Two cases of neonatal haemochromatosis, a rare and often fatal metabolic disorder, presenting with acute liver failure, are reported. Both presented in the first week of Life with hypoglycaemia, jaundice, and coagulopathy, with rapid deterioration of Liver function. Both received a transplantation using reduced Liver grafts. One child was well 18 months later. Few survivors have been reported and despite the difficult perioperative management, liver transplantation is the best treatment for neonatal haemochromatosis.
LIVER-TRANSPLANTATION FOR NEONATAL HEMOCHROMATOSIS / MUIESAN P; RELA M; KANE P; DAWAN A; BAKER A; BALL C; MOWAT AP; WILLIAMS R; HEATON ND. - In: ARCHIVES OF DISEASE IN CHILDHOOD. - ISSN 0003-9888. - 73:(1995), pp. F178-F180. [10.1136/fn.73.3.F178]
LIVER-TRANSPLANTATION FOR NEONATAL HEMOCHROMATOSIS
MUIESAN P;
1995
Abstract
Two cases of neonatal haemochromatosis, a rare and often fatal metabolic disorder, presenting with acute liver failure, are reported. Both presented in the first week of Life with hypoglycaemia, jaundice, and coagulopathy, with rapid deterioration of Liver function. Both received a transplantation using reduced Liver grafts. One child was well 18 months later. Few survivors have been reported and despite the difficult perioperative management, liver transplantation is the best treatment for neonatal haemochromatosis.
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