Journal ArticleDOI
The effects of postponing prophylactic treatment on long-term outcome in patients with severe hemophilia.
Krista Fischer,Johanna G. van der Bom,Eveline P. Mauser-Bunschoten,Goris Roosendaal,Robert Prejs,Piet de Kleijn,Diederick E. Grobbee,Marijke van den Berg +7 more
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TLDR
The effect of postponing prophylaxis on long-term arthropathy was studied in a cohort of 76 patients with severe hemophilia born between 1965 and 1985, finding that most patients have their first joint bleed after the age of 2 years.About:
This article is published in Blood.The article was published on 2002-04-01. It has received 250 citations till now. The article focuses on the topics: Arthropathy & Hemarthrosis.read more
Citations
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Journal ArticleDOI
The role of synovectomy in the management of a target joint.
TL;DR: The number of bleeds occurring in the musculoskeletal system must be kept to a minimum to prevent the permanent inflammation of the synovial tissue known as chronic synovitis.
Journal ArticleDOI
Towards the goal of prophylaxis: experience and treatment strategies from Sweden, France and Hungary.
TL;DR: The barriers to prophylaxis are discussed, such as the perceived need, costs and availability, and difficulty of venous access, and the authors' experiences with the therapy are described.
Journal ArticleDOI
Clinical use of factor VIII and factor IX concentrates.
TL;DR: Replacement therapy with intravenously delivered plasma-derived or recombinant FVIII and FIX concentrates, aimed at correcting the coagulation factor deficiency in the case of, or in order to prevent, bleeding is the cornerstone of management of patients with haemophilia A and haemophile B.
Journal ArticleDOI
Changing Paradigm of Hemophilia Management: Extended Half-Life Factor Concentrates and Gene Therapy.
TL;DR: Seminal developments in the past 5 years, including the commercial availability of extended half-life factor concentrates and the publication of successful results of gene therapy for patients with hemophilia B, promise to further revolutionize Hemophilia care over the next few decades.
Journal ArticleDOI
Hemophilic joint disease – current perspective and potential future strategies
TL;DR: A combination of serological and imaging evidence of early joint disease might ultimately impact on the optimal management of hemophilic joint disease.
References
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Journal ArticleDOI
Longitudinal data analysis using generalized linear models
Kung Yee Liang,Scott L. Zeger +1 more
TL;DR: In this article, an extension of generalized linear models to the analysis of longitudinal data is proposed, which gives consistent estimates of the regression parameters and of their variance under mild assumptions about the time dependence.
Journal ArticleDOI
Twenty-five years' experience of prophylactic treatment in severe haemophilia A and B
TL;DR: It appears to be possible to prevent haemophilic arthropathy by giving effective continuous prophylaxis from an early age, and preventing the VIII:C or IX:C concentration from falling below 1% of normal.
Journal ArticleDOI
A longitudinal study of orthopaedic outcomes for severe factor‐VIII‐deficient haemophiliacs
TL;DR: It is hypothesized that no direct relationship exists between increasing factor dosage and orthopaedic outcomes over time and optimal therapeutic regimens for persons with haemophilia are established.
Journal ArticleDOI
Haemophilia prophylaxis in young patients–a long‐term follow‐up
TL;DR: To review long‐term prophylactic factor treatment in young patients with severe haemophilia A and B, focusing on the orthopaedic and radiological outcome.
Journal ArticleDOI
Primary prophylaxis in severe haemophilia should be started at an early age but can be individualized.
TL;DR: The frequency of joint bleeds and orthopaedic joint scores were evaluated in 121 patients with severe haemophilia who had started prophylactic treatment with clotting factor concentrates at least once weekly before the age of 10.
Related Papers (5)
Prophylaxis versus episodic treatment to prevent joint disease in boys with severe hemophilia.
Marilyn J. Manco-Johnson,Marilyn J. Manco-Johnson,Thomas C. Abshire,Amy D. Shapiro,Brenda Riske,Michele R. Hacker,Ray F. Kilcoyne,J. David Ingram,M L Manco-Johnson,Sharon Funk,Linda J. Jacobson,Leonard A. Valentino,W. Keith Hoots,George R. Buchanan,Donna DiMichele,Michael Recht,Deborah L Brown,Cindy A. Leissinger,Shirley Bleak,Alan R. Cohen,Prasad Mathew,Alison Matsunaga,Desiree Medeiros,Diane J. Nugent,Gregory Thomas,Alexis A. Thompson,Kevin McRedmond,J. Michael Soucie,Harlan Austin,Bruce L. Evatt +29 more