• 文献检索
  • 文档翻译
  • 深度研究
  • 学术资讯
  • Suppr Zotero 插件Zotero 插件
  • 邀请有礼
  • 套餐&价格
  • 历史记录
应用&插件
Suppr Zotero 插件Zotero 插件浏览器插件Mac 客户端Windows 客户端微信小程序
定价
高级版会员购买积分包购买API积分包
服务
文献检索文档翻译深度研究API 文档MCP 服务
关于我们
关于 Suppr公司介绍联系我们用户协议隐私条款
关注我们

Suppr 超能文献

核心技术专利:CN118964589B侵权必究
粤ICP备2023148730 号-1Suppr @ 2026

文献检索

告别复杂PubMed语法,用中文像聊天一样搜索,搜遍4000万医学文献。AI智能推荐,让科研检索更轻松。

立即免费搜索

文件翻译

保留排版,准确专业,支持PDF/Word/PPT等文件格式,支持 12+语言互译。

免费翻译文档

深度研究

AI帮你快速写综述,25分钟生成高质量综述,智能提取关键信息,辅助科研写作。

立即免费体验

相似文献

1
Diagnosis and care of patients with mild haemophilia: practical recommendations for clinical management.轻度血友病患者的诊断和护理:临床管理的实用建议。
Blood Transfus. 2018 Nov;16(6):535-544. doi: 10.2450/2017.0150-17. Epub 2017 Nov 14.
2
Mild haemophilia: a disease with many faces and many unexpected pitfalls.轻度血友病:一种具有多种表现形式和许多意外陷阱的疾病。
Haemophilia. 2010 Jul;16 Suppl 5:100-6. doi: 10.1111/j.1365-2516.2010.02306.x.
3
Haemophilia and inhibitors. 1: Diagnosis and treatment.血友病与抑制物。1:诊断与治疗。
Nurs Times. 2008;104(26):26-7.
4
Cost-effectiveness of recombinant activated factor VII vs. plasma-derived activated prothrombin complex concentrate in the treatment of mild-to-moderate bleeding episodes in patients with severe haemophilia A and inhibitors in Spain.在西班牙,重组活化因子 VII 与血浆源性活化凝血酶原复合物浓缩物治疗重型血友病 A 伴抑制物患者轻至中度出血发作的成本效益比较。
Haemophilia. 2013 Nov;19(6):841-6. doi: 10.1111/hae.12199. Epub 2013 Jun 11.
5
Successful eradication of a FVIII inhibitor in a 60-year-old patient with mild haemophilia A using single-agent prednisolone.成功使用单药泼尼松龙治疗 60 岁轻度血友病 A 伴 FVIII 抑制剂患者。
Hamostaseologie. 2012;32 Suppl 1:S48-51.
6
The perspective of patients with haemophilia with inhibitors and their care givers: preferences for treatment characteristics.伴有抑制剂的血友病患者及其照护者的观点:对治疗特征的偏好。
Haemophilia. 2011 May;17(3):476-82. doi: 10.1111/j.1365-2516.2010.02401.x. Epub 2010 Nov 23.
7
Tranexamic acid as adjunct therapy to bypassing agents in haemophilia A patients with inhibitors.氨甲环酸作为旁路制剂的辅助疗法用于治疗有抑制物的甲型血友病患者。
Haemophilia. 2014 May;20(3):369-75. doi: 10.1111/hae.12318. Epub 2013 Nov 19.
8
Inhibitor incidence after intensive FVIII replacement for surgery in mild and moderate haemophilia A: a prospective national study in the Netherlands.在荷兰进行的一项前瞻性全国研究:重度和中度血友病 A 患者手术强化 FVIII 替代治疗后的抑制剂发生率。
Br J Haematol. 2012 Jun;157(6):747-52. doi: 10.1111/j.1365-2141.2012.09119.x. Epub 2012 Apr 26.
9
Risk of inhibitor development in mild haemophilia A increases with age.轻型血友病 A 患者的抑制剂发生风险随年龄增长而增加。
Haemophilia. 2012 Mar;18(2):263-7. doi: 10.1111/j.1365-2516.2011.02629.x. Epub 2011 Aug 19.
10
Acquired haemophilia: management of bleeds and immune therapy to eradicate autoantibodies.获得性血友病:出血的管理及根除自身抗体的免疫治疗
Haemophilia. 2005 Sep;11(5):510-5. doi: 10.1111/j.1365-2516.2005.01136.x.

