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Haemophilia A and B – evaluation of the Swedish prophylactic regimen by magnetic resonance imaging

INTRODUCTION: Sweden has been a pioneer in the prophylactic treatment of haemophilia. Magnetic resonance imaging (MRI) can detect small changes in joints and can therefore give an indication of a risk of developing arthropathy. AIM: To use MRI to evaluate the outcome of the Swedish ‘high‐dose regime...

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Autores principales: Lundin, Björn, Baghaei, Fariba, Holmström, Margareta, Petrini, Pia, Müller, Gunilla, Månsson, Sven, Ljung, Rolf
Formato: Online Artículo Texto
Lenguaje:English
Publicado: John Wiley and Sons Inc. 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10107095/
https://www.ncbi.nlm.nih.gov/pubmed/36469433
http://dx.doi.org/10.1111/hae.14693
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author Lundin, Björn
Baghaei, Fariba
Holmström, Margareta
Petrini, Pia
Müller, Gunilla
Månsson, Sven
Ljung, Rolf
author_facet Lundin, Björn
Baghaei, Fariba
Holmström, Margareta
Petrini, Pia
Müller, Gunilla
Månsson, Sven
Ljung, Rolf
author_sort Lundin, Björn
collection PubMed
description INTRODUCTION: Sweden has been a pioneer in the prophylactic treatment of haemophilia. Magnetic resonance imaging (MRI) can detect small changes in joints and can therefore give an indication of a risk of developing arthropathy. AIM: To use MRI to evaluate the outcome of the Swedish ‘high‐dose regimen’ and correlate the findings to age, bleeds, joint score and physical activity. METHODS: The study group comprised 48 Swedish male patients, mean age 25 years (range 12–33 years), with severe or moderate haemophilia A or B. Data on the Haemophilia Joint Health Score (HJHS) were available and physical activity was evaluated by a self‐reported questionnaire. RESULTS: MRI score was recorded in 188 joints. Twenty out of 48 patients had a score of ≥1 (range 1–13) in 31 joints of which 3/31 scores were in the knees and 28/31 in the ankles. No correlation was found between the number of recorded bleeds and the MRI score or between HJHS and MRI score. There was no correlation between the physical activity and the number of joint bleeds per se, but a trend (OR 3.0) that those most physically active (19/48; 39.6%), more frequently had an MRI score of ≥1 with an overweight for the right ankle. CONCLUSION: The Swedish prophylactic model offers protection against haemophilia joint arthropathy but will still not prevent osteochondral changes in some patients at young age. MRI of the ankles can signal risk of future arthropathy and indicate need to modify the prophylactic regimen.
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spelling pubmed-101070952023-04-18 Haemophilia A and B – evaluation of the Swedish prophylactic regimen by magnetic resonance imaging Lundin, Björn Baghaei, Fariba Holmström, Margareta Petrini, Pia Müller, Gunilla Månsson, Sven Ljung, Rolf Haemophilia Original Articles INTRODUCTION: Sweden has been a pioneer in the prophylactic treatment of haemophilia. Magnetic resonance imaging (MRI) can detect small changes in joints and can therefore give an indication of a risk of developing arthropathy. AIM: To use MRI to evaluate the outcome of the Swedish ‘high‐dose regimen’ and correlate the findings to age, bleeds, joint score and physical activity. METHODS: The study group comprised 48 Swedish male patients, mean age 25 years (range 12–33 years), with severe or moderate haemophilia A or B. Data on the Haemophilia Joint Health Score (HJHS) were available and physical activity was evaluated by a self‐reported questionnaire. RESULTS: MRI score was recorded in 188 joints. Twenty out of 48 patients had a score of ≥1 (range 1–13) in 31 joints of which 3/31 scores were in the knees and 28/31 in the ankles. No correlation was found between the number of recorded bleeds and the MRI score or between HJHS and MRI score. There was no correlation between the physical activity and the number of joint bleeds per se, but a trend (OR 3.0) that those most physically active (19/48; 39.6%), more frequently had an MRI score of ≥1 with an overweight for the right ankle. CONCLUSION: The Swedish prophylactic model offers protection against haemophilia joint arthropathy but will still not prevent osteochondral changes in some patients at young age. MRI of the ankles can signal risk of future arthropathy and indicate need to modify the prophylactic regimen. John Wiley and Sons Inc. 2022-12-05 2023-01 /pmc/articles/PMC10107095/ /pubmed/36469433 http://dx.doi.org/10.1111/hae.14693 Text en © 2022 The Authors. Haemophilia published by John Wiley & Sons Ltd. https://creativecommons.org/licenses/by-nc-nd/4.0/This is an open access article under the terms of the http://creativecommons.org/licenses/by-nc-nd/4.0/ (https://creativecommons.org/licenses/by-nc-nd/4.0/) License, which permits use and distribution in any medium, provided the original work is properly cited, the use is non‐commercial and no modifications or adaptations are made.
spellingShingle Original Articles
Lundin, Björn
Baghaei, Fariba
Holmström, Margareta
Petrini, Pia
Müller, Gunilla
Månsson, Sven
Ljung, Rolf
Haemophilia A and B – evaluation of the Swedish prophylactic regimen by magnetic resonance imaging
title Haemophilia A and B – evaluation of the Swedish prophylactic regimen by magnetic resonance imaging
title_full Haemophilia A and B – evaluation of the Swedish prophylactic regimen by magnetic resonance imaging
title_fullStr Haemophilia A and B – evaluation of the Swedish prophylactic regimen by magnetic resonance imaging
title_full_unstemmed Haemophilia A and B – evaluation of the Swedish prophylactic regimen by magnetic resonance imaging
title_short Haemophilia A and B – evaluation of the Swedish prophylactic regimen by magnetic resonance imaging
title_sort haemophilia a and b – evaluation of the swedish prophylactic regimen by magnetic resonance imaging
topic Original Articles
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10107095/
https://www.ncbi.nlm.nih.gov/pubmed/36469433
http://dx.doi.org/10.1111/hae.14693
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