پيوند كليه بدون دياليز قبلي در يك بيمار مبتلا به سندرم باردشبيدل

Preemptive renal transplantation in a Bardet-Biedl patient: a case report

Bardet- Biedl syndrome is a rare autosomal recessive trait with retinitis pigmentosa, hypogonadism of various etiologies, polydactyly, obesity, learning difficulties and renal defects. We describe here the case of a 38 year old girl with Bardet - BiedL syndrome, diagnosed during adolescense according to retinitis pigmentosa, polydactyly, hypogonadism, obesity and learning difficulties. Three years ago, she presented with hypertension and renal failure. At imaging study, kidneys were enlarged with innumerable cysts scattered diffusely throughout renal cortex and medulla. The patient received conservative therapy until one year ago when she was referred to our hospital for kidney transplantion. She received preemptive renal allograft and was prescribed a triple immunosuppresive regimen. The course of the operation was complicated with wound infection and Guillain Barre syndrome. The patient was discharged in a good condition with normal kidney function. At our center, a Bardet- BiedLe patient with renal failure and cystic kidney disease successfully received a renal allograft.