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erta!a a(in a se"a#ian "esar %atan# e ru!ah sa it%en#an #e a(a e%e!a pa%a e(opa !ata) %a%a) perut)
e stre!itas) s rotu!) %an (a"ia
Sin%ro! ne8roti %i"a#i !en a%i5Sin%ro! Ne8roti pri!er5 Sin%ro! Ne8roti se un%er
Le"ih serin# %i #o(on# an e respon terha%ap steroi%5 Sin%ro! ne8roti sensiti8 steroi%5 Sin%ro! ne8roti resisten steroi%
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*na!nesis5
K; • >en# a pa%a e(opa !ata) perut) %an pun##un# a i
KT • Su(it >er a(an
R S
• asien !erupa an ru u an %ari RS Lho seu!a?e& I"u pasien!en#e(uh an "a%an ana n a !e!"en# a pa%a !ata %an perut se a3 "u(an an# (a(u& >en# a %ia?a(i %i "a#ian e(opa !ata& *?a(n ahan a %i !ata) e!u%ian !en a(ar e se(uruh tu"uh %an s rotu!&>en# a ti!"u( %i pa#i hari saat "an#un ti%ur) %an "er uran# saatana n a "era ti8itas@"er!ain& Oran# tua !en#ata an "en# a ti!"u( 1"u(an sete(ah ana n a atuh %ari etin##ian A@5 1 !eter %i ru!ahn a&
• Oran# tua pasien u#a !en#e(uh an en
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*na!nesis
6
R D• ernah "en# a u!ur 7 "u(an %an %i"a?a e pen#o"atan a(ternati8
R O• ara
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*na!nesis
7
Ri?a atKeha!i(a
n
• I"u pasien teratur *N9 e "i%an
Ri?a atersa(ina
n
• asien ana e%ua %ari %ua "ersau%ara (ahir se>L 2 00 #ra!
Ri?a atI!unisasi
• Ti%a (en# ap
Ri?a atMa anan
• 05/ "u(an $ *SI)• /512 "u(an $ susu 8or!u(aAM *SI)• 1 tahun B se aran# $ !a anan e(uar#a
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e!eri saan Fisi
8
•
Kea%aan u!u! $ Sa it se%an#• Kesa%aran $ 9o!pos !entis• Te anan %arah $ =0@/0 !!'#• Fre uensi na%i $ 120 C@!enit• Fre uensi napas $ 20 C@!enit• Te!peratur $ 3/) 9• Tas Forerat "a%an $ 7)4 #• Tin##i "a%an $ /:
>>I $ 12 #• '* $ sesuai usia / "u(an
Data *ntropo!etri
• >erat "a%an@;!ur $ 53 SD• Tin##i "a%an@;!ur $ 53 SD• >erat >a%an @Tin##i >a%an $ 51 s&% 0 SD• Kesan Status iGi $ >ai ) !a(nutrisi roni
Status iGi
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e!eri saan Fisi9
=
Nor!o
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Darah Len# ap
La"oratoriu!
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;rina(isa
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'or!on Tiroi%
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Ra%io(o#i
Foto po(os a"%o!enT#( +1 @0:@201 ,
Kesan$ Ti%a ta!pa "atura%iopa & Distri"usi #as
usus nor!a(& Foto %a(a!"atas nor!a(
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;(trasono#ra8i
Kesan $ aren
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Dia#nosis >an%in#E%e!a e< %% 1& Sin%ro! Ne8roti A 'ipotiroi% A Stuntin# A
Ma(nutrisi Kronis2& Sin%ro! Ne8riti
3& * ut Ki%ne In urA 'ipotiroi% A Stuntin# A Ma(nutrisi Kronis
Dia#nosis Ker aE%e!a e< Sin%ro! Ne8roti A 'ipotiroi% A Stuntin# AMa(nutrisi Kronis
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Tata(a sanaFarmakologi
I FD D J / #tt@I !a roMeti( re%niso(on 4 !#) 3C1 ta"In 9e8triaCon 300 !#@12 a!
