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LAPORAN KASUS

SINDROMA NEFROTIK

DOKTER PEMBIMBING

dr. Mas Wishnuwardhana, Sp. A

DISUSUN OLE

Muha!!ad Fa"hri Ridha #r$an

%&%.'%.'(%

RUMA SAKIT UMUM DAERA BEKASI

ILMU KESEATAN ANAK

FAKULTAS KEDOKTERAN UNI)ERSITAS TRISAKTI

PERIODE *+ DESEMBER *%' - MARET *%'

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DAFTAR ISI

BAB I PENDAULUAN.........................................................................................................&

BAB II LAPORAN KASUS..................................................................................................../

BAB III TIN0AUAN PUSTAKA...........................................................................................'(

ANATOMI GINJAL.............................................................................................................19

FISIOLOGI GINJAL............................................................................................................22

SINDROMA NEFROTIK....................................................................................................24

Definisi..............................................................................................................................24

Epidemioloi.....................................................................................................................24E!ioloi..............................................................................................................................24

Kl"sifi#"si.........................................................................................................................2$

%em&"i"n %"!oloi An"!omi............................................................................................2'

%"!ofisioloi......................................................................................................................29

Ge("l" Klinis.....................................................................................................................)2

T"!"l"#s"n"........................................................................................................................)*

Kompli#"si........................................................................................................................)9

%+onosis...........................................................................................................................41

DAFTAR PUSTAKA............................................................................................................../*

2

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BAB I

PENDAULUAN

Sind+om nef+o!i# ,SN- "d"l" se#/mp/l"n m"nifes!"si #linis 0"n di!"nd"i ole

p+o!ein/+i" m"sif ,le&i d"+i )* 13) m2 l/"s pe+m/#""n !/&/ pe+ "+i- ipo"l&/minemi"

,#/+"n d"+i ) dl- edem" ipe+lipidemi" lipid/+i" d"n ipe+#o"/l"&ili!"s. e+d"s"+#"n

e!ioloin0" SN d"p"! di&"i men("di SN p+ime+ ,idiop"!i#- 0"n &e+/&/n"n den"n

#el"in"n p+ime+ lome+/l/s den"n se&"& !id"# di#e!"/i d"n SN se#/nde+ 0"n dise&""n

ole pen0"#i! !e+!en!/.1

%"d" "n"#5"n"# ,6 1$ !"/n- p"lin se+in di!em/#"n nef+op"!i lesi minim"l ,3*75

'*7- den"n /m/+ +"!"5+"!" 2* !"/n '87 6 $ !"/n s""! di"nosis di&/"! d"n l"#i5l"#i d/"

#"li le&i &"n0"# d"+ip"d" "ni!". %"d" o+"n de"s" p"lin &"n0"# nef+op"!i mem&+"nos"

,)875*87- /m/+ +"!"5+"!" )85*8 !"/n d"n pe+&"ndin"n l"#i5l"#i d"n "ni!" 2 : 1.

Ke("di"n SN idiop"!i# 25) #"s/s188.888 "n"#!"/n sed"n#"n p"d" de"s"

)1888.888!"/n. Sind+om nef+o!i# se#/nde+ p"d" o+"n de"s" !e+&"n0"# dise&""n ole

di"&e!es melli!/s.2

%en0e&"& SN se#/nde+ s"n"! &"n0"# di "n!"+"n0" pen0"#i! infe#si #e"n"s"n o&"!5

o&"!"n pen0"#i! m/l!isis!em d"n ("+in"n i#"! +e"#si "le+i pen0"#i! me!"&oli# pen0"#i!

e+edi!e+5f"mili"l !o#sin !+"nspl"n!"si in("l !+om&osis ;en" +en"lis s!enosis "+!e+i +en"lis

o&esi!"s m"ssif. Di #lini# ,3*75'87- #"s/s SN m"si me+/p"#"n SN p+ime+ ,idiop"!i#-.)

)

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BAB II

LAPORAN KASUS

I. IDENTITAS PASIEN

Da1a Pasi#n A2ah I3u

N"m" An. < Tn. M N0. =

>m/+ 2 !"/n )$ !"/n )4 !"/n

Jenis Kel"min L"#i5 l"#i L"#i5l"#i %e+emp/"n

Al"m"! e#"si

A"m" Isl"m Isl"m Isl"m

S/#/ &"ns" 5 J"" J""

%endidi#"n SD S1 SMA

%e#e+(""n 5 G/+/ I&/ R/m" T"n"

Ke!e+"n"n D Ko!" e#"si.

a4 K#$uhan U1a!a5

%"sien d"!"n #"+en" sel/+/ &"d"nn0" &en#"# se("# d/" min/ se&el/m m"s/#

+/m" s"#i!.

34 Riwa2a1 P#n2a6i1 S#6aran75

%"sien d"!"n #e poli "n"# RS>D Ko!" e#"si den"n #el/"n sel/+/ &"d"n

&en#"# se("# 2 min/ 0"n l"l/. en#"# ""ln0" dim/l"i d"+i #ed/" #elop"#

m"!" p"sien #em/di"n #el"min d"n #ed/" !/n#"i. %e+/! di+"s"#"n le&i mem&es"+.

en#"# !e+/!"m" p"d" p"i "+i s""! &"n/n !id/+ #em/di"n &e+#/+"n s""! si"n

"+i d"n so+e "+i. Sel"m" &en#"# o+"n !/" men0"d"+i &"" "i+ #en?in p"sien

men("di &e+"+n" #/nin #e+/ d"n sedi#i! &e+&/s". F+e#/ensi AK 45* #"li se"+i.

Kel/"n se+in !e+&"n/n m"l"m "+i /n!/# #en?in dis"n#"l. Ses"# n"p"s

4

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dis"n#"l. An"# !id"# pe+n" m/n!"5m/n!" dem"m d"n #e("n. Sel"m" s"#i!

p"sien !id"# pe+n" !"mp"# p/?"! lem" les/ "!"/ #eil"n"n n"fs/ m"#"n. %"sien

semp"! menel/#"n "d"n0" A ?"i+ ) "+i se&el/m &en#"# !im&/l #el/"n n0e+i

pe+/! dis"n#"l. Se#"+"n A no+m"l.

"4 Riwa2a1 P#n2a6i1 Dahu$u5

P#n2a6i1 U!ur P#n2a6i1 U!ur P#n2a6i1 U!ur

Ale+i 5 Dif!e+i" 5 J"n!/n 5

"?in"n 5 Di"+e 5 Gin("l 5

DD 5 Ke("n 5 D"+" 5

T0poid 5 M"" 5 R"d"n p"+/ 5

Sind+om

nef+o!i#5 B"+i?el" 5 T/&e+#/losis 5

%"+o!is 5 Asm" 5 Mo+&ili 5

Kes"n: p"sien !id"# pe+n" m"s/# +/m" s"#i! se&el/mn0". Sese#"li pe+n" &"!/#

d"n pile# n"m/n sem&/ den"n min/m o&"! d"+i p/s#esm"s.

d4 Riwa2a1 K#ha!i$an dan K#$ahiran5

KEAMILAN Mo+&idi!"s #e"mil"n An"# #e!i"

%e+""!"n "n!en"!"l %e+i#s" #e &id"n 1 #"li !i"p &/l"n

KELAIRAN Temp"! #el"i+"n id"n

%enolon pe+s"lin"n id"n

"+" pe+s"lin"n No+m"l

M"s" es!"si 48 min/

%en0/li! Tid"# "d"

Ke"d""n &"0i

e+"! l"i+ ).288 +"m

%"n("n &"d"n !id"# in"!

Lin#"+ #ep"l" !id"# in"!

L"ns/n men"nis

*

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Nil"i "p"+ !id"# !"/

Tid"# "d" #el"in"n &"""n

Kes"n : Ri"0"! #e"mil"n d"n #el"i+"n p"sien &"i#

#4 Riwa2a1 K#$uar7a5

Da1a A2ah I3u

N"m" Tn. M N0. =

%e+#"in"n #e %e+!"m" %e+!"m"

>m/+ )$ !"/n )4 !"/n

Ke"d""n #ese"!"n "i# "i#

84 Riwa2a1 P#r1u!3uhan dan P#r6#!3an7an5

%e+!/m&/"n ii I : 5 ,no+m"l *59 &/l"n-

%si#omo!o+

Men"n#"! #ep"l" : 5 ,no+m"l 15) &/l"n-

Ten#/+"p : 5 ,no+m"l 15) &/l"n-

D/d/# : $ &/l"n ,no+m"l $ &/l"n-

e+di+i : 11 &/l"n ,no+m"l 9512 &/l"n-

e+("l"n : 1) &/l"n ,no+m"l 1) &/l"n-i?"+" : 12 &/l"n ,no+m"l 9512 &/l"n-

Kes"n : Ri"0"! pe+!/m&/"n d"n pe+#em&"n"n p"sien ses/"i /si"

74 Riwa2a1 Ma6anan5

U!ur 93u$an4 ASI:PASI Buah:3is6ui1 Bu3ur susu Nasi 1i!

