Seremetis S V
Kravis Women's and Children's Center, Mount Sinai Medical Center, Women's Health Program, 5 E 98th St, 5th Floor, Box 1521, New York, NY 10029, USA.
J Gend Specif Med. 2001;4(2):59-64.
Disorders of thrombosis and hemostasis represent both diagnostic and therapeutic challenges for the clinician, in part because of sex-based differences in incidence and presentation. The hemophilias are characterized by specific sex-linked patterns of inheritance, and there are sex differences in the presentation of the autosomally inherited disorders, particularly von Willebrand's disease. The diagnosis of these disorders can be affected by variations in either endogenous or exogenous estrogens, and the hemostatic stresses presented by menstruation and childbirth render any coagulopathy more severe in females than in males. Women are also at increased risk for developing thrombotic and embolic problems while on exogenous estrogens and during pregnancy. This article presents recommendations about the most appropriate and cost-effective ways to screen for the inherited disorders of both thrombosis and hemostasis in men and women. Recommendations are also developed for the treatment of women with these disorders, particularly in the context of pregnancy, contraception, uterine bleeding, and postmenopausal management.
血栓形成和止血障碍给临床医生带来了诊断和治疗方面的挑战,部分原因是发病率和临床表现存在性别差异。血友病具有特定的性连锁遗传模式,常染色体显性遗传疾病的表现也存在性别差异,尤其是血管性血友病。这些疾病的诊断可能会受到内源性或外源性雌激素变化的影响,月经和分娩带来的止血压力使女性的任何凝血病都比男性更严重。女性在使用外源性雌激素期间以及怀孕期间发生血栓形成和栓塞问题的风险也会增加。本文提出了关于筛查男性和女性血栓形成和止血遗传性疾病的最合适且最具成本效益方法的建议。还针对患有这些疾病的女性的治疗制定了建议,特别是在妊娠、避孕、子宫出血和绝经后管理的背景下。