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Nephrotic Syndrome in an African American Female

An 11-year-old African American female with no significant past medical history presented to the clinic for evaluation of edema for 1 week. She had an afebrile upper respiratory infection 2 weeks prior to admission. Rapid strep test was negative and no specific treatment was required. A few days later she developed progressive facial and lower extremity edema, abdominal distension, and a mild decrease in urine output for the last 2 days. Patient denied any respiratory distress, abdominal pain, or fever. There was no history of gross hematuria or dysuria. No history of arthralgia, rash, or oral ulcers. She denied taking any medications, including nonsteroidal anti-inflammatory drugs. Family history was unremarkable. On physical exam, she was an obese female with anasarca. She weighed 86 kg (>95%) and her blood pressure was 110/67 without an orthostatic component. She had moderate facial and periorbital edema, pitting edema in her lower extremities and abdominal distension. No lymphadenopathy was appreciated. Laboratory evaluation showed that her creatinine was 0.9 mg/dL, blood urea nitrogen 5 mg/dL, albumin 1.1 g/dL with normal liver function tests. White blood cell count was 5.000/μL with normal differential, and hemoglobin of 12.5 g/dL. Urine analysis showed +3 protein, with protein/creatinine ratio of 12.3, and no red blood cells. Urine pregnancy test was negative. Lipid panel showed triglycerides 245 mg/dL, high-density lipoprotein 46 mg/dL, and low-density lipoprotein 340 mg/dL. The C3 level was 185 mg/dL (normal range 82-180 mg/dL) and C4 was 44 mg/dL (range 18-45 mg/dL). The erythrocyte sedimentation rate was 103 mm/h, and C-reactive protein was 0.4 mg/dL. Her ANA, HIV Elisa and hepatitis B/C panel were all negative. Lupus panel came back negative for lupus antibodies (anti ds-DNA, anti-SSA, anti-SSB, anti-smith, anti-cardiolipin, JO-1 antibodies, and lupus inhibitor antibodies). Serum immunoglobulin titers showed IgG 394 mg/dL (range 595-1275), IgM 140 mg/dL (range 28-184), and IgA 142 mg/dL (range 43-207). Renal ultrasound showed a horseshoe kidney, with normal echogenicity of renal parenchyma and no hydronephrosis. Patient underwent a kidney biopsy, which showed diffused and marked increase in mesangial cellularity with segmental endothelial proliferation and capillary basement membrane thickening with occasional wire loops. There were no glomerular crescents. Interstitium showed mild to moderate increase in connective tissue, along with a patchy infiltrate of lymphocytes and plasma (Figures 1 and 2). Immunofluorescence studies showed deposition of IgG, IgM, IgA, C3, and C1q.