang gatal. Li%a(en)'ati *ang unul terja(i

    n)(u# li%atiku# +elakang telinga. Li%a(en)'ati eru'akan #uatu

    gejala klini# *ang 'enting 'a(a Ru+ella. Pe+e#aran kelenjar li%e

    +i#a ti+ul 80 "ari #e+elu ti+ul ek#antea. Bia#an*a

    'e+engkakan kelenjar geta" +ening itu +erlang#ung #elaa 8/

    "ari.$

    Pa(a "ari ke(ua atua ketiga5 unul ')la rua Ru+ella

    kale(i()#k)'i. Mula8ula akula era" u(a *ang irreguler 

    6+ia#an*a (ala 2, ja7 ti+ul (i le"er5 +a(an5 lengan (an

    ak"irn*a (i kaki. Pa(a "ari +erikutn*a le#i ini en*atu5 e+entuk

    k)')nen akul)'a'ular (an enja(i #kar atini%)ri#. Muka

    #ering +e+a# rua 'a(a #aat rua 'enu" #a'ai tungkai +a

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    :; in%ek#i aternal unul #e+elu u#ia ge#ta#i 11 inggu5 $$;

    'a(a u#ia ge#ta#i 11812 inggu5 11; 'a(a u#ia ge#ta#i 1$81,

    inggu5 2,; 'a(a u#ia ge#ta#i 181- inggu5 (an ; 'a(a u#ia

    ge#ta#i le+i" (ari 1- inggu.1

    Tab!* (. 42n,!nita* d!f!5ts and *at! manif!stati2ns 2f r-b!**a

    inf!5ti2n

    Pr!s!nt at birt/

     Au(i)l)gi an)alie# 6-0;7

    • Sen#)rineural (ea%ne##

    ar(ia (e%et# 612;7• Pul)nar* #ten)#i#

    • Patent (utu# arteri)#u#

    • @entriular #e'tal (e%et

    4'"t"ali (e%et# 612;7

    • Retin)'at"*

    • atarat#

    • Mir)'"t"alia

    • Pigentar* an( )ngenital

    glau)a

    entral ner9)u# #*#te 612;7

    • Mental retar(ati)n

    • Mir)e'"al*

    • Mening)ene'"aliti#

    4t"er#

    • T"r)+)*t)'enia

    • He'at)#'len)egal*

    • Ra(i)luent +)ne (i#ea#e

    • "arateri#ti 'ur'ura

    6Blue+err* u%%in a''earane7

    Lat! manif!stati2ns

    Dia+ete# ellitu#

    T"*r)i(iti#

    Gr)

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    'neu)nia inter#titial5 kelainan jantung k)ngenital 6'atent (utu#

    ateri)#u# a(ala" kelainan *ang 'aling #ering (iteukan75 glauk)a

    atau katarak k)ngenital5 (an ikr)#e%ali.0

    Pa(a gejala extended   (a'at unul Cerebral Palsy 5 retar(a#i

    ental5 keterla+atan 'ertu+u"an (an +er+iara5 kejang5 ikteru#

    (an gangguan iun)l)gi. Pa(a delayed symptoms  (a'at terja(i

    'anen#e%aliti#5 DM ti'e 15 atau ganguan ata'en(engaran *ang

    +aru unul laa #etela" in%ek#i 'rier.10

    &.6. Dia,n2sis

    !)n%ira#i (iagn)#i# in%ek#i Ru+ella akut 'a(a

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    1. P)l*era#e "ain Reati)n 6PR7Ba"an 'eerik#aan (ia+il (ari air ketu+an 6airan ani)n7

    atau (ara" janin. Penga+ilan #a'el air ketu+an atau 'un

    (ara" janin "aru# (ilakukan )le" ()kter a"li kan(ungan (an

    ke+i(anan5 (an "an*a (a'at (ilakukan #etela" u#ia ke"ailan (i

    ata# 22 inggu.2. ELISA

    IgM #en#iti% unutuk in%ek#i akut 'rier (an rein%ek#i. IgM

    Ter(etek#i 'a(a 18 "ari #etela" unul rua5 ena'ai

    'unak 'a(a "ari ke8-812 (an +erta"an "ingga 18, inggu. Titer 

    turun5 ti(ak ter(etek#i #etela" -812 inggu. IgG (a'at (i (etek#i

    'a(a 18$ "ari #etela" unul gejala5 +erta"an #euur "i(u'.$. La+)rat)riu Heaglutina#i Pa#i% 

    Bila ter(a'at aglutina#i aka te(a'at anti+)(i #'e#i%ik ter"a(a'

    Ru+ella.,. Uji He)li#i# Ra(ial

    J)na 'a(a le'engan te# enunjukkan a(an*a iunita#

    anti+)(i ter"a(a' 9iru# Ru+ella 6J)na "e)li#i# 'a(a le'engan

    k)ntr)l terentang antara $58 7.

