Pathogenesis of haemophilic arthropathy

Roosendaal, G.; Lafeber, Floris P. J. G.

doi:10.1111/j.1365-2516.2006.01268.x

Cited by 241 publications

(260 citation statements)

References 34 publications

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“…Hemophilia is characterized by frequent intra-articular and intramuscular bleeding episodes, causing damage to joint [1,2] and soft tissue, therefore, predisposing to arthropathy and musculoskeletal dysfunction [3,4]. Treatment of hemophilia with factor replacement aims to minimize structural damage to joints and maximize patients' functional independence and quality of life [5].…”

Section: Introductionmentioning

confidence: 99%

Functional independence score in hemophilia: A cross‐sectional study assessment of Mexican children

Tlacuilo‐Parra¹,

Villela-Rodriguez²,

Garibaldi-Covarrubias³

et al. 2009

Pediatric Blood & Cancer

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BackgroundThe Functional Independence Score in Hemophilia (FISH) is a performance‐based assessment tool used to measure the patients functional ability so far only used in patients with severe hemophilia. Its aim is to determine if FISH is useful in patients with mild and moderate disease.ProcedureIn a cross‐sectional study 90 children 60 hemophilic and 30 sex‐, race‐, and age‐matched healthy males were assessed. Patients between 5 and 16 years of age were selected each patient was evaluated in seven activities under three categories: self‐care (grooming and eating, bathing, and dressing), transfers (chair and squat), and locomotion (walking and step climbing). Each activity was graded from 1 to 4 according to the amount of assistance required to perform the activity with total scores ranging from 7 to 28.ResultsAs a whole, the mean age of the patients was 10.0 ± 3.4 years with a mean FISH of 25.8 ± 3.6 (range 15–28). There were no differences in the FISH between healthy males and patients with mild hemophilia; however, the score was significantly higher in patients with mild hemophilia (28 ± 0) than patients with moderate (26.2 ± 2.5; P = 0.004) or severe hemophilia (24.0 ± 4.7; P = 0.0006). The most affected activities were squatting, walking, and step climbing.ConclusionsA significant decrease in functional ability was demonstrated according to the severity of hemophilia, especially for those activities involving weight‐bearing demands like locomotion and step climbing. Of seven activities evaluated, changes were observed in the group with moderate and severe hemophilia, but no changes detected in patients with mild disease. Pediatr Blood Cancer 2010;54:394–397. © 2009 Wiley‐Liss, Inc.

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Section: Introductionmentioning

confidence: 99%

Functional independence score in hemophilia: A cross‐sectional study assessment of Mexican children

Tlacuilo‐Parra¹,

Villela-Rodriguez²,

Garibaldi-Covarrubias³

et al. 2009

Pediatric Blood & Cancer

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“…Grey-scale US can also be used to follow the progression or regression of soft tissue hematomas [4,5,31,32] and pseudotumours. The latter entity is a rare complication that occurs in 1-2% of haemophiliacs.…”

Section: Ultrasonographymentioning

confidence: 99%

“…The pathogenetic mechanisms involved in haemophilic arthropathy are unknown, but most likely multifactorial [5]. Some authors [6][7][8][9] suggest that intra-articular blood has a precursor direct effect on cartilage, as a result of the iron-catalysed formation of destructive oxygen metabolites, subsequently affecting the synovium.…”

Section: Introductionmentioning

confidence: 99%

State‐of‐the‐art imaging techniques for the evaluation of haemophilic arthropathy: present and future

Doria

2010

Haemophilia

102

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Summary. In spite of the fact that the diagnosis of haemophilia is essentially clinical and laboratorybased, imaging has become an important tool for the evaluation of complications, diagnostic confirmation and/or complementation and therapeutic follow-up in haemophilic arthropathy. Radiography remains the workforce horse in the diagnosis and follow-up of haemophilic arthropathy. The radiographical findings in arthropathy follow an expected sequence of events and are overall similar in different joints. Magnetic resonance imaging (MRI) has advantages over radiography based on its capability of visualizing soft tissue and cartilage changes in haemophilic joints. The recent development and standardization of MRI scoring systems for measuring soft tissue and cartilage abnormalities may enable the comparison of pathological joint findings in clinical trials conducted at different institutions across the world. The implementation of high-frequency transducers and colour/power Doppler capabilities has provided new insights for clinical applications of ultrasonography (US) in haemophilic arthropathy. In spite of the imaging modality's technical challenges such as operator-dependency, US has advantages over MRI. One of these advantages is its ability of differentiating synovium hypertrophy and hemosiderin deposition, which is not possible with MRI given the presence of susceptibility artefacts from extracellular hemosiderin on gradient-echo MR images. In addition to the aforementioned conventional imaging modalities, novel imaging techniques (blood oxygen level dependent, ultrasmall superparamagnetic ironoxide contrast-enhanced, and T1 and T2 mapping MRI, ultrasound biomicroscopy, microbubble contrast-enhanced US and positron emission tomography, among others) hold promise for early assessment of haemophilic arthropathy in the future upon completion of their clinical validation.

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“…1 Boys with severe HA suffer recurrent bleeding into joints, leading to arthropathy and physical disability that seriously impacts their quality of life. 2,3 Prophylaxis treatment with regular FVIII infusion prevents bleeding and their associated complications, and is considered the standard of care by the World Federation of Haemophilia (WFH). 4,5 Prophylaxis is beneficial when started at any age/stage of disease, but the most benefit will come from primary prophylaxis to start with no more than one large joint bleed with no joint damage and before age 3.…”

Section: Introductionmentioning

confidence: 99%

Influence of medical insurance schemes and charity assistance projects on regular prophylaxis treatment of the boys with severe haemophilia A in China

Zhao

et al. 2017

Haemophilia

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Pathogenesis of haemophilic arthropathy

Cited by 241 publications

References 34 publications

Functional independence score in hemophilia: A cross‐sectional study assessment of Mexican children

Functional independence score in hemophilia: A cross‐sectional study assessment of Mexican children

State‐of‐the‐art imaging techniques for the evaluation of haemophilic arthropathy: present and future

Influence of medical insurance schemes and charity assistance projects on regular prophylaxis treatment of the boys with severe haemophilia A in China

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