Goswami Deepti, Conway Gerard S
Department of Endocrinology, The Middlesex Hospital, London W1T 3AA, UK.
Hum Reprod Update. 2005 Jul-Aug;11(4):391-410. doi: 10.1093/humupd/dmi012. Epub 2005 May 26.
Premature ovarian failure (POF) causing hypergonadotrophic hypogonadism occurs in 1% of women. In majority of cases the underlying cause is not identified. The known causes include: (a) Genetic aberrations, which could involve the X chromosome or autosomes. A large number of genes have been screened as candidates for causing POF; however, few clear causal mutations have been identified. (b) Autoimmune ovarian damage, as suggested by the observed association of POF with other autoimmune disorders. Anti-ovarian antibodies are reported in POF by several studies, but their specificity and pathogenic role are questionable. (c) Iatrogenic following surgical, radiotherapeutic or chemotherapeutic interventions as in malignancies. (d) Environmental factors like viral infections and toxins for whom no clear mechanism is known. The diagnosis is based on finding of amenorrhoea before age 40 associated with FSH levels in the menopausal range. Screening for associated autoimmune disorders and karyotyping, particularly in early onset disease, constitute part of the diagnostic work-up. There is no role of ovarian biopsy or ultrasound in making the diagnosis. Management essentially involves hormone replacement and infertility treatment, the only proven means for the latter being assisted conception with donated oocytes. Embryo cryopreservation, ovarian tissue cryopreservation and oocyte cryopreservation hold promise in cases where ovarian failure is foreseeable as in women undergoing cancer treatments.
卵巢早衰(POF)导致高促性腺激素性性腺功能减退,在1%的女性中出现。在大多数情况下,潜在病因不明。已知病因包括:(a)基因畸变,可能涉及X染色体或常染色体。大量基因已被筛选为可能导致POF的候选基因;然而,已确定的明确致病突变很少。(b)自身免疫性卵巢损伤,POF与其他自身免疫性疾病的关联提示了这一点。多项研究报道POF患者存在抗卵巢抗体,但其特异性和致病作用存在疑问。(c)恶性肿瘤等手术、放疗或化疗干预后的医源性因素。(d)病毒感染和毒素等环境因素,其确切机制尚不清楚。诊断基于40岁前出现闭经且FSH水平处于绝经范围。筛查相关自身免疫性疾病和进行核型分析,特别是在早发性疾病中,是诊断检查的一部分。卵巢活检或超声检查对诊断无帮助。治疗主要包括激素替代和不孕症治疗,后者唯一经证实的方法是使用捐赠卵母细胞进行辅助受孕。在可预见卵巢衰竭的情况下,如接受癌症治疗的女性,胚胎冷冻保存、卵巢组织冷冻保存和卵母细胞冷冻保存具有前景。