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Long-term outcome of childhood IgAnephropathy with minimal proteinuria
Pediatric Nephrology
December 2015, Volume 30,Issue 12
pp 2121-212
!sumi "iga, #u$o %hima, &a$etsugu "ama, 'asashi %ato, "iro(obu 'u$aiyama, "iro$o &oga)a,
*yo+iro &a(a$a , a(dai Nou , 'ayumi %a$o
1
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Daftar Singkatan
!./I !(giote(si(-co(erti(g e(ymei(hibitor
!* !(giote(si( II receptor bloc$er
.D .hro(ic id(ey Disease
D'P diuse mesa(gial proli4eratio( /%*D /(d stage re(al disease
'P 4ocal mesa(gial proli4eratio(
Ig!N Immu(oglobuli( Nephropathy '6! mi(or glomerular ab(ormalities
2
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PENDAHULUAN
Nefropati Imunoglobulin A (IgAN) :
6lomerulo(e4ritis terba(ya$ didu(ia
%alah satu pe(yebab gagal gi(+al tahap a$hir7/%*D8 disemua usia de(ga( pe(ya$it glomerularprimer
Donadio 2002 Ale!opoulu" 200#
3
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Pe(elitia( di 9epa(g pada 2:1 a(a$ de(ga( Ig!N,11; /%*D dalam 15 th
!(a$ de(ga( difuse mesangial prolieration
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Pe(elitia( lai(
beberapa pasie( de(ga( Ig!N
glomerular mi(or ya(g ab(ormal atau ocalmesangial proleration 7'6!>'P8 remisispo(ta( ta(pa obat
Prog(osis Ig!N de(ga( protei(uria mi(imal 7'P-Ig!N8 saat diag(osis prog(osis bai$
'hima 20&
?uara( +a(g$a pa(+a(g pada 'P-Ig!N tida$di$etahui
5
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TUJUAN
ara$teristi$ da( luara( +a(g$a pa(+a(gpasie( de(ga( diag(osa 'P-Ig!N saat masaa(a$
@
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METODE
Desai( pe(elitia( %tudy retrospe$ti4Aa$tu pe(elitia( 9u(i 1B@ C 9uli 200B
?o$asi pe(elitia( a(a$ usia 20 th di u(iersitasobe da( Aa$ayama da( pasie( ya(g me(+ala(i
biopsi sebelum terapi dimulaiE
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Diag(osis Ig!N Ditemu$a( Ig! imu(oglobuli( predomi(a( di
mesa(gium glomerular de(ga( tida$ ada(ya$elai(a( sistemi$
%emua partisipa( didiag(osa oleh satui(estigator de(ga( $riteria ya(g sama
iopsi protei(uria ya(g me(etap de(ga( atauta(pa hematuria
=
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6ambar 1E !lur sele$si pasie(
515 biopsi de(ga( Ig! (e4ropatia(tara +u(i 1B@ C 9uli 200B
130 tida$ meme(uhi$riteria
3=5 pasie(didiag(osa a(atra
desember 1B@ da(
+uli 200B
2B pasie( de(ga((o(-mi(imalprotei(uria
10@ pasie( de(ga(mi(imal protei(uria
B
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Definisi patologi
la"i*%a"i +!ford : Mesangial Hypercellularity
Segmental glomerulosclerosis atauadhesi
Endocapilliary hypercellularity
Tubular atrophy/interstitial brosis Cresents
Global glomerulosclerosis
Arterial intima thicening
10
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Protei(uria mi(imal pada biopsi protei(uria 0E5g>hari>1E3 m2
"ematuria
5 atau lebih *. 7red blood cell8 dalam spesime(
uri( ya(g dise(tri4ugasi Mean arterial pressure
2>3 te$a(a( diastoli$ F1>3 te$a(a( sistoli$E
Glomerular ltration rate
/stimasi me(ggu(a$a( 4ormula Sch!art" de(ga($o(sta(ta 0E55 G 0E u(tu$ la$i la$i rema+a
11
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Definisi patologi
lasiH$asi Mesangial hypercellularity
Segmental glomerulosclerosis / adhesi
Endocapillary hypercellualrity
Tubular atrophy/ interstitial brosis Global glomerulosclerosis da( arterial intima
thicening
12
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Pada pra$te$ $li(is pe(elitia( i(i 2 $elompo$ A"
'6! 7mi(or glomerular ab(ormalities 8>'P 7ocalmesangial proleration#
$ifuse mesangial prolieration7D'P8
