Women's and Newborn Program, Monash Health, Clayton, Victoria, Australia.
Department of Obstetrics and Gynaecology, Monash University, Clayton, Victoria, Australia.
Prenat Diagn. 2023 Feb;43(2):261-271. doi: 10.1002/pd.6261. Epub 2022 Nov 12.
Turner syndrome (TS), a common chromosomal abnormality affecting females, is associated with partial or complete loss of the second sex chromosome. Although the classic karyotype is 45, X, the detection of mosaic TS is increasing. TS is a multi-system disorder with significant endocrine, cardiovascular and reproductive impacts. Accelerated ovarian follicular loss leads to primary amenorrhoea or premature ovarian insufficiency and infertility. Early diagnosis and counselling regarding hormone replacement therapy and future reproductive capacity, including fertility preservation, are essential to improve reproductive outcomes. Pubertal induction or estrogen replacement is usually required to optimise long-term health outcomes; however, initiation may be delayed due to delayed diagnosis. Spontaneous pregnancy occurs in a small number of women; however, many require donor oocytes and assisted reproductive technology to achieve a pregnancy. Pregnancy is a high risk especially when associated with congenital heart disease. Prepregnancy counselling by the multidisciplinary team (MDT) to identify contraindications and optimise pre-existing health issues is essential. Pregnancy management should be led by a maternal-fetal medicine unit with input from the MDT. This review examines reproductive health outcomes in women with TS and how best to manage them to reduce health risks and improve maternal and neonatal outcomes.
特纳综合征(TS)是一种常见的女性染色体异常疾病,与第二性染色体的部分或完全缺失有关。虽然经典核型为 45,X,但嵌合体 TS 的检出率正在增加。TS 是一种多系统疾病,对内分泌、心血管和生殖系统有重大影响。卵巢卵泡加速丢失导致原发性闭经或卵巢早衰和不孕。早期诊断和咨询激素替代治疗和未来生育能力,包括生育力保存,对于改善生育结局至关重要。青春期诱导或雌激素替代通常需要优化长期健康结局;然而,由于诊断延迟,启动可能会延迟。少数妇女会自然怀孕;然而,许多妇女需要供体卵和辅助生殖技术来实现妊娠。妊娠风险很高,尤其是伴有先天性心脏病时。多学科团队(MDT)进行孕前咨询以识别禁忌症和优化现有健康问题至关重要。妊娠管理应由母体胎儿医学单位主导,并由 MDT 提供意见。本文综述了 TS 妇女的生殖健康结局,以及如何最好地管理这些问题,以降低健康风险,改善母婴结局。