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周期性库欣病及其在丙戊酸钠治疗下的成功控制

Cyclical Cushing's disease and its successful control under sodium valproate.

作者信息

Beckers A, Stevenaert A, Pirens G, Flandroy P, Sulon J, Hennen G

机构信息

Endocrinology, Université de Liège, Belgium.

出版信息

J Endocrinol Invest. 1990 Dec;13(11):923-9. doi: 10.1007/BF03349657.

Abstract

Several subgroups of Cushing's disease were recently described (anterior or intermediate lobe origin, hyper-or hypo-pulsatility of cortisol, presence or absence of response after GRH or TRH, cyclical Cushing's disease). We present here a detailed case report on a patient suffering from Cushing's disease whose endocrine functions were extensively investigated. Treatment with bromocriptine, as well as subsequent transsphenoidal surgery, were followed by rapid but transient reversal of symptoms. When clinical manifestations reoccurred, daily measurements of free urinary cortisol revealed a cyclic pattern of cortisol hyperexcretion. A study of ultradian rhythm revealed hyperpulsatility of cortisol secretion. More interestingly, a treatment with sodium valproate, a drug known to inhibit CRH production, was followed by a rapid and longstanding normalization of clinical and biological data for 2 years. Based on these data, and on information from the literature, the present case of Cushing's disease exhibits characteristics suggesting a possible hypothalamic origin.

摘要

最近描述了库欣病的几个亚组(起源于垂体前叶或中间叶、皮质醇高脉冲或低脉冲、促性腺激素释放激素或促甲状腺激素释放激素刺激后有无反应、周期性库欣病)。我们在此展示一份关于一名患有库欣病患者的详细病例报告,该患者的内分泌功能得到了广泛研究。使用溴隐亭治疗以及随后的经蝶窦手术之后,症状迅速但短暂地得到缓解。当临床表现再次出现时,每日测定游离尿皮质醇显示出皮质醇分泌过多的周期性模式。一项超日节律研究显示皮质醇分泌存在高脉冲性。更有趣的是,使用已知可抑制促肾上腺皮质激素释放激素分泌的丙戊酸钠进行治疗后,临床和生物学数据迅速且持续两年保持正常。基于这些数据以及文献资料,本病例的库欣病表现出提示可能起源于下丘脑的特征。

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