Prof. Dr. dr.

Syarifuddin Rauf, SpA(K)

Jabatan : Ketua Bagian Ilmu Kesehatan Anak FK-UNHAS

Spesialisasi : Anak Konsultan Ginjal & Hipertensi

 NEPHROTIC SYNDROME

1. Generalized oedema

2. Heavy proteinuria

3. Hypoproteinemia

4. Hyperlipidaemia (Hypercholestrolemia)
INCIDENCE

Wilawirya (1992): 6 cases/100.000 population < 14 yr old/yr

Sex ratio : ♂: ♀: = 1,5 – 2 : 1

Age incidence : - Highest Inc. = 2-5 years

- Less common : > 5 years

Department of Child Health, Hasanuddin University /

General Hospital Wahidin Sudirohusodo : 1-2 cases/month

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ETIOLOGY

Unknown

Genetic factors : - Congenital NS (mutation

on chromosom 19)

- HLA antigens : HLA-DR7

Predisposition : Allergy

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CLASSIFICATION / CAUSES

Primary Glomerular diseases

1. Minimal Change NS (MCNS)  70-80%

2. Focal Segmental Glomerulosclerosis (FSGS)

3. Mesangiocapillary Glomerulonephritis

4. Membranous Nephropathy

Secondary diseases

1. Henoch-Schoenlein Purpura

2. Systemic Lupus Erythematosus

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PATOMECHANISM

Soluble antigen- antibody complex

Electrochemic theory

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PATOMECHANISM NEPHROTIC SYNDROME

2 Autoimmune
Glomerulonephritis

1 Soluble AG-AB
Complex

Antigen

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 ELECTROCHEMIS THEORY

GBM:negative
Epithel cell charge

GBM

Endothel cell 1

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CLINICAL MANIFESTATIONS

Congenital NS (Finlandia type)

Placenta enlargement
Massif oedema
Genetic mutation on chromosome 19
Steroid sensitive NS
Responsif to corticosteroid
Minimal change NS (MCNS) : 70-80%
Steroid resistant
No/minimal response to corticosteroid
Focal glomerulosclerosis (FSGS) 8
SYMPTOMS & SIGNS
1. Oedema :

Pitting oedema

Generalized : starting in periorbital regions

 face  abdomen (ascites)  extremities

Pleural effusions

Massive anasarca  scrotal or vulval oedema

2. No hypertension or hematuria

3. Normal renal function

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MANAGEMENT

1. Hypoalbuminaemia (< 2 gr%)

Salt-poor human albumin (plasbumin) 1 gr/kgBW
2. Febrile / feels unwell / abdominal pain :
Antibiotics
3. Diuretic : Indications : severe oedema that causes
dyspnoe
4. Specific treatment : corticosteroid
Protocol : International Study of Kidney Disease
in Children (ISKDC) 8
PROTOCOL THERAPY OF NS
First attack :
CD = 4 weeks

AD/ID = 4 weeks Tap. off

Stop
1 2 3 4 5 6 7 8
remission remission

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PROTOCOL THERAPY OF RELAPS NS

CD

AD/ID Tap. off

Stop
1 2 3 4

remission remission

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 Any
Question
s?