Lecture Notes: Nephrotic Syndrome
Overview
- Nephrotic syndrome is a feature of several kidney diseases, not a disease itself
- Key characteristics:
- Marked proteinuria (> 3.5 grams/day)
- Hypoalbuminemia
- General edema
- Hyperlipidemia
Kidney Structure and Function
- Normal kidney has 1-2 million nephrons
- Glomerular structure includes:
- Fenestrated endothelium
- Glomerular basement membrane
- Protozoites
- Nephron barriers:
- Size barrier
- Negative charge barrier
- Glomerulus permits small molecules (e.g., sodium, water) but not large or negatively charged molecules (e.g., proteins)
Pathophysiology of Nephrotic Syndrome
- Damage to glomerulus increases permeability
- Plasma proteins leak into urine (proteinuria > 3.5 grams/day)
Types of Nephrotic Syndrome
- Primary Nephrotic Syndrome: Specific to kidneys
- Minimal change nephropathy (common in children)
- Focal glomerulosclerosis
- Membranous nephropathy
- Hereditary nephropathies
- Secondary Nephrotic Syndrome: Manifestation of systemic illnesses
- Diabetes mellitus (diabetic nephropathy)
- Lupus erythematosus
- Viral infections (hepatitis B, C, HIV)
- Amyloidosis, paraproteinemias
- Preeclampsia
- Alloantibodies from enzyme replacement therapy
Pathophysiological Effects
- Loss of albumin causes:
- Hypoalbuminemia
- Lowered plasma oncotic pressure
- Hypovolemia
- Activation of renin-angiotensin-aldosterone system → sodium reabsorption
- Generalized edema (anasarca)
- Loss of immunoglobulins decreases immunity
- Loss of thyroxine-binding proteins leads to impaired iron transport
- Hypercoagulatability due to loss of antithrombin III and increased fibrinogen
- Hyperlipidemia due to increased hepatic synthesis
Differential Diagnosis
- Acute glomerulonephritis
- Acute and chronic renal failure
- Kwashiorkor
- Allergic reactions
- Congestive cardiac failure
- Liver disease with hypoalbuminemia
- Protein-losing enteropathies
Diagnostic Investigations
- Urinalysis: hematuria, proteinuria
- Complement C3 levels
- Urinary protein measurement
- Serum albumin levels
- Serologic studies for infections and immune abnormalities
- Renal ultrasonography and biopsy
Management
- Dietary Management:
- Normal protein diet considering nutritional status
- Low salt intake
- Medication:
- Corticosteroids (e.g., prednisone)
- Immunosuppressants (e.g., cyclophosphamide, cyclosporine)
- Diuretics (e.g., furosemide, spironolactone)
- ACE inhibitors (e.g., captopril, enalapril)
- Angiotensin II receptor blockers (e.g., losartan)
- Complication Management:
- Hypertension treatment (short-term nifedipine, hydralazine)
- Prevent thrombosis through mobilization
- Infection management with prophylactic antibiotics (e.g., penicillin)
- Albumin infusion may be used to manage hypovolemia
Conclusion
- Understanding nephrotic syndrome involves recognizing its role in kidney pathology and systemic implications
- Management includes dietary adjustments, pharmacological intervention, and addressing complications
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