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טיפול מונע בילדים חולי המופיליה PowerPoint PPT Presentation


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טיפול מונע בילדים חולי המופיליה. ד"ר גילי קנת מנהלת יחידת הקרישה שיבא, תל השומר. Optimal haemophilia care versus the reality- P Bolton Maggs, BJH 2006.

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טיפול מונע בילדים חולי המופיליה

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Optimal haemophilia care versus the reality p bolton maggs bjh 2006

Optimal haemophilia care versus the reality- P Bolton Maggs, BJH 2006

  • Optimal care for severe haemophilia includes accurate diagnosis, early and adequate factor replacement for bleeding episodes and the provision of prophylaxis from an early age to prevent joint bleeding and the consequent arthropathy.


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Prevention and treatment of musculoskeletal disease in the haemophilia population: role of prophylaxis and synovectomy.

Hemophilia 2006


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Management and prevention of recurrent hemarthrosis in patients with hemophilia.Curr Opin Hematol 2005

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A comparison between prophylaxis and on demand treatment for severe haemophilia. Clin lab hematol 2005

  • The study sample consisted of 133 patients with severe haemophilia A and B treated in the Katharine Dormandy Haemophilia Centre at the Royal Free Hampstead NHS Trust in London.

  • The total number of bleeds per year was significantly (P = 0.021) less for prophylactically (7.4) vs. on-demand treated patients (11.4).

  • This suggests that prophylaxis reduces the risk of arthropathies, the number of future hospital visits and orthopaedic surgeries, and is thus the optimal modality of treatment for patients with severe haemophilia.


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29 studies; four studies (including 37 participants) eligible for inclusion.

  • 3 / 4 evaluated hemophilia A;

  • one showed a decrease in frequency of joint bleeds with prophylaxis compared to placebo with a rate difference (RD) -10.80 (95% CI -16.33 to 5.27) bleeds per year.

  • one study showed no difference in joint bleed frequency, and another failed to demonstrate an advantage of factor VIII dosing based on individual pharmacokinetic data over the standard prophylaxis regimen with RD -0.14 (95% CI -1.34 to 1.05) bleeds per year.

  • The fourth study evaluated hemophilia B and showed fewer joint bleeds with weekly (15 IU/kg) versus bi-weekly (7.5 IU/kg) prophylaxis, RD -3.30 (95% CI -5.50 to - 1.10) bleeds per year.


Cochrane databese 2006

Cochrane databese 2006

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Well-designed RCTs are needed to assess the effectiveness of prophylactic clotting factor concentrates.


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Knowledge, attitudes, and behaviors of youths in the US hemophilia population: results of a national survey.-Am J Public Health. 2006

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  • Exercise may decrease further destruction in the adult haemophilic joint.

    Haemophilia 2006


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