Using the Hemophilia Joint Health Score for assessment of children: Reliability of the Spanish version

Cuesta‐Barriuso, Rubén; Torres-Ortuño, Ana; Pérez‐Alenda, Sofía; J, Carrasco Juan; Querol, Felipe; Nieto-Munuera, Joaquín; Ja, López-Pina

doi:10.1080/09593985.2018.1443356

Cited by 3 publications

(3 citation statements)

References 28 publications

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“…This is unlikely to cause significant bias as the literature suggests good inter-observer reliability of the tool provided specific training has been given. 3,19 One study demonstrated interobserver discrepancies in HJHS assessment both within and between centres, 7 reinforcing the importance of training and standardisation of assessment. This is an important consideration in the context of HTCs with constrained physiotherapy resources.…”

Section: Discussionmentioning

confidence: 99%

“…This study also assumes equal accuracy in performance of the HJHS across sites. This is unlikely to cause significant bias as the literature suggests good inter‐observer reliability of the tool provided specific training has been given 3,19 . One study demonstrated inter‐observer discrepancies in HJHS assessment both within and between centres, 7 reinforcing the importance of training and standardisation of assessment.…”

Section: Discussionmentioning

confidence: 99%

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Haemophilia joint health score (HJHS) usage, patterns and outcome data in patients with haemophilia A and haemophilia B in Australia: A descriptive study using the Australian Bleeding Disorders Registry (ABDR)

George,

Parikh,

Carter

et al. 2023

Haemophilia

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Introduction: Assessment of joint health is an essential component of haemophilia management. A variety of clinical tools have been developed to standardise this assessment process. One such tool, the Haemophilia Joint Health Score (HJHS), is embedded for use within the Australian Bleeding Disorders Registry (ABDR). This provides a unique opportunity to analyse patterns of tool usage as well as associations between scores, demographics and clinical outcome factors. Aims: To characterise clinician practices regarding use of HJHS in routine clinical assessment of persons with haemophilia (PWH), to examine relationships between HJHS, and age, inhibitor status and body mass index (BMI), and to identify potential barriers to HJHS tool usage. Methods: A national, retrospective study was conducted using data extracted from the ABDR between 2014 and 2020, complemented by a qualitative questionnaire exploring haemophilia treatment centre (HTC) structure, resourcing and clinician perspectives about HJHS. Results: 28.1% (622/2220) of PWH had at least one HJHS recorded in the ABDR during the defined study period (546 haemophilia A, 76 haemophilia B). HJHS were recorded more in children than adults and performed more in severe than non-severe haemophilia. Multivariate analysis demonstrated significant association of age, severity and inhibitor status with HJHS. No association was identified between BMI and HJHS. Qualitative surveys revealed significant variation in physiotherapy funding, availability and methods of tool use between HTCs. Conclusion:This study provides valuable insights into joint health assessment in Australia. It improved our understanding of factors influencing long-term joint outcomes.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Haemophilia joint health score (HJHS) usage, patterns and outcome data in patients with haemophilia A and haemophilia B in Australia: A descriptive study using the Australian Bleeding Disorders Registry (ABDR)

George,

Parikh,

Carter

et al. 2023

Haemophilia

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“…The function of elbows, ankles and knees (total six joints per patient) was graded. 14,15 The scoring criteria included joint swelling, swelling duration, pain, muscle atrophy, muscle strength, joint friction sound during movement, joint flexion, and extension range of motion and gait score. Examination and scoring were performed by specialized physicians of the Rehabilitation Physiotherapy Department.…”

Section: Hjhsmentioning

confidence: 99%

Long‐term joint outcomes of regular low‐dose prophylaxis in Chinese children with severe haemophilia A

Zhou

et al. 2021

Haemophilia

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Severe haemophilia A is a hereditary disease with clotting factor VIII (FVIII) <1%. These severe patients suffer spontaneous bleeding in muscles and joints. 1,2 Recurrent joints bleeding can lead to arthropathy. Iron and haemoglobin deposited on the surface of the joint synovium activate inflammatory reactions, leading to synovitis. 3,4 As a result, the joint is more prone to recurrent bleeding, becoming a target joint. Eventually, cartilage and subchondral bone damage develop. These pathogenic processes result in fibrotic joints, loss of joint function, and even disability, which seriously reduces the quality of life (QOL) of the affected children with severe haemophilia. 5,6 In the past, due to the limitation of drug sources and economic conditions, most children with haemophilia in China were treated on

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Clinical and ultrasound evaluation of patients with haemophilia on prophylaxis

et al. 2021

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Haemophilia is characterised by articular bleeding, mainly in elbows, knees and ankles. Repeated bleeding episodes induce synovial hypertrophy and progressive osteochondral changes that may lead to degenerative arthropathy. This is the most common complication in patients with haemophilia, and it causes disability and loss of quality of life. [1][2][3] Primary prophylaxis is the current gold standard in haemophilia care for the prevention of bleeding and ensuing joint damage. Dosage and treatment regimens vary across countries. Due to the limited access to factor concentrates and financial resources in developing countries, many children and young adults with haemophilia are treated with secondary or tertiary prophylaxis. [4][5][6] According to evidence, many patients experience joint degenerative changes despite prophylaxis, which suggests that subclinical bleeding occurs even with this type of treatment. 7,8 Early detection of joint bleeding, whether symptomatic or subclinical, preferably during childhood, helps prevent joint deterioration

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Using the Hemophilia Joint Health Score for assessment of children: Reliability of the Spanish version

Abstract: The interrater reliability of the Spanish population version of the HJHS is high. This scale should be used generically in evaluating musculoskeletal pediatric patients with hemophilia.

Cited by 3 publications

References 28 publications

Haemophilia joint health score (HJHS) usage, patterns and outcome data in patients with haemophilia A and haemophilia B in Australia: A descriptive study using the Australian Bleeding Disorders Registry (ABDR)

Haemophilia joint health score (HJHS) usage, patterns and outcome data in patients with haemophilia A and haemophilia B in Australia: A descriptive study using the Australian Bleeding Disorders Registry (ABDR)

Long‐term joint outcomes of regular low‐dose prophylaxis in Chinese children with severe haemophilia A

Clinical and ultrasound evaluation of patients with haemophilia on prophylaxis

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