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Long-acting recombinant fusion protein linking coagulation factor IX with albumin (rIX-FP) in children: Results of a phase 3 trial

The result's identifiers

  • Result code in IS VaVaI

    <a href="https://www.isvavai.cz/riv?ss=detail&h=RIV%2F00064203%3A_____%2F16%3A10333367" target="_blank" >RIV/00064203:_____/16:10333367 - isvavai.cz</a>

  • Alternative codes found

    RIV/00216208:11130/16:10333367 RIV/00843989:_____/16:E0106485

  • Result on the web

    <a href="http://dx.doi.org/10.1160/TH16-03-0179" target="_blank" >http://dx.doi.org/10.1160/TH16-03-0179</a>

  • DOI - Digital Object Identifier

    <a href="http://dx.doi.org/10.1160/TH16-03-0179" target="_blank" >10.1160/TH16-03-0179</a>

Alternative languages

  • Result language

    angličtina

  • Original language name

    Long-acting recombinant fusion protein linking coagulation factor IX with albumin (rIX-FP) in children: Results of a phase 3 trial

  • Original language description

    A global phase 3 study evaluated the pharmacokinetics, efficacy and safety of a recombinant fusion protein linking coagulation factor IX with albumin (rIX-FP) in 27 previously treated male children (1-11 years) with severe and moderately severe haemophilia B (factor IX [FIX] activity LESS-THAN OR EQUAL TO2 IU/dl). All patients received routine prophylaxis once every seven days for up to 77 weeks, and treated any bleeding episodes on-demand. The mean terminal half-life of rIX-FP was 91.4 hours (h), 4.3-fold longer than previous FIX treatment and clearance was 1.11 ml/h/kg, 6.4-fold slower than previous FIX treatment. The median (Q1, Q3) annualised spontaneous bleeding rate was 0.00 (0.00, 0.91) and was similar between the <6 years and GREATER-THAN OR EQUAL TO6 years age groups, with a weekly median prophylactic dose of 46 IU/kg. In addition, patients maintained a median trough level of 13.4 IU/dl FIX activity on weekly prophylaxis. Overall, 97.2 % of bleeding episodes were successfully treated with one or two injections of rIX-FP (95 % CI: 92 % to 99 %), 88.7 % with one injection, and 96 % of the treatments were rated effective (excellent or good) by the Investigator. No patient developed FIX inhibitors and no safety concerns were identified. These results indicate that rIX-FP is safe and effective for preventing and treating bleeding episodes in children with haemophilia B with weekly prophylaxis. Routine prophylaxis with rIX-FP at treatment intervals of up to 14 days are currently being investigated in children with severe and moderately severe haemophilia B. Clinicaltrials.gov (NCT01662531). (C) Schattauer 2016.

  • Czech name

  • Czech description

Classification

  • Type

    J<sub>x</sub> - Unclassified - Peer-reviewed scientific article (Jimp, Jsc and Jost)

  • CEP classification

    FD - Oncology and haematology

  • OECD FORD branch

Result continuities

  • Project

  • Continuities

    I - Institucionalni podpora na dlouhodoby koncepcni rozvoj vyzkumne organizace

Others

  • Publication year

    2016

  • Confidentiality

    S - Úplné a pravdivé údaje o projektu nepodléhají ochraně podle zvláštních právních předpisů

Data specific for result type

  • Name of the periodical

    Thrombosis and Haemostasis

  • ISSN

    0340-6245

  • e-ISSN

  • Volume of the periodical

    116

  • Issue of the periodical within the volume

    4

  • Country of publishing house

    DE - GERMANY

  • Number of pages

    10

  • Pages from-to

    659-668

  • UT code for WoS article

    000384964200012

  • EID of the result in the Scopus database

    2-s2.0-84989158710