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先天性非溶血性黄疸直应胆红素增高Ⅱ型,又称Rotor氏综合征,是一种遗传性疾病,相当少见。截至1976年总共才报道31个家族发病,国内仅在1964年报道2个家族(共5例病人)。我们遇到一个家族在一代中有6人罹患本病(见家系表)。I_1及I_3之父母已故,不能肯定患病情况。6例患者中1例(Ⅱ_7)不在本地未做调查,其余5例均曾住我院诊治。2例做了剖腹探查术,其中1例做了肝活组织检查。本文旨在报告我们的研究结果并复习文献作简要讨论。
Congenital non-hemolytic jaundice should be elevated bilirubin Type II, also known as Rotor’s syndrome, is a genetic disease, quite rare. As of 1976, a total of 31 families were reported, and in China, only 2 families were reported in 1964 (a total of 5 patients). We encountered a family in a generation of six people suffering from the disease (see family tables). The parents of I_1 and I_3 are deceased, and I am not sure of the illness. One of the 6 patients (Ⅱ_7) did not investigate in the local area, and the remaining 5 patients were treated in our hospital. Two cases underwent exploratory laparotomy, of which 1 had liver biopsy. This article aims to report our findings and review the literature for a brief discussion.