    Diagn)#i# 'renatal (ilakukan (engan eerik#a a(an*a IgM (ari

    (ara" janin elalui @S atau k)r()#ente#i#. !)n%ira#i in%ek#i

    %etu# 'a(a trie#ter I (ilakukan (engan eneukan a(an*a

    antigen #'e#i%ik Ru+ella (an RNA 'a(a @S.

    Ber(a#arkan gejala klinik (an teuan #er)l)gi5 SR! (a'at

    (ikla#i%ika#ikan #e+agai +erikut

    1051/

    1. !)n%ira#i SR! +ila te# PR atau IgM ')#iti%.2. SR! k)'ati+el +ila a(a (e%ek k)ngenital teta'i #er)l)gi#

    negati%.$. SRI +ila ti(ak a(a (e%ek k)ngenital teta'i #er)l)gi# ')#iti%.,. Stillbirth +ila (i#e+a+kan Ru+ella aternal.. Bukan SR!.

     Alg)rita 'enanganan i+u "ail *ang ter'ajan1

    16

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    &.7. P!nata*a+sanaan

    Tera'i (ari SR! +ia#an*a #u')rti%5 self limited  (i#ea#e5 ti(ak (a'at

    (i)+ati "an*a +i#a (iega" (engan 9ak#ina#i. Baru8+aru ini5

    a(aantanain "i(r)kl)ri(a 6aanta(in7 tela" (ila')rkan e%ekti% invitro  (ala eng"a+at #ta(iu a

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    "aru# (i#i'an (ala leari e# 'a(a #u"u , (an (igunakan

    #e#egera 9ak#in ini (ilarutkan ke+ali. @ak#in (i+erikan #e+agai

    #atu injek#i #u+kutan.1:

    Ta+el 2. ara 'en*i'anan 9ak#in MMR (an MMR@

    Pa(a )rang *ang rentan5 'r)tek#i 'a#i% (ari atau 'elea"an

    'en*akit (a'at (i+erikan #eara +er9aria#i (engan injek#i

    intrau#kuler Gl)+ulin Iun Seru 6GIS7 *ang (i+erikan (engan

    ()#i# +e#ar 65285 Lkg atau 512852 Ll+7 (ala 08/ "ari

    'a#a 'eajanan. E%ekti9ita# gl)+ulin iun ti(ak (a'at (iraalkan.

    Ta'akn*a tergantung 'a(a ka(ar anti+)(i 'r)(uk *ang (igunakan

    (an 'a(a %akt)r *ang +elu (iketa"ui. Man%aat GIS tela"

    (i'ertan*akan karena 'a(a +e+era'a kea(aan rua (iega" (an

    ani%e#ta#i klini# ti(ak a(a atau inial

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    Se+elu "ail #e+aikn*a eerik#a keke+alan tu+u" ter"a(a'

    Ru+ella5 #e'erti juga ter"a(a' in%ek#i T4RH lainn*a. &ika anti8

    Ru+ella IgG #aja *ang ')#iti%5 +erarti 'erna" terin%ek#i atau #u(a"

    (i9ak#ina#i ter"a(a' Ru+ella5 ti(ak ungkin terkena Ru+ella lagi5

    (an janin 1; aan. &ika anti8Ru+ella IgM #aja *ang ')#iti% atau

    anti8Ru+ella IgM (an anti8Ru+ella IgG ')#iti%5 +erarti +aru terin%ek#i

    Ru+ella atau +aru (i9ak#ina#i ter"a(a' Ru+ella. Pa#ien (i#arankan

    untuk enun(a ke"ailan #a'ai IgM enja(i negati%5 *aitu

    #elaa $8- +ulan. &ika anti8Ru+ella IgG (an anti8Ru+ella IgM

    negati% +erarti ti(ak e'un*ai keke+alan ter"a(a' Ru+ella.Pa#ien +ila +elu "ail akan (i+erikan 9ak#in Ru+ella (an

    enun(a ke"ailan #elaa $8- +ulan. Bila #u(a" terlanjur "ail5

    "an*a (a'at eng"in(ari %akt)r ri#ik).22

    BAB IIIPENUTUP

    0.(. K!sim$-*an1. Sin(r)a Ru+ella !)ngenital 6SR!7 a(ala" in%ek#i

    tran#'la#enta 'a(a janin (engan Ru+ella5 +ia#an*a 'a(a

    ke"ailan trie#ter 'ertaa5 *ang (i#e+a+kan )le" in%ek#iaternal.

