Abstract

Blood-induced joint damage as a hallmark of haemophilia continues to occur despite the widespread prophylaxis. Pre-cise assessment and follow-up of joint status are crucial for tailoring their treatment. To study the correlation between the bleeding phenotype, the functional joint status, and the magnetic resonance imaging score in pediatric patients with haemophilia. Eighty-six joints (ankles, knees, and elbows) in patients aged 10.7±0.5 (range 4 - 20) years with severe/moderate haemophilia A, severe haemophilia B and haemophilia A with inhibitors were included in the study. The joints were assessed by Haemophilia Joint Health Score 2.1 (HJHS2.1) one month after the last hemarthrosis in a non-bleeding state. The magnetic reso-nance imaging was performed on 40 (46.5%) of the examined hemophilic joints (16 ankles, 11 knees and 13 elbows). Joint bleeds were present in 37 (38.9%) of the joints with ankles being the most commonly affected. Sixty joints (69.8%) had normal HJHS2.1 score. Only the loss of flexion score differed significantly between the joints and the ankles had highest score. The cumulative number of hemarthrosis in the joint correlated moderately with hemosiderin deposition and strongly with the formation of subchondral cysts on magnetic resonance imaging. The magnetic resonance imaging scores for soft tissue and osteochondral domains correlated moderately with the cumulative number of hemarthrosis in the joint and only with the presence of pain and crepitus of mo-tion from the physical examination. Magnetic resonance imaging is more sensitive than the bleeding phenotype and physical examination in detecting early signs of haemophilic arthropathy.

Highlights

  • Blood-induced joint damage as a hallmark of haemophilia continues to occur despite the widespread prophylaxis

  • Blood-induced joint damage continues to occur. Detection of this hemophilic arthropathy is crucial for the choice or modification of the treatment approach with the goal to prevent or postpone deterioration of the joint status

  • Several studies have investigated the correlation between the magnetic resonance imaging (MRI)-detected structural joint alterations and the findings from the physical examination in patients with hemophilia, but the results are controversial.[5,6,7]

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Summary

Introduction

Blood-induced joint damage as a hallmark of haemophilia continues to occur despite the widespread prophylaxis. The magnetic resonance imaging scores for soft tissue and osteochondral domains correlated moderately with the cumulative number of hemarthrosis in the joint and only with the presence of pain and crepitus of motion from the physical examination. That is why much research has been currently devoted to exploring the diagnostic sensitivity of imaging studies for early hemophilic arthropathy detection and the impact of the identified joint alterations on tailoring the prophylactic treatment.[1,2,3] The magnetic resonance imaging (MRI) of the six index joints (ankles, knees and elbows) is accepted as the gold standard for depicting the soft tissue and osteochondral changes in haemophilia.[4] Several studies have investigated the correlation between the MRI-detected structural joint alterations and the findings from the physical examination in patients with hemophilia, but the results are controversial.[5,6,7]

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