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Iranian Journal of Pediatrics
Tehran University of Medical Sciences Press
ISSN: 1018-4406
EISSN: 2008-2150
Vol. 16, No. 1, 2006, pp. 57-162
Bioline Code: pe06009
Full paper language: Farsi
Document type: Research Article
Document available free of charge

Iranian Journal of Pediatrics, Vol. 16, No. 1, 2006, pp. 57-162

 en The outcome of renal transplantation in Bardet-Biedl syndrome
Sharifian, M; Dadkhah, M; Einollahi, B; Nafar, M; Simfroosh, N; Basiri, A & Otukesh, H

Abstract


Background: Bardet-Biedl syndrome (BBS) is an autosomal recessive disorder characterized by obesity, polydactyly of the hands and feet, retinitis pigmentosa, hypogenitalism and various degrees of intellectual impairment and renal anomalies. Other clinical features include: speech disorder, brachydactyly, developmental delay, polyuria/polydipsia, ataxia, poor coordination/clumsiness, diabetes mellitus, left ventricular hypertrophy, hepatic fibrosis. Spasticity and mental retardation fulfill the criteria for Laurence-Moon-Bardet-Biedl syndrome (LMBBS).
Methods: From March 1998 to April 2003, five patients (4 females) with LMBBS were transplanted with kidneys taken from living unrelated donors (LURD). All 5 patients had Retinitis pigmentosa and obesity with a body mass index (BMI) up to 39.33.
Findings: 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 mofetile (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 was 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 79 (range 38 to 137) based on Schwartz formula.
Conclusions: Renal transplantation is safe and successful and renal replacement (RR) therapy 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.

Keywords
Bardet Biedl , Syndrome (BBS) , Renal failure , Renal transplantation , Children

 
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