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[突尼斯的1型神经纤维瘤病:66例病例]

[Type 1 neurofibromatosis in Tunisia: 66 cases].

作者信息

Gouider R, Azaiz M I, Kchouk M, Lasram L, Ben Osman A, Mrabet A

机构信息

Unité de Neurologie, Hôpital Charles-Nicolle, Tunis.

出版信息

Ann Dermatol Venereol. 1994;121(2):185-9.

PMID:7979037
Abstract

A multidisciplinary transversal descriptive study was conducted from June to October 1992 to determine the clinical manifestations and laboratory findings observed in 66 patients with type 1 neurofibromatosis. Diagnostic criteria were those of the National Institute of Health as recommended for subjects over 20 years of age. All patients over the age of 25 had café au lait spots, neurofibromas, lentigines and nodules. Occurrence of lesions of the central nervous system was significantly earlier than peripheral nervous manifestations. The optic glioma was the most frequent lesion of the central nervous system. Complications were observed during the first twenty years of the disease. Based on these findings, we propose a clinical and laboratory work-up designed as a function of age.

摘要

1992年6月至10月进行了一项多学科横向描述性研究,以确定66例1型神经纤维瘤病患者的临床表现和实验室检查结果。诊断标准采用美国国立卫生研究院为20岁以上受试者推荐的标准。所有25岁以上的患者均有牛奶咖啡斑、神经纤维瘤、雀斑和结节。中枢神经系统病变的出现明显早于周围神经表现。视神经胶质瘤是中枢神经系统最常见的病变。在疾病的头二十年观察到了并发症。基于这些发现,我们提出了一项根据年龄设计的临床和实验室检查方案。

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