In Furose!i% !#@12 a!Spirono(a ton 2C10 !#
(as"u!in 20J 40
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(annin#Konsu( Di6isi En%o rino(o#i %an iGi *na;rina(isa tiap 4 hariKu(tur ;rin
9e %arah rutin) a("u!in) ;reu! Kreatinin
ro#nosisuo a% 6ita! $ %u"ia et "ona!uo a% 8un
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Foto K(inis
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Tin auan usta aDEFINISI
Sin%ro!a (ini %en#an #e a(a
5 roteinuria !asi8 +H40 !#@!2
L >@ a!,5 Rasio protein %an reatinin urin se?a tu H2!#@!(5 ; i %ipsti urin H A25 hipoa("u!ine!ia + 2& #@%L,
5 E%e!a5 'iper o(estero(e!ia+2 0 !#@ L,
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Etiologi
1& Kon#enita(Finnish-type congenital nephrotic syndrome +NPHS1 ) nephrin, Denys-Drash syndrome +WT1 , Frasier syndrome +WT1 , Diff se mesangial sclerosis +WT1! P"#E1 ,
$ tosomal recessi%e! familial FS&S +NPHS' ) po%o
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2& I%iopati @ ri!er
Sin%ro! Ne8roti Ke(ainan Mini!a( +SNKM,(o!eru(os (erosis 8o a( se#!enta( + SFS,
+esangial Proliferati%e Dif se 2+PD3(o!eru(one8ritis Me!"ranopro(i8erati8 + NM ,
Ne8ropati Me!"ranosa + NM,
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3& Se un%er (upus eri!atosus siste!i +LES,
e#anasan) seperti (i!8o!a %an (eu e!ia6as u(itis) seperti #ranu(o!atosis Qe#ener +#ranu(o!atosis %en#anpo(ian#itis,) sin%ro! 9hur#5Strauss +#ranu(o!atosis eosino8i(i %en#anpo(ian#itis,) po(iartritis no%osa) po(ian#itis !i ros opi ) purpura 'eno
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>atasan)emisi *pa"i(a proteinuri ne#ati8 atau trace +proteinuria 4 !#@! 2L >@ a!, 3 hari "erturut5turut %a(a! satu !in##u) !a a%ise"ut re!isi&
)elaps *pa"i(a proteinuria 2A +H40 !#@! 2L >@ a! atau rasio protein@ reatinin pa%a urin se?a tu H2 !#@!#, 3 hari"erturut5turut %a(a! satu !in##u) !a a %ise"ut re(aps&
Sindrom nefroti. sensitif steroid 2SNSS3Sin%ro! ne8roti an# apa"i(a %en#an pe!"erian pre%nison %osis penuh +2!#@ #@hari, se(a!a 4 !in##u!en#a(a!i re!isi&
Sindrom nefroti. resisten steroid 2SN)S3Sin%ro! ne8roti an# apa"i(a %en#an pe!"erian pre%nison %osis penuh +2!#@ #@hari, se(a!a 4 !in##u ti%a!en#a(a!i re!isi&
Sindrom nefroti. relaps 5arangSin%ro! ne8roti an# !en#a(a!i re(aps 2 a(i %a(a! / "u(an se a respons a?a( atau 4 a(i %a(a! 1 tahun&
Sindrom nefroti. relaps seringSin%ro! ne8roti an# !en#a(a!i re(aps 2 a(i %a(a! / "u(an se a respons a?a( atau 4 a(i %a(a! 1 tahun&
Sindrom nefroti. dependen steroidSin%ro! ne8roti an# !en#a(a!i re(aps %a(a! 14 hari sete(ah %osis pre%nison %iturun an !en a%i 2@3 %osispenuh atau %ihenti an %an ter a%i 2 a(i "erturut5turut&
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KlasifikasiSin%ro! Ne8roti Sensiti8 Steroi% +SNSS,Sin%ro! Ne8roti Resisten Steroi% +SNRS,
Patofisiologienin# atan per!ea"i(itas %in%in# api(er #(o!eru(usenipisan (uas %ari prosesus po%ositan##uan siste! i!un
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e!eri saan Fisi
en#u uran >>) T>) %an L tiap harien#u uran 6ita( si#n teruta!a TD
Men
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e!eri saan enun an#;rina(isis u(tur urin
rotein urin uantitati8 e!eri saan %arah
La u 8i(trasi #(o!eru(us
Ru!us S
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Tata(a sana
Gambar 3.3 Terapi inisial kortikosteroid 16
Gam!ar "#4 en#o"atan Sin%ro! Ne8roti re(aps 1/