852 ASI 5 5 5

254 ASI 5 5 5

45$ ASI 5 5 5

$5' ASI /" C &is#/i! /&/+ s/s/ 5

'518 ASI C S/s/

fo+m/l"

/" C &is#/i! /&/+ s/s/ N"si !im

Kes"n : Ke&/!/"n ii p"sien !e+pen/i ?/#/p &"i#

h4 Riwa2a1 I!unisasi5

%"sien mend"p"!#"n im/nis"si se?"+" len#"p.

$

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i4 Riwa2a1 P#ru!ahan dan Sani1asi5

Tin"l di +/m" sendi+i !e+d"p"! 2 #"m"+. Ben!il"si d"n ?""0" m"!""+i ?/#/p "i+

min/m &e+"s"l d"+i "i+ #em"s"n "i+ m"ndi &e+"s"l d"+i "i+ %AM.

Kes"n : Kese"!"n lin#/n"n !emp"! !in"l p"sien ?/#/p &"i#

III. PEMERIKSAAN FISIK

Ke"d""n /m/m : ompos men!is

AB%> : Ale+!

%edi"!+i? Assesmen! T+i"nle%AT

T"nd" Bi!"l

o F+e#/ensi n"di : 112meni!

o Te#"n"n d"+" : 118'8mm

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'

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Kep"l"

o en!/# : no+mo?ep"li

o R"m&/! : i!"m !id"# m/d" di?"&/! dis!+i&/si &"i#

o M"!" : #on(/n!i;" "nemis 55 s#le+" i#!e+i# 55 p/pil iso#o+ RL C

C RTL CC #d#!a pa$p#3ra ;:;o Telin": no+mo!i" se+/men 55 pe+d"+""n 55

9

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o 485*4 7 M#nurun

T+om&osi! 488.888/L 1*8.8885

488.888/L

No+m"l

i1un7 ?#nis

18

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"sofil 8 7 6 1 7 No+m"l

Eosinofil ) 7 15) 7 No+m"l

"!"n 2 7 25$ 7 No+m"l

Semen! *2 7 *2538 7 No+m"l

Limfosi! )9 7 28548 7 No+m"l

Monosi! 4 7 25' 7 No+m"lE+i!+osi! 4.3$ (/!"/L 45* (/!"/L No+m"l

Ki!ia K$ini6

%+o!ein To!"l /,%% $$538 dL M#nurun

Al&/min %,+ )*54* dL M#nurun

Glo&/lin &,'/ 1*5)8 dL M#nin76a1

Fun7si Gin?a$

>+e/m 28 28548 No+m"l

K+e"!inin 8* 8*51) No+m"l

L#!a6

Koles!e+ol !o!"l //* 6 288 mdL M#nin76a1E$#61r+o&ilinoen 82 >E 88582 >E

Ni!+i!"#!e+i Ne"!if Ne"!if

Le/#osi! /+in C Ne"!if

S#di!#n

Le/#osi! sedimen 85* lp& 6 * lp&

E+is!+osi! 852 lp& 6 2lp&

Silinde+ %osi!if Ne"!if

Epi!el Gepen ,C- Gepen ,C-

K+is!"l %osi!if Ne"!if

"#!e+i Ne"!if Ne"!if

L"in5l"in Ne"!if Ne"!if

). RESUME

%"sien An.

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en#"# !e+/!"m" p"d" p"i "+i s""! &"n/n !id/+ #em/di"n &e+#/+"n s""! si"n "+i d"n

so+e "+i. Sel"m" &en#"# o+"n !/" men0"d"+i &"" "i+ #en?in p"sien men("di &e+"+n"

#/nin #e+/ d"n sedi#i! &e+&/s". F+e#/ensi AK 45* #"li se"+i. ) "+i se&el/m &en#"#

p"sien menel/ A ?"i+ n"m/n se#"+"n A no+m"l.

D"+i peme+i#s""n fisi# did"p"!#"n #e"d""n /m/m ?ompos men!is den"n !e#"n"n

d"+": 118'8 mm

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S//

%i!!in Edem"

)$4

C

)$)

C

)$4

C

)$8

C

A: Sind+om"

nef+o!i#

Sind+om"

nef+o!i#

Sind+om"

nef+o!i#

Sind+om"

nef+o!i#

%: IBFD D*7

F/+osemide

218m

Amo?ilin

))88m i;

IBFD D*7

F/+osemide

218m

Amo?ilin

))88m i;

IBFD D*7

F/+osemide

218m

%+ednison

2m#

"+i ,25251-

IBFD D*7

F/+osemide

218m

Amo?ilin

))88m i;

%+ednison

2m#

"+i ,25251-

T+"nsf/si

"l&/min 287

@. ANALISA KASUS

D"+i "n"mnesis did"p"!#"n p"sien d"!"n #e poli "n"# RS>D Ko!" e#"si den"n

#el/"n &en#"# di sel/+/ !/&/ se("# 2 min/ 0"n l"l/. O+"n !/" p"sien men"!"#"n

&"" &en#"# dim/l"i d"+i #elop"# m"!" #em/di"n #e !/n#"i pe+/! d"n #em"l/"n.

Se&el/mn0" p"sien semp"! menel/ A ?"i+ se#i!"+ ) "+i se&el/m d"!"n #e poli

n"m/n se#"+"n A s/d" no+m"l.

Ri"0"! ses"# n"f"s dem"m &"!/# pile# d"n ?ep"! lel" dis"n#"l. %"sien AK

se#i!"+ * #"li se"+i se("# s"#i! d"n #en?inn0" &e+"+n" #/nin #e+/ se+!" &e+&/s".

Kel/"n ini &"+/ pe+!"m" #"li di"l"mi p"sien. Se&el/mn0" p"sien &el/m pe+n" s"#i!

&e+"! in" "+/s m"s/# +/m" s"#i!.

%eme+i#s""n fisi# did"p"!#"n #e"d""n /m/m#es"d"+"n: !"mp"# s"#i! sed"n ?ompos

men!is !"mp"# &en#"# p"d" "(" pe+/! d"n #ed/" !/n#"i. D"!" An!+opome!+i

o e+"! &"d"n : 1$ #

o Tini &"d"n : '$ ?m

o S!"!/s ii men/+/! D:

> 1$1) 1887 12)7 ,ii le&i-

T> '$'' 1887 937 ,ii &"i#-

T 1$1)* 1887 11' 7

Kes"n O&esi!"s ,#"+en" p"sien men"l"mi oedem"-

1)

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%"d" m"!" did"p"!#"n edem" p"lpe&+"l ,CC-. A&domen !"mp"# ?em&/n &isin /s/s

,C- p"d" p"lp"si !id"# di!em/#"n "d"n0" o+"nome"li "!"/ n0e+i !e#"n %e+#/si "&domen

did"p"!#"n "d"n0" sif!in d/llness ,C-.

%"d" peme+i#s""n eni!"li"n e#s!e+n" did"p"!#"n "d"n0" edem" p"d" s?+o!/m. %"d"

#ed/" !/n#"i did"p"!#"n pi!!in edem".

%e+("l"n"n pen0"#i! p"d" sind+om" nef+o!i# ""ln0" meni#/!i sind+om p"d" p"sien

ini sepe+!i &en#"# p"d" p"lpe&+"l d"n oli/+i" #em/di"n se!el" &e&e+"p" "+i edem"

sem"#in (el"s d"n men("di edem" "n"s"+#".

D"+i "sil peme+i#s""n pen/n("n p"d" s""! p"sien pe+!"m" m"s/# RS did"p"!#"n

"sil nil"i "l&/min 0"n +end" 0"i!/ 8'$ d"n nil"i #oles!e+ol !o!"l 0"n !ini 442. D"+i

"sil /+in"lisis did"p"!#"n "d"n0" "l&/min d"l"m /+in ,CC- sein" d"+i !em/"n di "!"s

di"nosis sind+om" nef+o!i# d"p"! di!e"##"n.

