Cushing综合征的病因诊断与临床特点分析

Clinical study and etiologic diagnosis of Cushing's syndrome

目的分析Cushing综合征患者的不同病因、临床表现及内分泌实验室检查和影像学检查的特点,评价各种病因鉴别诊断方法的效率。方法对经手术病理确诊为Cushing综合征的42例患者的临床资料和实验室检查、影像学检查等资料进行回顾分析。结果 42例患者的病因以Cushing病占的比例最高,主要临床表现在不同病理类型之间发生率差异无统计学意义。但满月脸、紫纹、皮肤瘀斑在肾上腺瘤中的发生率比其他病理类型为高,64.3%的病人有典型的Cushing外貌,血糖异常、高血压的发生率分别为61.9%、61.9%。Cushing综合征诊断试验的敏感性从高到低依次为不被午夜1mg地塞米松抑制、小剂量地塞米松抑制试验、24h尿游离皮质醇升高、血皮质醇昼夜节律消失、清晨血皮质醇升高。在Cushing综合征的病因鉴别诊断中,93.3%的肾上腺腺瘤病人不被8mg大剂量地塞米松抑制,71.4%的病人被8mg大剂量地塞米松实验抑制。在影像学检查中,肾上腺CT可100%检查出肾上腺腺瘤。结论 Cushing病和肾上腺腺瘤是Cushing综合征最常见的病因。在临床上关注一些有倾向性的表现,并结合ACTH检测、8mg地塞米松抑制试验以及影像学检查,方能对Cushing综合征的病因作出准确的鉴别诊断。

Objective To explore the characteristics of Cushing’s syndrome in clinical manifestations and endocrinological inspection and imaging examination,and evaluate the efficiencies of various assays in diagnosis and differential diagnosis.Methods The medical records of a total of 42 patients with Cushing’ s syndrome were retrospectively reviewed.Results Cushing’s disease was the most common cause of Cushing’s syndrome.About 64.3% patients had typical Cushing symptoms.The prevalence of glucose intolerance and hypertension were 61.9% and 61.9%,respectively.The sensitivity for diagnosis from high to low was lack of cortisol suppression after 1 mg overnight dexamethasone test,low-dose dexamethasone inhibitory test,increased 24 h urinary free cortisol,loss of the normal diurnal rhythm of the plasma cortisol,raised morning plasma cortiso1.In the differential diagnosis,93.3% patients with adrenal adenoma can not be suppressed by high dose dexamethasone suppression test,but 71.4% patients with Cushing’s disease can be suppressed.In the imaging examination,CT of the adrenal glands can detect 100% adrenal neoplasm.Conclusion Cushing’s disease and adrenal adenoma are the most common causes in Cushing’s syndrome.We can concern about the tendentious clinical manifestations of Cushing’ s syndrome.A combination of ACTH,8 mg dexamethasone suppression test as well as the image assay is necessary for the differential diagnosis for Cushing’s syndrome.