Literature DB >> 8958282

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

H Egawa1, W Berquist, R Garcia-Kennedy, K Cox, A S Knisely, C O Esquivel.   

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.

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Year:  1996        PMID: 8958282     DOI: 10.1097/00007890-199611270-00023

Source DB:  PubMed          Journal:  Transplantation        ISSN: 0041-1337            Impact factor:   4.939


  1 in total

1.  Classification and genetic features of neonatal haemochromatosis: a study of 27 affected pedigrees and molecular analysis of genes implicated in iron metabolism.

Authors:  A L Kelly; P W Lunt; F Rodrigues; P J Berry; D M Flynn; P J McKiernan; D A Kelly; G Mieli-Vergani; T M Cox
Journal:  J Med Genet       Date:  2001-09       Impact factor: 6.318

  1 in total

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