肾上腺性征综合征(附15例报告)  

Adrenogenital syndrome (report of 15 cases)

作　　者：许建业[1] 曹振中 吴春华[1] 韩恩善[3] 

机构地区：[1]宁夏医学院附属医院泌尿外科,银川750004 [2]宁夏青铜峡市人民医院外科,青铜峡751600 [3]宁夏医学院附属医院病理科,银川750004

出　　处：《现代泌尿外科杂志》2005年第6期333-335,共3页Journal of Modern Urology

摘　　要：目的提高对肾上腺性征综合征的认识.方法总结15例肾上腺性征综合征患者的临床资料,其中先天性肾上腺皮质增生13例,肾上腺皮质癌2例.皮质激素替代治疗9例,手术治疗6例(皮质激素加阴蒂切除4例,肾上腺癌根治2例).结果所有患者随访6个月至5年.皮质激素替代治疗9例中,5例婴儿出院后死亡,4例女童身高发育正常.手术治疗6例中,2例皮质癌术后1～2年肿瘤复发、恶病质死亡,其余4例先天性肾上腺皮质增生者身高及第二性征发育正常,其中3例青春期后有月经产生.结论对儿童先天性肾上腺皮质增生所致的性征异常需尽早使用糖皮质激素替代治疗,视年龄及外生殖器畸形程度予以手术矫正,肾上腺皮质癌所致的性征异常需根据患者全身情况决定治疗方法.Objective To investigate adrenogenital syndrome. Methods The clinical data of 15 patients were summarized including 13 cases of congenital adrenal hyperplasia （CAH）, and 2 cases of adrenocortical carcinoma. 9 cases were treated with hormonal replacement and 6 with surgery （4 with hormonal replacement after resection of clitoris with oral deltacortelan, and 2 with radical operation of adrenocortical carcinoma）. Results All patients were followed up for 1 to 5 years. Of the 9 cases treated with hormonal replacement, 5 infants died after leaving hospital, other 4 female babies were normal in physical built. Of the 6 cases with surgical treatment, 2 had adrenocortical carcinoma and died after 1 to 2 years of operation, and the rest 4 cases with CAH were normal in physical built and sex characteristics, of whom 3 had menstruation at puberty. Conclusion Hormonal replacement treatment should be carried out as soon as possible when child suffers from CAH. Considering the condition of patients external genital or vulva malformation and age, orthomorphia should be performed. The treatment should be determined by the individual condition.

关 键 词：肾上腺疾病 性征 

分 类 号：R586.22[医药卫生—内分泌]