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首页> 外文期刊>Archives of Iranian medicine >Renal transplantation in patients with Bardet-Biedl syndrome.
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Renal transplantation in patients with Bardet-Biedl syndrome.

机译：Bardet-Biedl综合征患者的肾脏移植。

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BACKGROUND: Bardet-Biedl syndrome is an autosomal recessive disorder characterized by obesity, polydactyly of hands and feet, retinitis pigmentosa, hypogenitalism, 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, and renal hypoplasia/dysplasia. If spasticity and mental retardation present, it fulfills the criteria for Laurence-Moon-Bardet-Biedl syndrome. METHODS: Between July 1985 and January 2005, 2,282 kidney transplantations were performed at Labbafinejad Medical Center, of whom 262 were children under 15 years of age (61% males). Among children, five (four females) had Bardet-Biedl syndrome who were transplanted preemptively. Kidneys were taken from living unrelated donors. RESULTS: All five patients had retinitis pigmentosa and obesity with body mass index up to 39.33 kg/m(2). The cause of end-stage renal failure was reflux nephropathy in one, neurogenic bladder in one, and renal hypoplasia/dysplasia in three patients. The mean age at transplantation was 11 years (range: 6-17 years). Immunosuppressives administered were prednisolone, cyclosporine, and mycophenolate mofetil. All of them suffered at least one episode of acute rejection shortly after transplantation but reversed with methylprednisolone pulses; in the last follow-up, the mean creatinine was 1.2 (range: 0.6 - 2.3 mg/dL). The mean glomerular filtration rate before transplantation was 10 mL/min/1.73 m(2); in the last follow-up it was 79 (range: 38-137).CONCLUSION: Renal transplantation is a safe and successful procedure and renal replacement therapy of choice in patients with Bardet-Biedl syndrome and end-stage renal failure, but special attention should be paid to body mass index and steroid-free immunosuppression if other suitable drugs, such as sirolimus and basiliximab are affordable.
机译：背景：Bardet-Biedl综合征是一种常染色体隐性遗传疾病，其特征为肥胖，手脚多指，色素性视网膜炎，性欲低下，各种程度的智力障碍和肾脏异常。其他临床特征包括言语障碍，肢体近距离发育，发育迟缓，多尿/多饮，共济失调，协调性/直觉性差，糖尿病，左心室肥大，肝纤维化和肾发育不全/发育不良。如果出现痉挛和智力低下，则符合Laurence-Moon-Bardet-Biedl综合征的标准。方法：从1985年7月至2005年1月，在Labbafinejad医疗中心进行了2282例肾脏移植，其中262例为15岁以下的儿童（男性占61％）。在儿童中，有五名（四名女性）患有Bardet-Biedl综合征，这些人被抢先移植。肾脏取自不相关的活体捐献者。结果：5例患者均患有色素性视网膜炎和肥胖症，体重指数高达39.33 kg / m（2）。终末期肾衰竭的原因是：反流性肾病1例，神经源性膀胱1例，肾发育不全/发育不良3例。移植的平均年龄为11岁（范围：6-17岁）。给予的免疫抑制剂为泼尼松龙，环孢霉素和霉酚酸酯。他们所有人在移植后不久都遭受了至少一次急性排斥反应，但被甲基泼尼松龙脉冲逆转了。在最后一次随访中，平均肌酐为1.2（范围：0.6-2.3 mg / dL）。移植前平均肾小球滤过率是10 mL / min / 1.73 m（2）;在最后一次随访中为79（范围：38-137）。结论：对于Bardet-Biedl综合征和终末期肾功能衰竭的患者，肾移植是一种安全，成功的方法，并且可以选择肾脏替代治疗，但要特别注意如果可以负担得起其他合适的药物，如西罗莫司和巴利昔单抗，应考虑体重指数和无类固醇的免疫抑制。

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