Assessment of the progression of haemophilic arthropathy in children

Abstract

Arthropathy is considered as an irreversible and progressive complication in patients with haemophilia, even in children on prophylaxis. To estimate the progression of haemophilic arthropathy, 85 joints of 24 boys with severe (n = 18) and moderate (n = 6) haemophilia (A: 22, B: 2) were investigated with clinical examination, X-rays and magnetic resonance imaging (MRI) at two time periods (time 0 and 1). Patients' age at time 0 was 10.5 +/- 3.6 years and time elapsed to time 1 was 3.8 +/- 1.4 years. At time 0: all investigated joints had more than three bleeds. Sixteen boys were on secondary prophylaxis for 5.4 +/- 2.8 years. Clinical score (a modification of World Federation of Haemophilia's scale): 2.0 +/- 3.6, X-ray score (Pettersson): 2.1 +/- 2.8, MRI score (Denver): 4.5 +/- 3.8. After the first evaluation, prophylaxis was intensified in 11 children and initiated in four. At time 1: clinical score: 1.5 +/- 3.1, X-ray: 1.7 +/- 2.7, MRI score: 5.1 +/- 4.1. On average, the clinical and X-ray scores showed a significant improvement (26% and 40% of the joints respectively, P < 0.01) and the number of haemarthroses evidenced a threefold reduction from time 0 to 1 (P < 0.01), findings that could be associated with the modification of prophylaxis after time 0. MRI findings showed deterioration in 34% of the joints. Conversely, 14 joints (16.5%) with mild or moderate synovitis without cartilage degradation at time 0 showed an improvement at time 1. The information carried by the three scales could be divided into information shared by the three scores and information specific to each score, thus giving a more complete picture of joint damage caused by bleedings.

Biochemical markers of joint tissue damage increase shortly after a joint bleed; an explorative human and canine in vivo study

“…Blood-induced damage is a rather aggressive form of joint damage with features of both OA and RA 11,23 . Severely damaged joints are found at a much younger age than in case of OA or RA 24,25 and the pathologic processes are initiated by a clear trigger. Clearly, it cannot be excluded that there are pathways of joint damage (including cartilage, bone, and/or synovium) specific for certain joint disease and not generic for all.…”

Hemofilia: ejercicio y deporte

“…2), los hematomas y las sinovitis las lesiones musculoesqueléticas más comunes 8 . Los sangrados intraarticulares o hemartrosis son la manifestación clínica más frecuente y más conocida de la hemofilia, tanto grave como moderada, representando el 65-80% de todas las hemorragias 9,10 . Las articulaciones afectadas en mayor proporción son las rodillas, los codos y los tobillos, que suponen el 60-80% de todas las hemartrosis 3,11 .…”

Evaluating the Effectiveness of Prophylactic Replacement Therapy in Children With Hemophilic Arthropathy in Dynamics on Magnetic Resonance Imaging

“…Это позволяет осуществлять мониторинг суставов на ранних стадиях заболевания, проводить в динамике исследование у пациентов детского возраста [2,[9][10][11][12].…”