原发性醛固酮增多症合并肾上腺性库欣综合征的临床特点

Clinical characteristics of primary aldosteronism with concurrent adrenal Cushing′s syndrome

目的:分析总结原发性醛固酮增多症(PA)合并肾上腺性库欣综合征(ACS)患者的临床特点。方法:回顾性分析2013年1月1日至2016年12月31日北京大学第一医院收治的87例PA患者的临床、影像及病理资料。其中男45例(51.7%)、女42例(48.3%),年龄15~70(52.7±10.5)岁。根据是否合并ACS,将患者分为PA合并ACS组(n=11)、单纯PA组(n=55),总结PA合并ACS患者的临床特点。结果:11例PA合并ACS患者中有10例亚临床ACS。与单纯PA组比较,PA合并ACS组患者就诊年龄更大[(55.6±9.1)岁比(48.4±10.2)岁,P=0.033],肾上腺肿瘤最大直径更大[(2.3±1.6)cm比(1.4±0.6)cm,P=0.015],皮质醇/促肾上腺皮质激素(ACTH)分泌节律中血清皮质醇水平更高[0∶00皮质醇:158(92,217)nmol/L比71(43,128)nmol/L,P=0.004],血浆ACTH水平更低[8∶00 ACTH:0.80(<0.22,1.45)pmol/L比4.40(2.58,5.93)pmol/L,P<0.001;16∶00 ACTH:0.25(<0.22,0.91)pmol/L比2.03(0.74,3.69)pmol/L,P<0.001;0∶00 ACTH:<0.22(<0.22,0.44)pmol/L比1.27(<0.22,2.66)pmol/L,P<0.001],1 mg过夜地塞米松抑制试验后血清皮质醇水平更高[95(73,142)nmol/L比30(22,41)nmol/L,P<0.001]。31例患者行单侧肾上腺手术治疗,其中PA合并ACS组3例(3/8)及单纯PA组3例(3/23)术后出现肾上腺皮质功能不全并给予糖皮质激素替代治疗。结论:PA合并ACS在PA中并不罕见,以合并亚临床ACS为主,极易被漏诊。建议对高龄、肾上腺肿瘤比较大的PA患者,积极检测皮质醇和ACTH分泌节律并行1 mg过夜地塞米松抑制试验筛查ACS。PA合并ACS患者行肾上腺手术治疗后易出现肾上腺皮质功能不全,建议术后密切随访,及时给予糖皮质激素替代治疗。

Objective To analyze and summarize the clinical characteristics of primary aldosteronism(PA)with concurrent adrenal Cushing′s syndrome(ACS).Methods The clinical,imaging and pathological data of 87 patients with PA admitted to Peking University First Hospital from January 1st,2013 to December 31th,2016 were reviewed.There were 45 males(51.7%)and 42 females(48.3%)with an average age of(52.7±10.5)years(range:15-70 years).According to whether they were diagnosed with ACS,the patients were divided into PA with concurrent ACS group(n=11)and pure PA group(n=55).The clinical characteristics of PA with concurrent ACS were summarized.Results Ten of the eleven patients in PA with concurrent ACS group were diagnosed with subclinical ACS.Compared with these in pure PA group,the patients in PA with concurrent ACS group were older[(55.6±9.1)years vs(48.4±10.2)years,P=0.033],with larger diameters of adrenal tumors[(2.3±1.6)cm vs(1.4±0.6)cm,P=0.015],higher serum cortisol levels in the circadian rhythm[158(92,217)nmol/L vs 71(43,128)nmol/L at 0∶00,P=0.004],lower plasma adrenocorticotropic hormone(ACTH)levels in the circadian rhythm[0.80(<0.22,1.45)pmol/L vs 4.40(2.58,5.93)pmol/L at 8∶00,P<0.001;0.25(<0.22,0.91)pmol/L vs 2.03(0.74,3.69)pmol/L at 16∶00,P<0.001;<0.22(<0.22,0.44)pmol/L vs 1.27(<0.22,2.66)pmol/L at 0∶00,P<0.001],and higher serum cortisol levels after 1 mg overnight dexamethasone suppression test[95(73,142)nmol/L vs 30(22,41)nmol/L,P<0.001].Unilateral adrenalectomy was performed in 31 patients,of whom 3 patients in PA with concurrent ACS group(3/8)and 3 patients in pure PA group(3/23)presented with adrenal insufficiency and received glucocorticoid replacement therapy after adrenalectomy.Conclusions PA with concurrent ACS is not rare in PA patients,which are mainly complicated with subclinical ACS and easily misdiagnosed.It is recommended that elderly PA patients with large adrenal tumors should be screened for ACS by means of cortisol and ACTH circadian rhythm and 1 mg overnight dexamethasone suppression test.Postoperative adrenal insufficiency is prone to occur in those patients,who should be followed up closely after adrenalectomy,and receive glucocorticoid replacement therapy in time.