• Corpus ID: 21211386

Primary prophylaxis and early secondary prophylaxis in children

@inproceedings{Coppola2017PrimaryPA,
  title={Primary prophylaxis and early secondary prophylaxis in children},
  author={Antonio Coppola and Massimo Franchini and Annarita Tagliaferri},
  year={2017}
}
A four-decade clinical experience and recent evidence from randomised controlled studies definitively recognised primary prophylaxis, i.e. the regular infusion of factor concentrates started after the first haemarthrosis and/or before the age of two years, as the first-choice treatment in children with severe haemophilia. The available data clearly show that preventing bleeding since an early age enables to avoid or reduce the clinical impact of muscle-skeletal impairment from haemophilic… 

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References

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No conclusion can be drawn from the data presented herein whether primary prophylaxis or an early start of secondary prophYLaxis is superior with respect to joint outcome in children with severe haemophilia A.

Primary prophylaxis in severe haemophilia should be started at an early age but can be individualized

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

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The aim of treatment is to afford children with haemophilia the ability to live a nearly normal life with a minimal number of bleeding events and to promote psychosocial development without overprotection.

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Summary.  The benefits of prophylactic infusion of clotting factor in haemophilia A and B in delaying the onset of arthropathy are supported by retrospective data, but the possibility of selection

Secondary prophylaxis therapy: what are the benefits, limitations and unknowns?

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  • Medicine
    Haemophilia : the official journal of the World Federation of Hemophilia
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Irrespective of age at initiation, long‐term secondary prophylaxis appears to reduce the frequency of bleeding episodes even in patients with existing target joints whose bleeding diathesis persists during the early phases of secondary Prophylactic therapy.

Prophylaxis for severe haemophilia: clinical challenges in the absence as well as in the presence of inhibitors

The challenge is to optimize efficiency by individualizing prophylactic dose and frequency according to lifestyle and bleeding pattern to promote adherence and continue to prevent bleeds during this important period of rapid growth.

The impact of prophylactic treatment on children with severe haemophilia

Twenty‐seven children with severe haemophilia receiving regular prophylactic factor concentrate were evaluated to examine the overall effectiveness of prophylaxis in modern haemophilia care. The

When should prophylactic treatment in patients with haemophilia A and B start?— The German experience

Surprisingly, worsening joint scores could be detected despite ongoing prophylaxis after the 3‐year interval (median orthopaedic score 4, median radiological score 8), which is a sign of progressing osteoarthropathic alterations in patients reporting more than five joint haemorrhages before onset of Prophylactic treatment.

Comparing outcomes of different treatment regimens for severe haemophilia

Data indicate that both prophylaxis regimens resulted in significantly improved long-term outcomes, as assessed by pain, clinical and radiological assessment scores, suggesting that the improvement in long‐term clinical outcomes and reduced risk of arthropathy may lead to reduced factor consumption in adult patients who received early proPHylactic therapy.

Can long‐term prophylaxis for severe haemophilia be stopped in adulthood? Results from Denmark and the Netherlands

One‐third of young adults with severe haemophilia on a prophylactic regimen discontinued Prophylaxis in early adulthood, while maintaining a low joint bleed frequency and similar arthropathy after 4 years.
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