NEPHROTIC SYNDROME

S YAK I B B AK R I

Glomerulopathies

Glomerulopathy : a group of diverse conditions including, but not lim

to, glomerulonephritis having in common the fact that the dis

process begins in the glomerulus or that the glomerulus is the m

importantly diseased part of the nephron.

Glomerulopathies are the most common causes of end-stage renal dis

Clinical presentation of glomerular diseas

I.

Acute glomerulonephritis

II.

Rapidly progressive glomerulonephritis

III. Chronic glomerulonephritis

IV. Persistent urinary abnormalities with few or no symp

V.

Nephrotic syndrome

Nephrotic syndrome

Clinical entity having miltiple causes and characterize

by increased glomerular permeability manifested
by massive proteinuria and lipiduria.
Massive proteinuria > 3.5 g/day/1.73m2 body surface
in the absence of a depressed GFR.

Clinical Features of The Nephrotic Syndrome

Manifestations of the nephrotic syndrome itself

Signs and symptoms determined by the underlying disease

involving the kidney

Clinical manifestation of nephrotic syndro

Oedema
Hypertension
Dyslipidemia
Hypercoagulable state
Hypoproteinemia / proteinuria
Progressive renal failure
Trace metal deficiencies
Endocrine disturbances
Infectious / immunodeficiency states

Pathophysiology of the Nephrotic Syndrome

sification of the disease states associated with the development

nephrotic syndrome

I. Idiopathic nephrotic syndrome due to Primary Glomerular Disease

II.Nephrotic syndrome associated with spesific etiologic events or in w

glomerular disease arises as a complication of other disease
1.
2.
3.
4.
5.
6.
7.

Medications
Allergens, venoms, immuization
Infection ( bacterial, viral, protozoal, helminthic )
Neoplasmic ( solid tumors, leukemia and lymphoma )
Multisystem disease
Heredofamilial and metabolic disease
Miscellaneous

iagnostic approach in nephrotic syndrom

I. Clinical
II. Laboratory studies
III. Renal biopsy

I. Clinical
History
Preexisting disease
Previous infection
Drug ingestion
Arthritis, rash
Current pregnancy
Family history of renal disease
Physical examination
Severe obesity
Rash, arthritis
Diabetic retinopathy
Hypertension
Evidence of malignancy
Lipodystrophy
Lymphoadenopathy/hepatosplenomegaly

II. Laboratory Studies

Urinalysis
In all cases ( nondiagnostic )
Creatinine clearance
Serum protein electrophoresis
Serum tota;cholesterol, lipoprotein
Serum ionized calcium
Parathyroid hormone

In selected cases ( to establish the diagnostic )

Complement level
Antinuclear antibody assay
Cryoglobulins
Hepatitis and HIV serology
Serum and urine immunoelectrophoresis

III. Renal biopsy

Minimal change disease

Focal segmental glomerulosclerosis
Membranous nephropathy
Membranoproliferative glomerulonephritis
Other glomerulonephritis

Suggested approach for initial treatment

( Minimal change disease )
Children

Prednisone 60 mg/m2/day until remission, then 40 mg/m2/48 h for

12 weeks, then reduce by 5-10 mg/m2/48 h every month.

Adults

Prednisone 1mg/kg/day until remission or for 6 weeks, then 1.6 mg/kg/

for 1 month, then reduce by 0.2-0.4 mg/kg/48 h.

Elderly

Prednisone 1 mg/kg/day until remission or for 4 weeks, then 0.8 mg/kg

for 2 weeks, then 1.6 mg/kg/48 h for 2 weeks. Then reduce by 0.4 mg/
every 2 weeks. If no remission continue with 1.2 mg/kg/48 h for anothe
4 weeks then reduce.

Contraindications to prednisone

Cyclophosphamide 2 mg/kg/day or chlorambucil 0.15mg/kg/day for 8-1

weeks

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