Periorbital edema, ankle swelling, headaches, and upper abdominal discomfort
Patient: David Chang MRN: C55359541 Attending Physician: Evangeline DeRupo, MD HPI: A 13-year-old boy had been well until 4 weeks before admission, when he developed a cough, periorbital edema, ankle swelling, headaches, and upper abdominal discomfort. On admission, he was febrile with facial and ankle edema; there were generalized, superficial lymphadenopathy; numerous adventitial sounds in the lungs; and mild hypertension (BP 140/110). His hemoglobin was 72 g/1 with a normal white-cell count and an erythrocyte sedimentation rate (ESR) of 137 mm/h. His blood urea was high (27.5 mmoll) with a low serum bicarbonate (13.6 mmoll) and serum albumin (19 g/1). His creatinine clearance was 45 ml/min/m: with urinary protein loss of 6.7 g/day. His serum CH, was low (14 U/ml; NR. 25-45), as was his C3 level (0.20 g/1; NR. 0.8-1.4); his C4 level was normal (0.30 g/1; NR. 0.2-0.4). A chest x-ray showed several rounded opacities in both lungs. These were presumed to be infective and treated with amoxycillin and flucloxacillin with resolution of the radiological findings. The association of a low C3 with acute glomerulonephritis suggested acute poststreptococcu disease as the most likely diagnosis, although no streptococcuswere isolated and streptococc antibodies were not raised. Over the following 3 weeks, his blood urea fell but the proteinuria and hypertension persisted. CURRENT ADMISSION: Although he feels better, he still has heavy proteinuria with a low serum albumin (22 g/1; NR 35-50). Surprisingly, the serum CH, and C3 levels are still low at 18 U/ml and 0.4 g/1, respectively. This pattern was not consistent with the working diagnosis. It suggested continued complement activation via the alternate pathway, either due to some circulating activating factor or because of a regulatory defect caused by absence of the inhibitors I or H. However, his serum levels of I and H are normal. Electrophoresis of fresh serum and plasma showed the presence of C3 breakdown products, and his serum was able to break down C3 in normal serum due to the presence of C3 nephritic factor. C3 nephritic factor shows a strong association with membranoproliferative glomerulonephritis, but not with acute poststreptococcuslomerulonephritis. Because these conditions have different prognoses, a bilateral renal biopsy was performed yesterday. The results show 11 glomeruli, all of which were swollen with proliferation of mesangial, endothelial, and epithelial cells. On electron microscopy, the capillary loops showed basement membrane thickening with electron- dense deposits within the GBM. On immunofluorescence, intense C3 deposition is present in the GEM without immunoglobulin staining. These appearances, together with the finding of circulating C3 nephritic factor, are characteristic of membranoproliferative glomerulonephritis with dense intramembranous deposits (type II MPGN). Alternate-day prednisolone 10 mg PO per day was started, with prescription for 5 mg PO every other day for 30 days, although this disease nearly always shows a slow progression to chronic renal failure. ANSWER:
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