Factors affecting the Haemophilia Joint Health Score in children with severe haemophilia

Haemophilia ◽  
2013 ◽  
Vol 19 (4) ◽  
pp. 626-631 ◽  
Author(s):  
M. Bladen ◽  
E. Main ◽  
N. Hubert ◽  
E. Koutoumanou ◽  
R. Liesner ◽  
...  
Keyword(s):  
Severe Haemophilia ◽  
Factors Affecting ◽  
Health Score ◽  
Joint Health

Musculoskeletal Pathologies in Children with Haemophilia Evaluated using a Standardised Physical Joint Scoring System to Assess Disability

2011 ◽  
Vol 07 (01) ◽  
pp. 76 ◽  
Author(s):  
Sonata Saulyte Trakymiene ◽  
Keyword(s):  
Treatment Efficacy ◽  
Scoring System ◽  
Joint Damage ◽  
Joint Disease ◽  
Severe Haemophilia ◽  
International Consensus ◽  
Health Score ◽  
Haemophilic Arthropathy ◽  
Joint Health ◽  
Joint Assessment

Repeated bleeding into the joints leads to the development of chronic and progressive joint disease in 90% of people with severe haemophilia. Owing to recurrent haemarthroses, musculoskeletal outcome remains an important hallmark of treatment efficacy in haemophilia. Physical joint assessment is commonly used to measure structural and functional joint damage. With the development and introduction of prophylaxis, the most widely used instruments for the assessment of haemophilic arthropathy were found to lack sensitivity to detect the earliest signs of joint disease. To address these inadequacies, a new international consensus tool, the Haemophilia Joint Health Score (HJHS), was developed. The HJHS appears to be a sensitive, reliable and valid tool. However, the HJHS needs further evaluation in various patient populations to assess its applicability and usefulness.

Discontinuing early prophylaxis in severe haemophilia leads to deterioration of joint status despite low bleeding rates

2016 ◽  
Vol 115 (05) ◽  
pp. 931-938 ◽  
Author(s):  
Annelies Nijdam ◽  
Wouter Foppen ◽  
Piet de Kleijn ◽  
Evelien P. Mauser-Bunschoten ◽  
Goris Roosendaal ◽  
...  
Keyword(s):  
Functional Limitations ◽  
Objective Assessment ◽  
Haemophilia A ◽  
Severe Haemophilia ◽  
On Demand ◽  
Recommended Treatment ◽  
Objective Monitoring ◽  
Joint Health ◽  
Observational Cohort

SummaryProphylaxis is the recommended treatment for children with severe haemophilia A, but whether prophylaxis should be continued in adulthood is still under debate. Previous studies with limited follow-up have suggested that some patients may be able to stop prophylaxis in adulthood, while maintaining good joint health. This single-centre observational cohort study examined patients with severe haemophilia A born 1970–1988 without inhibitor development, and assessed the long-term consequences of discontinuing prophylaxis. Patient-initiated changes in prophylaxis, including all switches to on-demand treatment lasting a minimum of two consecutive weeks, were recorded from the time self-infusion began until the last evaluation. Sixty-six patients were evaluated at a median age of 32.4 years: 26 of patients had stopped prophylaxis for a median of 10 years, 15 had interrupted prophylaxis and 59 had continued prophylaxis. Annual joint bleeding rate (AJBR), Haemophilia Joint Health Score (HJHS-2.1; 0–124 points), radiological Pettersson score (0–78 points) and Haemophilia Activities List score (HAL; 100–0 points) were compared between patients who stopped and patients who continued prophylaxis. Although self-reported bleeding rates and functional limitations were similar in both groups (AJBR: 1.5 vs 1.2 and HAL: 84 vs 84 for those who stopped and continued prophylaxis, respectively), objective assessment of joint status showed increased arthropathy after 10 years of on-demand treatment in patients who stopped prophylaxis compared with those who continued (HJHS: 23 vs. 14 and Pettersson: 16 vs 5, respectively; P< 0.01). These results support continuation of long-term prophylaxis in adults and demonstrate the need for objective monitoring of joint status.Trial registration: Dutch Trial Registry number 3098; UTN U1111–1121–7069.