Sind+om nef+o!i# di!e"##"n &e+d"s"+#"n 4 e("l" #lini# 0"n #"s 0"i!/ :

1. %+o!ein/+i" m"sif "!"/ p+o!ein/+i" nef+o!i#

Dim"n" d"l"m /+in !e+d"p"! p+o!ein H 48 mm2 lp&("m "!"/ *8 m# 24 ("m

"!"/ +"sio "l&/min #+e"!inin p"d" /+in se"#!/ 2 mm "!"/ dips!i# H 2C. %+o!ein/+i"

p"d" sind+om nef+o!i# #el"in"n minim"l +el"!if sele#!if 0"n !e+&en!/# !e+/!"m" ole

"l&/min.

2.

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!e+("di +emisi p"d" 4 min/ pe+!"m" m"#" pem&e+i"n s!e+oid dil"n(/!#"n den"n 4 min/

#ed/" den"n dosis 48mm2,2) dosis ""l- se?"+" "l!e+n"!in ,sel"n se"+i- 1 #"li se"+i

se!el" m"#"n p"i. il" se!el" 4 min/ peno&"!"n s!e+oid dosis pen/ !id"# !e+("di

+emisi p"sien din0"!"#"n se&""i +esis!en s!e+oid.

%em&e+i"n di/+e!i# di"n(/+#"n sel"m" "d" edem" &e+"!. i"s"n0" di&e+i#"n loop di/+e!i?

sepe+!i f/+osemide 152 m#"+i &il" pe+l/ di#om&in"si#"n den"n spi+onol"#!on

,"n!"onis "ldos!e+on di/+e!i# em"! #"li/m- 25) m#"+i. %"d" pem"#"i"n di/+e!i#

le&i l"m" d"+i 152 min/ pe+l/ dil"#/#"n pem"n!"/"n ele#!+oli! d"+" ,#"li/m d"n

n"!+i/m-.

BAB III

TIN0AUAN PUSTAKA

I. ANATOMI GIN0AL

Gin("l !e+le!"# di d"l"m +/"n +e!+ope+i!one/m sedi#i! di "!"s #e!ini"n /m&ili#/s

d"n #is"+"n p"n("n se+!" &e+"!n0" &e+!/+/!5!/+/! d"+i #i+"5#i+" $ ?m d"n 24 p"d" &"0i

?/#/p &/l"n s"mp"i 12 ?m "!"/ le&i d"+i 1*8 p"d" o+"n de"s". Gin("l memp/n0"i

l"pis"n l/"+ korteks0"n &e+isi lome+/li !/&/l/s #on!o+!/s p+o#sim"lis d"n dis!"lis d"n

d/#!/s #ole#!i;/s se+!" di l"pis"n d"l"m medula 0"n men"nd/n &"i"n5&"i"n !/&/l/s

0"n l/+/s len#/n ,"ns"-

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%/n?"# p0+"mid med/ll" menon(ol #e d"l"m dise&/! p"pil in("l 0"n me+/p"#"n

/(/n #"li#s mino+. e&e+"p" d/#!/s #oliens &e+m/"+" p"d" d/#!/s p"pil"+is ellini 0"n

/(/nn0" &e+m/"+" di p"pil in("l d"n men"li+#"n /+in #e d"l"m #"li#s mino+. K"+en" "d"

1'524 l/&"n m/"+" d/#!/s elini p"d" /(/n p"pil m"#" d"e+" !e+se&/! !e+li"! se&""i

!"pis"n &e+"s d"n dise&/! (/" den"n "+e" #+i&os"

An!"+" d/" p0+"mid !e+se&/! !e+d"p"! ("+in"n #o+!e#s !emp"! m"s/#n0" ?"&"n5?"&"n

"+!e+i +en"lis dise&/! #ol/mn" e+!ini. e&e+"p" #"li#s mino+ mem&en!/# #"li#s m"0o+ 0"n

&e+s"!/ men("di pi"l" ,pel;is- in("l d"n #em/di"n &e+m/"+" #e d"l"m /+e!e+. >+e!e+ #"n"n

d"n #i+i &e+m/"+" di #"nd/n #emi 0"n (/" dise&/! &/li5&/li "!"/ ;esi#" /+in"+i". >+in

di#el/"+#"n d"+i #"nd/n #emi mel"l/i /+e!+".

13

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Si+#/l"si Gin("l

Se!i"p in("l mene+im" #i+"5#i+" 2*7 isi se#/n?/p ("n/n. il" dipe+&"ndin#"n

den"n &e+"! o+"n in("l "l ini me+/p"#"n s/pl"i d"+" !e+&es"+ did"l"m !/&/ m"n/si".

S/pl"i d"+" p"d" se!i"p in("l &i"s"n0" &e+"s"l d"+i "+!e+i +en"lis /!"m" 0"n #el/"+ d"+i

"o+!" "+!e+i +en"lis m/l!ipel &/#"nn0" !id"# l"im di(/mp"i. A+!e+i +en"lis /!"m" mem&"i

men("di med/l" #e &"!"s "n!"+" #o+!e#s d"n med/l". %"d" d"e+" ini "+!e+i in!e+lo&"+is

&e+?"&"n mem&en!/# "+!e+i "+#/"!" d"n mem&en!/# "+!e+iole "fe+en lome+/l/s. Sel5sel

o!o! 0"n !e+spesi"lis"si d"l"m dindin "+!e+iole "fe+en &e+s"m" den"n sel l"?is d"n &"i"n

dis!"l !/&/l/s ,m/#/l" dens"- 0"n &e+de#"!"n den"n lome+/l/s mem&en!/# "p"+"!/s

(/#s!"lome+/le+ 0"n menend"li#"n se#+esi +enin. A+!e+iole "fe+en mem&"i men("di

"n0"m"n #"pile+ lome+/l/s 0"n #em/di"n &e+"&/n men("di "+!e+iole efe+en. A+!e+iole

efe+en lome+/l/s de#"! med/l" ,lome+/l/s (/#s!"med/ll"+is- le&i &es"+ d"+i p"d" "+!e+iole

di #o+!e#s se&el" l/"+ d"n mem&e+i#"n p"so#"n d"+" ,;"s" +"#!"- #e !/&/l/s d"n med/l".

S!+/#!/+ Nef+on

Ti"p in("l men"nd/n #/+"n le&i 1 (/!" nef+on ,lome+ol/s d"n !/&/l/s 0"n

&e+/&/n"n den"nn0"-. %"d" m"n/si" pem&en!/##"n nef+on seles"i p"d" ("nin )* min/.

Nef+on &"+/ !id"# di&en!/# l"i se!el" l"i+. %e+#em&"n"n sel"n(/!n0" "d"l" 0pe+pl"si"d"n ipe+!+ofi s!+/#!/+ 0"n s/d" "d" dise+!"i m"!/+"si f/nsion"l. %e+#em&"n"n p"lin

?ep"! !e+("di p"d" * !"/n pe+!"m" se!el" l"i+. Ole #"+en" i!/ &il" p"d" m"s" ini !e+("di

"n/"n mis"ln0" infe#si s"l/+"n #emi "!"/ +efl/#s m"#" "l ini d"p"! men"n/

pe+!/m&/"n in("l.*

Ti"p nef+on !e+di+i "!"s lome+ol/s d"n #"ps/l" &om"n !/&/l/s p+o#sim"l "ns"

enle d"n !/&/l/s dis!"l. Glome+ol/s &e+s"m" #"ps/l" om"n (/" dise&/! &"d"n M"lpii.