引用本文的文献

1
A Systematic Review of Modelling Approaches in Economic Evaluations of Treatments for Inherited Bleeding Disorders.遗传性出血性疾病治疗经济评估中建模方法的系统评价
Appl Health Econ Health Policy. 2025 Aug 31. doi: 10.1007/s40258-025-00996-3.
2
Experiences and Needs Regarding Information Provision in Children With Haemophilia: A Qualitative Study on Caregivers' and Healthcare Providers' Perspectives.血友病患儿信息提供方面的经验与需求:一项关于照顾者和医疗服务提供者观点的定性研究
Haemophilia. 2025 Jul;31(4):657-667. doi: 10.1111/hae.70063. Epub 2025 May 19.
3
Multidisciplinary management of a significant quadriceps haematoma in an adolescent with mild haemophilia.一名患有轻度血友病的青少年股四头肌严重血肿的多学科管理。
BMJ Case Rep. 2025 Apr 17;18(4):e263253. doi: 10.1136/bcr-2024-263253.
4
Extending health equity to people with moderate and mild hemophilia A: revisiting the HAVEN 6 trial.将健康公平扩展至中度和轻度甲型血友病患者:重新审视HAVEN 6试验。
Res Pract Thromb Haemost. 2024 Nov 29;9(1):102648. doi: 10.1016/j.rpth.2024.102648. eCollection 2025 Jan.
5
Design of an international, phase IV, open-label study of simoctocog alfa in women/girls with hemophilia A undergoing surgery (NuDIMENSION).一项关于simoctocog alfa在接受手术的甲型血友病女性/女孩中的国际IV期开放标签研究(NuDIMENSION)的设计。
Ther Adv Hematol. 2024 Dec 1;15:20406207241300040. doi: 10.1177/20406207241300040. eCollection 2024.
6
Exercise-based telerehabilitation for the management of chronic pain in people with severe haemophilia: a mixed-methods feasibility study.基于运动的远程康复用于重度血友病患者慢性疼痛的管理:一项混合方法可行性研究
Pilot Feasibility Stud. 2024 Oct 8;10(1):128. doi: 10.1186/s40814-024-01550-z.
7
Prevalence of Hemophilia and Clinicodemographic Characteristics of Hemophilic Patients Aged ≤ 18 Years in Thi-Qar, Iraq.伊拉克济加尔省18岁及以下血友病患者的患病率及临床人口统计学特征
Glob Pediatr Health. 2024 Sep 13;11:2333794X241280119. doi: 10.1177/2333794X241280119. eCollection 2024.
8
Anesthetic considerations for joint replacement surgery in hemophilic arthropathy: a comprehensive review.血友病性关节病关节置换手术的麻醉考量:综述
Anesth Pain Med (Seoul). 2024 Jul;19(3):194-208. doi: 10.17085/apm.24047. Epub 2024 Jul 23.
9
Nonsevere Hemophilia: The Need for a Renewed Focus and Improved Outcomes.非重度血友病:需要重新聚焦并改善治疗结果。
Semin Thromb Hemost. 2025 Feb;51(1):58-67. doi: 10.1055/s-0044-1786358. Epub 2024 May 11.
10
Risk of Intracranial Hemorrhage in Persons with Hemophilia A in the United States: Real-World Retrospective Cohort Study Using the ATHNdataset.美国甲型血友病患者颅内出血风险:使用ATHN数据集的真实世界回顾性队列研究
J Blood Med. 2024 Apr 24;15:191-205. doi: 10.2147/JBM.S443380. eCollection 2024.

