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Kuliah Glomerulopati
pada anak
Muhammad HeruMuryawan,dr,SpA(K)
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Pokok bahasan
Glomerulopati1. Sindrom nerotik
!. Glomeruloneritis
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Ge"ala #an##uan #in"al anak yan#serin# di"umpai
• $dema
• %enal
• Kardial• Hepatal
• &utrisional
• Hematuria (#in"al'di luar #in"al)
• Proteinuria
• Hipertensi (primer'sekunder)
• Penurunan a"u ltrasi #lomerulus.
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G*M$%+*PAH-
MA& /A+S$ *0 K&$- 0A+%$ & /H%$&
$0&*&2 in3amatory 4han#es in #lomerulus due to
immunolo#i4 me4hanism
/&/A MA&0$SA*&S• solated proteinuria
• Proteinuria 5 edema (i.e.&ephroti4syndrome)
• solated haematuria• Hypertension 5'6 proteinuria'haematuria
• %enal ailure
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Classifcation
/on#enital
Primary'
idiopathi4
A47uired
• Alport Syndrome• /on#enital &ephroti4 Syndrome
Se4ondary
1. Minimal change (MCNS)2. Focal segmentalglomerulosclerosis3. Mesangial prolierativeglomerulonephriti4. Memrano!prolierativeglomerunephritis
". Memranous glomerulonephritis#. $g% Nephropath&'. lomerulonephritis others
• ost $nection * ost streptococcalglomerulonephritis
• Mulis&stem +iseases, -/ 0S• $ntoicaation, +rugs/ metal• Neoplasms
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Massi8e Proteinuria6 9 :; m#'k# body wei#ht 'day,or6 9 !,: #'d
$dema
Hyperlipidemia (=!;;m#?)
Roth KS. Nephrotic syndrome: Pathogenesis and management. Ped in Rev 2002;23(7):237!7
Nephrotic S&n+rome
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$pidemiolo#y
• n4iden4e – n4iden4e !6@ new 4ases per 1;,;;;
– Pre8alen4e 1:.@ 4ases per 1;,;;;
• A#e – M/ !.: years median a#e
– 0SGS years median a#e
• SeB – C2! DoysE Girls in 4hildren > yo
– $7ual ratio in those older
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/lassi4ation1. /on#enital.!. Primary nephroti4 syndrome
he term applied to disease limited tothe kidney
• Minimal 4han#e, lipoid disease, nildisease• 0o4al se#mental s4hlerosis• Membranous nephropathy
• Prolierati8e nephritis (mesan#ial,o4al, diFuse)
C. Se4ondary nephroti4 syndrome• upus nephritis
• Heno4h6S4honlein purpura
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/lassi4ation o &ephroti4 syndrome
Response to steroid
"arratt #$. Steroid responsive nephrotic syndrome. %n: "arratt #$& editor. Pediatric nephro'ogy. !th
edition. "a'timore:ippincot i'iams * i'+ins;,---. p. 732.
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CONGENITAL NEPHROTIC SYNDROME
4lini4al onset in the rst C months o lie
proteinuria in utero or at birth ele8ated amnioti4 3uid le8el o alpha6etoprotein beore !; weeks #estation /lassi4ation 2
Primary 0innish type iFuse mesan#ial s4lerosis Minimal 4han#es &S 0o4al se#mental #lomerulos4lerosis
Secondary 4on#enital syphilis, toBoplasmosis, 4ytome#alo8irus - #onadal dys#enesis and Iilms tumour nephroblastoma et4
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Etiologi
J; ? idiopathi4 nephroti4 syndrome
@: ? minimal 4han#e nephroti4 syndrome(M/&S)
1; ? o4al se#mental #lomerulos4lerosis(0SGS)
> : ? membranous nephropathi
1; ? Membrano prolierati8e
#lomerulonephritis
*PAH/'P%MA%-&$PH%*/ S-&%*M$
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$M$-5
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Pathophysiology
he underlyin# patho#eneti4 abnormalityo &S is proteinuria due to an in4rease in
#lomerular 4apillary wall permeability.
