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[艾迪生氏病、贲门失弛缓症与无泪症的关联。附2例报告]

[An association of Addison's disease, achalasia of the cardia and alacrimation. Apropos of 2 cases].

作者信息

Hammami A, Trabelsi M, Bennaceur B, Boukhris R

出版信息

Ann Pediatr (Paris). 1989 Apr;36(4):279-82.

PMID:2662879
Abstract

We report two cases of a syndrome combining Addison disease, cardial achalasia, and alacrimation in two girls aged 4 and 5 1/2 years. Moreover, these patients exhibited some unusual features: both also had mineralocorticoid deficiency, and one had loss of tongue papillae and absence of the pupillary reflex. This syndrome is infrequent. Etiology is unknown. Pathophysiology is under debate; autonomic nervous system dysfunction seems likely. We have analyzed the features of this syndrome on the basis of our two observations and the 15 previously reported cases.

摘要

我们报告了两例患有艾迪生病、贲门失弛缓症和流泪综合征的病例,患者为两名4岁和5岁半的女孩。此外,这些患者还表现出一些不寻常的特征:两人都有盐皮质激素缺乏,其中一人舌乳头缺失且无瞳孔反射。这种综合征并不常见。病因不明。病理生理学仍在争论中;自主神经系统功能障碍似乎很有可能。我们根据这两例观察结果以及之前报告的15例病例分析了该综合征的特征。

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