Laurence-Moon-Bardet-Biedl syndrome (LMBBS) is an autosomal recessive disorder characterized by obesity, polydactyly on the hands and feet, retinitis pigmentosa, hypogenitalism and various degrees of mental retardation and renal anomalies.
Other clinical features include: Speech disorder, Brachydactyly, Developmental delay, Polyuria/polydipsia, Ataxia, Poor coordination/clumsiness, Diabetes mellitus, Left ventricular hypertrophy, Spasticity and Hepatic fibrosis. Between March 1998 and April 2003, five patients (4 female) with Laurence Moon Bardet Biedl Syndrome (LMBBS) were transplanted with kidneys taken from Living Unrelated Donors (LURD). All 5 patients had Retinitis Pigmentosa and obesity with Body Mass Index up to 39.33. The cause of End Stage Renal Failure (ESRF) was Reflux Nephropathy in one, Neuropathic bladder in one and renal hypoplasia/dysplasia in three patients. Mean age at Transplantation was 11 years (Range 6 to 17 years. Immunosuppressives were Prednisolone, Cyclosporine (Neoral) and Mycophenolate Mofetil (Cellcept). All of them suffered at least one episode of acute rejection shortly after transplantation but reversed with Methyl Prednisolone pulses and in the last follow-up the mean creatinine is 1.2 (range 0.6 to 2mg/dl. Mean Glomerular Filtration Rate(GFR) before transplantation was 10ml/min/1.73m2 and in the last follow-up is 79(range 38 to 137) based on schwarts formula. Renal transplantation is safe and successful and Renal Replacement Therapy (RRT) of choice in patients with LMBBS and ESRF but special attention should be paid to BMI and steroid free immunosuppression should be considered if other suitable drugs such as Rapamycin are affordable.