nephrotic syndrome.ppt
TRANSCRIPT
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
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)
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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)
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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 :