1. he 4apillary wall loss the ne#ati8e4har#e
#ly4oprotein barries!. n4rease #lomerular permeability to
proteins
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Gambar 1. Penampan# #in"al
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Patosiolo#i S&
Kehilan#an muatan ne#ati i membran basalis
Proteinuria
Hipoalbunemiahiperkolesterolemia
edema
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n prin4iple edema may de8elop
by two me4hanism 2
A. he 4apillary hydrauli4 pressurein4reases as a result o 4onstant
ele8ation o plasma 8olume 2o8er3owL 4on4ep (nehpriti4edema)
D. he 4olloid osmoti4 pressure inplasma drops 2 underllin# theory (nephroti4 edema
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Nephritic e+ema Nephrotice+ema
%enal salt and water %lteration oStarling orces retention (Capillar& colloi+ osmotic
pressure )
$Bpansion o 4ir4ulatory 8olume $dema ormation
Alteration o Starlin# or4es olume 4ontra4tion(/apillary hydrauli4 pressure ↑)
$dema ormation %enal salt and waterretention Proposed s4heme o edema ormation in
patiens with #lomerular disease
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Clinical manifestation
$+iopathic nephrotic s&n+rome
• Pre8alen4y male 2 emale N ! 2 1• Most 4ommonly between the a#e o !& ys
• $dema, initially noted around theeyes, and in the lower eBtremities is pittin#L. t be4omes #eneraliOed
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Generelised edema(anasar4a)
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Older child withnephrotic !ndro"e
Pittin# peripher$loede"$
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&ephroti4 Syndrome
As4ites
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Laboratory Test in N S
o 4onrm &SSerum (albumin, #lobulin, 4holesterol)+rine protein 2 7ualitati8e (dipsti4k 2 albumin)
7uantitati8e (!
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Management
A. General Prin4iples• &o sistemati4 dietary ad8i4e is ne4essaryin simple 4ases o S%&S
• Antibioti4 is indi4ated in 4ellulitis, peritonitis, septi4emia,et4.• iureti42 $dematous 4hild in the absen4e o hypo8olemia
diureti4 2 urosemide (16! m# 'k#DI'day)• Albumin inusions 2 $Bpensi8e 4an haOardous but may belie sa8in#, its indi4ations in4lude 2
• Hypo8olemia (abdominal pain, hypotension, oli#uria)• %enal insuQ4ien4y
/ompli4ation 2Infections 2 S.pneumoniae, 4hi4kenpoB and measlesThrombocytosis 2 Hyper4oa#ulable state.
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%$AM$&
1. Medi4ation
1. S$%*
!. +%$/S
C. MM+&*S+P%$SS$ AG$&S
!. ietary (nephroti4 diet)
*I SA (16! #'day)
P%*$& !6C #'k#'day
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8.Corticosteroi+
• Prednisolon an a4ti8e metabolit o
prednison.
• Doth ha8e been widely used but
remains un4lear whether their mode
o a4tion is 6 anti6in3amatory,
6 immunosupressi8e,
6 or both
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$nitial 7reatment
a. ntrodu4tion o remission Prednisolon ; m#'m!'day or ! m#'k#DI'
day at least < weeks daily bein# re7uired
b. Iithdrawal 2 here are two alternati8e 2 6 Modied SK/ re#imen 2
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S7%N%6 76%7MN7C67$CS76$ (6N$SN)
4 MINGGU
IMMUNOSUPRESSIVE AGENTS
Prednison FD: 60 mg/m2/day
Prednison AD: 40 mg/m2/day
REMISSION (-) REMISSION (+)
STEROID RESISTANT
STEROID
SENSITIVE
4 MINGGU
FULL DOSE ALTERNATING
$N$7$%- 76%7MN7
THE INTERNATIONAL COMMITTEE OF KIDNEY DISEASE IN CHILDREN (1967)
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Indications for hospital admission or patient with nephrotic syndrome
• &ewly dia#nosed patiens
• Se8ere dehydrations (poor intake,persistent 8omitin#)
• +neBplained e8er (suspe4ted ba4terialine4tion)
• %era4tory edema (respiratory distress)
• Peritonitis
• %enal insuQ4ien4y (ele8ated serum
4reatinine)
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utcome
Mortality
he mortality rate or S%&S is 1 to !,: ?usually rom sepsis, hypo8olemia, and
thrombo4ytosis.
%elapses
n most 4ases the relapses e8entually
4ease he earlier the onset o S%&S, the morelikely that the disease will be protra4ted
enitions
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enitions
• %emission – +rinary protein > < m#' m!hr or AlbustiB N
;'ra4e or C 4onse4uti8e days
• Steroid %esponsi8e
– %emission with steroids alone
• %elapse – +rinary protein =
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• Steroid ependen4e – wo 4onse4uti8e relapses o44urrin#
durin# 4orti4osteroid treatment or within
1< days o its 4essation• Steroid %esistan4e
– 0ailure to a4hie8e response in spite o <
weeks o prednisone ; m#'m!
day
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6NS$S
6N%- FNC7$N gra+uall&
ailure
rapi+/ aout" * 1> &ears
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