周期性库欣综合征的临诊应对  

Approach to the patients with cyclic Cushing＇s syndrome

作　　者：李佳[1] 臧丽[1] 周涛[2] 杜锦[1] 吕朝晖[1] 母义明[1] 

机构地区：[1]解放军总医院内分泌科,北京100853 [2]解放军总医院神经外科,北京100853

出　　处：《中华内分泌代谢杂志》2017年第12期1063-1067,共5页Chinese Journal of Endocrinology and Metabolism

摘　　要：周期性库欣综合征是库欣综合征的一种特殊表现形式,皮质醇呈周期性分泌增多.常见病因包括库欣病、异位促肾上腺皮质激素（adrenocorticotropic hormone,ACTH）综合征及原发肾上腺疾病.皮质醇分泌周期长短不一,临床症状可自发缓解并反复发作,定性及定位诊断均较为困难.常用监测方法包括血清皮质醇、24 h尿游离皮质醇、清晨尿皮质醇/肌酐、唾液皮质醇及头发节段皮质醇含量的测定等,无论采用哪种监测手段,动态而重复性的检测对于明确诊断是非常必要的.本文详细分析1例由垂体ACTH瘤引起周期性库欣综合征的诊治过程,并总结周期性库欣综合征的临床特点、监测手段和诊治策略.Cyclic Cushing＇s syndrome is a special manifestation of Cushing＇s syndrome, of which the serum cortisol is only periodically increased. The causes include Cushing＇ s disease, ectopic adrenocorticotropic hormone （ ACTH）-producing syndrome, and primary adrenal disease. It is difficult to diagnose because the cycle length varies from hours to years and the clinical signs may show spontaneous remissions and recurrent attacks. The detection methods include either measurement of serum cortisol, 24 h urinary free cortisol, early-morning urinary cortisol/creatinine, salivary cortisol or hair cortisol. No matter which method to be used, repeated and continuous measurement is essential. Here, we present a female patient who was diagnosed as cyclic Cushing＇s syndrome due to a pituitary ACTH adenoma. The clinical features, detection methods, diagnosis, and treatment strategies of cyclic Cushing＇ s syndrome are also reviewed in this article.

关 键 词：库欣综合征 垂体ACTH瘤 正电子发射计算机断层显像 

分 类 号：R586[医药卫生—内分泌]