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[儿童库欣病:经蝶窦腺瘤切除术后治愈的一例报告]

[Cushing's disease in childhood: apropos of a case cured after trans-sphenoidal adenomectomy].

作者信息

Barrios Castellanos R, Argente Oliver J, Alonso Blanco M, Yturriaga Matarranz R

出版信息

An Esp Pediatr. 1984 Mar 15;20(4):387-94.

PMID:6732065
Abstract

Authors describe one case of Cushing's disease in a old eight-year boy, with growth deficiency, pubic hair and obesity. He had hypercortisolism unresponsive to dexamethasone suppression (1 mg). The more interesting fact for localization of the lesion was the more than 50% suppression with 8 mg of dexamethasone, while tomographic studies of sella turcicaland CAT were normal. A 3 mm microadenoma was removed at transsphenoidal surgery. After surgery the patient had diabetes insipida and adrenal insufficiency. One year later all endocrinologic studies were normal. This fact underlines the importance that transsphenoidal surgery can have in the treatment of Cushing's disease.

摘要

作者描述了一例8岁大龄男孩患库欣病的病例,该男孩存在生长发育迟缓、阴毛生长及肥胖问题。他患有皮质醇增多症,对1毫克地塞米松抑制试验无反应。对于病变定位而言,更有意思的是,给予8毫克地塞米松后抑制率超过50%,而蝶鞍断层扫描和计算机断层扫描(CAT)结果正常。经蝶窦手术切除了一个3毫米的微腺瘤。术后患者出现尿崩症和肾上腺功能不全。一年后所有内分泌检查均恢复正常。这一事实突显了经蝶窦手术在库欣病治疗中的重要性。

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