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双侧岩下窦静脉插管取血(bilateral inferior petrosal sinus sampling, BIPSS)及去氨加压素(DDAVP)兴奋试验是库欣病诊断的金标准。但有少数库欣病患者BIPSS呈假阴性结果,临床上易被误诊为异位ACTH综合征。本文报告1例BIPSS假阴性的库欣病病例,并复习文献,以提高临床医师对ACTH依赖性库欣综合征的鉴别诊断及治疗水平。“,”Bilateral inferior petrosal sinus sampling(BIPSS), with or without desmopressin stimulation, is the gold standard in the diagnosis of Cushing′s disease. A few of patients with Cushing′s disease present a false negative result in BIPSS. These patients are often misdiagnosed as ectopic adrenocorticotropin(ACTH) syndrome(EAS). Here we report a case of Cushing′s disease with a false negative BIPSS, in the hope to aid clinical physicians in the differential diagnosis and treatment of ACTH-dependent Cushing′s syndrome.