16691_glomerulopati Bengkulu April 2015

8/18/2019 16691_glomerulopati Bengkulu April 2015

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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

16691_glomerulopati Bengkulu April 2015

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