靶腺切除治疗异位ACTH综合征

目的　探讨肾上腺切除治疗异位ACTH综合征的临床效果。　方法　异位ACTH综合征 2 3例 ,男 14例 ,女 9例 ,平均年龄 38岁。 2 3例临床表现、生化测定及影像学检查均符合库兴综合征的诊断。行肾上腺全切 16例 ,7例未手术者行氨基导眠能治疗。　结果　 16例行肾上腺全切患者术后库兴综合征症状及体征均有明显缓解 ,1、2及 5年存活率分别为 6 7%、38%及 19% ;7例未手术治疗患者中失访 3例 ,其余 4例无 1例存活 >1年。　结论　双侧肾上腺全切加激素替代疗法是治疗定位困难的异位ACTH综合征患者的一种有效方法。

Role of adrenolectomy in ectopic ACTH syndrome

Objective To evaluate the role of adrenolectomy in ectopic ACTH syndrome. Methods Clinical data of 23 cases of ectopic ACTH syndrome were analysed.Of them,14 were males and 9 were female,the average age was 38.All of the 23 cases had clinical and biochemical and imaging evidences for the diagnosis of Cushing's syndrome.16 of the 23 cases were treated with total adrenalectomy and the other 7 were treated without surgical intervention. Results Sixteen cases,having no identifiable source of ectopic hormone production, treated with bilateral or unilateral total adrenalectomy,had resolution of their presenting signs and symptoms,1-year survival was 67%,2-year survival was 44% and 5-year survival was 19%.Seven of the patients who were managed without surgical intervention,1-year survival was 0%. Conclusions To most patients with no identifiable source of ectopic hormone production, bilateral adrenolectomy with hormonal replacement is effective.