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Gambar 3.5 Pengobatan SN relaps sering dengan CPA oral 16
Gambar 3.5 Pengobatan SN relaps sering
dengan CPA oral16
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*na(isa Kasusasien "en# a pa%a !ata %an perut
se a 3 "u(an an# (a(u& >en# a %ia?a(i%i "a#ian e(opa !ata& >en# a%irasa an saat pa#i hari& *?a(n a han a%i !ata) e!u%ian !en a(ar e se(uruhtu"uh %an s rotu!&
;n%er8i((e% Theor
E%e!a %ise"a" an o(eh!enurunn a te anan on oti
intra6as u(er %an !en e"a" an
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No Kas$s %eori
2& a%a pe!eri saan urina(isis pasien
!enun u an proteinuria %en#anprotein 2A +24@:@201 ,) 3A +27@:@201 ,)
2A +31@:@201 ,) 1A +03@=@201 ,) %an 2A
+0:@=@201 ,
ene(itian an# %i(a u an o(eh
rati?i Dian ra!anaP% +2013, %an 'an%a ani
+2007, !enun u an "ah?a
a%ar protein urin
+se!i uantitati8, ter"an a pa%aana Sin%ro! Ne8roti a%a(ah
A3& roteinuria %ise"a" an
penin# atan per!ea"i(itas
api(er terha%ap protein a i"aterusa an #(o!eru(us
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No Kas$s %eori
3a%a pe!eri saan hati %an
e!pe%u pasien) %i%apat an a%ar
*("u!in 1)= #@%( +14@:@201 ,)
2)10 #@%( +24@:@201 ,) 2)1 #@%(
+01@=@201 ,) 2)30 #@%( +0:@=@201 ,
%an 2):2 #@%( +0=@=@201 ,
enurunan a%ar a("u!in pa%a pasien
Sin%ro! Ne8roti ter a%i a i"at penin# atan
8i(trasi #(o!eru(us terha%ap a("u!in sehin##a
ter a%i proteinuria !asi8 %an a%ar a("u!in
seru! !enurun) !es ipun sintesis a("u!in %i
hepar !enin# at sa!pai 354 a(i&
'ipoa("u!ine!ia ini u#a %ise"a" an o(eh
arena pro%u si a("u!in o(eh hepar ti%a
%apat !en#i!"an#i u!(ah protein an#
%i"uan# +e s resi, e #in a(
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No Kas$s %eori
a%a pe!eri saan i!unosero(o#i
tan##a( 20 *#ustus 201 ) T3 tota( $
0) !!o(@L) T4 tota( $ 43@32
!!o(@L) %an TS's $ 23)/ 0
I;@!(& Dari hasi( (a"oratoriu!
!enun u an "ah?a a%ar T4
%arah ren%ah %an a%ar TS's
tin##i an# !enun u an "ah?a
pasien !en#a(a!i hipotiroi%is!e
an##uan hor!ona( pa%a sin%ro!a ne8roti
arena a%an a proteinuria) sehin##a hor!on
an# teri at o(eh protein a an "er uran#
a%arn a) seperti hor!on tiroi%& 'or!on tiroi%
an# se"a#ian "esar +H==J, %a(a! %arah
teri at protein) %i!ana pa%a sin%ro!a ne8roti
ter a%i pen#e(uaran protein an# "an a )
!a a %en#an sen%irin a hor!on tiroi% a an
i ut e(uar "ersa!a protein pen#i atn a&
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No Kas$s &nalisa
/ FarmakologiI FD D J / #tt@I !a roMeti( re%niso(on 4 !#) 3C1 ta"In 9e8triaCon 300 !#@12 a!In Furose!i% !#@12 a!Spirono(a ton 2C10 !#
(as"u!in 20J 40
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Kesi!pu(anSin%ro! Ne8roti !erupa an !ani8estasi #(o!eru(us an# pa(in#serin# %ite!u an pa%a ana an# %itan%ai %en#an proteinuria !assi8+ 40 !#@!2 L >@ a! atau rasio protein@ reatinin pa%a urin se?a tuH 2 atau %ipsti< 2A,) hipoa("u!ine!ia + 2) #r@%L,) e%e!a) serta%apat %isertai hiper o(estero(e!ia +2 0 !#@uL,Dari hasi( ana!nesis) pe!eri saan 8isi %an pe!eri saan penun an#pasien !en%erita Sin%ro! Ne8roti %en#an e(uhan "en# a pa%ase(uruh tu"uh an# %ia?a(i %i "a#ian e(opa !ata pa%a saat "an#unpa#i) e!u%ian !en a(ar e se(uruh tu"uh %an s rotu!) %an hasi((a"oratoriu! pasien !enun u an proteinuria) hipoa("u!in %anhiper o(estero(e!ia
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Teri!a Kasih