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一名患有1a型糖原贮积病(冯·吉尔克病)的肾移植患者出现多发性腺瘤和肝细胞癌。

Multiple adenomas and hepatocellular carcinoma in a renal transplant patient with glycogen storage disease type 1a (von Gierke disease).

作者信息

Gossmann J, Scheuermann E H, Frilling A, Geiger H, Dietrich C F

机构信息

Medizinische Klinik II, Zentrum der Inneren Medizin, Klinikum der Johann Wolfgang Goethe-Universität, Theodor-Stern-Kai 7, 60590 Frankfurt a.M., Germany.

出版信息

Transplantation. 2001 Jul 27;72(2):343-4. doi: 10.1097/00007890-200107270-00033.

DOI:10.1097/00007890-200107270-00033
PMID:11477366
Abstract

We report on a 42-year-old female patient with glycogen storage disease type 1a (von Gierke disease, GSD 1a) who developed hepatic adenomas and finally a hepatocellular carcinoma 10 years after renal transplantation. The tumor was resected; however, the patient died 6 months later as a result of fulminant carcinoma recurrence. In patients who have GSD 1a with terminal renal failure, combined liver and kidney transplantation may be considered at an early stage of the disease.

摘要

我们报告了一名42岁的1a型糖原贮积病(冯·吉尔克病,GSD 1a)女性患者,她在肾移植10年后发生了肝腺瘤,最终发展为肝细胞癌。肿瘤被切除;然而,患者在6个月后因癌肿复发而死亡。对于患有终末期肾衰竭的GSD 1a患者,在疾病早期可考虑进行肝肾联合移植。

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