Mes#ip/n /l!+"fil!+"si pl"sm" !e+("di di lome+ol/s !e!"pi pe+"n"n !/&/l/s d"l"m

pem&en!/##"n /+in !id"# #"l" pen!inn0" d"l"m pen"!/+"n meli"/ in!e+n"l. F/nsi in("l

no+m"l !e+di+i "!"s ) #omponen 0"n s"lin &e+/&/n"n 0"i!/ :

1. >l!+"fil!+"si lome+ol/s

2. Re"&so+&si !/&/l/s !e+"d"p sol/!e d"n "i+

). Se#+esi !/&/l/s !e+"d"p "!5"! o+"ni? d"n non5o+"ni#

%op/l"si lome+ol/s "d" 2 m"?"m :

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1. Glome+ol/s #o+!e#s 0"n memp/n0"i "ns" enle 0"n pende# &e+"d" di&"i"n l/"+

#o+!e#s

2. Glome+ol/s (/#s!"med/l"+ 0"n memp/n0"i "ns" enle 0"n p"n("n s"mp"i #e

&"i"n d"l"m med/ll". Glome+ol/s sem"?"m ini &e+"d" dipe+&"!"s"n #o+!e#s d"n

med/ll" d"n me+/p"#"n 287 pop/l"si nef+on !e!"pi s"n"! pen!in /n!/# +e"&so+psi

"i+ d"n sol/!e.

II. FISI

OL

OGI

GIN0AL

Gin("l i#/! men"!/+ #eseim&"n"n &io#imi" !/&/ m"n/si" den"n ?"+" men"!/+

#eseim&"n"n "i+ men"!/+ #onsen!+"si "+"m d"l"m d"+" men"!/+ "s"m &"s" d"+"

pen"!/+"n e#s#+esi &""n &/"n"n d"n #ele&i"n "+"m d"n memp+od/#si o+mon 0"i!/ :

". Prostaglandin 0"n &e+f/nsi /n!/# pen"!/+"n "+"m d"n "i+ se+!"

mempen"+/i !e#"n"n ;"s#/le+.

&. Eritropoietin 0"n &e+f/nsi /n!/# me+"ns"n p+od/#si sel d"+" me+".

III.?. 1,! di"idroksikolekalsiferol 0"n &e+f/nsi mempe+#/"! "&so+psi #"lsi/m d"+i

/s/s d"n +e"&so+&si fosf"! ole !/&/l/s +en"lis.

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d. Renin 0"n &e+f/nsi &e#e+(" p"d" ("l/+ "nio!ensin /n!/# menin#"!#"n

!e#"n"n ;"s#/le+ d"n p+od/#si "ldos!e+on.

Ti" !""p pem&en!/#"n /+in:

1- Fil!+"si lome+/l"+

%em&en!/#"n #emi dim/l"i den"n fil!+"si pl"sm" p"d" lome+/l/s

sepe+!i #"pile+ !/&/ l"inn0" #"pile+ lome+/l/s se?"+" +el"!if &e+sif"!

impe+me"&le !e+"d"p p+o!ein pl"sm" 0"n &es"+ d"n ?/#/p pe+me"&le !e+"d"p

"i+ d"n l"+/!"n 0"n le&i #e?il sepe+!i ele#!+oli! "s"m "mino l/#os" d"n sis"

ni!+oen. Ali+"n d"+" in("l ,RF Ren"l lood Flo- "d"l" se#i!"+ 2*7 d"+i

?/+" ("n!/n "!"/ se#i!"+ 1288mlmeni!. Se#i!"+ sepe+lim" d"+i pl"sm" "!"/

se#i!"+ 12*mlmeni! di"li+#"n mel"l/i lome+/l/s #e #"ps/l om"n. Ini di#en"l

den"n l"(/ fil!+"si lome+/l/s ,GFR Glome+/l"+ Fil!+"!ion R"!e-. Ge+"#"n

m"s/# #e #"ps/l" &om"n dise&/! fil!+"!e. Te#"n"n fil!+"si &e+"s"l d"+i pe+&ed""n

!e#"n"n 0"n !e+d"p"! "n!"+" #"pile+ lome+/l/s d"n #"ps/l" &om"n !e#"n"n

id+os!"!i# d"+" d"l"m #"pile+ lome+/l/s mempe+m/d" fil!+"si d"n #e#/"!"n ini

dil""n ole !e#"n"n id+os!"!i# fil!+"!e d"l"m #"ps/l" &om"n se+!" !e#"n"n

osmo!i# #oloid d"+". Fil!+"si lome+/l/s !id"# "n0" dipen"+/i ole !e#"n"n

!e#"n"n #oloid di"!"s n"m/n (/" ole pe+me"&ili!"s dindin #"pile+.

2- Re"&so+psi

="!5"! 0"n difill!+"si in("l di&"i d"l"m ) &"i"n 0"i!/ : non ele#!+oli!

ele#!+oli! d"n "i+. Se!el" fil!+"si l"n#" #ed/" "d"l" +e"&so+psi sele#!if "!5"!

!e+se&/! #em&"li l"i "!5"! 0"n s/d" difil!+"si.

)- Se#+esi

Se#+esi !/&/l"+ meli&"!#"n !+"nsfo+ "#!if mole#/l5mole#/l d"+i "li+"n

d"+" mel"l/i !/&/l/s #ed"l"m fil!+"!. "n0"# s/&s!"nsi 0"n dise#+esi !id"#

!e+("di se?"+" "l"mi" d"l"m !/&/ ,mis"ln0" penisilin-. S/&s!"nsi 0"n se?"+"

"l"mi" !e+("di d"l"m !/&/ !e+m"s/# "s"m /+"! d"n #"li/m se+!" ion5ion

id+oen.

%"d" !/&/l/s dis!"lis !+"nsfo+ "#!if n"!+i/m sis!em ?"+ie+ 0"n (/" !eli&"!

d"l"m se#+esi id+oen d"n ion5ion #"li/m !/&/l"+. D"l"m /&/n"n ini !i"p

#"li ?"+ie+ mem&"" n"!+i/m #el/"+ d"+i ?"i+"n !/&/l"+ ?"+ie+n0" &is" id+oen

"!"/ ion #"li/m #ed"l"m ?"i+"n !/&/l"+ pe+("l"n"nn0" #em&"li ("di /n!/#

se!i"p ion n"!+i/m 0"n di"&so+psi id+oen "!"/ #"li/m "+/s dise#+esi d"nse&"li#n0". %ili"n #"!ion 0"n "#"n dise#+esi !e+"n!/n p"d" #onsen!+"si ?"i+"n

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e#s!+"!/&/l"+ ,ES- d"+i ion5ion ini ,id+oen d"n #"li/m-. %ene!"/"n !en!"n

pe+!/#"+"n #"!ion d"l"m !/&/l/s dis!"lis ini mem&"n!/ #i!" mem""mi &e&e+"p"

/&/n"n 0"n dimili#i ele#!+oli! den"n l"inn0". Se&""i ?on!o #i!" d"p"!

mene+!i men"p" &lo#e+ "ldos!e+on d"p"! men0e&""n ipe+#"lemi" "!"/

men"p" p"d" ""ln0" d"p"! !e+("di pen/+/n"n #"li/m pl"sm" #e!i#" "sidosis

&e+"! di#o+e#si se?"+" !e/+"pe/!i#.

I. DEFINISI

Sind+om nef+o!i# "d"l" s"l" s"!/ pen0"#i! in("l 0"n se+in di(/mp"i p"d"

"n"# me+/p"#"n s/"!/ #/mp/l"n e("l"5e("l" #linis 0"n !e+di+i d"+i p+o!ein/+i"

m"sif ipo"l&/minemi" ipe+#oles!e+olemi" se+!" sem&"&. "n dim"#s/d

p+o!ein/+i" m"sif "d"l" "p"&il" did"p"!#"n p+o!ein/+i" se&es"+ *85188 m# &e+"!

&"d"n"+i "!"/ le&i. Al&/min d"l"m d"+" &i"s"n0" men/+/n in" #/+"n d"+i 2*

+"mdl. Sel"in e("l"5e("l" #linis di "!"s #"d"n5#"d"n di(/mp"i p/l" ipe+!ensi

em"!/+i &"#"n #"d"n5#"d"n "o!emi".

II. EPIDEMIOLOGI

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Sind+om nef+o!i# 0"n !id"# men0e+!"i pen0"#i! sis!emi# dise&/! sind+om"

nef+o!i# p+ime+. %en0"#i! ini di!em/#"n 987 p"d" #"s/s "n"#. Ap"&il" ini !im&/l

se&""i &"i"n d"+ip"d" pen0"#i! sis!emi# "!"/ &e+/&/n"n den"n o&"! "!"/ !o#sin

m"#" dise&/! sind+om" nef+o!i# se#/nde+. Insidens pen0"#i! sind+om nef+o!i# p+ime+

ini 2 #"s/s pe+5!"/n !i"p 188.888 "n"# &e+/m/+ #/+"n d"+i 1$ !"/n den"n "n#"

p+e;"lensi #/m/l"!if 1$ !i"p 188.888 "n"#. Insidens di Indonesi" dipe+#i+"#"n $ #"s/s

pe+5!"/n !i"p 188.888 "n"# #/+"n d"+i 14 !"/n. R"sio "n!"+" lel"#i d"n pe+emp/"n

p"d" "n"# se#i!"+ 2:1. L"po+"n d"+i l/"+ nee+i men/n(/##"n 2) #"s/s "n"# den"n

SN di(/mp"i p"d" /m/+ #/+"n d"+i * !"/n.

