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
Cost‐effectiveness analysis of late prophylaxis vs. on‐demand treatment for severe haemophilia A in Italy
A. Coppola,A D'Ausilio,A Aiello,S Amoresano,Mondher Toumi,Prasad Mathew,Annarita Tagliaferri +6 more
TL;DR: The 5‐year prospective Italian POTTER study provided evidence of benefits in adolescents and adults of late prophylaxis vs. on‐demand therapy in reducing bleeding episodes and joint morbidity and improving quality of life; however, costs were increased.
Journal ArticleDOI
Enjeux d'une prise en charge pédiatrique précoce de l'hémophilie sévère
H. Chambost,S. Meunier +1 more
TL;DR: In this article, the early diagnosis of severe haemophilia and the involvement of expert caregivers in a multidisciplinary approach, are essential to make the acceptance of the disease easier.
Journal ArticleDOI
Hemophilic arthropathy: clinical, radiologic, and functional evaluation: a single-center experience in a limited resource country
TL;DR: A significant decrease in the functional ability was demonstrated on the basis of the severity of hemophilia and the FISH and Pettersson scoring systems are of great importance in assessing patients with hemophilic arthropathy.
Journal ArticleDOI
Preventing bleeds by treatment: new era for haemophilia changing the paradigm.
TL;DR: In an era where clotting factor concentrates are abundant and gene therapy a reality, all patients with severe haemophilia should be offered a strategy of bleeding prevention andEpisodic (‘on demand’) therapy as a treatment strategy for severe ha Hemophilia needs reconsideration.
Journal ArticleDOI
Hemophilia management in transfusion medicine.
TL;DR: The use of blood products, including plasma derived and recombinant coagulation factor concentrates (CFCs), and other hemostatic agents central to the management of bleeding, surgical procedures, prophylaxis and inhibitors are reviewed.
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.
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Prophylaxis versus episodic treatment to prevent joint disease in boys with severe hemophilia.
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