Post-transplantation nephrotic syndrome

A 60 year-old Caucasian female who developed chronic renal failure from focal segmental glomerulosclerosis (FSGS) received a living-related renal allograft from her 28-year-old son. The patient presented initially at age 48 with signs of nephrotic syndrome (peripheral edema, hypoalbuminemia, and hypercholesterolemia) and a serum creatinine of 1.5 mg/dl (132 mmol/l) (normal range 0.6-1.2 mg/dl (52.8-105.6 mmol/l)). She was treated with prednisone for six months, followed by cyclosporine A, with only partial remission of proteinuria. Renal function slowly declined over the ensuing decade. At the time of initial presentation, serologic test results were negative or within normal limits for antinuclear antibody, C3 and C4 complement levels, hepatitis B surface antigen, hepatitis C antibody, and serum and urine protein electrophoreses. Her past medical history was significant for hypertension for 25 years, and hypothyroidism. She had no history of childhood urinary tract infections or urinary reflux and she denied regular intake of over-the-counter medications. The patient's post-operative course was unremarkable and she was discharged seven days post-transplantation with a serum creatinine of 1 mg/dl. Immunosuppressive medications included FK506, mycophenolate mofetil, and prednisone. Four weeks post-transplant, the patient noted worsening lower extremity edema. A 24-hour urine collection contained 4.5 g protein (normal range 0-150 mg/day). Serum albumin was 3.6 g/dl (36 g/l) (normal range 3.5-5.0 g/dl (30-50 g/l)) and serum creatinine was 1.0 mg/dl (88.4 mu mol). FK506 levels were within therapeutic range. A renal ultrasound showed no signs of urinary tract outflow obstruction and normal venous and arterial blood flow. A renal biopsy was performed.