Follow-up after liver transplantation for protoporphyric liver disease.
Follow-up after liver transplantation for protoporphyric liver disease.
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原卟啉性肝病肝移植后的随访。
DOI：
10.1002/lt.500020404
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发表时间：
1996
期刊：
影响因子：
--
通讯作者：
Carithers,RL
中科院分区：
文献类型：
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作者：
Bloomer,JR;Rank,JM;Payne,WD;Snover,DC;Sharp,HL;Zwiener,RJ;Carithers,RL
原卟啉症是一种遗传性疾病，患者可能会发生严重的原卟啉诱导的肝损伤，需要移植。由于独特的问题发生在围手术期，因为过量生产的原卟啉的骨髓继续肝移植后，这种程序的疗效原卟啉性肝病是不确定的。我们对9例接受肝移植的患者进行了随访。两名患者在移植后2个月内死亡，一名死于腹部出血并发症，另一名死于肠穿孔后败血症。其余7例患者在移植后14个月至8年（平均3.8年）进行了随访。七人中有两人因暴露在手术室灯光下而皮肤烧伤，愈合后没有疤痕。3例在术后出现轴索神经病变，需要长时间机械通气，2例持续存在运动缺陷。5例患者在随访时（平均3.5年）肝脏化学正常，肝活检结果正常或显示轻度门静脉三联征异常，但红细胞原卟啉水平仍显著升高（1，765 ± 365 mcg/ dL;正常，<65）。另外两名患者，他们都有排斥反应，巨细胞病毒感染，胆道梗阻，需要内镜治疗，肝活检特征表明原发性肝病复发。1例在移植后5年死于肝病并发症。另一个在移植后3.3年稳定，正在监测可能的再次移植。因此，肝移植可以成功地在患有原卟啉性肝病的患者中进行，其中间存活率与一般移植人群相当。然而，疾病可能在移植物中复发，特别是如果有引起胆汁淤积的并发症。版权所有© 1996美国肝病研究协会。
Protoporphyria is a genetic disorder in which patients may develop severe protoporphyrin‐induced liver damage and require transplantation. Because unique problems occur in the perioperative period and because excess production of protoporphyrin by the bone marrow continues after liver transplantation, the efficacy of this procedure for protoporphyric liver disease is uncertain. We present follow‐up of nine patients who underwent liver transplantation. Two patients died within 2 months of transplantation, one from complications of abdominal bleeding and the other from sepsis after bowel perforations. The remaining seven patients had follow‐up at 14 months to 8 years after transplantation (mean, 3.8 years). Two of the seven had suffered skin burns from exposure to operating room lights, which healed without scarring. Three had axonal neuropathies in the postoperative period requiring prolonged mechanical ventilation, and motor defects persisted in two. Five patients had normal liver chemistries at follow‐up (mean, 3.5 years), with liver biopsy results normal or showing mild portal triad abnormalities, but erythrocyte protoporphyrin levels remained significantly elevated (1,765 ± 365 mcg/ dL; normal, <65). The other two patients, both of whom had rejection, cytomegalovirus infection, and biliary tract obstruction requiring endoscopic therapy, had a recurrence of protoporphyric liver disease as indicated by liver biopsy features. One died 5 years after transplantation from complications of the liver disease. The other was stable 3.3 years after transplantation and was being monitored for possible retransplantation. Thus, liver transplantation can be performed successfully in patients with protoporphyric liver disease, with intermediate survival rates comparable to the general transplant population. However, disease may recur in the graft, particularly if there are complications that cause cholestasis. Copyright © 1996 by the American Association for the Study of Liver Diseases.