D'P=0; glomerolus de(ga( proli4erasi mesa(gialselular seda(g >berat yi < 3 sel per area mesa(gialperi4er
erdasar$a( $lasiH$asi J4ord di ce(ter i(i
=1; pasie( '6!>'P'0
=@; pasie( D'P '1
13
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Hasil
,enemuan lini": Dari 3=5 a(a$ de(ga( Ig!N 10@ 72;8 'P Ig!NE
,-IgAN:
Program s$ri(i(g se$olah 7=, 3E@;8 gross hematuria 72= 2@E:;8,
'a(i4estasi ya(g berat !N da( atau N% tida$
terlihat di $elompo$ 'P-Ig!N
1:
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Non-MP-IgAN: Program s$ri(i(g se$olah 7221, BE2;8
gross hematuria 7:1, 1:E;8,
si(drom (e4riti$ a$ut 7!N8 72,0E;8,
si(drom (e4roti$ 7N%8 7B, 3E2;8 atau !N F N% 7@, 2E2;8
15
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ara%teri"ti%pa"ien
,-IgAN Non-,-IgAN , .alue
+n"et
Program s$ri(i(gse$olah/ro"" hematuria'indrom nefriti%a%ut'indrom nefroti%AN N'
= 73E@;821 (23#4)0(0304)0 (0304)0(0304)
2217BE2;8#& ()2 70E;8B 73E2;8@72E2;8
0E2030&0EBB0E00E1B
,enemuan %lini"6"ia on"et (tahun)Dura"i on"et %ebiop"i renalPerempua(
&e$a(a( arteri rata
rata 7mm"g8/pisode grosshematuriae6* 7ml>me(it>1E3m2
,roteinuria "aatbiop"i
(g7hari7&357m2
)
&&38 (930-&35)939 (#30-&539)
:5E3;E5 K 1@EB
5= 75:E;8111E@ K 23E@032 (032-03)
&03 (13&-&238)832 (231-&839)
:5EB;E= K 1E2
1:@ 752E3;810EB K 23E=&3# (039-231)
03005030
0EBB0EB3
0E30E2: 03000&
&abel 1E $ara$teristi$ pasie( de(ga( Ig!N de(ga( protei(uria mi(imal
diba(di(g$a( pasie( (o(-'P-Ig!N1@
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Penemuan patologi
Anali"i" hi"tologi%al ; %la"i*%a"i +!ford
Perbedaa( ya(g sig(iH$a( pada rasio '0>'1,/0>/1 da( %0>%1E
Perbedaa( sig(iH$a( 'P- da( (o( 'P-Ig!N
pada rasio ada>tida$ ada(ya global s$lerosisE &ida$ satupu( pasie( pe(ebala( arterial i(tima
Pasie( de(ga( 'P-Ig!N gambara( patologi$alsig(iH$a( lebih ri(ga( diba(di(g$a( (o(-'P-Ig!N
1
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ara%teri"ti%pa"ien
,-IgAN Non-,-IgAN , .alue
,enemuan,atologi
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?a%tor
patologi
Anali"i" uni.ariat
+dd" ratio 984 I ,.alue"
Anali"i" multi.ariat
+dd" ratio 984 I ,.alue"
07& 351 23&-35
03000& 23 &3&-880300
=07=& 351 2322-385
03000& 302 &3-831&03000
%0>%1 2E:B 1E5:-:E10
0E0002 1E55 0E@-3E230E23
&0>&1 0E3B 0E02-BEB0
0E51 0E0: 0E0-1E210E0@
@re"ent"(ada7tida%)
38 232&-83&
03000& 23#8 &3-#350300
/lobal"clero"i"
(ada7tida%
3 23-&539
03000& 838 &355-23203002
&abel 2E !(alisis multiariat da( u(iariat 4a$tor patologis u(tu$pasie( de(ga( 'P-Ig!N atau (o(-'P-Ig!N
1B
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Terapi
10@ pasie( de(ga( 'P-Ig!N '6! 723, 21E;8,'P
7:, @BE=;8 da( D'P 7B, =E5;8E
,rin"ip terapi dari ,-IgAN :
&ida$ ada terapi 73B pasie(, 3@E=;8, a(ti platelet da( atau a(ti$oagula( 71:, 13,2;8,
pred(isolo( 7K a(tiplatelet da( atau a(ti$oagula(L2, 1EB;8,
20
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pred(isolo( F imu(osupresa( 7K a(tiplatelet da(atau a(ti$oagula(L 2, 1EB;8,
"erbal .i(a B, =E5;
!(giote(si(-co(erti(g e(yme i(hibitor 7!./I8da( atau a(giote(si( II reseptor bloc$er 7!*8 732,30E2;8
&erapi ya(g tida$ di$etahui 7=, E5;8
Pada B= pasie( de(ga( terapi ya(g tida$di$etahui, remisi $li(is spo(ta( terlihat pada 20720E:;8
21
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,rin"ip terapi ,-IgAN(n&0)
Non-,-IgAN(n259)
, .alue
>anpa
medi%a"i
9 (314) #0 (") 03000&
!(tiplatelet da(ataua(ti$oaula(
1: 713E2;8 23 7=E2;8 0E1
,redni"olon
(Bantiplateletdan atauanti%oagulan)
2 (&394) # (&2324) 0300&
,redni"olon7imuno"upre"an
(Bantiplateletdan atauanti%oagulan)
2 (&394) 18 (0384) 0300&
"erbal .i(a B 7=E5;8 2:7=E@;8 0EBB
A@= inh dan
atau A
