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Combined paediatric liver-kidney transplantation: Analysis of our experience and literature review

机译:小儿肝肾联合移植:我们的经验分析与文献复习

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BACKGROUND: Renal insufficiency is increasingly common in end-stage liver disease and allocation of livers to this category of patient has escalated. The frequency of combined liver-kidney transplantation (CLKT) has consequently increased. Indications for CLKT in children differ from those for adults and typically include rare congenital conditions; subsequently limited numbers of this procedure have been performed in paediatric patients worldwide. Scant literature exists on the subject. METHODS: Subsequent to institutional approval, a retrospective chart analysis of all paediatric CLKTs performed at the Transplant Unit, Wits Donald Gordon Medical Centre, University of the Witwatersrand, Johannesburg, South Africa between January 2005 and July 2013 was conducted. RESULTS: Defining children as younger than 18 years of age, 43 patients had received a liver transplant since 2005, of whom 8 received a CLKT. Indications included autosomal recessive polycystic kidney disease (n=3), primary hyperoxaluria type 1 (n=4) and heterozygous factor H deficiency with atypical haemolytic uraemic syndrome (n=1). Graft combinations included whole liver and one kidney (n=5), whole liver and two kidneys (n=1) and left lateral liver segment and one kidney (n=2), all from deceased donors. Patient age ranged from 4 to 17 years (median 9) and included 4 females and 4 males. Weight ranged from 13 to 42 kg (median 22.5). We describe one in-hospital mortality. The remaining 7 patients were long-term survivors with a survival range from 6 to 65 months. CONCLUSIONS: Although rarely indicated in children, CLKT is an effective treatment option, appropriately utilising a scarce resource and significantly improving quality of life in the recipient.
机译:背景:肾功能不全在终末期肝病中越来越普遍,并且将肝脏分配给此类患者的情况有所增加。因此,肝肾联合移植(CLKT)的频率增加了。儿童的CLKT适应症与成人的适应症不同,通常包括罕见的先天性疾病。随后在全球范围内的儿科患者中进行了此过程的有限次数。关于这一主题的文献很少。方法:在获得机构批准后,对2005年1月至2013年7月在南非约翰内斯堡威特沃兹兰德大学Wits Donald Gordon医疗中心的Witt Donald Gordon医疗中心的移植部​​门进行的所有儿科CLKT进行了回顾性图表分析。结果:自2005年以来,定义了18岁以下儿童的43例患者接受了肝移植,其中8例接受了CLKT。适应症包括常染色体隐性隐性多囊肾疾病(n = 3),原发性高草酸尿症1型(n = 4)和具有非典型溶血性尿毒症的杂合子H缺乏症(n = 1)。移植物组合包括全肝和一个肾脏(n = 5),全肝和两个肾脏(n = 1)以及左侧肝段和一个肾脏(n = 2),所有这些均来自死者。患者年龄为4至17岁(中位数为9),其中包括4名女性和4名男性。重量范围从13到42公斤(中位数22.5)。我们描述了一种住院死亡率。其余7例患者是长期生存者,生存期从6到65个月不等。结论:尽管在儿童中很少使用CLKT,但CLKT是一种有效的治疗选择,可适当利用稀缺资源并显着改善接受者的生活质量。

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