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Definition

• Manifested by massive proteinuria,


hypoalbuminemia, marked albuminuria &
hyper- lipidemia
Types
• Congenital
• Primary
• Secondary
Congenital
• Associated with other congenital anomalies of
kidney
• Inherited as an autosomal recessive disease
• Primary: regarded as an autoimmune
phenomenon
• Minimal change nephrotic syndrome
• Mesangial proliferative nephrotic syndrome
• Focal sclerosis nephrotic syndrome
Secondary
• Causes: chronic golerulonephritis, diabetes
mellitus, hepatitis B, infective endocarditis,
HIV/ AIDS, drug toxicity, lymphomas, syphilis
Pathophysiology
Glomerular damage

Permeability of glomerular capillaries to proteins

Proteinuria

Hypoproteinemia

Plasma oncotic pressure Compensatory


synthesis of
proteins by liver

Fluid escapes into tissues Plasma volume

Glomerular filtration rate

Aldosterone secretion

Fluid retention
• Shift of fluid from plasma to interstitial space
• Reduces intravascular fluid volume
• Hypovolemia
• Stimulate renin angiotension axis
• Secrete aldosterone & antidiuretic hormone
• Increased reabsorption of sodium & water
• Edema
• Diminished oncotic pressure
• Inccreased hepatic lipoprotein synthesis
• Hyperlipidemia
Clinical manifestations
• Periorbital puffiness, edema, respiratory
distress, generalised edema
• Weight gain
• Reduced urine output, concentrated urine
• Vomiting, loss of appetite, diarrhea
• Fatigue, lethargy, pallor, irritability
• Hypertension, hematuria, hepatomegaly,
wasting of muscles
Diagnostic evaluation
• Urine examination
• Blood examination
• Renal biopsy
Management
• Bedrest & high protein diet
• Fluid restriction
• Steroid therapy (prednisolone)
• Antibiotic therapy
• Diuretics
• Albumin infusion
• Immunosuppressive drugs
• Renal transplantation

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