摘要
背景库欣综合征(CS)临床表现复杂,在CS的病因诊断中,库欣病(CD)和异位促肾上腺皮质激素(ACTH)综合征(EAS)难以鉴别诊断。而双侧岩下窦静脉采血(BIPSS)目前是鉴别诊断CD和EAS的金标准,但其在大多数医院无法开展。目前临床通常用大剂量地塞米松抑制试验(HDDST)诊断CD,但其灵敏度较低;有研究证明去氨加压素(DDAVP)具有促肾上腺皮质激素释放激素(CRH)样作用,其可促进垂体ACTH肿瘤分泌ACTH。目的探究外周DDAVP兴奋试验与HDDST在CD中的诊断价值。方法回顾性选取2015-01-01至2018-08-30于中国人民解放军总医院明确诊断为ACTH依赖性CS的患者72例为研究对象。收集患者一般资料及外周DDAVP兴奋试验、HDDST结果。计算DDAVP兴奋试验、HDDST诊断CD的灵敏度、特异度。结果72例ACTH依赖性CS患者中,CD 64例,EAS 8例。有63例ACTH依赖性CS患者进行了外周DDAVP兴奋试验(其中CD 55例,EAS 8例),其余9例予以剔除;外周DDAVP兴奋试验阳性51例,阴性12例;外周DDAVP兴奋试验诊断CD的灵敏度为92.7%,特异度为100.0%。72例ACTH依赖性CS患者中,HDDST阳性43例,阴性29例;HDDST诊断CD的灵敏度为67.2%,特异度为100.0%。同时行外周DDAVP兴奋试验与HDDST的63例患者中,外周DDAVP兴奋试验与HDDST同时阳性38例(均为CD患者),外周DDAVP兴奋试验与HDDST同时阴性9例(8例为EAS患者),外周DDAVP兴奋试验阳性、HDDST阴性16例,外周DDAVP兴奋试验阴性、HDDST阳性0例。同时行外周DDAVP兴奋试验与HDDST的55例CD患者中,外周DDAVP兴奋试验与HDDST同时阳性38例,外周DDAVP兴奋试验与HDDST同时阴性1例,外周DDAVP兴奋试验阳性、HDDST阴性16例,外周DDAVP兴奋试验阴性、HDDST阳性0例。结论外周DDAVP兴奋试验对CD有较高的诊断价值,临床中疑似CD患者HDDST为阴性时,进一步行外周DDAVP兴奋试验可明显降低漏诊率。
Background The clinical manifestations of Cushing's syndrome are complex.It is difficult to determine whether Cushing's syndrome is induced by Cushing's disease or ectopic ACTH syndrome.Although bilateral inferior petrosal sinus sampling,a recognized gold standard,can be used to differentiate the two,it can not be implemented in most hospitals.High-dose dexamethasone suppression test(HDDST)is commonly used to diagnose Cushing's disease,but its sensitivity is not satisfactory.Desmopressin has been reported to have the effect similar to corticotropin-releasing hormone,promoting the ACTH-producing pituitary tumor to secrete ACTH.Objective To explore the diagnostic value of desmopressin stimulation test versus HDDST in Cushing's disease.Methods 72 patients with a clear diagnosis of ACTH-dependent Cushing's syndrome were retrospectively recruited from Chinese PLA General Hospital during January 1st,2015 to August 30th,2018.General data,results of desmopressin stimulation test and HDDST were collected.The sensitivity and specificity of desmopressin stimulation test and HDDST in the diagnosis of Cushing's disease were evaluated.Results Of the 72 cases,64 were found with Cushing's disease,and 8 with ectopic ACTH syndrome.All of them underwent desmopressin stimulation test except 9 cases of Cushing's disease being excluded.51 had positive test results and other 12 showed negative test results.The desmopressin stimulation test showed a sensitivity of 92.7%and a specificity of 100.0%in diagnosing Cushing's disease.43 had positive results and other 29 had negative results in HDDST.The HDDST demonstrated a sensitivity of 67.2%and a specificity of 100.0%in diagnosing Cushing's disease.38 cases of Cushing's disease had positive results and 9 cases of ectopic ACTH syndrome(8 with ectopic ACTH syndrome)had negative results in both the tests.16 cases had positive results in desmopressin stimulation test but showed negative results in HDDST,and 0 showed negative results in desmopressin stimulation test but showed positive results in HDDST.Among the 55 cases of Cushing's disease undergoing both tests,38 showed positive response and 1 showed negative response to both tests,16 showed positive response to desmopressin stimulation test but showed negative response to HDDST,and 0 showed negative response to desmopressin stimulation test but showed positive response to HDDST.Conclusion Desmopressin stimulation test has a high diagnostic value for Cushing's disease.It may be used for those with suspected Cushing's disease but show negative response to HDDST,by which the possibility of missed diagnosis of Cushing's disease may be significantly reduced.
作者
李庞敏
陈康
杜锦
谷伟军
巴建明
吕朝晖
母义明
LI Pangmin;CHEN Kang;DU Jin;GU Weijun;BA Jianming;LYU Chaohui;MU Yiming(Department of Endocrinology,986 Hospital of Air Force,Xi'an 710000,China;Department of Endocrinology,Chinese PLA General Hospital,Beijing 100089,China)
出处
《中国全科医学》
CAS
北大核心
2020年第18期2314-2317,共4页
Chinese General Practice