III. ETIOLOGI

Sind+om nef+o!i# d"p"! dise&""n ole lome+/lonef+i!is p+ime+ d"n

se#/nde+ "#i&"! infe#si #e"n"s"n pen0"#i! ("+in"n pen/&/n ,?onne?!i;e !iss/e

dise"se- o&"! "!"/ !o#sin d"n "#i&"! pen0"#i! sis!emi# sepe+!i &e+i#/! :

A. Glome+/lonef+i!is ,GN- p+ime+:

Sindrom nefrotik primer f"#!o+ e!ioloin0" !id"# di#e!"/i. Di#"!"#"n sind+om

nef+o!i# p+ime+ ole #"+en" sind+om nef+o!i# ini se?"+" p+ime+ !e+("di "#i&"!

#el"in"n p"d" lome+/l/s i!/ sendi+i !"np" "d" pen0e&"& l"in. Golon"n ini

p"lin se+in di(/mp"i p"d" "n"#. Te+m"s/# d"l"m sind+om nef+o!i# p+ime+

"d"l"sindrom nefrotik kongenital 0"i!/ s"l" s"!/ (enis sind+om nef+o!i# 0"n

di!em/#"n se("# "n"# i!/ l"i+ "!"/ /si" di &"" 1 !"/n

GN lesi minim"l ,GNLM-

Glome+/los#le+osis fo#"l ,GSF-

GN mem&+"nos" ,GNMN-

GN mem&+"nop+olife+"!if ,GNM%-

GN p+olife+"!i;e l"in

. GN se#/nde+:

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Tim&/l se&""i "#i&"! d"+i s/"!/ pen0"#i! sis!emi# "!"/ se&""i "#i&"! d"+i

&e+&""i se&"& 0"n n0"!" sepe+!i mis"ln0" efe# s"mpin o&"!. %en0e&"& 0"n

se+in di(/mp"i "d"l" :

o Infe#si:

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Se?"+" #linis sind+om" nef+o!i# di&"i men("di ) #elompo# :

1. Sind+om nef+o!i# &"""n

Di!/+/n#"n se&""i +esesif "/!osom"l "!"/ #"+en" +e"#si m"!e+nofe!"l.

Ge("l"n0" "d"l" edem" p"d" m"s" neon"!/s. Sind+om nef+o!i# (enis ini

+esis!en !e+"d"p sem/" peno&"!"n. S"l" s"!/ ?"+" 0"n &is" dil"#/#"n

"d"l" pen?"n#o#"n in("l p"d" m"s" neon"!/s n"m/n !id"# &e+"sil.

%+onosis &/+/# d"n &i"s"n0" pende+i!" menin"l d"l"m &/l"n5&/l"n pe+!"m"

#eid/p"nn0".

2. Sind+om nef+o!i# se#/nde+ dise&""n ole :

M"l"+i" #/"+!"n" "!"/ p"+"si! l"in.

%en0"#i! #ol"en sepe+!i l/p/s e+i!em"!os/s disemin"!" p/+p/+"

"n"fil"#!oid.

Gl/me+onef+i!is "#/! "!"/ l/me+onef+i!is #+onis !+om&osis ;en"

+en"lis.

""n #imi" sepe+!i !+ime!"dion p"+"dion penisil"min "+"m em"s

sen"!"n le&" +"?/n o"# "i+ +"#s".

). Sind+om nef+o!i# idiop"!i# 0"n di&"i men("di 4 olon"n 0"i!/

Kel"in"n minim"l

Glome+ol/s !"mp"# no+m"l ,mi#+os#op &i"s"- "!"/ !"mp"# foo!

p+o?ess/s sel epi!el &e+p"d/,mi#+os#op ele#!+on-

Den"n imon/fl/o+esensi !id"# "d" IG "!"/ im/nolo&/lin

&e!"5I p"d" dindin #"pile+ lome+/l/s

Le&i &"n0"# !e+d"p"! p"d" "n"#

%+onosis &"i#

Nef+op"!i mem&+"nos"

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Glome+/l/s men/n(/#"n pene&"l"n dindin #"pile+ 0"n

!e+se&"+ !"np" p+olife+"si sel

%+onosis #/+"n &"i#

Glome+/lonef+i!is p+olife+"!i;e

E#s/d"!if dif/s: Te+d"p"! p+olif"+"si sel mes"ni"l d"n infil!+"si

polimo+fon/#le/s. %em&en#"#"n si!opl"sm" endo!el 0"n

men0e&""n #"pile+ !e+s/m&"!.

%ene&"l"n &"!"n lo&/l"+ ,lo&/l"+ s!"l# !i?#enin-: Te+d"p"!

p+olife+"si sel mes"ni"l 0"n !e+se&"+ d"n pene&"l"n &"!"nlo&/l"+.

Den"n &/l"n s"&i! ,?+es?en!-: %+olife+si sel mes"ni"l d"n

p+olife+"si sel epi!el s"mp"i #"ps/l"+ d"n ;is?e+"l.

Glomel/+os#le+osis mem&+"nop+olife+"!if: %+olife+"si sel

mes"ni"l d"n penemp"!"n fi&+in 0"n men0e+/p"i

mem&+"n" &"s"lis de meseni/m. Ti!e+ im/nolo&/lin &e!"5I

"!"/ &e!"5IA +end".

Glome+/los#le+osis Fo#"l Semen!"l

S#le+osis lome+/l/s d"n "!+ofi !/&/l/s

%+onosis &/+/#

). PEMBAGIAN PATOLOGI ANATOMI

1- Kel"in"n minim"l

"- Me+/p"#"n &en!/# /!"m" d"+i lome+/lonef+i!is dim"n" me#"nisme

p"!oene!i# im/n !"mp"# !id"# i#/! &e+pe+"n ,!id"# "d" &/#!i p"!oenesis

#omple#s im/n "!"/ "n!i5MG-.

2*

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&- Glome+ol/s !"mp"# foo! p+o?ess/s sel !e+p"d/ m"#" dise&/! (/"

nef+osis lipid "!"/ pen0"#i! podosi!.

?- Kel"in"n 0"n +el"!if (in"# "d"l" pen0e&"& sind+om nef+o!i# 0"n

p"lin se+in p"d" "n"#5"n"# /si" 15* !"/n.

d- Glome+/li !"mp"# no+m"l "!"/ "mpi+ no+m"l p"d" mi#+os#op ?""0"

sed"n#"n den"n mi#+os#op ele#!+on !e+li"! "d"n0" pen0"!/"n podosi!

"n0" &en!/# lome+ol/nef+i!is m"0o+ 0"n !id"# mempe+li"!#"n

im/nop"!oloi.

2- Nef+op"!i mem&+"nos" ,lome+/lonef+i!is mem&+"nos"-

"- %en0"#i! p+o+esif l"m&"! p"d" de"s" d"n /si" pe+!en""n se?"+"

mo+foloi #"s ole #el"in"n &e+&"!"s (el"s p"d" MG.

&- J"+"n di!em/#"n p"d" "n"#5"n"#.

?- Menen"i &e&e+"p" lo&/s lome+ol/s sed"n#"n 0"n l"in m"si no+m"l.

d- %e+/&""n is!oloi# !e+/!"m" "d"l" pene&"l"n mem&+"n" &"s"lis 0"n d"p"!

!e+li"! &"i# den"n mi#+os#op ?""0" m"/p/n ele#!+on.

)- Glome+/los#le+osis fo#"l semen!"l

"- Lesi ini p/n0" insidens em"!/+i" 0"n le&i !ini d"n ipe+!ensi

p+o!ein/+i" nonsele#!if d"n +esponn0" !e+"d"p #o+!i#os!e+oid &/+/#.

