库欣综合征208例

Cushingfs syndrome (Report of 208 cases)

目的:提高库欣综合征的诊治水平。方法:分析108例促肾上腺皮质激素(ACTH)非依赖性库欣综合征和100例ACTH依赖性库欣综合征患者的临床资料、诊断和手术效果。结果:ACTH非依赖性和依赖性库欣综合征均有库欣综合征的体征和血或尿皮质醇升高;腺瘤型库欣综合征主要显示大剂量地塞米松抑制试验(HDDST)不抑制和CT发现较小的肾上腺占位病变,99例行腺瘤切除术获治愈;肾上腺皮质癌主要表现瘤体直径>6cm及DHEAS升高,预后差;大结节样肾上腺皮质增生(AIMAH)的CT主要表现双侧肾上腺大结样增生,行双侧肾上腺切除可获治愈;库欣病可被HDDST抑制,CT显示双侧肾上腺增生或无变化,对69例垂体无阳性发现的患者行一侧肾上腺全切除加另一侧肾上腺次全切除症状缓解,20例轻型行一侧肾上腺切除加垂体放疗能获长期症状缓解,肾上腺全切分期进行较安全;异位ACTH综合征主要表现ACTH明显升高及发现分泌ACTH肿瘤。结论:对库欣综合征应行鉴别诊断,对不同类型库欣综合征采用适当手术方法可获较满意的手术效果。

Purpose:To develop the therapeutic ability on cushing's syndrome. Methods:To analyse the clinical data, diagnosis and surgical effect including 108 cases of corticotropin independent cushing's syndrome and 100 cases of corticotropin dependent cushing's syndrome. Results: All patients represented the symptoms of hypercorti-solism and had a high level of blood or urinary cortisol at the same time. The HDDST was negative when the pathological conclusion was adenoma while minor mass was detected in CT. Ninety nine patients were cured after resected the adenoma. Masses were found beyond 6 cm and the DHEAS went up in cases of adrenal cortical carcinoma in which the prognosis was very poor. The character of AIMAH on CT was bilateral adrenal macro-nodular hyperplasia. Only remove both side of the adrenal can heal the patients. The HDDST was positive in Cushing's disease. Each side of the adrenal showed hyperplastic or normal in CT. Total unilateral adrenal resection was performed in 69 cases with normal pituitary, other one received sub-total surgery. Their symptoms were alleviated. Twenty cases that had slight symptoms got the unilateral adrenal resection plus pituitary radioactive therapy. They have had a longterm alleviation. It was much safer to perform the total adrenal resection in different stages. As for ectopic ACTH syndrome, the serum corticotropin was high and tumors that excreting ACTH could be found. Conclusions: The diagnosis of cushing's syndrome should be identified. Different kind of this disease should be managed with different surgery in order to get the optimun effect.