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首页> 外文期刊>The Lancet >Combined kidney and liver transplantation for familial haemolytic uraemic syndrome.
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Combined kidney and liver transplantation for familial haemolytic uraemic syndrome.

机译:肾脏和肝脏联合移植治疗家族性溶血性尿毒症。

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摘要

Recurrent haemolytic uraemic syndrome (HUS) is a genetic form of thrombotic microangiopathy that is mostly associated with low activity of complement factor H. The disorder usually develops in families, leads to end stage renal disease, and invariably recurs after kidney transplantation. We did a simultaneous kidney and liver transplantation in a 2-year-old child with HUS and a mutation in complement factor H to restore the defective factor H, with no recurrence of the disease. The operation was successful, and at discharge, the child had healthy kidney and liver function, with no sign of haemolysis.
机译:复发性溶血性尿毒症综合征(HUS)是血栓性微血管病的一种遗传形式,通常与补体因子H活性低有关。该病通常在家庭中发生,导致终末期肾脏疾病,并且在肾脏移植后总是复发。我们在2岁的HUS和补体因子H突变的患儿中同时进行了肾脏和肝脏移植,以恢复有缺陷的因子H,而这种疾病没有复发。手术成功,出院时孩子的肾脏和肝脏功能正常,没有溶血迹象。

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