發(fā)布時(shí)間：2018-07-30 06:58

【摘要】：目的總結(jié)卵睪型性發(fā)育異常的臨床特點(diǎn)及診治經(jīng)驗(yàn)。方法回顧性分析1995年1月至2014年6月就診于解放軍總醫(yī)院院并經(jīng)手術(shù)證實(shí)的14例卵睪型性發(fā)育異常患者臨床資料和隨訪資料。結(jié)果社會(huì)性別:男性8例,女性6例;外生殖器表型:"女性"表現(xiàn)為陰蒂肥大3例,小陰莖2例,陰莖型尿道下裂1例;"男性"表現(xiàn)為尿道下裂,其中陰莖頭型2例,會(huì)陰型2例,陰莖型1例,陰囊型2例,陰莖短小1例。染色體核型分析:46,XX 9例,其中7例SRY基因陰性,46,XY 4例,46,XY/45,XO 1例;10例患兒睪酮水平低,3例青春期乳房發(fā)育者雌二醇、黃體生成素、卵泡刺激素水平在正常范圍;性腺畸形:雙側(cè)型3例,單側(cè)型6例,片側(cè)型5例。所有患者均切除不適當(dāng)?shù)男韵俳M織,4例青春期齡患者中2例予雌激素替代治療,2例予雄激素替代治療。7例患者進(jìn)行3個(gè)月至10年的隨訪,按男性撫養(yǎng)者4例,1例尚未進(jìn)入青春期,2例青春期發(fā)育正常,1例反復(fù)發(fā)生尿道狹窄;按女性撫養(yǎng)者3例,1例予雌激素替代治療后獲得青春期發(fā)育,1例處于青春期發(fā)育正常,1例發(fā)生陰道狹窄。結(jié)論早期診斷,根據(jù)內(nèi)、外生殖器優(yōu)勢(shì)選擇合適的性別,并行選擇性性腺手術(shù),對(duì)于維持患者正常的性生理、性心理及社會(huì)生活具有重要的意義。長(zhǎng)期隨訪對(duì)于解決患者在術(shù)后所遇到的心理、泌尿生殖系統(tǒng)問(wèn)題是必不可少的。
[Abstract]:Objective to summarize the clinical features, diagnosis and treatment of otorchidism dysplasia. Methods the clinical data and follow-up data of 14 patients with otorchidism dysplasia admitted to PLA General Hospital from January 1995 to June 2014 were retrospectively analyzed. Results gender: male 8 cases, female 6 cases; external genitalia phenotype: 3 cases of clitoris hypertrophy, 2 cases of small penis, 1 case of penile hypospadias, 2 cases of "male" hypospadias, 2 cases of penile head type. There were 2 cases of perineal type, 1 case of penile type, 2 cases of scrotal type and 1 case of short penis. Chromosomal karyotype analysis was performed in 9 cases, of which 7 cases were negative for SRY gene, 4 cases had low testosterone level in 10 cases and 10 cases had low testosterone level. The levels of estradiol, luteinizing hormone and follicle stimulating hormone were in normal range in 3 cases of pubertal breast development. There were 3 cases of bilateral type, 6 cases of unilateral type and 5 cases of lamellar type. All patients were treated with estrogen replacement therapy (EST) and androgen replacement therapy (androgen replacement therapy) for 3 months to 10 years. Urethral stricture occurred repeatedly in 4 male dependents, 1 case was not in puberty and 2 cases had normal puberty development. According to 3 female dependents, 1 case received estrogen replacement therapy, 1 case had normal puberty development and 1 case had vaginal stenosis. Conclusion early diagnosis, selection of suitable sex according to internal and external genitalia advantages, and selective gonadal surgery are of great significance in maintaining normal sexual physiology, sexual psychology and social life of patients. Long-term follow-up is essential for resolving the psychological and urogenital problems encountered by patients after surgery.
【作者單位】： 解放軍總醫(yī)院內(nèi)分泌科;
【分類號(hào)】：R711.1

【參考文獻(xiàn)】

相關(guān)期刊論文 前1條

1 黃魯剛,黃斌,王明和,陳紹基;兒童真兩性畸形診斷與治療:附9例報(bào)告[J];臨床兒科雜志;2004年06期

【共引文獻(xiàn)】

相關(guān)期刊論文 前2條

1 李永忠;溫曉玲;黃魯剛;邱邐;文曉蓉;莊華;;超聲診斷性別畸形的臨床價(jià)值[J];四川大學(xué)學(xué)報(bào)(醫(yī)學(xué)版);2007年06期

2 李瑾;付瑞鋒;陳珂;徐s，

本文編號(hào)：2154210