Hemophilia joint health score reliability study

Haemophilia ◽  
2006 ◽  
Vol 12 (5) ◽  
pp. 518-525 ◽  
Author(s):  
P. HILLIARD ◽  
S. FUNK ◽  
N. ZOURIKIAN ◽  
B.-M. BERGSTROM ◽  
C. S. BRADLEY ◽  
...  
Keyword(s):  
Reliability Study ◽  
Health Score ◽  
Score Reliability ◽  
Joint Health

P107 Prevalence of haemarthrosis and clinical impact on the musculoskeletal system in people with haemophilia in the United Kingdom: evaluation of UKHCDO and haemtrack patient reported data

Rheumatology ◽  
2020 ◽  
Vol 59 (Supplement_2) ◽  
Author(s):  
Richard A Wilkins ◽  
Heidi J Siddle ◽  
Graham Chapman ◽  
Anthony J Redmond ◽  
Hau Xiang ◽  
...  
Keyword(s):  
United Kingdom ◽  
Ankle Joint ◽  
Health Research ◽  
Clotting Factor ◽  
Haemophilia A ◽  
Severe Haemophilia ◽  
Research Support ◽  
Haemophilia B ◽  
The United Kingdom ◽  
Joint Health

Abstract Background Haemarthrosis whereby bleeding occurs within a joint is a significant feature of haemophilia. Despite the availability of prophylaxis clotting factor concentrates in the United Kingdom haemarthrosis is associated with worsening joint health and haemarthropathy in people with severe haemophilia. The ankle joint has been identified as the most affected joint followed by the knee and elbow. Whilst annual joint bleed rates (AJBR) are commonly reported in haemophilia research, bleed rates in individual affected joints and joint health status in paediatric and adult patients is yet to be established. Methods In 2018, paediatric (&lt;18 years) and adult (≥18 years) patients with severe non-inhibitor haemophilia A and B registered with the United Kingdom Haemophilia Centres Doctors Organisation (UKHCDO) National haemophilia Database (NHD) were evaluated for haemarthrosis prevalence and associated joint health. Fully itemised Haemophilia Joint Health Scores (HJHS) were obtained from NHD records in combination with AJBR from Haemtrack (HT) compliant patients. Haemarthrosis prevalence and AJBR were reported as a total and for individual joints (ankles, knees and elbows). Results During 2018, 2233 individuals were identified; 273 reported ≥75% simultaneous HT compliance and electronic fully itemised HJHS data. The median (range) age of children is 10 (6-14) years and adults 40 (25-51) years. The joint bleed prevalence of haemophilia A and B in children is 33% and 47% respectively, and in adults 42% and 60% respectively. In children with haemophilia A (n = 80) the knee (data) was the most common site of bleeding. In haemophilia A adults (n = 157) the ankle and elbow were equally affected. In haemophilia B children (n = 17) and adults (n = 19) the elbow was the most prevalent site. Total HJHS scores in children with haemophilia A and B were 0.00 (1.00 SD) and 0.40 (0.90 SD) respectively. Total HJHS scores in adults with haemophilia A and B were 21.20 (16.80 SD) and 15.40 (15.10 SD) respectively. Mean HJHS scores itemised by joint were higher in adults compared with children. In children with haemophilia A and haemophilia B, mean (3.80) and median (4.00) scores for the ankle joint were higher than for the knee (2.90 and 1.00) and elbow joint (3.30 and 1.00). Conclusion Whilst there are limitations to this self-select subset of individuals with severe haemophilia, the prevalence of haemarthrosis is evenly distributed in all adult joints with a trend towards the knee in paediatrics. Despite prophylaxis 30% of children and 60% adults still reported bleeding over a 12-month period. Irrespective of the prevalence of joint bleeds in children with haemophilia the HJHS does not appear to be clinically sensitive enough to detect changes in joint health. Adults deemed adherent with prophylaxis and haemtrack still demonstrate worsening HJHS scores despite a low AJBR. Disclosures R.A. Wilkins Grants/research support; Funded by the National Institute for Health Research. H.J. Siddle Grants/research support; Funded by the National Institute for Health Research. G. Chapman None. A.J. Redmond None. H. Xiang None. M. Scott None. M. Richards None. L. Horn None. B. Palmer None. D. Stephensen None.

