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[Netherton综合征的疾病分类学界限]

[Nosographic limits of the Netherton syndrome].

作者信息

Ottolenghi A, Pezzoli G, Altobelli R, Khodary I

出版信息

Pediatr Med Chir. 1985 Jan-Feb;7(1):141-5.

PMID:3841392
Abstract

This paper present the case of a 5 year old girl affected by ichthyosis vulgaris, atopic diathesis (asthma - eczema), hair anomalies as pili torti, trichorrhexis invaginata. Such data are compared with the most frequent symptoms Netherton's syndrome. Although some difference be extant, the authors deem it appropriate that this case be included within the framework of this syndrome. The problem is therefore posed once again of the boundaries of Netherton's syndrome. Also considered are the most interesting hypotheses recently set forth by different authors with the aim of better defining this disease whose biological mechanism is still unknown.

摘要

本文介绍了一名5岁患有寻常型鱼鳞病、特应性素质(哮喘 - 湿疹)、毛发异常如扭曲发、套叠性脆发的女孩病例。这些数据与Netherton综合征最常见的症状进行了比较。尽管存在一些差异,但作者认为将该病例纳入该综合征框架是合适的。因此,Netherton综合征的边界问题再次被提出。还考虑了不同作者最近提出的最有趣的假设,目的是更好地定义这种生物学机制仍不清楚的疾病。

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