摘要
目的探讨女性假两性畸形的病因、临床特点、诊断和治疗措施。方法收集5例女性假两性畸形患者的临床资料，对其病因、临床特点、诊断和治疗进行讨论。结果5例患者，诊断为外源性雄激素引起的女性假两性畸形1例，先天性肾上腺皮质增生引起的女性假两性畸形4例。均外阴发育异常，染色体核型为46XX。5例行保留阴蒂头及其血管神经蒂阴蒂成形术，4例术后予氢化可的松替代治疗，3个月后促肾上腺皮质激素、睾酮均降至正常。5例随访1～10年，外生殖器外观满意，阴蒂头血运、感觉良好，第二性征明显女性化，3例已基本上转变性别角色，2例尚存在不同程度的心理障碍。结论女性假两性畸形患者应尽早诊断，早期予糖皮质激素替代治疗，早期行阴蒂短缩术，以减轻患者心理负担，提高生活质量。
Objective To discuss the etiology, clinical features, diagnosis and treatment measures of female pseudohermaphroditism. Methods Five cases of female pseudohermaphroditism were collected. The etiology, clinical features, diagnosis and treatment measures were discussed. Results One case induced by exogenous androgenic hormones,4 cases induced by congenital adrenal hyperplasia. All the 5 cases presented with abnormal development of vulva and the sex chromosomes karyotype of 46XX. The combined treatment of reconstructive surgery （5 cases） and supplementary glucocorticoid （4 cases） were performed. After 3 months,the levels of adrenocorticotrophic hormone and virosterone were normal. Followed up for 1-10 years, The postoperative appearance of external genitalia was similar to the normal. The secondary sex characters were improved obviously, 3 cases were mainly feminilism, 2 cases existed different degree of psychologic obstacle. Conclusions The diagnosis and treatment in the early stage are very important. The combination treatment of surgery and medicine for the patients with female pseudohermaphroditism is satisfied.
出处
《中国医师进修杂志》
2010年第12期8-10,共3页
Chinese Journal of Postgraduates of Medicine
