肾上腺性性征异常的诊断与治疗
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The Diagnosis and Treatment of Virilizing and Feminizing Adrenal Syndrome
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    目的:探讨肾上腺性性征异常的诊断、鉴别诊断和治疗,特别是分泌性激素的肾上腺肿瘤的良恶性鉴别诊断和各种先天性肾上腺增生疾病的治疗原则.方法:报告1986年~1996年8例先天性肾上腺皮质增生和5例分泌性激素的肾上腺肿瘤的诊治经验.结果:先天性肾上腺皮质增生中有3例17-α羟化酶缺乏症,肿瘤患者包括2例女性男性化、3例男性女性化肾上腺肿瘤.分泌性激素肾上腺肿瘤均经手术切除.结论:分泌性激素的肾上腺皮质肿瘤的重量、直径(DHEA)、尿17酮和血性激素水平、CT表现和肿瘤浸润及转移可作为判断肿瘤良恶性的参考指标.对大体积肾上腺肿瘤首选改良肋缘下切口.先天性肾上腺皮质增生应根据疾病类型而选用不同皮质激素进行治疗,对于17-α羟化酶缺乏的病例.儿童期至青春期不宜应用性激素治疗.

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    Objective: To inquire into diagnosis, and treatment of virilizing and feminizing adrenal syndrome, differential diagnosis between benign and malignant sex hormone producing adrenal neoplasma and, treatment principles of congenital adrenal hyperplasia ( CAH ) . Methods: Eight cases of CAH and 5 cases of sex hormone producing adrenal neoplasma were admitted to hospital during 1986-1996. The former included 3 rare cases of 17 a hydroxylase deficiency. The latter included 3 cases of feminizing adrenal tumor and 2 cases of virilizing adrenal tumor. Results: Weight, size and CT of the tumor, DHEA, 17-ks, sex hormone levels, infiltration, and metastasis were closely related to the degree of differentiation of the tumors. Conclusion: Virilizing and feminizing adrenal neoplasm were removed surgically by different incisions. Modified subcostal incision was recommended as the best choice for huge adrenal mass. Corticoadrenal hormone treatment fo CAH should be individualized according to the different types of the disease. Sex hormones were not suitable for children suffering from 17 hydroxylase deficiency before puberty.

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钱立新,程双管,眭元庚,何戎华,吴宏飞,张炜,李强.肾上腺性性征异常的诊断与治疗[J].南京医科大学学报(自然科学版),2003,17(3):

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