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[体质性高胆红素血症与混合性胆红素。临床与功能研究]

[Constitutional hyperbilirubinemia and mixed bilirubin. Clinical and functional study].

作者信息

Rapaccini G L, Anti M, Miggiano G, De Vitis I, De Simone F, Fedeli G

出版信息

Minerva Med. 1980 Nov 10;71(44):3221-8.

PMID:7454089
Abstract

Three cases of non-haemolytic constitutional icterus with prevalently conjugated bilirubin have been studied: one case of Dubin-Johnson syndrome and two cases of Rotor's syndrome. Confirmation was obtained for the differences between the two syndromes from the physiopathological (by means of the BSF load test and determination of urinary excretion of porphyrin) and morphological (macroscopic, histological, ultrastructural) viewpoints. The following were carried out in all cases: a load curve with crystalline bilirubin, study of the course of the curve and application of compartmental analysis to the curve. Findings underlie the hypothesis that Rotor's syndrome contains an uptake defect and an accumulation change on the part of the liver cell of organic anions and that it is therefore possible to suggest some identity between Rotor's syndrome and the so-called "Hepatic storage disease".

摘要

对三例以结合胆红素为主的非溶血性体质性黄疸病例进行了研究

一例为杜宾-约翰逊综合征,两例为罗特综合征。从生理病理学(通过BSF负荷试验和卟啉尿排泄测定)和形态学(宏观、组织学、超微结构)角度证实了这两种综合征之间的差异。所有病例均进行了以下操作:结晶胆红素负荷曲线、曲线过程研究以及对曲线应用房室分析。研究结果支持以下假设,即罗特综合征存在有机阴离子在肝细胞部分的摄取缺陷和蓄积变化,因此有可能提出罗特综合征与所谓的“肝贮积病”之间存在某些一致性。

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Minerva Med. 1980 Nov 10;71(44):3221-8.
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