Understanding Nephrotic Syndrome and Management

Nov 26, 2024

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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