Hereditary complement C3 deficiency is associated with recurrent bacterial infections and proliferative glomerulonephritis. We describe a case of an adult with complete deficiency of complement C3 due to homozygous mutations in C3 gene: c.1811delT (Val604Glyfs*2), recurrent bacterial infections, crescentic glomerulonephritis, and end‐stage renal failure. Following isolated kidney transplantation he would remain C3 deficient with a similar, or increased, risk of infections and glomerulonephritis. As C3 is predominantly synthesized in the liver, with a small proportion of C3 monocyte derived and kidney derived, he proceeded to simultaneous liver‐kidney transplantation. The procedure has been successful with restoration of his circulating C3 levels, normal liver and kidney function at 26 months of follow‐up. Simultaneous liver‐kidney transplant is a viable option to be considered in this rare setting.

中文翻译：

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成功的同步肝肾移植治疗与遗传性补体 C3 缺乏症相关的肾功能衰竭。

遗传性补体 C3 缺乏与复发性细菌感染和增生性肾小球肾炎有关。我们描述了一例由于 C3 基因纯合子突变导致补体 C3 完全缺乏的成年人：c.1811delT (Val604Glyfs*2)、反复细菌感染、新月体性肾小球肾炎和终末期肾衰竭。在孤立的肾移植后，他将保持 C3 缺陷，并具有类似或增加的感染和肾小球肾炎风险。由于C3主要在肝脏合成，少量C3单核细胞来源和肾脏来源，他进行了同步肝肾移植。在 26 个月的随访中，该手术成功恢复了他的循环 C3 水平、正常的肝肾功能。