Comparison of joint status in children with haemophilia A using ultrasound and physical examination

Aspdahl, Magnus; Viljakainen, Heli; Petrini, Pia; Ranta, Susanna

doi:10.1080/21679169.2018.1438511

Cited by 10 publications

(24 citation statements)

References 13 publications

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“…Strong correlations of the total HEAD‐US score for ankles and knees with the jBR for three years were derived. Klukowska et al observed similar correlation ( r = 0.73) between the lifetime cumulative haemarthroses and US‐cartilage defects in knees of children aged 2‐16 years with >30 joint bleeds, contrary to other studies conducted mostly on childhood cohorts treated with regular prophylaxis, which demonstrated either weak correlations between bleeding episodes in the index joints and HEAD‐US score, or only a trend ( P = 0.05) for positive correlation . Our study showed no statistically significant correlation between HEAD‐US score in the elbow joints and the corresponding jBR.…”

Section: Outcome Data Related To Joint Bleeding Rate and Ultrasound Econtrasting

confidence: 61%

“…The scoring was based on three markers: synovitis (score 0-2), cartilage (score 0-4) and subchondral bone (score of 0-2) with a maximum score of eight points per joint. 4 The ultrasound examinations were conducted by the same medical doctor-paediatric haematologist, during his visits into the different haemophilia centres, on a single ultrasound scanner (type MyLab25 Gold; Esaote) with a linear probe (5)(6)(7)(8)(9)(10)(11)(12)(13)(14)(15)(16)(17)(18). Out of the 42 patients included in the study, 22% (n = 9) did not experience any haemarthroses for the observation period of 3 years.…”

Section: Dear Editormentioning

confidence: 99%

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Beyond the bleeding rates‐ HEAD‐US joint assessment in patients treated by late prophylaxis

et al. 2019

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Section: Outcome Data Related To Joint Bleeding Rate and Ultrasound Econtrasting

confidence: 61%

Section: Dear Editormentioning

confidence: 99%

Beyond the bleeding rates‐ HEAD‐US joint assessment in patients treated by late prophylaxis

et al. 2019

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“…Several studies have shown association between physical joint status and imaging techniques, whereas in other studies no association was found. 31 patients showed abnormal HEAD-US scores in healthy joints. Our findings for HEAD-US scores showed no significant difference between the two study groups at 0, 6 and 12 months, suggesting that the degree of joint changes detected by ultrasound did not affect joint function scored with HJHS.…”

Section: Discussionmentioning

confidence: 91%

“…Several studies have shown association between physical joint status and imaging techniques, whereas in other studies no association was found . Muca‐Perja et al found no correlation between ultrasound and orthopaedic joint score in patients of all ages and disease severity.…”

Section: Discussionmentioning

confidence: 96%

Comparison of the efficacy and safety of 12‐month low‐dose factor VIII tertiary prophylaxis vs on‐demand treatment in severe haemophilia A children

et al. 2019

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Introduction Prophylaxis has commonly become standard treatment for severe haemophilia patients. The World Federation of Hemophilia (WFH) recommends low‐dose prophylaxis in countries with resource constraints. Objective To determine efficacy and safety of low‐dose factor VIII (FVIII) tertiary prophylaxis compared to on‐demand treatment in severe haemophilia A children in Indonesia. Methods Eligible patients were randomly assigned to prophylaxis and on‐demand groups. Patients in the prophylaxis group received infusion of FVIII 10 IU/kg body weight, two times per week. Primary outcomes were the numbers of joint bleeding and total bleeding episodes; secondary outcomes were evidence of FVIII inhibitor, Hemophilia Joint Health Score (HJHS) and Hemophilia Early Arthropathy Detection Ultrasound (HEAD‐US) score. Patients were monitored for 12 months. Results Fifty patients, all with tertiary prophylaxis, 4‐18 years of age, were randomized into prophylaxis (n = 25) and on‐demand (n = 25) groups. The mean follow‐up time was 12.8 ± 0.86 vs 12.3 ± 0.54 months, respectively. Numbers of total and joint bleeding episodes were significantly lower in the prophylaxis group (P < 0.001, 95% CI −24.6;−10.7 and P < 0.001, 95% CI −14;−3, respectively). The prophylaxis group showed improvement of joint function (P = 0.004; CI 95% −3;−0.5); on the contrary, we found deterioration in the on‐demand group (P = 0.001; CI 95% 1;3). HEAD‐US scores showed improvement at month 6 in the prophylaxis group, but there was no significant difference between groups at month 12. Conclusion Low‐dose FVIII tertiary prophylaxis was effective in reducing joint bleeding episodes and improvement of HJHS compared to on‐demand FVIII treatment in severe haemophilia A children.

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“…Data on reliability 5,6 and validity 7 have supported the use among clinicians with limited ultrasound skills. In previous publications, HEAD‐US and HJHS have been considered as supplementary tools to evaluate joint health in haemophilia 8‐13 …”

Section: Introductionmentioning

confidence: 99%

Haemophilia early arthropathy detection with ultrasound and haemophilia joint health score in the moderate haemophilia (MoHem) study

et al. 2021

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Introduction Detection of early arthropathy is crucial for the management of haemophilia, but data on moderate haemophilia are limited. Therefore, we evaluated joint health and treatment modalities in Nordic patients with moderate haemophilia A (MHA) and B (MHB). Aim To explore and compare the Haemophilia Early Arthropathy Detection with Ultrasound (HEAD‐US) and Haemophilia Joint Health Score (HJHS) to detect early arthropathy in moderate haemophilia. Methods A cross‐sectional, multicentre study covering Nordic patients with MHA and MHB. Arthropathy was evaluated by HEAD‐US and HJHS 2.1. Results We assessed 693 joints in 118 patients. HEAD‐US scores (medians [interquartile ranges]) were as follows: elbows 0 points (0–0), knees 0 (0–0) and ankles 0 (0–1). Respectively, by HJHS: elbows 0 (0–1), knees 0 (0–1) and ankles 0 (0–1). Cartilage (14%) and bone (13%) were most commonly affected by HEAD‐US. Frequent HJHS findings were crepitus on motion in knees (39%), and loss of flexion (23%) and extension (13%) in ankles. HEAD‐US correlated strongly with HJHS (elbows r = .70, knees r = .60 and ankles r = .65), but 24% had discordant scores. Joints with HJHS zero points, 5% captured HEAD‐US ≥1 point. Moreover, 26% had HJHS findings without HEAD‐US pathology. Notably, 31% of knees had crepitus on motion and normal HEAD‐US. Conclusion Overall, the joints attained low scores implying good joint health. HEAD‐US correlated strongly with HJHS. In 5%, HEAD‐US detected subclinical pathology. Crepitus on motion was frequently reported despite normal HEAD‐US, thus not necessarily reflecting arthropathy. HEAD‐US therefore improves the joint assessment in moderate haemophilia.

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Comparison of joint status in children with haemophilia A using ultrasound and physical examination

Cited by 10 publications

References 13 publications

Beyond the bleeding rates‐ HEAD‐US joint assessment in patients treated by late prophylaxis

Beyond the bleeding rates‐ HEAD‐US joint assessment in patients treated by late prophylaxis

Comparison of the efficacy and safety of 12‐month low‐dose factor VIII tertiary prophylaxis vs on‐demand treatment in severe haemophilia A children

Haemophilia early arthropathy detection with ultrasound and haemophilia joint health score in the moderate haemophilia (MoHem) study

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