Dubin-Johnson综合征临床特点分析

Clinical Features of Dubin-Johnson Syndrome

目的探讨Dubin-Johnson综合征的临床特点。方法回顾性分析30例Dubin-Johnson综合征患者的临床表现、实验室检查及诊治方法。结果主要表现为无症状的长期轻-中度皮肤、巩膜黄染和小便发黄,慢性持续性或间歇性黄疸;总胆红素水平升高,平均(87.9±51.5)mol/L;尿胆红素均阳性;肝穿刺活检组织病理检查显示:中央区周围肝细胞胞质内易见较粗大的棕色色素颗粒沉积,一般无需特殊治疗,预后良好。结论对临床上反复发生以结合性胆红素增高为主的黄疸,同时血清转氨酶正常,并已排除肝内、外梗阻及其他各类肝病者,应及时行肝穿刺活检组织病理检查,以便确诊是否患有Dubin-Johnson综合征。

Objective To analyze the clinical features of Dubin-Johnson syndrome.Methods The clinical manifestations,laboratory tests,diagnosis and treatment were analyzed retrospectively in 30 Dubin-Johnson syndrome patients.Results The main manifestations were：long-term asymptomatic mild-moderate yellow skin,sclera and urine,and chronic persistent or intermittent jaundice;total bilirubin levels increased,averaging（87.9±51.5） mol/L;urinary bilirubins were positive;liver biopsy pathology tests showed that gross brown pigment granules were seen in cytoplasm of liver cells around central area,usually not needing treatment with good prognosis.Conclusion Timely liver biopsy should be performed to confirm whether having Dubin-Johnson syndrome in patients with jaundice repeatedly occurring mostly with conjugated hyperbilirubinemia and normal serum transaminase but without obstruction in or outside liver or other liver diseases.