新生儿血色病肝移植后肝细胞铁沉积症的快速发展。

Rapid development of hepatocellular siderosis after liver transplantation for neonatal hemochromatosis.

作者信息

Egawa H, Berquist W, Garcia-Kennedy R, Cox K, Knisely A S, Esquivel C O

机构信息

Pediatric Liver Transplant Program, Lucile Packard Children's Hospital, Stanford University, Palo Alto, California, USA.

出版信息

Transplantation. 1996 Nov 27;62(10):1511-3. doi: 10.1097/00007890-199611270-00023.

DOI:10.1097/00007890-199611270-00023

PMID:8958282

Abstract

A male infant with neonatal iron storage disease, also known as neonatal hemochromatosis (NH), underwent orthotopic liver transplantation (OLT) at the age of 55 days. The native liver contained an incidental hepatocellular carcinoma. Scant iron accumulation was found in a biopsy specimen of the implanted liver on the seventh postoperative day (POD); successive biopsies showed increasing siderosis. On POD 62, the patient died of a cardiac arrhythmia. Autopsy showed siderosis at many sites, including the implanted liver. We discuss the possibility that hemochromatosis recurred in the liver allograft and review possible factors contributing to the siderosis.

摘要

一名患有新生儿铁储存疾病（也称为新生儿血色素沉着症，NH）的男婴在55日龄时接受了原位肝移植（OLT）。其原生肝脏存在偶然发现的肝细胞癌。术后第7天（POD）在植入肝脏的活检标本中发现少量铁蓄积；后续活检显示铁沉积增加。在POD 62时，患者死于心律失常。尸检显示包括植入肝脏在内的多个部位存在铁沉积。我们讨论了肝移植中血色素沉着症复发的可能性，并回顾了导致铁沉积的可能因素。