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ara%teri"ti% >anpa terapiimuno"upre"an (n9#)
>erapiimuno"uoppre"i.e (n#)
Nilai p
>emuan %lini"
(set usia7tahu(8Dura"i darion"et %ebiop"i ginal
7bula(8Perse(tasiperempua('ea( !rterialPressure7mm"g8
6rosshematuriasebelum(ya 7;8/g4r7ml>me(it>1EB3m28
Protei(uria saatbiopsi
11E: 7=E=-13E8&032 (#-&53#)
:5E;
EB K 15E@
5E0
111E5 K 2:E:
0E2 70E2-0E38
10E5 7BE:-12E083& (&3#-#38)
25E0;
BE K 13EB
5E0
11@E0 K 1@E0
0E3 70E2-0E38
0E@B030
0E@3
0E2
0E@:
0E@@
0E0=
&abel :E ara$teristi$ pasie( de(ga( 'P-Ig!N berdasar$a(
pe(ggu(aa( terapi imu(osupresa(23
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ara%teri"ti% >ida% adaterapiimuno"upre"an (n9#)
>erapiimuno"uoppre"i.e (n#)
Nilai p
,enemuanpatologi(%riteriao!ford) (4)'esa(gial score7'0>'18
/(docapillaryhypercellularity7/0>/18%egme(talsclerosis da(adhesi 7%0>%18
!troH &ubularda( i(terstisialHbrosis7&0>&1>&28.rese(ts7ada>tida$8
6lobal s$lerosis7ada>tida$8
>23
@=>32
@>2:
BB>10
@5>35
BB>1
50>50
:3>5
50>50
100>0E0
25>5
100>0
0E25
0E25
0E2
0EBB
0E1:
0EBB
&abel :E ara$teristi$ pasie( de(ga( protei(uria mi(i(al berdasar$a(
pe(ggu(aa( terapi imu(osupresa(2:
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Luaran jangka panjang
*ata rata periode ti(da$ la(+ut 7K %D8
@E1 K :E2 th 'P-Ig!N G 5E= K :E1 tahu( No( 'P-Ig!NE
Anali"i" aplan E eier :
ebas dari .D IV-V, a(tara 2 $elompo$ p M 0E02 *asio surial bebas dari .D tahap III-V pada
setelah15 tahu( 100; pada 'P-Ig!N da(=E; pada (o(C'P-Ig!NE
Nilai te(gah e6* pasie( 'P-Ig!N
0E25K 5EBml>me(it>1E3 m2 > tahu(E
25
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Diskusi
Variabilitas da( berat(ya ma(i4estasi glomerularpada Ig!N $esulita( dalam predi$si prog(osispasie(
?uara( +a(g$a pa(+a(g pasie( Ig!N ge+ala $li(ismayor atau berat protei(uria berat, protei(uria
me(etap, ga(ggua( 4u(gsi gi(+al
eberapa pe(elitia( lai($li(is ri(ga( mi(imalprotei(uria, perubaha( mi(imal
2@
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Pe(elitia( i(i luara( pasie( Ig!N C'P saatdiag(osis lebih bai$ dari (o(-'P-Ig!N
3E@; pasie( de(ga( 'P-Ig!N teride(tiH$asimelalui program s$ri(i(g se$olah
9epa(g 1B: program pemeri(tah s$ri(i(g usia@ da( 1= tahu( diag(osis Ig!N lebih cepat
Pemeri$saa( uri( o(set sa$it periode 1 tahu(da( data homoge( pasie( de(ga( pe(ya$it ri(ga(tida$ terdapat ge+ala saat a(alisis
2
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ara$teristi$ $li(is perbedaa( sig(iH$a( usiasaat o(set da( durasi dari o(set $e biopsi gi(+alE
'P-Ig!N Pe(emua( patologis secara sig(iH$a(lebih ri(ga( D'P be(tu$ ya(g berat dari 'P-Ig!N de(ga( prog(osis buru$
eberapa pasie( de(ga( D'Pge+ala protei(uriami(imal pe(ti(g(ya biopsi re(al
2=
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2B
6ambar 2E *e(al surial probability determi(ed usi(g apla(C'eiera(alysisE 'P-Ig!N Ig! (ephropathy )ith mi(imal protei(uria, .Ico(Hde(ce i(teral
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&abel 5E *emisi da( medi$asi pasie( de(ga( 'P-Ig!N
>anpaedi%a"i(n9)
edi%a"i(n89) Nilai ,
*eimisi> tida$
remisi
20>1B 20>3B 0E10
30
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Perbedaa( +elas ditemu$a( pada pri(sip terapi'P-Ig!N da( No(-'P-Ig!N
'P-Ig!N pali(g ba(ya$ tida$ diterapi atau!./Is da( atau !*s, dima(a terapiimu(osupresa( lebih seri(g diberi$a( pada No(-
'P-Ig!N E
31
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Pe(elitia( i(i ta(pa terapi imu(osupresa( pada'P-Ig!N, hi(gga pemeri$saa( $li(is tera$hir7 herbal .i(a, a(tiplatelet da( a(ti$oagula(8 me(+adi alasa( perubaha( terapi pada 2 pasie(
: pasie( 73E=;8 terapi imu(osupresa( 2
