7 g/day). In serum, total protein was 4.4 g/dl, and albumin was 2.1 g/dl, indicating NS. Blood urea nitrogen (BUN) was 59 mg/dl and creatinine was 1.23 l, showing renal hypofunction. Urinary

β2-microglobulin (MG) was increased by 1,450 μg/day; however, the urine concentrating ability, osmotic pressure of the urine, and excretion of several minerals into the urine were normal. Steroid therapy (2 mg/kg/day) was Selleck I-BET-762 initiated, but urinary protein did not decrease. A renal biopsy specimen included 16 glomeruli; changes were minimal (Fig. 2a). However, marked cloudy degeneration OSI-027 supplier and vacuolation of uriniferous tubules and tubular epithelial cell detachment were noted, and the uriniferous tubules showed cystic changes (Fig. 2a, b). Immunofluorescence methods showed no deposition of any immunoglobulin type or of complement. Localization of nephrin and CD2AP was normal. The patient was diagnosed with steroid-resistant NS. Cyclosporin A (CyA) treatment was initiated, obtaining a type I incomplete remission. At 4 years of age, proteinuria was exacerbated by infection, and the patient was admitted for treatment. In a second kidney biopsy specimen, segmental sclerotic glomerular lesions were observed, leading to the diagnosis of FSGS (Fig. 2c). In a third biopsy specimen at 6 years of age, tubulointerstitial

and segmental sclerotic glomerular lesions had progressed selleck chemicals llc (Fig. 2d). In the specimen obtained at 4 years, the median diameter was 92.4 μm in 32 glomeruli evaluated, representing about 1.5 times that seen in age-matched children (55–60 μm); the number of glomeruli per unit area was 5.2/mm2, a value within the normal range. The number of glomeruli had decreased and glomerular diameter increased in the subsequent specimen. No non-functioning genotype of ECT2 was observed in his parents, suggesting a de novo case. Fig. 2 Histologic findings in patient 1. On initial biopsy at 3 years of age, tubulointerstitial alterations included

tubular cloudy degeneration, cystic dilatation of tubules, detachment of tubular epithelial cells, and interstitial mononuclear cell infiltration (a, b); however, glomeruli were essentially normal. At the time of the second biopsy, focal segmental sclerosis of glomeruli was observed (c). NADPH-cytochrome-c2 reductase These sclerotic lesions progressed together with tubulointerstitial changes in a specimen at age 8 (d) Patient 2 The patient is a man who is currently 24 years old. No abnormality had been noted in the perinatal period, nor was there any contributory or past medical history. His parents were unrelated; however, they were divorced soon after his birth. No inherited kidney disease or other congenital anomalies of the kidney were found in his maternal family members. The patient was brought to our department because of edema that developed after influenza at 3 years of age. Proteinuria, hypoproteinemia, and mild renal dysfunction were present, and the patient was admitted. On physical examination, facial edema was present, but ascites was absent.