肾上腺性征异常症的诊治（附16例报告）

Diagnosis and treatment of adrenogenital syndrome （report of 16 cases）

目的探讨肾上腺性征异常症的诊断和治疗。方法总结1999年2月-2008年3月收治16例肾上腺性征异常症患儿的临床资料，患者均为女性，年龄5～16岁，平均10．2岁。其中先天性肾上腺皮质增生（CAH）11例，肾上腺腺瘤4例，肾上腺皮质癌1例，CAH组均利用会阴皮瓣行阴蒂和阴道成形术，术后皮质激素替代治疗。5例男性化肾上腺肿瘤患者均手术切除肿瘤。结果11例CAH患者身高及第二性征发育正常，其中5例青春期后月经来潮，1例外阴成形术后出现阴道狭窄。男性化肾上腺肿瘤组术后男性化体征改善，其中4例肾上腺腺瘤随访2-4年无复发，1例肾上腺皮质癌患者术后1年内复发死亡。结论CAH所致的性征异常症应尽早使用糖皮质激素替代治疗，并行相应的会阴部成形术。及早发现并切除肿瘤是治疗皮质肿瘤导致肾上腺性征异常症的关键。腺瘤效果好，皮质癌预后差。

Objective To discuss the diagnosis and treatment of adrenogenital syndrome. Methods 16 cases of adrenogenital syndrome from 1999. 2 to 2008.3 were reviewed. They were all female, in the age range of 5 to 16 and the average is 10. 2. 11 cases were diagnosed CAH, 4 cases adrenal adenoma, 1 case adencarcinoma. CAH cases all underwent clitoroplasty and vaginoplasty with perineal skin flap. After operation all the patients were treated withhormonal replacement. The adrenal tumors were resected in the 5 virilizing adrenocortical tumor patients. Results the 11 patients with CAH were normal in physical built and sex characteristics, of whom 5had menstruation at puberty. I patient found vaginal stenosis after vaginoplasty. The virilizng signs were improved after resection of the adrenal tumors. No recurrencehappened in 4 cases of adrenal adenoma in 1-4 years follow-up visit. I case adencarcinoma were died of relapses of the tumor in 1 year after operation. Conclusionshormonal replacement treatment should be carried out as soon as possible when child suffers from CAH, and appropriate perineoplasty should be performed. Early detection and resection of tumor is the key point for treating adrenogenital syndrome caused by cortex tumor. The prognosis of adrenal adenoma is good, except adencarcinoma.