The need for speed in the management of haemophilia patients with inhibitors

被引:23
作者
Salek, S. Z. [1 ]
Benson, G. M. [2 ]
Elezovic, I. [3 ]
Krenn, V. [4 ]
Ljung, R. C. R. [5 ]
Morfini, M. [6 ]
Remor, E. [7 ]
Santagostino, E. [8 ]
Sorensen, B. [9 ,10 ]
机构
[1] Univ Hosp Ctr Zagreb, Natl Haemophilia Ctr, Zagreb 10000, Croatia
[2] No Ireland Haemophilia Comprehens Care Ctr, Belfast, Antrim, North Ireland
[3] Univ Belgrade, Sch Med, Clin Ctr Serbia, Inst Haematol, Belgrade, Serbia
[4] Ctr Histol Zytol & Mol Diagnost, Trier, Germany
[5] Malmo Univ Hosp, Dept Paediat & Coagulat Disorders, Malmo, Sweden
[6] Azienda Osped Univ Careggi, Dept Emergency & Recept, Reference Ctr Inherited Bleeding Disorders Tuscan, Agcy Hemophilia, Florence, Italy
[7] Univ Autonoma Madrid, Fac Psychol, Dept Psychobiol & Hlth, Madrid, Spain
[8] Univ Milan, Mangiagalli & Regina Elena Fdn, IRCCS Maggiore Hosp, Angelo Bianchi Bonomi Haemophilia & Thrombosis Ct, Milan, Italy
[9] Guys & St Thomas NHS Fdn Trust, Ctr Haemophilia & Thrombosis, Haemostasis Res Unit, London, England
[10] Kings Coll London, Sch Med, London WC2R 2LS, England
关键词
arthropathy; haemophilia; inhibitors; muscle bleed; quality of life; recombinant activated factor VII; RECOMBINANT FACTOR VIIA; QUALITY-OF-LIFE; HOME TREATMENT; CARTILAGE; HEMARTHROSES; EXPRESSION; SPORTS; OSTEOARTHRITIS; HEMATOMAS; NOVOSEVEN;
D O I
10.1111/j.1365-2516.2010.02265.x
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
Rapid control of bleeding is the key to reducing bleeding complications and thereby preserving joint and musculoskeletal function in haemophilia patients with inhibitors. However, this requires early diagnosis following the onset of bleeding and strategies for rapid treatment in an outpatient setting. Overarching themes on the need for speed in managing bleeds in haemophilia patients were examined by a panel of clinicians experienced in managing inhibitor patients and joint disease during the Third Zurich Haemophilia Forum on 8 May 2009. This report summarizes the opinions of the panel on how to achieve rapid bleeding control in inhibitor patients and areas that were identified by the panel for future research or as needing new consensus guidelines. The consensus was that home treatment should be established for haemophilia patients with inhibitors, as it is associated with a faster time to treatment, as well as improvements in the quality of life of patients and their carers. In addition, as improved haemostatic control now allows inhibitor patients to participate in a wider range of physical activities, specific guidelines are required on which types of sport and work are appropriate. It was agreed that clear, systematic approaches are needed for early diagnosis of joint and muscle bleeds in inhibitor patients, which could facilitate rapid treatment. There may be opportunities for exploiting new diagnostic techniques from osteoarthritis to enable earlier diagnosis of haemophilic arthropathy. Overall, it was concluded that greater emphasis should be placed on education and patients' psychological needs, to enable inhibitor patients to cope up more effectively with their disease.
引用
收藏
页码:95 / 102
页数:8
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