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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。
Objective To improve clinicians’ understanding of ectopic ACTH syndrome. Methods The clinical data and diagnosis and treatment of 6 patients with ectopic ACTH syndrome diagnosed in West China Hospital of Sichuan University from 1996 to 2005 were retrospectively analyzed. Results Pseudobulbar tumor in 1 case, neuroendocrine carcinoma in 1 case, 4 cases of lung cancer. Clinical characteristics of the course of 15d to 2 months, 6 patients had severe hypokalemia with alkali poisoning, 5 patients had hypertension and lower extremity edema. Cushing’s syndrome manifested full-moon face in 4 cases, ecchymosis and beard in 2 cases, multiple blood clots, acne, pigmentation in 1 case, blood cortisol (morning 8:00)> 1173nmol / L, high dose dexamethasone Inhibition test can not be inhibited. Conclusion For short course, severe hypokalemia, edema and obvious clinical manifestations of Cushing, Cortisol> 1000nmol / L should highly suspect ectopic ACTH syndrome. Looking for the cause, resection of the tumor as soon as possible, the recent good prognosis.