&- %en0"#i! ini m/l"5m/l" "n0" menen"i &e&e+"p" lome+/li ,is!il"

fo#"l- d"n p"d" pe+m/l""n "n0" lome+oli (/#s!"med/le+. Ji#" pen0"#i! ini

&e+l"n(/! m"#" sem/" &"i"n !e+#en".

?- Se?"+" is!oloi# di!"nd"i s#le+osis d"n i"linis"si &e&e+"p" "n0"m"n

did"l"m s"!/ lome+ol/s men0isi#"n &"i"n5&"i"n l"in. J"di

#e!e+li&"!"nn0" &"i# fo#"l d"n semen!"l.

d- Le&i ("+"n men0e&""n sind+om" nef+o!i#.

4- Glome+/lonef+i!is p+olife+"!i;e mem&+"nos" ,M%GN-

2$

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"- Di!"nd"i den"n pene&"l"n mem&+"n &"s"lis d"n p+olife+"si sel/le+

,ipe+sel/l"+i!"s- se+!" infil!+"si sel %MN.

&- Den"n mi#+os#op ?""0" MG mene&"l d"n !e+d"p"! p+olife+"si dif/s sel5

sel mes"ni"l d"n s/"!/ pen"m&""n m"!+i#s mes"ni"l.

?- %e+l/"s"n mes"ni/m &e+l"n(/! #e d"l"m #/mp"+"n #"pile+ pe+ife+

men0e&""n +ed/pli#"si mem&+"n" &"s"lis ,(e("#5!+em "!"/ #on!/+

len#"p-

d- Kel"in"n ini se+in di!em/#"n p"d" nef+i!is se!el" infe#si s!+ep!o?o??/s

0"n p+o+esif d"n p"d" sind+om nef+o!i#.

e- Ad" M%GN !ipe I d"n !ipe II.

*- Glome+/lonef+i!is p+olife+"!i;e fo#"l

"- %+olife+"!if lome+/le+ d"n "!"/ #e+/s"#"n 0"n !e+&"!"s p"d"

semen lome+/l/s indi;id/"l ,semen!"l- d"n menen"i "n0" &e&e+"p"

lome+/l/s ,fo#"l-.

&- Le&i se+in "d" den"n sind+om nef+i!i#.

)I. PATOFISIOLOGI

%+o!ein/+i" ,"l&/min/+i"- m"sif me+/p"#"n pen0e&"& /!"m" !e+("din0"

sind+om nef+o!i# sed"n#"n e("l" #linis l"inn0" di"n"p se&""i m"nifes!"si

se#/nde+. %en0e&"& !e+("din0" p+o!ein/+i" &el/m di#e!"/i &en"+ s"l" s"!/ !eo+i

0"n d"p"! men(el"s#"n "d"l" il"nn0" m/"!"n ne"!if 0"n &i"s"n0" !e+d"p"! di

sep"n("n endo!el #"pile+ lome+/l/s d"n mem&+"n &"s"l.

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%em&en!/#"n sem&"& p"d" sind+om nef+o!i# me+/p"#"n s/"!/ p+oses 0"n din"mi#

d"n m/n#in s"(" #ed/" p+oses underfill d"n overfill &e+l"ns/n &e+s"m""n "!"/

p"d" "#!/ &e+l"in"n p"d" indi;id/ 0"n s"m" #"+en" p"!oenesis pen0"#i!

lome+/l/s m/n#in me+/p"#"n s/"!/ #om&in"si +"ns"n"n 0"n le&i d"+i s"!/.9

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Sed"n#"n #"d"+ )8

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S!"!/s #linis Sind+om Nef+o!i# dise&""n ole in(/+i lome+/l/s di!"nd"i

den"n penin#"!"n pe+me"&ili!"s mem&+"n lome+/l/s !e+"d"p p+o!ein 0"n

men"#i&"!#"n #eil"n"n p+o!ein /+in"+i" 0"n m"ssif p+o!ein/+i" m"sif ,le&i d"+i *8

m#24 ("m "!"/ H )* "+i- ipop+o!ein/+i" ipo"l&/minemi" ,#/+"n d"+i )*

dl- ipe+lipidemi" d"n !"np" "!"/p/n dise+!"i edem" d"n ipe+#oles!e+olemi".

i"s"n0" sedimen /+in no+m"l n"m/n &il" did"p"!i em"!/+i" mi#+os#opi#

,28e+i!+osi!L%- di?/+i"i "d"n0" lesi lome+/l"+ ,mis"l : s#le+osis lome+/l/s

fo#"l-.

Ga!3aran $a3

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o %ene!"p"n (/ml" p+o!ein den"n ?"+" Es&"? ,modifi#"si Ts/?i0"- did"p"!#"n "sil

p+o!ein/+i" !e+/!"m" "l&/min ,'*59*7- se&"n0"# 1851* +"m"+i.

o %+o!ein/+i" &e+"! e#s#+esi le&i d"+i )* +"ml24("m.

o

%eme+i#s""n (/ml" /+in did"p"!#"n p+od/#si /+in &e+#/+"n "l ini &e+l"ns/nsel"m" edem" m"si "d".

o e+"! (enis /+in menin#"!.

o Sedimen /+in d"p"! no+m"l "!"/ &e+/p" !o+"# i"lin+"n/l" lipoid

o di!em/#"n o;"l f"! &odies me+/p"#"n p"!onomoni# sind+om nef+o!i# ,den"n

pe"+n""n S/d"n III-.

o Te+d"p"! le/#osi!

%eme+i#s""n d"+" 0"n did"p"!#"n1

:

o

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onresponder a'al

onresponder lambat

Resisten-steroid seja$ terapi a'al.

Resisten-steroid terjadi pada pasien "ang sebelumn"a responsif-steroid.

)III. TATALAKSANA

%eno&"!"n SN !e+di+i d"+i peno&"!"n spesifi# 0"n di!/(/#"n !e+"d"p

pen0"#i! d"s"+ d"n peno&"!"n non5spesifi# /n!/# men/+"ni p+o!ein/+i"

menon!+ol edem" d"n meno&"!i #ompli#"si. Di/+e!i# dise+!"i die! +end" "+"m d"n

!i+" &"+in d"p"! mem&"n!/ menon!+ol edem". F/+osemid o+"l d"p"! di&e+i#"n d"n&il" +esis!en d"p"! di#om&in"si den"n !i"id me!"l"on d"n "!"/ "se!"ol"mid.

Kon!+ol p+o!ein/+i" d"p"! mempe+&"i#i ipo"l&/minemi" d"n men/+"ni +isi#o

#ompli#"si 0"n di!im&/l#"n. %em&"!"s"n "s/p"n p+o!ein 8.'51.8 # "+i d"p"!

men/+"ni p+o!ein/+i". O&"! pen"m&"! enim #on;e+si "nio!ensin ,"nio!ensin

?on;e+!in en0me ini&i!o+s- d"n "n!"onis +esep!o+ "nio!ensin II ,"nio!ensin II

+e?ep!o+ "n!"onis!s- d"p"! men/+/n#"n !e#"n"n d"+" d"n #om&in"si #ed/"n0"

memp/n0"i efe# "di!if d"l"m men/+/n#"n p+o!ein/+i".

Risi#o !+om&oem&oli p"d" SN menin#"! d"n pe+l/ mend"p"! pen"n"n"n.

@"l"/p/n pem&e+i"n "n!i#o"/l"n ("n#" p"n("n m"si #on!+o;e+si"l !e!"pi p"d"

s"!/ s!/di !e+&/#!i mem&e+i#"n #e/n!/n"n. Dislipidemi" p"d" SN &el/m se?"+"

me0"#in#"n menin#"!#"n +isi#o pen0"#i! #"+dio;"s#/l"+ !e!"pi &/#!i #lini# d"l"m

pop/l"si men0o#on pend"p"! pe+l/n0" menon!+ol #e"d""n ini. O&"! pen/+/n lem"#

olon"n s!"!in sepe+!i sim;"s!"!in p+";"s!"!in d"n lo;"s!"!in d"p"! men/+/n#"n

#oles!e+ol LDL !+ilise+ide d"n menin#"!#"n #oles!e+ol

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Is!i+""! s"mp"i edem" &e+#/+"n ,pem&"!"s"n "#!i;i!"s-

Res!+i#si p+o!ein den"n die! p+o!ein 8' # ide"l"+i C e#s#+esi p+o!ein d"l"m

/+in24("m. il" f/nsi in("l s/d" men/+/n die! p+o!ein dises/"i#"n in" 8$

# ide"l"+i C e#s#+esi p+o!ein d"l"m /+in24 ("m. %em&"!"s"n "+"m "!"/ "s/p"n n"!+i/m s"mp"i 1 2 +"m"+i. Men/n"#"n

"+"m se?/#/pn0" d"l"m m"#"n"n d"n menid"+i m"#"n"n 0"n di"sin#"n.

