原发性双侧大结节样肾上腺增生症的临床特征探讨  

Clinical features of primary bilateral macronodular adrenal hyperplasia

作　　者：周薇薇[1] 苏颋为[1] 祝宇[2] 姜蕾[1] 孙福康[2] 蒋怡然[1] 戴军[2] 张翠[1] 何竑超[2] 钟旭[1] 武鲁铭 郑思畅 王卫庆[1] Zhou Weiwei;Su Tingwei;Zhu Yu;Jiang Lei;Sun Fukang;Jiang Yiran;Dai Jun;Zhang Cui;He Hongchao;Zhong Xu;Wu Luming;Zheng Sichang;Wang Weiqing(Department of Endocrine and Metabolic Diseases,Shanghai Institute of Endocrine and Metabolic Diseases,Ruijin Hospital,Shanghai Jiao Tong University School of Medicine,Shanghai National Clinical Research Center for Metabolic Diseases,Key Laboratory for Endocrine and Metabolic Diseases of the National Health Commission of the PR China,Shanghai Key Laboratory for Endocrine Tumor,State Key Laboratory of Medical Genomics,Shanghai,200025,China;Department of Urology,Ruijin Hospital,Shanghai Jiao Tong University School of Medicine,Shanghai 200025,China)

机构地区：[1]上海交通大学医学院附属瑞金医院内分泌代谢科,上海市内分泌代谢病研究所,国家代谢性疾病临床医学研究中心(上海),国家卫健委内分泌代谢病重点实验室,上海市内分泌肿瘤重点实验室,医学基因组学国家重点实验室,上海200025 [2]上海交通大学医学院附属瑞金医院泌尿外科,上海200025

出　　处：《中华内分泌代谢杂志》2023年第12期1023-1027,共5页Chinese Journal of Endocrinology and Metabolism

基　　金：国家重点研发计划(2021YFC2501600,2021YFC2501601,2021YFC2501603);国家自然科学基金重点项目(91857205,81730023);上海市医药卫生发展基金会糖尿病临床研究项目(Ⅱ期02研究);上海市申康医院发展中心项目(SHDC2020CR2002A,SHDC2020CR6015);上海市卫计委面上项目(201840049)。

摘　　要：目的探讨原发性双侧大结节样肾上腺增生症(primary bilateral macronodular adrenal hyperplasia,PBMAH)的临床特征及性别差异。方法回顾性纳入本中心2014年起诊断的PBMAH 140例,所有患者均行激素水平、影像学检查和代谢指标的评估。结果PBMAH队列中临床库欣综合征占76.4%,女性占47.9%。与亚临床组相比,临床组有着更高的血皮质醇和24 h尿游离皮质醇水平、更低的促肾上腺皮质激素、更高的小剂量地塞米松抑制试验血皮质醇抑制值和更大的肾上腺最大径,且肥胖、高血压、糖尿病和低钾血症比例更高(均P<0.05)。与男性相比,女性组的肾上腺最大径更小(P<0.001)、HbA_(1C)更低(P=0.003)、总胆固醇(P=0.005)和低密度脂蛋白胆固醇水平更高(P=0.035)。校正年龄和病程后,男性组的24 h尿游离皮质醇与体重指数呈负相关性;进一步校正体重指数后,与舒张压、空腹血糖、口服葡萄糖耐量试验(OGTT)2 h血糖和HbA_(1C)呈正相关性,与血钾水平呈负相关性。而女性组的24 h尿游离皮质醇仅与舒张压、空腹血糖和OGTT 2 h血糖呈正相关性(均P<0.05)。随访结果显示,单侧肾上腺切除术的缓解率为80.0%,复发率为17.9%。结论PBMAH相关代谢紊乱与临床库欣综合征和男性关系更密切,且单侧肾上腺切除术作为有效治疗方式能使大部分患者获益。Objective To investigate the clinical profile of primary bilateral macronodular adrenal hyperplasia(PBMAH)and sex difference.Methods One hundred and forty cases of PBMAH were recruited in our center from 2014,and all patients were evaluated for hormone secretion,adrenal imaging,and metabolic parameters.Results Overt Cushing′s syndrome accounted for 76.4%of PBMAH cohort and 47.9%were female.The overt group had higher serum cortisol and 24 h urinary free cortisol levels,lower adrenocorticotropic hormone,higher serum cortisol after low-dose dexamethasone suppression tests,larger total adrenal size,and a higher percentage of obesity,hypertension,diabetes mellitus,and hypokalemia than the subclinical group(all P<0.05).When compared with the male group,the female group had smaller adrenal size(P<0.001),lower HbA_(1C)(P=0.003),higher total cholesterol(P=0.005),and lower density lipoprotein-cholesterol levels(P=0.035).Further,24 h urinary free cortisol in the male group was found to be positively correlated with diastolic blood pressure,fasting glucose,2 h postprandial glucose after oral glucose tolerance test(OGTT),and HbA_(1C)after adjusted for age,body mass index,and onset duration,and was negatively correlated with body mass index and potassium levels.While 24 h urinary free cortisol in the female group was positively correlated only with diastolic blood pressure,fasting glucose,and 2 h postprandial glucose after OGTT(all P<0.05).During follow-up,80.0%of patients achieved remission after unilateral adrenalectomy,with a recurrence rate of 17.9%.Conclusion PBMAH related metabolic disorder is more pronounced in overt Cushing′s syndrome and males.Unilateral adrenalectomy as an effective treatment can benefit the majority of patients.

关 键 词：原发性双侧大结节样肾上腺增生症 代谢紊乱 性别差异 

分 类 号：R586[医药卫生—内分泌]