Transplantation 1996 Nov 27;62(10):1511-3

Rapid development of hepatocellular siderosis after liver transplantation for
neonatal hemochromatosis

Egawa H, Berquist W, Garcia-Kennedy R, Cox K, Knisely AS, Esquivel CO

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

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