Die! +end" #oles!+ol 6 $88 m"+i

%em&"!"s"n "s/p"n ?"i+"n !e+/!"m" p"d" pende+i!" +""! in"p 988 s"mp"i 1288

ml "+i.

M#di6a!#n1

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#"+en" d"p"! mene#"n o+mon on"d"l ,!e+/!"m" p"d" +em"(" p+ep/&e+!"s- d"p"!

!e+("di sis!i!is emo++"i# d"n mene#"n p+od/#si sel s/ms/m !/l"n.

S/"!/ /(i #lini# meli&"!#"n 3) p"sien den"n minim"l ?"ne nep+i!i?

s0nd+ome se?"+" "?"# mend"p"!#"n ?0?loposp"mide 2 m#"+i sel"m" ' "!"/ 12

min/ m"sin m"sin d"l"m #om&in"si den"n p+ednisone. Tid"# "d" pe+&ed""n

"n!"+" d/" #elompo# d"l"m /si" onse! nep+osis +"sio (enis #el"min l"m"n0" nep+osis

"!"/ (/ml" p"sien 0"n +el"p p"d" s""! m"s/# peneli!i"n. Dipe+ole "sil "n#" &e&"s

d"+i +el"p sel"m" * !"/n p"d" p"sien 0"n mend"p"! !e+"pi sel"m" ' min/ "d"l" 2*

7 se+/p" den"n 0"n mend"p"! !e+"pi 12 min/ 24 7. D"+i /(i #lini# !e+se&/! d"p"!

disimp/l#"n ?0?loposp"mide !id"# pe+l/ di/n"#"n le&i l"m" d"+i ' min/ den"n

dosis 2 m#"+i p"d" "n"# "n"# d"l"m #om&in"si den"n s!e+oid p"d" minim"l

#"ange nep"oti# s$ndrome.

Te+"pi inisi"l p"d" "n"# den"n sind+om nef+o!i# idiop"!i# !"np" #on!+"indi#"si

s!e+oid ses/"i den"n "n(/+"n ISKD "d"l" di&e+i#"n p+ednison $8 mm2L%"+i

"!"/ 2 m#&&"+i ,m"#sim"l '8 m"+i- d"l"m dosis !e+&"i /n!/# menind/#si

+emisi. Dosis p+ednison dii!/n ses/"i den"n &e+"! &"d"n ide"l ,&e+"! &"d"n !e+"d"p

!ini &"d"n-. %+ednison dosis pen/ ,f/ll dose- inisi"l di&e+i#"n sel"m" 4 min/. il"

!e+("di +emisi d"l"m 4 min/ pe+!"m" dil"n(/!#"n den"n 4 min/ #ed/" den"ndosis 48 mm2L% ,2) dosis ""l- "!"/ 1* m#&&"+i se?"+" "l!e+n"!in ,sel"n

se"+i- 1 se"+i se!el" m"#"n p"i. il" se!el" 4 min/ peno&"!"n s!e+oid dosis

pen/ !id"# !e+("di +emisi p"sien din0"!"#"n se&""i +esis!en s!e+oid.

%"d" "n"# 0"n !el" din0"!"#"n +el"ps se+in "!"/ dependen s!e+oid se!el"

+emisi den"n p+ednison dosis pen/ di!e+/s#"n den"n s!e+oid dosis 1* m#&&

se?"+" "l!e+n"!in. Dosis ini #em/di"n di!/+/n#"n pe+l""n&e+!""p 82 m#&& se!i"p

2 min/. %en/+/n"n dosis !e+se&/! dil"#/#"n s"mp"i dosis !e+#e?il 0"n !id"#

menim&/l#"n +el"ps 0"i!/ "n!"+" 81 8* m#&& "l!e+n"!in. Dosis ini dise&/! dosis

!+esold d"n d"p"! dipe+!""n#"n sel"m" $512 &/l"n #em/di"n di?o&" dien!i#"n.

>m/mn0" "n"# /si" se#ol" d"p"! &e+!ole+"nsi den"n p+ednison 8* m#&&

sed"n#"n "n"# /si" p+" se#ol" s"mp"i 1 m#&& se?"+" "l!e+n"!in. il" +el"ps

!e+("di p"d" dosis p+ednison "n!"+" 81 8* m#&& "l!e+n"!in m"#" +el"ps !e+se&/!

di!e+"pi den"n p+ednison 1 m#&& d"l"m dosis !e+&"i di&e+i#"n se!i"p "+i s"mp"i

!e+("di +emisi. Se!el" +emisi m"#" p+ednison di!/+/n#"n men("di 8' m#&&

di&e+i#"n se?"+" "l!e+n"!in #em/di"n di!/+/n#"n 82 m#&& se!i"p 2 min/

)*

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s"mp"i s"!/ !""p ,82 m#&&- di "!"s dosis p+ednison p"d" s""! !e+("di +el"ps 0"n

se&el/mn0" "!"/ +el"ps 0"n !e+"#i+. il" +el"ps !e+("di p"d" dosis p+ednison +/m"!

8* m#&& "l!e+n"!in !e!"pi 6 18 m#&& "l!e+n"!in!"np" efe# s"mpin 0"n

&e+"! d"p"! di?o&" di#om&in"si#"n den"n le;"misol sel"n se"+i 2* m#&&

sel"m" 4512 &/l"n "!"/ l"ns/n di&e+i#"n si#lofosf"mid ,%A-.

O&"! si!os!"!i#" 0"n p"lin se+in di/n"#"n p"d" peno&"!"n SN "n"# "d"l"

si#lofosf"mid ,%A- "!"/ #lo+"m&/sil. Si#lofosf"mid d"p"! di&e+i#"n pe+o+"l den"n

dosis 25) m#&&"+i d"l"m dosis !/n"l m"/p/n se?"+" in!+";en" "!"/ p/l;. %A

p/l; di&e+i#"n den"n dosis *88 3*8 mm2 L% 0"n dil"+/!#"n d"l"m 2*8 ml

l"+/!"n N"L 897 di&e+i#"n sel"m" 2 ("m. %A p/ls di&e+i#"n se&"n0"# 3 dosis

den"n in!e+;"l 1 &/l"n ,!o!"l d/+"si pem&e+i"n %A p/ls "d"l" $ &/l"n-. Efe#

s"mpin %A "d"l" m/"l m/n!" dep+esi s/ms/m !/l"n "lopesi" sis!i!is

emo+"i# "ospe+mi" d"n d"l"m ("n#" p"n("n d"p"! men0e&""n #e"n"s"n.

Ole #"+en" i!/ pe+l/ pem"n!"/"n peme+i#s""n d"+" !epi 0"i!/ #"d"+ emolo&in

le/#osi! !+om&osi! se!i"p 152 semin/. il" (/ml" le/#osi! 6)888/L emolo&in

6' dL i!/n !+om&osi! 6188.888/L o&"! dien!i#"n semen!"+" d"n di!e+/s#"n

#em&"li se!el" le/#osi! *.888/L emolo&in ' dL !+om&osi! 188.888/L. Efe#

!o#sisi!"s %A p"d" on"d d"n #e"n"s"n !e+("di &il" dosis !o!"l #/m/l"!if men?"p"iH2885)88 m#&&. %em&e+i"n %A o+"l sel"m" ) &/l"n memp/n0"i dosis !o!"l 1'8

m#&& d"n dosis ini "m"n &"i "n"#.1

I@. KOMPLIKASI

1. Kel"in"n #o"/l"si d"n !im&/ln0" !+om&osis. D/" me#"nisme #el"in"n

emos!"sis p"d" sind+om nef+o!i#:

%enin#"!"n pe+me"&ili!"s lome+/l/s men"#i&"!#"n:

Menin#"!n0" de+"d"si +en"l d"n il"nn0" p+o!ein did"l"m /+in sepe+!i AT III

p+o!ein S &e&"s pl"sminoen d"n P "n!ipl"smin.

