库欣综合征37例临床分析

Analysis of 37 patients with Cushing＇ s syndrome

目的通过对37例库欣综合征（cs）患者临床资料的分析提高对cs的认识。方法对我院2003年12月-2013年12月收治的37例库欣综合征患者的临床表现、诊断方法和治疗措施进行回顾性分析。结果37例患者平均年龄（35．19±11．86）岁，病程中位数2年，男：女=1．0：2．7。最常见的临床表现为高血压、向心性肥胖及多血质外貌。午夜皮质醇水平、过夜小剂量（1mg）地塞米松抑制试验和24小时尿游离皮质醇（UFC）诊断库欣综合征的敏感性分别为100．00％、100．00％和91．30％。37例cs患者中库欣病（CD）13例，肾上腺性库欣综合征（aCS）15例，异位促。肾上腺皮质激素（ACTH）综合征（EAS）9例。以晨8时ACTH是否〉1．10μmol／L区分ACTH依赖性与非ACTH依赖性cs的敏感性为100．00％，特异性为100．00％。在ACTH依赖性cs中，大剂量地塞米松抑制试验（HDDST）皮质醇抑制率区分CD和EAS的最佳切点为42．87％，其敏感度为81．80％，特异度为93．30％。CD组13例、aCS组手术11例以及EAS组4例行手术治疗。结论本组资料显示cs多发生于中青年女性，病情较隐匿。最常见临床表现为高血压、向心性肥胖和多血质外貌。皮质醇分泌节律紊乱和负反馈调节异常是诊断cs的关键。高敏感度的ACTH测定可很好地区分ACTH依赖性与ACTH非依赖性cs。在ACTH依赖性cs中HDDST可较好地区分CD与EAS。CS的治疗关键是明确病因后手术治疗。

Objective To improve the understanding for Cushing＇ s syndrome（CS） by analyzing the clinical materials of 37 CS patients. Methods The clinical manifestations,diagnostic and therapeutic methods of 37 inpatients with Cushing＇ s syndrome in our hospital from December 2003 to December 2013 were retrospectively analyzed. Results The average age of the patients was （35.19 ± 11.86 ） years old, and the ratio of male to female was 1.0： 2.7. The median duration of the disease was 2 years. The most common clinical manifestations were hypertension, central obesity and plethora. The diagnostic sensitivity of midnight cortisol level,overnight low-dose（ 1 mg） dexamethasone suppression test and 2g-hour urinary free cortisol（UFC） for CS was 100.00%, 100. 00% and 91. 30% respevtively. In the 37 cases of Cushing＇s syndrome, 13 were Cushing disease（ CD）, 15 were adrenal Cushing＇s syndrome（aCS） ,9 were ectopic ACTH syndrome（EAS）. The adrenocortocotropic hormone（ACTH） cut-off value of 1.10 pmol/L could well differentiate the ACTH-dependent from ACTH independent Cushing＇ s syndrome. The cortisol suppression rate after high-dose dexamethasone suppression test （ HDDST ） of 42. 87% could well differentiate CD and EAS with the sensitivity of 81.80% and specificity of 93.30%. 13 cases in CD group, 11 cases in aCS and 4 cases in EAS group were surgically treated. Conclusion The resuhs demonstrated Cushing＇ s syndrome generally occurs in middle-aged women. The most common clinical manifestations are hypertension, central obesity and plethora. Disorder in cortisol secretion rhythm and negative feedback were more sensitive for the diagnosis of Cushing＇ s syndrome. The morning ACTH levels may well distinguish ACTH-dependent and ACTH independent Cushing＇ s syndrome. The HDDST is optimal to distinguish CD and the EAS and surgical operation is the first choice treatment for CS.