A male patient with steroid-dependent nephrotic syndrome for 25 years and obesity-associated focal segmental glomerulosclerosis.

An obese male patient with steroid-dependent nephrotic syndrome since age 6 years had a thirty-third relapse at the age of 29 years. Renal biopsy showed focal segmental glomerulosclerosis. Proteinuria disappeared after treatment with prednisolone. He went into complete remission and renal function remained normal at the age of 31 years. Focal segmental glomerulosclerosis associated with obesity was suspected. Long-term follow-up with renal biopsy is necessary in obese children with steroid-dependent nephrotic syndrome.

Living related kidney transplantation in a patient with autosomal-recessive Alport syndrome.

We discuss a patient with Alport syndrome who received a renal transplant from a donor with thin basement membrane disease. A 30-year-old woman, diagnosed with Alport syndrome on the basis of sensorineural hearing loss, characteristic renal biopsy findings and a family history of microhaematuria, entered chronic haemodialysis therapy. She then received a renal transplant donated from her father, who had sensorineural hearing loss and persistent microhaematuria. On the day of renal transplantation, a 1-h graft biopsy after reperfusion showed thin basement membrane disease. We re-tested the patient's native kidney biopsy specimen by immunohistochemical staining using alpha-chain-specific collagen type IV monoclonal antibodies. There was no expression of collagen type IV alpha3-, alpha4- and alpha5-chain on glomerular basement membrane, but positive staining of alpha5-chain on Bowman's capsular basement membrane was noted. A diagnosis of autosomal-recessive Alport syndrome was made. We concluded that this family might display different phenotypic expressions of the same genotype: one suffered end-stage renal disease and the other thin basement membrane disease.