16例异位ACTH综合征的临床特点分析

目的 通过分析异位ACTH综合征的临床特点,加深对其的认识,提高诊治水平.方法 回顾性分析解放军总医院2000-2009年确诊的16例异位ACTH综合征患者的临床资料.结果 (1)异位ACTH综合征最常见的病因为肺部肿瘤及胸腺类癌;(2)临床表现以糖代谢异常、高血压、低血钾、下肢水肿最为突出;(3)实验室检查提示血皮质醇、促肾上腺皮质激素(ACTH)及24 h尿游离皮质醇(24hUFC)水平显著升高及严重的低血钾碱中毒;(4)大剂量地塞米松抑制试验、促肾上腺皮质激素释放激素(CRH)兴奋试验及岩下窦取血测定岩下静脉血ACTH与外周静脉血ACTH的比值对定性诊断具有较高价值;(5)X线胸片、胸部CT能发现大多数原发病灶;(6)手术切除原发肿瘤病灶是首选治疗方法.结论 异位ACTH综合征诊断困难,其治疗应首选手术切除原发肿瘤.

The clinical features of ectopic ACTH syndrome: a report of 16 cases

Objectives To improve the diagnostic and therapeutic ability of ectopic ACTH syndrome by analysing its clinical features.Methods Sixteen cases of ectopic ACTH syndrome diagnosed in Chinese PLA General Hospital from 2000 to 2009 were analyzed retrospectively.Results ( 1 ) The main causes of ectopic ACTH syndrome were lung tumor and thymic carcinoid;(2) Abnormal glucose metabolism, hypertension, hypokalemia and edema of both lower limbs were the most common clinical symptoms;(3) Laboratory examination showed a significant increase of serum cortisol, adrenocorticotropic hormone(ACTH) and 24 h urinary free cortisol (24hUFC) together with severe hypokalemia and alkalosis;(4) High dose dexamethasone suppressing test, corticotrophin-releasing hormone(CRH) stimulating test and petrosal sinus sampling were the most meaningful diagnostic methods;(5) Most of the primary lesions might be detected with chest film and CT;(6) Resection of the primary lesion was the treatment of first choice.Conclusion The diagnosis of ectopic ACTH syndrome is very hard.Resection of the primary lesion is the best treatment.