女性假两性畸形内外科联合治疗经验探讨  

Medical and Surgical Summary Treatment Experience of Female Pseudohermaphroditism

作　　者：崔西春[1] 王志芳[2] 范应中[1] 李泸平[1] 张胜利[1] 姚浩宇[1] 李福凯[1] 王家祥[1] CUI Xi-chun WANG Zhi-fang FAN Ying-zhong et al(Pediatric Surgery Department, the First Affiliated Hospital of Zhengzhou University, Zhengzhou 450052, Chin)

机构地区：[1]郑州大学第一附属医院小儿外科,河南郑州450052 [2]郑州大学第一附属医院内分泌科,河南郑州450052

出　　处：《医学与哲学（B）》2016年第12期37-39,68,共4页Medicine & Philosophy(B)

摘　　要：探讨先天性肾上腺皮质增生症（CAH）致女性假两性畸形的临床诊断及内外科联合治疗方法。回顾性分析2000年9月~2013年12月收治的67例CAH致女性假两性畸形患儿病例资料,就诊年龄1岁3个月~14岁,平均7岁2个月。均经醋酸氢化可的松替代治疗后,根据患者原发病、年龄、社会性别及家长意愿进行性别选择及外科手术治疗,评估其长期治疗效果。67例患儿最终矫治为女性62例,矫治为男性者5例,随访2年~15年,62例矫治为女性者5例失访、3例停用糖皮质激素再次出现不同程度阴蒂肥大,余生长发育良好。5例矫治为男性者2例其身高较同龄人偏矮,余3例进入青春期后加用雄激素后有胡须生长、声音低沉,阴茎牵拉长4cm,但不能勃起。CAH致女性假两性畸形并非罕见疾病,内分泌治疗结合外科矫正手术可以取得较好的疗效。矫治为男性需慎重。To evaluate the medical and surgical treatment of female pseudohermaphroditism caused by congenital adrenalhyperplasia.Retrospective analysis the clinical data of 67 cases of female pseudohermaphroditism caused by congenital adrenalhyperpl-asia in our hospital from Sept.2000 to Dec.2013.Giving glococorticoid replacement therapy.The gender selection and the following surgical treatment were provided according to patients＇ protopathy,age,social gender,their desires and their parents＇ wishes.Out of 67 cases,62cases were operated to women.5cases were operated to man.After 2~15years following up,five of the 62 cases were operated to women of female pseudohermaphrodite missed,3cases clitoridauxe recurrence after withdrawal GC and other groething good.Three of the 5cases were operated to man of female pseudohermaphrodite had already entered adolescent,had given androgens and the effect was good,but astyphia,other 2cases height weaker than their peers.Female Pseudohermaphroditism caused by congenital adrenalhyperplasia is not orphan disease.It should be firstly treated with GC therapy,then giving proper corrective procedure,and keeped lifelong medication to obtain good prognosis.Treatments for men should be careful.

关 键 词：先天性肾上腺皮质增生症 女性假两性畸形 内分泌治疗 外科矫正手术 

分 类 号：R691.1[医药卫生—泌尿科学]