ACTH非依赖性库欣综合征术后糖皮质激素替代治疗方法分析

On the postoperative steroid replacement in patients with ACTH independent Cushing＇s Syndrome

目的 ACTH非依赖性库欣综合征术后糖皮质激素替代方案及其影响因素分析.方法 纳入2003 ～2013年瑞金医院内分泌科临床诊断为ACTH非依赖性库欣综合征,病理明确为肾上腺皮质腺瘤患者181例.通过评估术前内分泌水平、影像学及临床随访资料,分析术后糖皮质激素替代剂量及替代时间的影响因素.结果 所有患者术中及术后均需要激素替代,醋酸可的松平均替代时间为12个月（6～ 15个月）,平均替代累积剂量为（8.12±5.07）g.Spearman相关性分析显示发病年龄分别与术后激素替代时间（r=0.250,P=0.010）及激素替代累积剂量（r=0.244,P=0.013）呈正相关,进一步行多元逐步回归分析提示发病年龄是术后可的松替代时间及替代剂量的独立影响因素（P＜0.05）.术后ACTH先于皮质醇恢复并在术后9个月达到高峰,当肾上腺皮质功能恢复正常后ACTH降至正常范围.2.88％（3/104）患者出现激素撤退综合征.库欣综合征体征、体重指数、血压、血糖及血脂在术后半年内得到显著改善.结论 ACTH非依赖性库欣综合征术后需要醋酸可的松替代,发病年龄越大的患者术后激素替代时间越长并且累积剂量越大.激素减量过程中需注意激素撤退综合征的发生.

Objective To investigate the preoperative factors in influencing the postoperative steroid replacement in patients with ACTH-independent Cushing＇s syndrome.Methods One hundred and eighty-one patients with adrenal cortical adenoma underwent unilateral adrenalectomy from 2003 to 2013.All preoperative and postoperative follow-up clinical parameters were acquired and analyzed.Results All patients need glucocorticoid （GC） replacement during and after surgery.At the median follow-up of 32 months （14-64 months）,104 patients withdrew the cortisone acetate replacement,with a median replacement duration of 12 months （6-15 months） and an average cumulative dosage of（8.12 ± 5.07） g.Spearman correlation analysis showed that the age at diagnosis was positively correlated with the duration of GC replacement therapy （r =0.250,P =0.010） as well as the cumulative dosage of GC（r=0.244,P =0.013）.Multiple stepwise regression analysis indicated that age at diagnosis was the independent factor that influenced both the duration and cumulative dosage of postoperative cortisone acetate replacement（P＜0.05）.Plasma ACTH levels gradually increased and peaked during the 9th month,there was a lag of several months in the recovery of normal adrenal responsiveness.After that period,corticosteroid rose to normal levels,and followed by plasma ACTH concentrations,which fell to normal.2.88％ （3/104） of the patients presented with steroid withdrawal syndrome （SWS）.Most features of hypercortisolism resolved within 6 months after surgery.Conclusion An empirical experience in Ruijin hospital in using cortisol replacement therapy for patients with ACTH-independent Cushing＇s syndrome after unilateral adrenalectomy was herewith reported.The older patients tended to prolong the replacement therapy of steroids and thus had to deal with a larger cumulative dosage.We should be aware of the risk of development of SWS postoperatively.