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3兄弟中的沃纳综合征样疾病。早老综合征的鉴别诊断

[Werner syndrome-like disease in 3 brothers. Differential diagnosis of progeria syndrome].

作者信息

Mensing H, Schaeg G, Burck U, Meigel W

出版信息

Hautarzt. 1982 Oct;33(10):542-7.

PMID:7152896
Abstract

A report is given on a progeroid disease affecting three brothers, 11, 13, and 14 years old. The difficult differential diagnosis of such progeroid syndromes is discussed. The clinical signs were mostly consistent with Werner's syndrome. Since some of the symptoms of this disease were not present, probably because of the young age of the patients, this progeroid syndrome was classified as Werner syndrome-like. Electronmicroscopic findings of the patients skin showed changes of the blood vessels and nerves, which were until now not described in Werner's syndrome or other progerias. The latter findings may give some hints with regard to the pathogenesis of this disease.

摘要

报告了一例影响三名年龄分别为11岁、13岁和14岁兄弟的早老样疾病。讨论了此类早老样综合征的鉴别诊断难点。临床体征大多与沃纳综合征相符。由于该疾病的一些症状未出现,可能是因为患者年龄较小,这种早老样综合征被归类为类沃纳综合征。患者皮肤的电子显微镜检查结果显示血管和神经有变化,这在沃纳综合征或其他早老症中迄今尚未见描述。后一发现可能为该疾病的发病机制提供一些线索。

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