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[迟发性威尔逊病]

[Late-onset Wilsońs disease].

作者信息

Campos Franco J, Domínguez Santalla M J, Tomé Martínez de Rituerto S, Otero Antón E, González Quintela A

机构信息

Servicio de Medicina Interna. Hospital Clínico. Complejo Universitario Santiago de Compostela. A Coruña, Spain.

出版信息

An Med Interna. 2003 Aug;20(8):416-8.

Abstract

A 58-year old male with a past history of psychiatric disorders was studied for a persistent serum transaminase increase. Low serum ceruloplasmin level (lower than 3 mg/dL), increased urinary copper excretion, and increased liver tissue copper concentration (1050 mcg/g dry weight) confirmed the diagnosis of Wilsońs disease. Slit lamp examination did not show Kayser-Fleischer rings. D-penicilamin therapy was followed by serum transaminase normalization. Similar late-onset cases of Wilsońs disease are exceptional, but confirm the clinical heterogeneity of the disease.

摘要

一名有精神疾病病史的58岁男性因血清转氨酶持续升高接受检查。血清铜蓝蛋白水平低(低于3mg/dL)、尿铜排泄增加以及肝组织铜浓度升高(1050mcg/g干重)确诊为威尔逊病。裂隙灯检查未发现凯-弗环。青霉胺治疗后血清转氨酶恢复正常。威尔逊病类似的迟发性病例很罕见,但证实了该疾病的临床异质性。

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