Prof. Dr. dr. Syarifuddin Rauf, SpA(K)Prof. Dr. dr. Syarifuddin Rauf, SpA(K)

Jabatan : Ketua Bagian Ilmu Kesehatan Anak FK-UNHAS

Spesialisasi : Anak Konsultan Ginjal & Hipertensi

1. Generalized oedema

2. Heavy proteinuria

3. Hypoproteinemia

4. Hyperlipidaemia (Hypercholestrolemia)

NEPHROTIC SYNDROME

2

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

3

ETIOLOGY

Unknown

Genetic factors : - Congenital NS (mutation

on chromosom 19)

- HLA antigens : HLA-DR7

Predisposition : Allergy

4

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

5

PATOMECHANISM

Soluble antigen- antibody complex

Electrochemic theory

6

PATOMECHANISM NEPHROTIC SYNDROME

AutoimmuneGlomerulonephritis

Antigen

2

Soluble AG-ABComplex

1

7

2

1

ELECTROCHEMIS THEORY

Epithel cell

GBM

Endothel cell

GBM:negative charge

8

CLINICAL MANIFESTATIONS

Congenital NS (Finlandia type)Placenta enlargementMassif oedemaGenetic mutation on chromosome 19

Steroid sensitive NSResponsif to corticosteroidMinimal change NS (MCNS) : 70-80%

Steroid resistantNo/minimal response to corticosteroidFocal glomerulosclerosis (FSGS)

9

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

8

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)

9

PROTOCOL THERAPY OF NS

CD = 4 weeks

AD/ID = 4 weeks Tap. off

Stop1 2 3 4 5 6 7 8

remission remission

First attack :

10

PROTOCOL THERAPY OF RELAPS NS

CD

AD/ID Tap. off

Stop1 2 3 4

remission remission

Any Questions

?