    2. Ber(a#arkan (ata (ari 3H45 'aling ti(ak 2$-. ka#u# SR!

    terja(i #etia' ta"un (i negara8negara +erke+ang (an (a'at

    eningkat 1 kali li'at 'a(a #aat terja(i e'i(ei.$. Pen*e+a+ Ru+ella a(ala" 9iru# Ru+ella (ari genu# t)ga9iri(ae.,. Diagn)#i# SR! #ulit (ik)n%ira#i aki+at gejalan*a *ang

    a#i't)atik atau #u+klini# 'a(a aternal #e"ingga ti(ak a(a

    alar untuk e'er"atikan k"u#u# janin *ang (ikan(ung.

    . Tera'i (ari SR! +iaan*a #u')rti% (an self limited  disease.

    19

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    -. SR! "an*a (a'at (iega" (engan iuni#a#i #e+elu

    ke"ailan.

    DAFTAR PUSTAKA

    1. D)ntign* L5 Ar#enault MK Martel M&. Rubella in pregnancy . SOGC 

    Clinical Practice Guidelines 2/ 2$ 18.2. ED. Survey on rubella, rubella in pregnancy and congenital rubella

    sureillance systems in !"# contries. C$C 21$ 18$:.$. D. T"e Pink B))k )ur#e tet+))k 12 t" E(iti)n Se)n(

    Printing. C$C 212 1 182.,. Dee'ika D5 Ra"na R5 Saran S5 R)* !! Neena M. $iagnosis of 

    acute rubella infection during pregnancy . % Obstet Gynecol &ndia 2-

    -617 ,,8,-.. 3H4. liminating measles and rubella and preventing congenital 

    rubella infection. '(O 21$ 18$.-. MM3R. Prevention of measles, rubella, congenital rubella syndorme,

    and mumps. C$C 21$ 18,.0. Ga++e SG5 Nie+*l &R5 Si'#)n &L5 e(#. Obstetrics)normal and 

     problem pregnancies. ,t" e(. Ne< K)rk "ur"ill Li9ing#t)ne5

    In.221$2/$./. Batti#ti A5 K)#er &D5 Ple9ka P5 3inkler D5 Pra#a( @M5 !u"n R&5 et al.

    Cryo)electron tomography of rubella virus. % *irol+ 212 2 0/8/.:. !ujala. T"e t)ga9iru# RNA Re'liati)n )'le. !niversity of (elsini 

    2 182.

    1. Gre+er. Mechanisms of virus uncoating .

    20

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    11. Na#iriR5 K)#e%%i &5 !"aje(al)e M5 Ka(i MS Delg)#"aei F. Congenital 

    rubella syndrome after rubella vaccination in -). /ees

     periconceptiona period . &ndian % Ped 2: 0- 20:82/2.

    12. En(er# G5 Nikerl8Pa"er U5 Miller E5 ra()k83at#)n &E. Outcome

    of confirmed periconceptional maternal rubella. Lanet 1://11,,

    0.1$. D"ian N5 Harala+ie9a I5 @ierkant RA5 PankratC @S5 R*an &E5

    &a)+#)n RM5 et al. Predominant &nflammatory Cytoine Secretion

    Pattern &n Response 0o 0/o $ose Of 1ive Rubella *accine &n (ealthy 

    *accinees. Cytoine 21 617 2,82:.1,. enter# %)r Di#ea#e )ntr)l. Control and prevention of rubella2

    evaluation and management of suspected outbreas, rubella in

     pregnant /omen, and surveillance for congenital rubella syndrome.

    MM3R Re) Re' &ul* 1$5 216RR12712$.1. Ree% SE5 Pl)tkin S5 )r(er) &F5 !atC M5 ))'er L5 S"