Henne-Bruns D, Kremer B
Chirurgische Universitätsklinik, Hamburg.
Klin Wochenschr. 1988 Jul 1;66(13):596-8. doi: 10.1007/BF01720835.
In a 33 years old woman an orthotopic liver transplantation was performed because of an endstage posthepatic liver cirrhosis. Postoperatively the unconjugated bilirubin levels remained elevated although liver enzyme values were within normal range. Because hemolysis, rejection, infection or biliary obstruction could be excluded we suspected a Gilbert's Syndrome and were able to confirm this diagnosis by low caloric intake and nicotinic acid test. This case report therefore describes the first time the transplantation of a clinically harmless metabolic disorder but inborn error of metabolism by liver grafting into the recipient.
一名33岁女性因终末期肝后性肝硬化接受了原位肝移植。术后,尽管肝酶值在正常范围内,但非结合胆红素水平仍持续升高。由于可排除溶血、排斥反应、感染或胆道梗阻,我们怀疑是吉尔伯特综合征,并通过低热量摄入和烟酸试验证实了这一诊断。因此,本病例报告首次描述了通过肝脏移植将一种临床无害的代谢紊乱即先天性代谢缺陷移植到受者体内的情况。