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肾上腺功能不全/无泪/贲门失弛缓症(3A)综合征中的神经和肾上腺功能障碍。

Neurological and adrenal dysfunction in the adrenal insufficiency/alacrima/achalasia (3A) syndrome.

作者信息

Grant D B, Barnes N D, Dumic M, Ginalska-Malinowska M, Milla P J, von Petrykowski W, Rowlatt R J, Steendijk R, Wales J H, Werder E

机构信息

Hospital for Sick Children, London.

出版信息

Arch Dis Child. 1993 Jun;68(6):779-82. doi: 10.1136/adc.68.6.779.

Abstract

Review of 20 patients with glucocorticoid deficiency (three cases also with salt loss) associated with absent tear secretion (19 cases) and achalasia of the cardia (15 cases) revealed neurological abnormalities in 17 including hyper-reflexia, muscle weakness, dysarthria, and ataxia together with impaired intelligence and abnormal autonomic function, particularly postural hypotension. These findings indicate that significant neurological problems are common in this multisystem disorder.

摘要

对20例伴有泪液分泌缺失(19例)和贲门失弛缓症(15例)的糖皮质激素缺乏患者(3例还伴有失盐)进行回顾性研究发现,17例存在神经学异常,包括反射亢进、肌肉无力、构音障碍和共济失调,同时伴有智力受损和自主神经功能异常,尤其是体位性低血压。这些发现表明,在这种多系统疾病中,严重的神经学问题很常见。

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本文引用的文献

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Selective ACTH insensitivity, achalasia, and alacrima: a multisystem disorder presenting in childhood.
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J Clin Endocrinol Metab. 1980 Feb;50(2):268-70. doi: 10.1210/jcem-50-2-268.
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