Devroey P, Wisanto A, Camus M, Van Waesberghe L, Bourgain C, Liebaers I, Van Steirteghem A C
Centre for Reproductive Medicine, Universiteit Brussel, Belgium.
Hum Reprod. 1988 Aug;3(6):699-704. doi: 10.1093/oxfordjournals.humrep.a136769.
The clinical, hormonal and cytogenetic findings in 36 women with primary ovarian failure, referred for oocyte or embryo donations are reported. Fifteen women were suffering from ovarian dysgenesis and 11 from premature menopause. Six of these 26 patients showed X-chromosome abnormalities. One patient had a Noonan syndrome. The remaining 10 had surgical menopause. The mean duration of their infertility was 6.5 +/- 3.2 years (+/- SD). All patients had elevated serum gonadotrophins within the menopausal range. Hypothalamic, pituitary and thyroid function were found to be intact. In one of the 15 ovarian biopsies on the patients with chromosomal competent ovarian failure, primordial follicles were found. Hysterosalpingograms revealed a normal uterine cavity in all patients. In view of oocyte donation, careful evaluation of the obstetric risk was mandatory in the six patients with X-chromosome aberrations and in the patient with the Noonan syndrome, because of their short stature and possible concomitant cardiovascular and renal disease. After substitution therapy with oestradiol valerate and natural progesterone, 13 pregnancies were established, seven patients delivered (one set of twins), eight healthy children were born, three pregnancies aborted and three pregnancies are progressing normally.
本文报告了36例因卵母细胞或胚胎捐赠而转诊的原发性卵巢功能衰竭女性的临床、激素和细胞遗传学检查结果。15名女性患有卵巢发育不全，11名患有过早绝经。这26名患者中有6名显示X染色体异常。1名患者患有努南综合征。其余10名患者因手术导致绝经。她们不孕的平均时长为6.5±3.2年（±标准差）。所有患者血清促性腺激素均在绝经范围内升高。下丘脑、垂体和甲状腺功能均正常。在15例染色体正常的卵巢功能衰竭患者的卵巢活检中，有1例发现了原始卵泡。子宫输卵管造影显示所有患者子宫腔正常。鉴于卵母细胞捐赠，对于6例X染色体畸变患者和1例努南综合征患者，由于她们身材矮小且可能伴有心血管和肾脏疾病，必须仔细评估产科风险。在用戊酸雌二醇和天然孕酮替代治疗后，13例患者成功受孕，7例患者分娩（其中1例为双胞胎），8名健康婴儿出生，3例妊娠流产，3例妊娠进展正常。
