Progression and potential regression of glomerulosclerosis.

illicit drugs. He had not noted any peri-orbital or leg edema, shortness of breath, chest pains, hematuria, or urinary symptoms. His only medication was fosinopril, 10 mg orally each day. The physical examination revealed a well-developed white male in no apparent distress. The blood pressure was 146/ 84 mm Hg; pulse, 84 beats/min; respiratory rate, 16 breaths/ min; temperature, 98.68F; weight, 215 lbs; and height, 599′′. The physical examination was unremarkable. Ultrasound tests showed an atrophic 3.4 cm echogenic right kidney, and a 12.6 cm left kidney with normal echogenicity. Laboratory evaluation revealed: serum glucose, 97 mg/dL; cholesterol, 137 mg/dL; triglycerides, 672 mg/dL; albumin, 4.1 g/dL; total protein, 7.4 g/dL; hematocrit, 46%; platelets, 361,000/mm; serum creatinine, 1.2 mg/dL; BUN, 15 mg/dL; and normal electrolytes. Creatinine clearance was 126 mL/min. An open renal biopsy disclosed early lesions of focal segCASE PRESENTATIONS mental glomerulosclerosis (FSGS) and glomerulomegaly, charPatient 1 acteristic of secondary FSGS (Fig. 1). The renal changes most A 13-year-old white boy was noted to have proteinuria, likely were related to his unilateral renal atrophy. He was 1 g/24 hr, eight years ago on a routine physical examination treated with a higher dose of the same ACE inhibitor. At latest for participation in school sports. The serum creatinine and follow-up nearly two years later, the serum creatinine was blood glucose levels were normal, and serologic tests were 1.2 mg/dL, and proteinuria was ,500 mg/24 hr. negative. Ultrasound examination demonstrated a very small, atrophic right kidney and hypertrophy and normal echogenicity Patient 2 of the left kidney. A diagnosis of solitary functioning kidney A 5-year-old white girl presented 17 years ago with an 8was made. month history of relapsing nephrotic syndrome. The first two The patient was treated with dietary protein restriction and episodes were steroid-sensitive, but subsequent relapses were an angiotensin-converting enzyme (ACE) inhibitor. At routine steroid-dependent, and she was admitted for renal biopsy. Labfollow-up at age 19, the proteinuria had increased to 3.7 g/24 oratory tests revealed normal serum creatinine (0.8 mg/dL), hr, and he was referred for further evaluation. The medical hypoalbuminemia (0.8 g/dL), proteinuria (2 g/24 hrs), negahistory was significant for a bilateral inguinal hernia repair at tive ASO titers, and normal complement levels. Physical exage 10 months, and a poorly documented history of vesicoureamination showed 31 edema, and her blood pressure was teral reflux as a child. The family history was significant for 120/70 mm Hg. microhematuria detected in the father three months prior to The renal biopsy showed no segmental sclerosis in the 23 this evaluation, a maternal grandmother with hypertension, glomeruli sampled. Immunofluorescence studies were negative, and a paternal grandfather with diabetes. There was no family and electron microscopy showed extensive foot process effacehistory of visual abnormalities or hearing loss. No hypertension ment. An initial diagnosis of minimal change disease was made. had been noted at regular follow-ups. The patient had gained Corticosteroid treatment was continued with no response even 25 pounds over the previous 18 months. A college student, he at a higher dose (40 mg daily). Addition of a six-week course worked part-time in an ice cream store. He had smoked for of chlorambucil resulted in remission (serum albumin, 4.3 g/dL; one month one year previously and did not drink alcohol or use proteinuria, 420 mg/24 hr). She remained in remission for 18 months and had a normal serum creatinine (0.3 mg/dL) when she was 7 years old. She then had a relapse following an upper