本文引用的文献

1
Haemophilia and joint disease: pathophysiology, evaluation, and management.血友病与关节疾病:病理生理学、评估及管理
J Comorb. 2011 Dec 27;1:51-59. doi: 10.15256/joc.2011.1.2. eCollection 2011.
2
Comorbidities of cardiovascular disease and cancer in hemophilia patients.血友病患者的心血管疾病和癌症合并症。
Thromb J. 2016 Oct 4;14(Suppl 1):34. doi: 10.1186/s12959-016-0097-x. eCollection 2016.
3
WFH 2016 World Congress Abstracts, Orlando, Florida, USA, July 24-28, 2016.
Haemophilia. 2016 Jul;22 Suppl 4:3-138. doi: 10.1111/hae.12980.
4
Joining the patient on the path to customized prophylaxis: one hemophilia team explores the tools of engagement.与患者一同踏上定制预防之路:一个血友病治疗团队探索参与工具。
J Multidiscip Healthc. 2015 Dec 7;8:527-34. doi: 10.2147/JMDH.S93579. eCollection 2015.
5
A critical appraisal of one-stage and chromogenic assays of factor VIII activity.关于因子 VIII 活性的一步法和显色法检测的批判性评价。
J Thromb Haemost. 2016 Feb;14(2):248-61. doi: 10.1111/jth.13215. Epub 2016 Feb 1.
6
The incidence and treatment of bleeding episodes in non-severe haemophilia A patients with inhibitors.非重型血友病 A 伴抑制物患者出血事件的发生率和治疗。
Thromb Haemost. 2016 Mar;115(3):543-50. doi: 10.1160/TH15-03-0212. Epub 2015 Nov 19.
7
Haemophilia A carriers experience reduced health-related quality of life.甲型血友病携带者的健康相关生活质量下降。
Haemophilia. 2015 Nov;21(6):761-5. doi: 10.1111/hae.12690. Epub 2015 Apr 30.
8
Inhibitors in nonsevere haemophilia A: outcome and eradication strategies.非重型血友病 A 中的抑制剂:结局和清除策略。
Thromb Haemost. 2015 Jul;114(1):46-55. doi: 10.1160/TH14-11-0940. Epub 2015 Apr 16.
9
Hemophilia B: molecular pathogenesis and mutation analysis.血友病B:分子发病机制与突变分析
J Thromb Haemost. 2015 Jul;13(7):1184-95. doi: 10.1111/jth.12958. Epub 2015 May 18.
10
A cross-sectional study of bleeding phenotype in haemophilia A carriers.甲型血友病携带者出血表型的横断面研究。
Br J Haematol. 2015 Jul;170(2):223-8. doi: 10.1111/bjh.13423. Epub 2015 Apr 1.

轻度血友病患者的诊断和护理:临床管理的实用建议。

Diagnosis and care of patients with mild haemophilia: practical recommendations for clinical management.

机构信息

Haemophilia and Thrombosis Centre, Belfast City Hospital, Belfast, Northern Ireland, United Kingdom.

Klinikum Bremen-Mitte, Professor Hess Children's Hospital, Bremen, Germany.

出版信息

Blood Transfus. 2018 Nov;16(6):535-544. doi: 10.2450/2017.0150-17. Epub 2017 Nov 14.

DOI:10.2450/2017.0150-17
PMID:29328905
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC6214819/
Abstract

Mild haemophilia is defined by factor levels between 0.05 and 0.40 IU/mL and is characterised by traumatic bleeds. Major issues associated with mild haemophilia are that it may not present for many years after birth, and that awareness, even within families, may be low. Methodological problems exist in diagnosis, such as inconsistencies in results obtained from different assays used to measure factor levels in mild haemophilia. Advances in genetic testing provide insight into diagnosis as well as the likelihood of inhibitor development, which is not uncommon in patients with mild or moderate haemophilia and can increase morbidity. The management of patients with mild haemophilia is a challenge. This review includes suggestions around formulating treatment plans for these patients, encompassing the full spectrum from clinical care of the newly diagnosed neonate to that of the ageing patient with multiple comorbidities. Management strategies consider not only the vast differences in these patients' needs, but also risks of inhibitor development and approaches to optimally engage patients.

摘要

轻度血友病的定义为因子水平在 0.05 至 0.40 IU/mL 之间,其特征是创伤性出血。与轻度血友病相关的主要问题是,它可能在出生后多年才出现,甚至在家庭内部,意识也可能较低。在诊断方面存在方法学问题,例如用于测量轻度血友病因子水平的不同检测方法获得的结果不一致。基因检测的进步为诊断以及抑制剂发展的可能性提供了深入了解,而抑制剂在轻度或中度血友病患者中并不罕见,并且会增加发病率。轻度血友病患者的管理是一个挑战。本综述包括了针对这些患者制定治疗计划的建议,涵盖了从新诊断的新生儿的临床护理到患有多种合并症的老年患者的全面范围。管理策略不仅考虑了这些患者需求的巨大差异,还考虑了抑制剂发展的风险以及使患者最佳参与的方法。