【摘要】 目的提高临床医生对异位ACTH综合征的认识。方法对四川大学华西医院1996—2005年确诊的6例异位ACTH综合征患者的临床资料及诊治过程进行回顾性分析。结果副节瘤1例,神经内分泌癌1例,肺癌4例。临床特征病程15d至2个月,6例患者均有严重低血钾伴碱中毒,5例患者有高血压和双下肢水肿。Cushing综合征表现满月脸4例,淤斑和长胡须2例,多血质、痤疮、色素沉着各1例,血皮质醇(晨起8时)>1173nmol/L,大剂量地塞米松(8mg)抑制试验均不能抑制。结论对病程短,严重低血钾碱中毒,水肿明显而Cushing临床表现不明显者,血皮质醇>1000nmol/L应高度怀疑异位ACTH综合征。寻找病因,尽快手术切除肿瘤,近期预后较好。更多还原

【Abstract】 Objective To deepen clinicians’ recognition to ectopic ACTH syndrome.Methods Six patients with ectopic ACTH syndrome were reviewed retrospectively.Results The cause of disease:1 patient with chromaffinoma,1 patient with neuroendocrine carcinoma,4 patients with pulmonary carcinoma.Clinical features:The course of disease:half a month to more than 2 months;6 patients had serious hypokalemic alkalosis;5 patients had hypertension and the edema of lower limbs;4 patients had moon-shaped face;2 patients had beard and ecchymosis;1 patient had sanguineous;1 patient had acne;1 patient had hyperpigmentation;plasma corticosteroid >1 173 nmol/L;6 patients did not respond to 8mg dexamethasone suppression.Conclusion Patients with the following clinical features should be suspected as ectopic ACTH syndrome:srious hypokalemic alkalosis and obvious edema of lower limbs without typical symptoms of Cushing’s syndrome;plasma corticosteroid>1 000 nmol/L.We should search the cause of disease and perform tumor execution as soon as possible.The short-term prognosis is good.更多还原