青少年库欣综合征的临床表现和定性诊断  

Clinical manifestations and qualitative diagnosis of Cushing＇ s syndrome in adolescence

作　　者：陈适[1] 卢琳[1] 童安莉[1] 潘慧[1] 朱慧娟[1] 茅江峰[1] 陆召麟[1] 

机构地区：[1]中国医学科学院,北京协和医学院,北京协和医院内分泌科,卫计委内分泌重点实验室,100730

出　　处：《中华神经外科杂志》2015年第11期1131-1134,共4页Chinese Journal of Neurosurgery

基　　金：国家临床重点专科建设项目（WBYZ2011-873）

摘　　要：目的分析青少年库欣综合征（CS）的临床特点和定性诊断方法。方法对1990年1月至2012年3月北京协和医院内分泌科收治的50例青少年CS患者的临床特点和实验室检查结果进行回顾性分析。结果患者就诊年龄（14±4）岁，男：女=1．00：1．17。50例CS患者中，36例（72％）患有库欣病，为最常见的病因。CS患者临床表现典型，锁骨上脂肪垫、满月脸、向心性肥胖、水牛背症状出现比例均超过90％；63％的青少年CS患者身高低于第3百分位，42％的患者体质量高于第75百分位。高血压、骨质疏松、糖代谢紊乱、高脂血症也较常见。若采用1．8μg/dl为切点，则午夜血清皮质醇诊断敏感性最高（100％）；经典小剂量地塞米松抑制试验、24h尿游离皮质醇和过夜小剂量地塞米松抑制试验的敏感性分别为95％、88％和70％。结论青少年CS患者的最常见病因为库欣病，临床表现较典型，包括身材矮胖等。午夜血清皮质醇在切点为1．8μg/dl时，其诊断敏感性最高。Objective To analyze the clinical features and qualitative diagnosis methods of Cushing＇s syndrome （CS） in adolescence. Methods The clinical features and laboratory findings of 50 adolescent patients with CS admitted to the Department of Endocrinology, Peking Union Medical College Hospital from January 1990 to March 2012 were analyzed retrospectively. Results The age of medical treatment was 14 ＋4 years. Male ： female = 1.00 ： 1.17. Thirty-six adolescent patients （72%） had CS, and it was the most common cause. The proportions of the typical clinical manifestations, supraclavicular fat pads, moon face, centripetal obesity, and buffalo hump of the adolescent patients were more than 90%. The height of 63% adolescent patients with CS was below the third percentile, but the body mass of 42% adolescent patients was higher than the 75th percentile. Hypertension, osteoporosis, glucose metabolism disorder, and hyperlipidemia were also more common. If the 1. 8 μg/dl cut-off point was used, the diagnostic sensitivity of midnight serum cortisol was the highest （ 100% ） ; followed by the classic low-dose dexamethasone suppression test, 24 h urine free cortisol and overnight low-dose dexamethasone suppression test, their sensitivities were 95%, 88%, and 70%, respectively. Conclusions The most common cause is CS in adolescent patients with CS. The clinical manifestations are more typical, including dumpy figure, etc. When the midnight serum cortisol cut-off point is 18.8 μg/dl, its diagnostic sensitivity is the highest.

关 键 词：库欣综合征 青少年 定性诊断 敏感性与特异性 

分 类 号：R725.8[医药卫生—儿科]