库欣综合征188例分析。

T C Liou, H C Lam, L T Ho
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摘要

从1962年1月到1988年4月,188名库欣综合征患者在退伍军人总医院住院治疗。医源性库欣综合征123例(65.4%)。促肾上腺皮质激素(ACTH)分泌过量43例(22.9%),良恶性肾上腺皮质肿瘤22例(11.7%)。评价医源性库欣综合征患者的临床特点和肾上腺功能储备。回顾性分析各种内分泌评价和影像学检查的诊断率。库欣综合征的治疗方法有四种:(1)垂体手术,(2)垂体照射,(3)肾上腺手术,(4)药物治疗。对这四种治疗方式的临床和激素反应进行了评估。本文报告25例库欣病经垂体手术治疗的内分泌学、影像学、外科及病理经验。5例(20%)内分泌异常(20%假阴性)。24例患者接受垂体CT扫描,16例(66.7%)CT表现阳性。在这25例患者中,19例病理证实为微腺瘤(小于1cm), 1例为大腺瘤(大于1cm), 5例没有腺瘤组织的证据。22例肾上腺库欣综合征患者中,4例(18%)为肾上腺皮质癌,18例(82%)为肾上腺良性肿瘤。左侧肾上腺腺瘤的发生率约为右侧肾上腺腺瘤的两倍。单侧肾上腺切除术对肾上腺皮质腺瘤的治愈率较高,但对肾上腺皮质癌的治愈率较低。
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Cushing's syndrome: analysis of 188 cases.

From January 1962 through April 1988, 188 patients with Cushing's syndrome were hospitalized at the Veterans General Hospital. Iatrogenic Cushing's syndrome was found in 123 (65.4%) of the patients. The other 43 patients (22.9%) had excess secretion of adrenocorticotropin (ACTH) and 22 patients (11.7%) had benign or malignant adrenocortical tumors. The clinical features and the functional reserve of the adrenal glands in those patients with iatrogenic Cushing's syndrome were evaluated. A retrospective analysis of the diagnostic rate of the various endocrine evaluations and the radiological examinations was also carried out. Four approaches were used in the management of Cushing's syndrome: (1) pituitary surgery, (2) pituitary irradiation, (3) adrenal surgery, and (4) drug therapy. Both the clinical and hormonal responses to these four management types were evaluated. The endocrinological, radiological, surgical and pathological experiences of 25 patients with Cushing's disease treated with pituitary surgery are presented. Five patients (20%) had an atypical endocrine pattern (20% false-negative). Twenty-four patients received a pituitary CT scan and 16 patients (66.7%) had positive CT findings. Of these 25 patients, 19 had pathology-proven microadenomas (less than 1cm), one had macroadenoma (greater than 1cm) and 5 had no evidence of adenomatous tissue. Among the 22 patients with adrenal Cushing's syndrome, 4 (18%) were suffering from adrenocortical carcinoma and 18 (82%) with benign adrenal tumors. The incidence of left adrenal adenoma was approximately twice that of right adrenal adenoma. The cure rate of adrenocortical adenoma by surgical treatment with unilateral adrenalectomy was excellent, but for adrenocortical carcinoma was poor.

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