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老年血友病患者。

The ageing patient with haemophilia.

作者信息

Canaro Mariana, Goranova-Marinova Veselina, Berntorp Erik

机构信息

Thrombosis and Haemostasis Unit, Son Espases University Hospital, Palma de Mallorca, Spain.

出版信息

Eur J Haematol. 2015 Feb;94 Suppl 77:17-22. doi: 10.1111/ejh.12497.

Abstract

Older patients with haemophilia (PWH) face many challenges related not only to haemophilia but also to general comorbidities associated with ageing. This article discusses the clinical experience published about the high prevalence of diseases in older PWH. These conditions are managed in the general population by healthcare workers with little training in haemophilia. Haemophilic arthropathy is common in elderly PWH. Prophylaxis starting at an early age in sufficient dose regimens to prevent arthropathy did not occur in patients who are now older than around 40 yr. Many PWH above this age thus have limitations in their activities of daily life. Cardiovascular diseases have become increasingly common in the growing, ageing cohort of PWH. Lifestyle issues such as sexual dysfunction may be exacerbated by the medical issues and psychological problems associated with haemophilia. Hepatitis C virus is a leading problem in PWH. Coinfection with HIV accelerates the progression to end-stage liver disease. Acute and chronic renal failure is more common in adult PWH than in general population. Other comorbidities are reviewed. The evidence is scarce, so it is imperative to report any experience regarding the diagnosis and treatment of these entities, to improve the quality of life of older PWH.

摘要

老年血友病患者(PWH)面临诸多挑战,这些挑战不仅与血友病相关,还与衰老相关的一般合并症有关。本文讨论了已发表的关于老年PWH中疾病高患病率的临床经验。在普通人群中,这些疾病由对血友病几乎没有培训的医护人员管理。血友病性关节病在老年PWH中很常见。在现在年龄超过40岁左右的患者中,未在早期以足够剂量方案开始预防关节病的治疗。因此,许多这个年龄以上的PWH在日常生活活动中存在限制。在不断增长的老年PWH队列中,心血管疾病越来越普遍。性功能障碍等生活方式问题可能会因与血友病相关的医学问题和心理问题而加剧。丙型肝炎病毒是PWH中的一个主要问题。与艾滋病毒合并感染会加速进展至终末期肝病。急性和慢性肾衰竭在成年PWH中比在普通人群中更常见。还对其他合并症进行了综述。证据很少,因此必须报告有关这些疾病诊断和治疗的任何经验,以改善老年PWH的生活质量。

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