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[罗特综合征:与肝内(酒精性)胆汁淤积相比,尿中粪卟啉异构体测定的相关性]

[Rotor syndrome: relevance of the determination of coproporphyrin isomers in the urine in comparison with intrahepatic (alcohol-induced) cholestasis].

作者信息

Both P, Frank M, Merkel H G, Doss M

机构信息

Abteilung für Klinische Biochemie im Fachbereich Humanmedizin, Universität Marburg.

出版信息

Z Gastroenterol. 1988 Aug;26(8):416-20.

PMID:3218284
Abstract

Porphyrin isomer examinations have been performed in two patients with Rotor syndrome (RS), one patient with Gilbert-Meulengracht syndrome and 12 patients with alcohol toxic cholestasis. Under both conditions, cholestasis and RS, total urinary coproporphyrin excretion as well as coproporphyrin isomer I was relatively and absolutely increased. Despite the different degree of the increase of coproporphyrin isomer I excretion between RS (69 vs. 72%) and cholestasis (47% on average), there are single cases with a coproporphyrin isomer I portion around 60%. In such cases, the differential diagnosis is quite difficult, so that the diagnosis "Rotor syndrome" should never be gained by one distinct examination; it is a diagnosis performed by exclusion of other diseases.

摘要

对两名 Rotor 综合征(RS)患者、一名 Gilbert-Meulengracht 综合征患者和 12 名酒精性中毒性胆汁淤积患者进行了卟啉异构体检测。在胆汁淤积和 RS 这两种情况下,尿中总粪卟啉排泄量以及粪卟啉异构体 I 相对和绝对增加。尽管 RS(69%对 72%)和胆汁淤积(平均 47%)之间粪卟啉异构体 I 排泄增加程度不同,但有个别病例粪卟啉异构体 I 比例在 60%左右。在这种情况下,鉴别诊断相当困难,因此“Rotor 综合征”的诊断绝不能仅通过一项特定检查得出;它是通过排除其他疾病而做出的诊断。

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