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目的探讨女性假两性畸形的诊断和治疗的时机及方法。方法回顾性分析近23年来收治的31例患儿的诊疗资料。1～6岁19例,7～14岁12例。性染色体均为46,XX。腕骨骨龄大于实际年龄的18例。出生后均见阴蒂肥大,27例为尿生殖窦单一开口,4例分别见到尿道和阴道口,半数已长出阴毛、声粗、痤疮,最小者2岁,3例14岁患儿身高不足150cm。行B超检查及尿生殖窦造影的病例均有正常女性生殖道。失盐型3例,在新生儿期出现危象而确诊。结果剖腹探查的9例有正常卵巢及内生殖道。26例作了阴蒂缩短成形或肥大阴蒂切除,小阴唇成形。除1例外,其余30例均在确诊后接受内科药物治疗。结论典型的女性肾上腺性征异常诊断虽不难,但就诊年龄多较晚,药物治疗滞后,对患儿的生理和心理有严重的负面影响。典型患儿不必作剖腹探查。失盐型患儿多在新生儿期出现危象,应尽早诊疗。
Objective To investigate the timing and methods of diagnosis and treatment of female pseudohermaphroditism. Methods Retrospective analysis of diagnosis and treatment of 31 cases of children admitted to the past 23 years. 19 cases of 1 ~ 6 years old, 12 cases of 7 ~ 14 years old. Sex chromosomes are 46, XX. Carpal bone age greater than the actual age of 18 cases. After birth are seen clitoral hypertrophy, 27 cases of urogenital sinus a single opening, 4 cases were seen urethra and vaginal mouth, half have grown pubic hair, rough voice, acne, the smallest of 2 years old, 3 cases of 14-year-old children underweight 150cm. Line B ultrasound and urine reproductive sinus imaging cases have normal female genital tract. Loss of salt in 3 cases, the emergence of crisis in the neonatal diagnosis. Results 9 cases of laparotomy have normal ovarian and endogenous. Twenty-six patients underwent clitoral shortening or clitoris excision and labia minora forming. In addition to one exception, the remaining 30 patients were diagnosed after receiving medical treatment. Conclusion Although it is not difficult to diagnose the abnormalities of adrenal glands in typical female patients, the treatment ages are late and the drug treatment lags behind, which has a serious negative impact on children ’s physiology and psychology. Typical children do not have to do laparotomy. Loss of salt-prone children in the neonatal crisis, early diagnosis and treatment.