Literature DB >> 19125934

Haemophilia care then, now and in the future.

J Oldenburg1, G Dolan, G Lemm.   

Abstract

Epidemiological data show the benefits of dramatically improved haemophilia care in all life-stages. There are improved administration techniques and dosing regimens, a shift from on-demand treatment to prophylaxis, successful treatment protocols for immune tolerance induction in patients with inhibitors and enhanced approaches to overall patient management. Improvements also include the introduction of virus inactivation methods for plasma derived clotting factor concentrates and the development of recombinant factor VIII therapy, which practically eliminated the risk of infectious disease transmission. Recombinant factor concentrates are recommended as treatment of choice by several guidelines today. All these developments have resulted in increased health-related quality of life and life expectancy in haemophilia patients, who are transitioning from childhood to adulthood with healthy joints and an overall healthy status today. Because of increased life expectancy, these patients are expected to experience age-related clinical problems that were not previously observed in this population. With respect to this, the spectrum of haemophilia care will be extended to diseases of older ages with the need of including further disciplines in comprehensive haemophilia care programmes. Despite these advances, the short half-life of factor VIII, requiring re-administration every 2 or 3 days and the development of inhibitors remains a challenge. Bayer's research and development currently focuses on the optimization of recombinant coagulation factors to address these challenges. Haemophilia care has experienced significant improvements within the past decades. Novel technologies and continued clinical research have facilitated the development of treatment regimen that resulted in dramatic increases in the life expectancy and quality of life of haemophilia patients. To set the scene for the following papers dealing with haemophilia care from paediatrics to geriatrics, developments behind these improvements and some aspects of future research will be presented in this paper.

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Year:  2009        PMID: 19125934     DOI: 10.1111/j.1365-2516.2008.01946.x

Source DB:  PubMed          Journal:  Haemophilia        ISSN: 1351-8216            Impact factor:   4.287


  15 in total

1.  The role of disease severity in influencing body mass index in people with haemophilia: a single-institutional cross-sectional study.

Authors:  M McNamara; A Antun; C L Kempton
Journal:  Haemophilia       Date:  2013-10-14       Impact factor: 4.287

Review 2.  The history and evolution of the clinical effectiveness of haemophilia type a treatment: a systematic review.

Authors:  Hector E Castro; María Fernanda Briceño; Claudia P Casas; Juan David Rueda
Journal:  Indian J Hematol Blood Transfus       Date:  2012-11-04       Impact factor: 0.900

3.  Perioperative safety and hemostatic efficacy of Advate® in patients with hemophilia A in a postmarketing surveillance in Japan.

Authors:  Keiji Nogami; Hideyuki Takedani; Midori Shima; Akira Yoshioka; Tadashi Matsushita; Junki Takamatsu; Masashi Taki; Katsuyuki Fukutake; Haruhiko Uchikawa; Hiroshi Takagi; Morio Arai; Werner Engl; Akira Shirahata
Journal:  Int J Hematol       Date:  2018-03-28       Impact factor: 2.490

4.  Recombinant factor VIII in the management of hemophilia A: current use and future promise.

Authors:  Jerry S Powell
Journal:  Ther Clin Risk Manag       Date:  2009-05-20       Impact factor: 2.423

Review 5.  Novel therapies and current clinical progress in hemophilia A.

Authors:  Pauline Balkaransingh; Guy Young
Journal:  Ther Adv Hematol       Date:  2017-12-28

6.  An inventory of healthy weight practices in federally funded haemophilia treatment centres in the United States.

Authors:  E Adams; J Deutsche; E Okoroh; S Owens-McAlister; S Majumdar; M Ullman; M L Damiano; M Recht
Journal:  Haemophilia       Date:  2014-03-16       Impact factor: 4.287

7.  Prevention of the musculoskeletal complications of hemophilia.

Authors:  E C Rodriguez-Merchan
Journal:  Adv Prev Med       Date:  2012-06-14

Review 8.  Evidence supporting the use of recombinant activated factor VII in congenital bleeding disorders.

Authors:  Pär I Johansson; Sisse R Ostrowski
Journal:  Drug Des Devel Ther       Date:  2010-07-21       Impact factor: 4.162

9.  Multiple Comorbid Conditions among Middle-Aged and Elderly Hemophilia Patients: Prevalence Estimates and Implications for Future Care.

Authors:  Aroub A Khleif; Nidra Rodriguez; Deborah Brown; Miguel A Escobar
Journal:  J Aging Res       Date:  2011-09-07

10.  Matching-adjusted indirect comparisons of annualized bleeding rate and utilization of BAY 94-9027 versus three recombinant factor VIII agents for prophylaxis in patients with severe hemophilia A.

Authors:  Katharine Batt; Wei Gao; Rajeev Ayyagari; Céline Deschaseaux; Parth B Vashi; Zhiwen Yao; Yao Wang; Sophia Kessabi; Robert Klamroth
Journal:  J Blood Med       Date:  2019-06-20
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