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For further information, see CMDT Part 22-14: Nephrotic Spectrum Glomerular Diseases

Key Features

  • Proteinuria > 3 g/day

  • Albumin < 3 g/dL

  • Edema

  • Typically hyperlipidemia

Clinical Findings

  • Peripheral edema with serum albumin < 3 g/dL

  • Edema can become generalized

  • Dyspnea caused by pulmonary edema, pleural effusions, and diaphragmatic compromise with ascites

  • Abdominal distention from ascites

Diagnosis

  • Urinalysis: proteinuria; few cellular elements or casts

  • Oval fat bodies appear as "grape clusters" under light microscopy and "Maltese crosses" under polarized light

  • Serum albumin < 3 g/dL, serum protein < 6 g/dL

  • Hyperlipidemia

  • Elevated erythrocyte sedimentation rate

  • Send serum complement levels, serum and urine protein electrophoresis, antinuclear antibodies, and serologic tests for hepatitis as indicated

  • Kidney biopsy indicated in adults with new-onset idiopathic nephrotic syndrome if a primary renal disease is suspected

  • Four most common lesions

    • Minimal change disease

    • Focal glomerular glomerulosclerosis

    • Membranous nephropathy

    • Membranoproliferative glomerulonephropathy

Treatment

  • In those with sub-nephrotic proteinuria or mild nephrotic syndrome, dietary protein restriction may be helpful in slowing progression of renal disease

  • In those with very heavy proteinuria (> 10 g/day) protein malnutrition may occur and daily protein intake should replace daily urinary protein losses

  • Salt restriction for edema

  • Loop and thiazide diuretics in combination

  • Angiotensin-converting enzyme inhibitors and angiotensin receptor blockers

  • Antilipidemic agents

  • Corticosteroids, cytotoxic agents as indicated for primary renal lesion

  • Warfarin in patients with thrombosis for at least 3–6 mo

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