pasie( $embali $e terapi herbal .i(a, a(tiplateletda( a(ti$oagula(
32
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!./Is I(h C< me(gura(gi e$s$resi protei(uria,mempertaha($a( 4u(gsi re(al pada pasie(de)asa de(ga( Ig!N
*a(domied .o(trol &rial 7*.&8 !./Is secarasig(iH$a( berma(4aat u(tu$ per$emba(ga( Ig!N
pada a(a$ da( ora(g de)asa muda
33
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*e$ome(dasi id(ey Disease>Improi(g 6lobalutcomes 7DI68 tida$ me(ggu(a$a( !./Ispada a(a$ de(ga( 'P-Ig!N 7 0E5 g>hari>1E3m2 8
!./Is dapat me(+adi piliha( terapi pada pasie(a(a$ de(ga( Ig!N ya(g me(u(+u$a( '6!>'PE
'es$ipu( demi$ia( pasie( de(ga( 'P-Ig!N ya(gme(u(+u$a( '6!>'P obserasi +a(g$apa(+a(g
3:
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Pe(elitia( i(i memba(di(g$a( pe(emua( $li(isda( patologis pada : pasie( de(ga( 'P-Ig!Nya(g me(daoat$a( terapi im(u(osupresa( da( B:pasie( de(ga( 'P-Ig!N ya(g me(erima terapi(o( imu(osupresi4
35
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&ida$ terdapat perbedaa( pada pe(emua( $li(is,$ecuali durasi dari o(set $e biopsi re(al da( padasemua pe(emua( patologiE
!(alisis luara( +a(g$a pa(+a(g pasie( de(ga( 'P-Ig!N atau (o(- 'P-Ig!N lebih bai$ pada pasie(
de(ga( 'P-Ig!N diba(di(g$a( (o(- 'P-Ig!N&ida$ satupu( pasie( de(ga( 'P-Ig!N me(galami
gagal gi(+al $ro(is atau pe(ya$it gi(+al tahap a$hirdalam 15 tahu(E
3@
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Pe(elitia( di %pa(yol protei(uria da( s$lerosissegme(tal adalah 4a$tor prog(osis $etaha(a(gi(+al da( remisi $li(is pada pasie( 'P-Ig!N
Pada pe(elitia( terdahulu, pe(eliti me(elitiada(ya cresents sebagai 4a$tor i(depe(de(
ya(g sig(iH$a( u(tu$ $etaha(a( gi(+al
3
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Aa$tu biopsi mu(g$i( salah satu pe(yebab hasilpe(elitia( i(i berbeda de(ga( pe(elitia( lai(
eterbatasa( pe(elitia( i(i C< e$s$lusi130 pasie($are(a tida$ tersedia(ya spesime( yag tersediada( ealuasi data berdasar$a( $lasiH$asi J4ord
5; dari a(a$ de(ga( 'P-Ig!N di pe(elitia( i(i program s$iri(i(g se$olah 9epa(g
3=
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Kesimpulan
"asil pe(elitia( i(i luara( +a(g$a pa(+a(g a(a$de(ga( 'P-Ig!( adalah bai$
'es$ipu( demi$ia( terapi imu(osupresidiperlu$a( sebagai tambaha( dari terapi a)alselama pe(ya$it $are(a pe(i(g$ata( protei(uria
leh $are(a itu obserasi +a(g$a pa(+a(gdiperlu$a( mes$ipu( de(ga( 'P-Ig!(
3B
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TERIMA KASIH
:0
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:1
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:2
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:3
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::
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:5
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:@
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:
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:=
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:B
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50
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51
% &n this glomerulus' in addition to mesangial hypercellularity'there are segments !ith endocapillary prolieration' in(ammatorycells' and diminution o capillary lumina% The arro! indicates asmall epithelial circumscribed crescent
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52
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53
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5:
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55
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