Cushing病与ACTH非依赖性Cushing综合征的临床特征分析  

Analysis on clinical characteristics of Cushing disease and ACTH-independent Cushing’s syndrome

作　　者：张娜娜 戚瑞 王琼 刘涛 ZHANG Nana;QI Rui;WANG Qiong;LIU Tao(Department of Endocrinology and Metabolism,Xijing Hospital of Air Force Military Medical University,Xi’an,Shaanxi 710032,China)

机构地区：[1]空军军医大学西京医院内分泌代谢科,西安710032

出　　处：《重庆医学》2023年第8期1198-1201,1207,共5页Chongqing medicine

摘　　要：目的分析Cushing病(CD)与促肾上腺皮质激素(ACTH)非依赖性Cushing综合征(Non-ACTH)患者的临床表现、内分泌实验室检查和影像学检查的特点,以提高对该类疾病的诊治水平。方法收集2010-2021年在该院住院确诊为Cushing综合征(CS)患者的病历资料96例,其中CD 58例(CD组),Non-ACTH 38例(Non-ACTH组)。回顾性分析患者的临床表现、内分泌实验室检查及影像学检查结果。结果2组患者均以女性多见。CD组较Non-ACTH组更易合并高雄激素血症。CD组患者ACTH均值为76.61pg/mL,经典小剂量地塞米松抑制试验(LDDST)后皮质醇均值为19.97μg/dL,20.7%的患者为大剂量地塞米松抑制试验(HDDST)不可抑制;82.6%的患者通过垂体动态增强MRI可检出垂体瘤,77.7%的患者出现肾上腺的双侧或单侧增生。Non-ACTH组患者ACTH均值为9.39 pg/mL,LDDST后皮质醇均值为21.26μg/dL,100%的患者为HDDST不可抑制,肾上腺CT检查出肾上腺腺瘤或大结节样增生100%,其中68.4%的患者为左侧肾上腺腺瘤。结论女性CS发病率高于男性,CD典型特点为ACTH、皮质醇水平高,CT表现为肾上腺增生,垂体MRI示垂体瘤。Non-ACTH CS的典型临床特点为皮质醇水平高,ACTH偏低或在正常范围,CT表现为单侧肾上腺腺瘤。Objective To analyze the clinical manifestations and characteristics of endocrine laboratory detection and imaging examinations of the patients with Cushing disease(CD)and ACTH-independent Cushing’s syndrome(Non-ACTH),so as to improve the diagnosis and treatment level of these diseases.Methods The data of 96 inpatients with definitely diagnosed with Cushing’s syndrome(CS)in this hospital during 2010-2021 were collected,including 58 cases of CD and 38 cases of Non-ACTH.The clinical manifestations,endocrinological laboratory and imaging examinations results were analyzed retrospectively.Results The patients in both groups were mostly female.The CD group was more likely to be complicated with hyperandrogenemia.The mean ACTH value of the CD group was 76.61 pg/mL,and the mean cortisol of the classic low-dose dexamethasone suppression test was 19.97μg/dL.20.7%of the patients were not suppressed by the high-dose dexamethasone suppression test(HDDST).Pituitary tumors could be detected by pituitary dynamic enhanced magnetic resonance in 82.6%of the patients,and 77.7%of the patients developed bilateral or unilateral adrenal hyperplasia.The mean ACTH value in the patients with Non-ACTH was 9.39 pg/mL,and the mean cortisol value after LDDST was 21.26μg/dL.100%of the patients were not suppressed by HDDST,adrenal CT could detect 100%of adrenal adenoma or macronodular hyperplasia,among them 68.4%of the patients were left side adrenal adenoma.Conclusion The incidence rate of CS in female is higher than that in male,the typical characteristics of CD are high ACTH and cortisol levels,the CT manifestations show adrenal hyperplasia and the pituitary MRI shows pituitary tumor.The typical clinical characteristics of Non-ACTH are high cortisol level,ACTH is low or in the normal range,and CT manifested by the one side adrenal adenoma.

关 键 词：CUSHING综合征 临床特征 地塞米松抑制试验 垂体瘤 肾上腺皮质腺瘤 皮质醇 

分 类 号：R586.2[医药卫生—内分泌]