CAJ | 학술논문

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.
목적 탐토녀성가량성기형적병인、림상특점、진단화치료조시.방법 수집5례녀성가량성기형환자적림상자료,대기병인、림상특점、진단화치료진행토론.결과 5례환자,진단위외원성웅격소인기적녀성가량성기형1례,선천성신상선피질증생인기적녀성가량성기형4례.균외음발육이상,염색체핵형위46XX.5례행보류음체두급기혈관신경체음체성형술,4례술후여경화가적송체대치료,3개월후촉신상선피질격소、고동균강지정상.5례수방1～10년,외생식기외관만의,음체두혈운、감각량호,제이성정명현녀성화,3례이기본상전변성별각색,2례상존재불동정도적심리장애.결론 녀성가량성기형환자응진조진단,조기여당피질격소체대치료,조기행음체단축술,이감경환자심리부담,제고생활질량.
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 ofreconstructive surgery (5 cases)and supplementary glucocorticoid (4 cases)wefe performed.After 3months,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.