INTRODUCTION: Hemophilic arthropathy is the most important cause of morbidity in patients with hemophilia. The earliest alterations that occur during the development of hemophilic arthropathy can be shown using magnetic resonance imaging (MRI). In addition, various tools have been developed to monitor joint health. AIM: : The purpose of this study was to determine the correlation between these tools when used to assess hemophilia patients. METHODS: This cross-sectional study enrolled 38 hemophilia patients between 2 and 18 years of age. Hemophilia Joint Health Score (HJHS) and radiologic scores (Pettersson and Arnold-Hilgartner) were used to evaluate the joints of the patients (n=236). Magnetic resonance imaging (MRI) was performed on 46 joints that were pathologic according to the HJHS. These joints were imaged bilaterally; therefore, 14 normal joints were imaged. In addition, the Functional Independence Score in Hemophilia (FISH) was used to evaluate the joints of 33 patients. RESULTS: The HJHS scores were correlated with the MRI and FISH scores. The annual bleeding rate was not correlated with any scores; however, the number of painful joints was correlated with the MRI scores. The radiologic scores were correlated weakly with progressive score and HJHS. CONCLUSION: The agreement between the HJHS scores and the MRI scores suggests that the HJHS may be used safely as a first-line tool. We recommend that the FISH should be used in the routine follow-up of hemophilia patients as a functional evaluation tool. Painful joints may be useful in deciding to apply MRI, whereas the bleeding frequency may not be useful.
INTRODUCTION:Hemophilic arthropathy is the most important cause of morbidity in patients with hemophilia. The earliest alterations that occur during the development of hemophilic arthropathy can be shown using magnetic resonance imaging (MRI). In addition, various tools have been developed to monitor joint health. AIM: : The purpose of this study was to determine the correlation between these tools when used to assess hemophiliapatients. METHODS: This cross-sectional study enrolled 38 hemophiliapatients between 2 and 18 years of age. Hemophilia Joint Health Score (HJHS) and radiologic scores (Pettersson and Arnold-Hilgartner) were used to evaluate the joints of the patients (n=236). Magnetic resonance imaging (MRI) was performed on 46 joints that were pathologic according to the HJHS. These joints were imaged bilaterally; therefore, 14 normal joints were imaged. In addition, the Functional Independence Score in Hemophilia (FISH) was used to evaluate the joints of 33 patients. RESULTS: The HJHS scores were correlated with the MRI and FISH scores. The annual bleeding rate was not correlated with any scores; however, the number of painful joints was correlated with the MRI scores. The radiologic scores were correlated weakly with progressive score and HJHS. CONCLUSION: The agreement between the HJHS scores and the MRI scores suggests that the HJHS may be used safely as a first-line tool. We recommend that the FISH should be used in the routine follow-up of hemophiliapatients as a functional evaluation tool. Painful joints may be useful in deciding to apply MRI, whereas the bleeding frequency may not be useful.
Authors: Matteo Nicola Dario Di Minno; Mariasanta Napolitano; Anna Chiara Giuffrida; Erminia Baldacci; Christian Carulli; Elena Boccalandro; Clarissa Bruno; Eleonora Forneris; Irene Ricca; Walter Passeri; Marco Martinelli; Gianna Franca Rivolta; Luigi Piero Solimeno; Carlo Martinoli; Angiola Rocino; Gianluigi Pasta Journal: Br J Haematol Date: 2021-12-19 Impact factor: 8.615