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原位肝移植后非结合胆红素血症的病因及发病率

Etiology and incidence of unconjugated hyperbilirubinemia after orthotopic liver transplantation.

作者信息

Gates L K, Wiesner R H, Krom R A, Steers J, Gores G J, Hay J E, Porayko M K

机构信息

Mayo Clinic Liver Transplant Unit, Mayo Foundation, Rochester, Minnesota.

出版信息

Am J Gastroenterol. 1994 Sep;89(9):1541-3.

PMID:8079934
Abstract

OBJECTIVES

Gilbert's syndrome, or slow bilirubin glucuronidation phenotype, is a common cause of benign hyperbilirubinemia in the general population. There have been only two previously reported cases of Gilbert's syndrome occurring in patients after liver transplantation. This study was conducted to determine the frequency of Gilbert's syndrome in liver transplant recipients.

METHODS

The charts of all patients followed by the Mayo Liver Transplant Clinic for 1 yr or more after transplantation, as of June 1992, were reviewed to identify all patients with a consistent pattern of unconjugated hyperbilirubinemia greater than two times the upper limits of normal and with a normal conjugated bilirubin level. These patients were further evaluated to exclude all other causes of hyperbilirubinemia, including biliary obstruction, rejection, viral infection, cholestatic liver disease, and hemolysis.

RESULTS

Five of 229 patients (2.2%) had a consistent pattern of unconjugated hyperbilirubinemia. Only three patients (1.3%) had no other identifiable cause of hyperbilirubinemia.

CONCLUSIONS

This study was performed to determine the incidence of unconjugated hyperbilirubinemia and particularly to determine the incidence of Gilbert's disease in liver transplant recipients. The apparently low frequency of Gilbert's after liver transplantation may reflect the masking of the diagnosis by other transplant-associated pathology or donor selection bias because of unexplained hyperbilirubinemia. Post-transplant patients who fit the Gilbert's syndrome profile of unconjugated hyperbilirubinemia should have a postprandial bilirubin drawn as a first step. The awareness of this syndrome may avoid a costly and invasive evaluation in the liver transplant recipient.

摘要

目的

吉尔伯特综合征,即胆红素葡萄糖醛酸化缓慢表型,是普通人群中良性高胆红素血症的常见原因。此前仅有两例肝移植患者发生吉尔伯特综合征的报道。本研究旨在确定肝移植受者中吉尔伯特综合征的发生率。

方法

回顾截至1992年6月梅奥肝移植诊所随访1年或更长时间的所有患者病历,以识别所有具有持续非结合性高胆红素血症模式且高于正常上限两倍且结合胆红素水平正常的患者。对这些患者进行进一步评估,以排除高胆红素血症的所有其他原因,包括胆道梗阻、排斥反应、病毒感染、胆汁淤积性肝病和溶血。

结果

229例患者中有5例(2.2%)具有持续的非结合性高胆红素血症模式。只有3例患者(1.3%)没有其他可识别的高胆红素血症原因。

结论

本研究旨在确定非结合性高胆红素血症的发生率,特别是确定肝移植受者中吉尔伯特病的发生率。肝移植后吉尔伯特综合征的发生率明显较低,可能反映了其他移植相关病理对诊断的掩盖或由于不明原因的高胆红素血症导致的供体选择偏倚。符合吉尔伯特综合征非结合性高胆红素血症特征的移植后患者应首先检测餐后胆红素。认识到这种综合征可以避免对肝移植受者进行昂贵且有创的评估。

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