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Treatment outcomes ...
Treatment outcomes in persons with severe haemophilia B in the Nordic region : The B-NORD study
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- Kihlberg, Kristina (författare)
- Lund University,Lunds universitet,Klinisk koagulationsmedicin, Malmö,Forskargrupper vid Lunds universitet,Clinical Coagulation, Malmö,Lund University Research Groups,Skåne University Hospital
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- Baghaei, Fariba (författare)
- Sahlgrenska University Hospital
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- Bruzelius, Maria (författare)
- Karolinska Institutet,Karolinska Institute,Karolinska University Hospital
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- Funding, Eva (författare)
- Copenhagen University Hospital,University of Copenhagen
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- Andre Holme, Pål (författare)
- University of Oslo,Oslo university hospital
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- Lassila, Riitta (författare)
- University of Helsinki,Helsinki University Central Hospital
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- Nummi, Vuokko (författare)
- University of Helsinki,Helsinki University Central Hospital
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- Ranta, Susanna (författare)
- Karolinska Institutet
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- Osooli, Mehdi (författare)
- Lund University,Lunds universitet,Allmänmedicin och klinisk epidemiologi,Forskargrupper vid Lunds universitet,Family Medicine and Clinical Epidemiology,Lund University Research Groups
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- Berntorp, Erik (författare)
- Lund University,Lunds universitet,Klinisk koagulationsmedicin, Malmö,Forskargrupper vid Lunds universitet,Clinical Coagulation, Malmö,Lund University Research Groups
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- Astermark, Jan (författare)
- Lund University,Lunds universitet,Klinisk koagulationsmedicin, Malmö,Forskargrupper vid Lunds universitet,Clinical Coagulation, Malmö,Lund University Research Groups,Skåne University Hospital
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(creator_code:org_t)
- 2021-03-29
- 2021
- Engelska 9 s.
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Ingår i: Haemophilia. - : Wiley. - 1351-8216 .- 1365-2516. ; 27:3, s. 366-374
- Relaterad länk:
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http://dx.doi.org/10... (free)
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https://onlinelibrar...
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https://lup.lub.lu.s...
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https://doi.org/10.1...
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http://kipublication...
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Abstract
Ämnesord
Stäng
- Introduction: Data on outcome in persons with haemophilia B (PwHB) are limited and mainly extrapolated from studies of haemophilia A (HA). Aim: To characterize treatment outcomes in persons with severe HB in the Nordic region, with a focus on joint health, compared with matched controls with HA. Methods: PwHB attending haemophilia centres in Denmark, Finland, Norway and Sweden were enrolled and matched with controls with HA. Joint assessment using Haemophilia Joint Health Score (HJHS) and ultrasound according to Haemophilia Early Arthropathy Detection protocol (HEAD-US) was conducted. Adherence was evaluated using the Validated Haemophilia Regimen Treatment Adherence Scale (VERITAS). Results: Seventy-nine males with HB, with median age of 30 years (range 1–75), were enrolled. Eleven patients (14%) had a history of or current inhibitor. Twenty-nine PwHB (37%) reported joint bleeds during the prior year, and 35% had previously undergone joint surgery. Ninety-five per cent were on prophylaxis, and 70% used recombinant concentrates, with a median factor consumption of 3,900 IU/kg/year for standard half-life products. Only two patients had a VERITAS score corresponding to ‘non-adherence'. Joint health, assessed with HJHS, showed a significant lower score among PwHB compared with HA controls, explained by a difference in the 18–49 age group, without observed differences in older or younger subgroups. The HEAD-US scores were overall low. Conclusion: The Nordic cohort of PwHB is well treated by prophylaxis, but the goal of zero bleeds for all is not reached. Our findings suggest that patients with severe HB suffer from a milder arthropathy than patients with severe HA.
Ämnesord
- MEDICIN OCH HÄLSOVETENSKAP -- Klinisk medicin -- Hematologi (hsv//swe)
- MEDICAL AND HEALTH SCIENCES -- Clinical Medicine -- Hematology (hsv//eng)
Nyckelord
- adherence
- arthropathy
- coagulation factor IX
- haemophilia B
- joint score
- phenotype
- ultrasound
Publikations- och innehållstyp
- art (ämneskategori)
- ref (ämneskategori)
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Till lärosätets databas
- Av författaren/redakt...
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Kihlberg, Kristi ...
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Baghaei, Fariba
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Bruzelius, Maria
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Funding, Eva
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Andre Holme, Pål
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Lassila, Riitta
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visa fler...
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Nummi, Vuokko
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Ranta, Susanna
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Osooli, Mehdi
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Berntorp, Erik
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Astermark, Jan
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visa färre...
- Om ämnet
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- MEDICIN OCH HÄLSOVETENSKAP
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MEDICIN OCH HÄLS ...
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och Klinisk medicin
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och Hematologi
- Artiklar i publikationen
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Haemophilia
- Av lärosätet
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Lunds universitet
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Karolinska Institutet