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2. Infe#si se#/nde+ !e+/!"m" infe#si #/li! ole s!+ep!o?o??/s

s!"p0lo?o??/s &+on#opne/moni" T. E+/psi e+isipel"s p"d" #/li! pe+/! "!"/

p"" se+in di!em/#"n. %ini+"n #el"in"n #/li! ini &"!"sn0" !e"s !"pi #/+"n

menon(ol sepe+!i e+isipel"s d"n &i"s"n0" !id"# di!em/#"n o+"nisme "p"&il"

#el"in"n #/li! di&i"#"n.

). G"n/"n !/&/l/s +en"lis

G"n/"n #li+ens "i+ &e&"s p"d" p"sien sind+om nef+o!i# m/n#in dise&""n

#/+"nn0" +e"&so+&si n"!+i/m di !/&/l/s p+o#sim"l d"n &e+#/+"nn0" "n!"+"n

n"!+i/m d"n "i+ #e "ns" enle !e&"l. G"n/"n pen"s"m"n /+in di!"nd"i den"n

#e!id"#m"mp/"n men/+/n#"n p< /+in ses/d" pem&e+i"n &e&"n "s"m.

4. G""l in("l "#/!.

Te+("di &/#"n #"+en" ne#+osis !/&/l/s "!"/ f+"#si fil!+"si &e+#/+"n !"pi #"+en"

edem" in!e+s!isi"l den"n "#i&"!n0" menin#"!n0" !e#"n"n !/&/l/s p+o#sim"lis

0"n men0e&""n pen/+/n"n LFG.

*. Anemi"

Anemi" ipo#+om mi#+osi!i# #"+en" defisiensi Fe 0"n !ipi#"l n"m/n +esis!en

!e+"d"p peno&"!"n p+ep"+"! Fe.

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e#s+esi #"lsi/m d"l"m feses le&i &es"+ d"+ip"d" pem"s/#"n.

!+om&osis di&e+i#"n !+om&oli!i#.

2. e" infe#si. Ji#" !e+("di infe#si se#/nde+ m"/p/n pe+i!oni!is di&e+i#"n

"n!i&io!i# !e+/!"m" 0"n &e+spe#!+/m l/"s .

). %em&e+i"n f/+osemid /n!/# menin#"!#"n "n!"+"n #e !/&/l/s dis!"l. Sel"in

i!/ f/+osemid 2(/" di&e+i#"n &il" edem" !id"# &e+#/+"n den"n pem&"!"s"n

"+"m. Dosis f/+osemid 1 m##"li &e+"n!/n p"d" &e+"!n0" edem" d"n

+espons peno&"!"n. il" +ef+"#!e+ d"p"! di/n"#"n id+o#lo+!i"id ,2*5*8

m"+i-. Sel"m" peno&"!"n di/+e!i# pe+l/ dip"n!"/ #em/n#in"n

ipo#"lemi" "l#"losis me!"&oli# "!"/ #eil"n"n ?"i+"n in!+";"s?/l"+ &e+"!.

4. Ji#" !e+("di ""l in("l "l ini mem&/!/#"n p+oses di"lisis "!"/ ?"n#o#in("l.

*. Ko+!i#os!e+oid d"p"! di&e+i#"n /n!/# men/+"ni infl"m"si infe#si #/li!.

%+ednison dosis pen/ : $8 mm2 l/"s pe+m/#""n &"d"n"+i "!"/ 2

m#"+i ,m"#sim"l '8 m#"+i- sel"m" 4 min/ dil"n(/!#"n

pem&e+i"n p+ednison dosis 48 mm2 l/"s pe+m/#""n &"d"n"+i "!"/ 2)

dosis pen/ 0"n di&e+i#"n ) "+i &e+!/+/!5!/+/! d"l"m seminn/ "!"/ sel"n

se"+i sel"m" 4 min/ #em/di"n dien!i#"n !"np" !"pe+in off. il" +el"ps

&e+i#"n p+ednison dosis pen/ sepe+!i !e+"pi ""l s"mp"i !e+("di +emisi

#em/di"n dosis di!/+/n#"n men("di 2) dosis pen/. il" +el"ps se+in "!"/

+esis!en s!e+oid l"#/#"n &iopsi in("l.

$. 12*m #"lsife+ol se"+i ,*8.888 /ni!- /n!/# "!"si ipo#"lsemi" !"pi m"si

d"l"m !""p pe+?o&""n.

@. PROGNOSIS

)'

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%+onosis m"#in &"i# (i#" d"p"! di di"nosis see+". %eno&"!"n see+" d"p"!

men/+"ni #e+/s"#"n lome+ol/s le&i l"n(/! "#i&"! me#"nisme #ompens"si in("l

m"/p/n p+oses "/!oim/n. %+onosis (/" &"i# &il" pen0"#i! mem&e+i#"n +espons 0"n

&"i# !e+"d"p #o+!i#os!e+oid d"n ("+"n !e+("di +el"ps. Te+"pi "n!i&"#!e+i d"p"!

men/+"ni #em"!i"n "#i&"! infe#si !e!"pi !id"# &e+d"0" !e+"d"p #el"in"n in("l

sein" "#i+n0" d"p"! !e+("di ""l in("l.

%en0em&/"n #linis #"d"n5#"d"n !e+d"p"! se!el" peno&"!"n &e+!"/n5!"/n

den"n #o+!i#os!e+oid.

K e l"in"n minim"l ,minim"l lesion-: %+onosis le&i &"i# d"+ip"d" olon"n

l"inn0" s"n"! &"i# /n!/# "n"#5"n"# d"n o+"n de"s" &"#"n &"i me+e#"

0"n !e+"n!/n s!e+oid.

Nef+op"!i mem&+"nos" ,lom+ol/nef+i!is mem&+"nos"- :%+onosis #/+"n &"i#

9*7 p"sien men"l"mi "o!emi" d"n menin"l "#i&"! /+emi" d"l"m "#!/

18528 !"/n.

Glome+/los#le+osis fo#"l semen!"l5 Le&i ("+"n men0e&""n sind+om"

nef+o!i#. %+onosis &/+/#

Glome+ol/nef+i!is p+olife+"!if mem&+"nos" ,M%GN-5 Kel"in"n ini se+in

di!em/#"n p"d" nef+i!is se!el" infe#si s!+ep!o?o??/s 0"n p+o+esif d"n p"d"

sind+om nef+o!i#.

DAFTAR PUSTAKA

1. A&doe++"?m"n M.I. 1993 ')25')*.

)9

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2. "+!" A. G/n""n. Sind+om Nef+o!i#: %"!oenesis d"n %en"!"l"#s"n""n. e+min D/ni"

#edo#!e+"n. No 1*8. 288$. *). Q?i!ed 281$ J"n/"+0 '. A;"il"&le:

!!p:.#"l&e.?o.idfiles?d#files1'1*8Sind+om"Nef+o!i#%"!oenesis.pdf1'1*

8Sind+om"Nef+o!i#%"!oenesis.!ml.

). L"!ief A. 2888. Di"nosis fisi# p"d" An"#. J"#"+!": S"/n Se!o.

4. S: FA D";is

omp"n0 2883.

$. Anon0m. 0?loposp"mide /n!/# sind+om" nef+o!i# Q"+!i#el. @e&si!e: Indonesi"

Kidne0 "+e l/&. Q?i!ed 281$ J"n/"+0 2. A;"il"&le:

!!p:.i#??.o+.id?on!en!.pp?2id138

3. A Repo+! of !e In!e+n"!ion"l S!/d0 of Kidne0 Dise"se in ild+en 19'1. Te p+im"+0

nep+o!i? s0nd+ome in ?ild+en : Iden!ifi?"!ion of p"!ien!s i! minim"l ?"nenep+o!i? s0nd+ome f+om ini!i"l +esponse !o p+ednison. J %edi"!+ 9' : *$1.

'. K"0sen GA 1992. %+o!ein/+i" "nd !e nep+o!i? s0nd+ome. In : S?+ie+ R@ edi!o+.

Ren"l "nd ele?!+ol0!e diso+de+s. 4!edi!ion. os!on : Li!!le +on "nd omp"n0 pp.

$'1532$.

9. @il" @i+0" IG 2882. Sind+om nef+o!i#. In: Al"!"s I pp. )'15

42$.

18. T+";is L 2882. Nep+o!i? s0nd+ome. Emed J. 2882 ) : )

11. Ni"/de! % 2888. T+e"!men! of idiop"!i? nep+o!i? s0nd+ome in ?ild+en.

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