Farrell F J, Nguyen M, Woodley S, Imperial J C, Garcia-Kennedy R, Man K, Esquivel C O, Keeffe E B
Department of Medicine, California Pacific Medical Center, San Francisco 94115.
Hepatology. 1994 Aug;20(2):404-10.
Recent preliminary reports suggest a poor outcome of orthotopic liver transplantation for patients with hemochromatosis. We analyzed an institutional experience with orthotopic liver transplantation for hemochromatosis, focusing on factors contributing to increased morbidity and mortality. Between March 1988 and October 1992, nine of 249 adults (3.6%) undergoing orthotopic liver transplantation had hemochromatosis. Mean age was 53 yr (range, 42 to 62 yr), and eight of nine patients were men. The diagnosis of hemochromatosis was based on transferrin saturation > 62% and hepatic iron index > 2.0. Only two patients were known to have hemochromatosis before liver transplantation. All nine patients underwent standard cardiac evaluation before transplantation, and no patient had detectable pre-existing cardiac disease. One patient had a major operative cardiac complication as a result of pulmonary embolism and made a full recovery. Postoperatively, congestive heart failure developed in three patients and four patients had arrhythmias. One patient is undergoing phlebotomy for post-transplant cardiac complications from hemochromatosis. Two patients had primary hepatic tumors in the explant liver. There were four deaths caused by multiorgan failure with congestive heart failure (1), infection (2), and/or malignancy (2). Five patients are alive 3 to 25 mo post-transplant. The actuarial survival of the nine patients was 53% at 25 mo vs. 89% for 18 age- and sex-matched control transplant recipients (p = 0.1) and 81% for all other adult liver transplant recipients (p < 0.01). In five of seven patients, post-transplant liver biopsies revealed hepatic iron accumulation.(ABSTRACT TRUNCATED AT 250 WORDS)
近期的初步报告显示，肝血色素沉着症患者原位肝移植的预后较差。我们分析了本机构对肝血色素沉着症患者进行原位肝移植的经验，重点关注导致发病率和死亡率增加的因素。1988年3月至1992年10月期间，249例接受原位肝移植的成人中有9例（3.6%）患有肝血色素沉着症。平均年龄为53岁（范围42至62岁），9例患者中有8例为男性。肝血色素沉着症的诊断基于转铁蛋白饱和度>62%和肝脏铁指数>2.0。肝移植前仅2例患者已知患有肝血色素沉着症。所有9例患者在移植前均接受了标准的心脏评估，且无患者有可检测到的既往心脏病。1例患者因肺栓塞出现严重的手术心脏并发症，但已完全康复。术后，3例患者发生充血性心力衰竭，4例患者出现心律失常。1例患者因肝血色素沉着症的移植后心脏并发症正在接受放血治疗。2例患者的切除肝脏中有原发性肝肿瘤。有4例患者死于多器官功能衰竭，伴有充血性心力衰竭（1例）、感染（2例）和/或恶性肿瘤（2例）。5例患者在移植后3至25个月存活。9例患者的25个月精算生存率为53%，而18例年龄和性别匹配的对照移植受者为89%（p = 0.1），所有其他成人肝移植受者为81%（p < 0.01）。7例患者中有5例移植后肝活检显示有肝铁沉积。（摘要截短于250字）
