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
Evolution of clotting factor concentrates prescriptions and impact of recommendations of prophylaxis in children with haemophilia.
TL;DR: The significant reduction of haemarthrosis in this cohort can be linked to a first infusion age and a prophylaxis introduction much earlier, as well as the evolution of the patients' number and regimen type introduced: increase of proPHylaxis and decrease of on demand regimen.
Journal ArticleDOI
An Assessment of Mental Wellbeing and Health-Related Quality of Life Among Youth Living in Central Mexico
TL;DR: In this article, a study was conducted to assess the connection between mental wellbeing and health-related quality of life among a sample of children in junior high school and found that mental wellbeing scores significantly predicted scores on the physical subscale of the quality-of-life measure, but did not predict overall wellbeing or psychosocial wellbeing.
Dissertation
Variation in FVIII/FIX activity in haemophilia: classification and clinical implications
TL;DR: Hemophilia is an inherited, x-linked bleeding disorder that causes recurrent joint bleeding, eventually leading to severe crippling arthropathy and the need for lifelong treatment.
Dissertation
Explaining variation in bleeding pattern of severe haemophilia
TL;DR: The age at first joint bleed may be used as an indicator of bleeding pattern and the effect of the underlying defect in the gene encoding for factor VIII was studied in 241 patients with severe haemophilia, finding these factors were not, or only to a limited extent, associated with bleeding pattern.
Dissertation
FVIII prophylaxis in children with severe haemophilia A : A nationwide survey of outcome and costs in Finland
TL;DR: Regular high-dose primary prophylaxis of PUPs with severe HA leads to excellent long-term joint health, annualised bleeding rates being near zero, and real-world data on total treatment costs per body weight in non-inhibitor and inhibitor patients is provided.
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.
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