Literature DB >> 22032268

Identification and long-term observation of early joint damage by magnetic resonance imaging in clinically asymptomatic joints in patients with haemophilia A or B despite prophylaxis.

M Olivieri1, K Kurnik, T Pfluger, C Bidlingmaier.   

Abstract

Severe haemophilia is associated with recurrent joint bleeds, which can lead to haemophilic arthropathy. Subclinical joint bleeds have also been associated with joint damage detected using magnetic resonance imaging (MRI). We investigated the development of early changes in clinically asymptomatic joints using MRI in haemophilia A or B patients receiving prophylactic therapy. In this single-centre retrospective cohort study, patients with clinical evidence of joint damage in one ankle and one clinically asymptomatic ankle, in which we performed an MRI scan of both ankles in one session, were enrolled. MRI findings were graded using a 4-point scoring system (0 = normal findings and III = severe joint damage). Since 2000, 38 MRIs in 26 patients have been performed. Starting at a median age of 4 years, 23 patients received prophylaxis 2-3 times weekly. On-demand treatment was performed in three patients. Eight patients (31%) presented with an MRI score of 0, 12 (46%) had a score of I, four (15%) had a score of II, and two (8%) had a score of III in the clinically unaffected ankle. The six patients with MRI scores of II and III had started regular prophylaxis between the ages of 2 years and 15 years; none had developed an inhibitor or experienced a clinically evident bleed in the asymptomatic ankle. During our study, five of 26 patients had a worsening of MRI findings without experiencing a joint bleed. Early morphological changes in clinically asymptomatic ankles can be detected using MRI, despite adequate prophylaxis.
© 2011 Blackwell Publishing Ltd.

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Year:  2011        PMID: 22032268     DOI: 10.1111/j.1365-2516.2011.02682.x

Source DB:  PubMed          Journal:  Haemophilia        ISSN: 1351-8216            Impact factor:   4.287


  13 in total

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Authors:  Tine Wyseure; Tingyi Yang; Jenny Y Zhou; Esther J Cooke; Bettina Wanko; Merissa Olmer; Ruchi Agashe; Yosuke Morodomi; Niels Behrendt; Martin Lotz; John Morser; Annette von Drygalski; Laurent O Mosnier
Journal:  JCI Insight       Date:  2019-10-03

2.  Haemophilia A carriers demonstrate pathological and radiological evidence of structural joint changes.

Authors:  L Gilbert; L Rollins; M Hilmes; Y Luo; D Gailani; M R Debaun; R F Sidonio
Journal:  Haemophilia       Date:  2014-09-23       Impact factor: 4.287

Review 3.  Achieving and maintaining an optimal trough level for prophylaxis in haemophilia: the past, the present and the future.

Authors:  Victor Jiménez-Yuste; Günter Auerswald; Gary Benson; Thierry Lambert; Massimo Morfini; Eduardo Remor; Silva Zupančić Salek
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4.  Hemophilia without prophylaxis: Assessment of joint range of motion and factor activity.

Authors:  Michael Wang; Michael Recht; Neeraj N Iyer; David L Cooper; J Michael Soucie
Journal:  Res Pract Thromb Haemost       Date:  2020-07-06

5.  FVIII activity following FVIII protein infusion or FVIII gene transfer predicts the bleeding risk in hemophilia A rats.

Authors:  Karin M Lövgren; Malte S Larsen; Shannon M Zintner; Juliana C Small; Mads Kjelgaard-Hansen; Mattias Häger; Maj Petersen; Bo Wiinberg; Paris Margaritis
Journal:  J Thromb Haemost       Date:  2020-04-16       Impact factor: 5.824

6.  Components in Plasma-Derived Factor VIII, But Not in Recombinant Factor VIII Downregulate Anti-Inflammatory Surface Marker CD163 in Human Macrophages through Release of CXCL4 (Platelet Factor 4).

Authors:  Anne Bertling; Martin F Brodde; Mayken Visser; Janina Treffon; Michelle Fennen; Anke C Fender; Reinhard Kelsch; Beate E Kehrel
Journal:  Transfus Med Hemother       Date:  2017-08-11       Impact factor: 3.747

7.  Issues in assessing products for the treatment of hemophilia - the intersection between efficacy, economics, and ethics.

Authors:  Albert Farrugia; Declan Noone; Uwe Schlenkrich; Steffen Schlenkrich; Brian O'Mahony; Josephine Cassar
Journal:  J Blood Med       Date:  2015-06-15

Review 8.  Treatment and Prevention of Bleeds in Haemophilia Patients with Inhibitors to Factor VIII/IX.

Authors:  Angiola Rocino; Massimo Franchini; Antonio Coppola
Journal:  J Clin Med       Date:  2017-04-17       Impact factor: 4.241

Review 9.  How to discuss gene therapy for haemophilia? A patient and physician perspective.

Authors:  Wolfgang Miesbach; Brian O'Mahony; Nigel S Key; Mike Makris
Journal:  Haemophilia       Date:  2019-05-21       Impact factor: 4.287

10.  Controlled, cross-sectional MRI evaluation of joint status in severe haemophilia A patients treated with prophylaxis vs. on demand.

Authors:  J Oldenburg; R Zimmermann; O Katsarou; G Theodossiades; E Zanon; B Niemann; E Kellermann; B Lundin
Journal:  Haemophilia       Date:  2014-12-02       Impact factor: 4.263

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