Muiesan P, Rela M, Kane P, Dawan A, Baker A, Ball C, Mowat A P, Williams R, Heaton N D
Liver Transplant Surgical Service, King's College Hospital, Camberwell, London.
Arch Dis Child Fetal Neonatal Ed. 1995 Nov;73(3):F178-80. doi: 10.1136/fn.73.3.f178.
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.
本文报告了两例新生儿血色素沉着症，这是一种罕见且常致命的代谢紊乱疾病，表现为急性肝衰竭。两例患儿均在出生第一周出现低血糖、黄疸和凝血功能障碍，并伴有肝功能迅速恶化。两人均接受了减体积肝移植。其中一名患儿在18个月后情况良好。据报道，该病幸存者寥寥无几，尽管围手术期管理困难，但肝移植仍是新生儿血色素沉着症的最佳治疗方法。