Haemophilia Joint Health Score in healthy adults playing sports

Haemophilia ◽  
2013 ◽  
Vol 20 (2) ◽  
pp. 282-286 ◽  
Author(s):  
D. Sluiter ◽  
W. Foppen ◽  
P. de Kleijn ◽  
K. Fischer
Keyword(s):  
Healthy Adults ◽  
Health Score ◽  
Joint Health

scholarly journals The Weight Assessment and its Relation to Hemophilia Joint Health Score in Hemophilic Patients

2021 ◽  
Vol 83 (1) ◽  
pp. 876-881
Author(s):  
Osama Roshdi El-Safy ◽  
Ahmed Mohammed Abdel Moneim ◽  
Nora Mohammed Ali Mohammed
Keyword(s):  
Health Score ◽  
Joint Health

scholarly journals Joint Health Status in Hemophilia Patients Using Hemophilia Joint Health Score and Pettersson Score

2018 ◽  
Vol 25 (2) ◽  
pp. 108-115
Author(s):  
Yun Young Roh ◽  
Young Ha Choi ◽  
Mina Park ◽  
Jung Hwa Hahn ◽  
Sun Hee Kim ◽  
...  
Keyword(s):  
Health Status ◽  
Health Score ◽  
Joint Health

scholarly journals Correlation between Hemophilia Early Arthropathy Detection with Ultrasound (HEAD-US) score and Hemophilia Joint Health Score (HJHS) in patients with hemophilic arthropathy

PLoS ONE ◽  
2021 ◽  
Vol 16 (4) ◽  
pp. e0248952
Author(s):  
Marcel Prasetyo ◽  
Ratna Moniqa ◽  
Angela Tulaar ◽  
Joedo Prihartono ◽  
Stefanus Imanuel Setiawan
Keyword(s):  
Joint Destruction ◽  
Medical Rehabilitation ◽  
Primary Data ◽  
Median Score ◽  
Moderate Correlation ◽  
Scoring Method ◽  
Health Score ◽  
Episodic Treatment ◽  
Hemophilic Arthropathy ◽  
Joint Health

Background Hemophilic arthropathy, a condition manifested as joint destruction due to spontaneous joint bleeding, is one complication of hemophiliac patients. Early detection and intervention may improve the outcome, in which ultrasonography can be an ideal modality with the introduction of HEAD-US (Hemophilia Early Arthropathy Detection with Ultrasound) protocol. Studies have shown US benefit in hemophiliac patients, including its potential as an alternative for the Hemophiliac Joint Health Score (HJHS) system. However, many of the studies were conducted in countries with better management of hemophilia using prophylaxis treatment. It is unclear whether HEAD-US has a correlation with HJHS in countries using episodic treatment only, like in Indonesia. Purpose This study aimed to explore the correlation between HEAD-US and HJHS in hemophiliac patients with joint problems in Indonesia. Materials and methods A cross-sectional correlation study between HEAD-US and HJHS was performed with primary data collected from 120 hemophilic patients. US examination was performed on elbow, knee and ankle joints using the HEAD-US scoring method by a musculoskeletal radiologist. HJHS examination was conducted by a trained physiotherapist and a medical rehabilitation specialist. All examiner is member of multidisciplinary Hemophiliac Management Team in Cipto Mangunkusumo General Hospital in Jakarta, Indonesia. Results The mean age of the participant was 9.3 (5–14) years old. The median score of HEAD-US was 8 (1–28) with most of the joint abnormalities found on the ankles. The median score of HJHS was 3 (0–35), with most joint abnormalities found on the knees. There was a moderate correlation between HEAD-US and HJHS score (p < 0.05, r = 0.65). Conclusion HEAD-US shows a moderate correlation to HJHS in hemophiliac patients who received episodic treatment. HEAD-US can provide additional value in the anatomical evaluation of the joint and could be complementary to HJHS in assessing the joint status in hemophilic patient

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