ArticleBlood transfusion = Trasfusione del sangue2020
Physical activity improved by adherence to prophylaxis in an Italian population of children, adolescents and adults with severe haemophilia A: the SHAPE Study.
Article in Blood transfusion = Trasfusione del sangue, 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 15 papers, 4 of them syntheses that pooled it.
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Who cites it
15 citing papers in PubMed, 4 syntheses or guidelines pooled it, 23 citations in OpenAlex.
- Clotting factor concentrates for preventing bleeding and bleeding-related complications in previously untreated or minimally treated children with hemophilia A or B.The Cochrane database of systematic reviews · 2025Pooled it
- Systematic Literature Review of Outcomes Associated With Adherence to Haemophilia Drug Therapy.Haemophilia : the official journal of the World Federation of Hemophilia · 2025Pooled it
- A systematic review of physical activity in people with haemophilia and its relationship with bleeding phenotype and treatment regimen.Haemophilia : the official journal of the World Federation of Hemophilia · 2021Pooled it
- Factor VIII replacement prophylaxis in patients with hemophilia A transitioning to adults: a systematic literature review.Orphanet journal of rare diseases · 2021Pooled it
- Long-Term Efficacy and Safety of Damoctocog Alfa Pegol Prophylaxis in Patients with Haemophilia A Aged 12-<18 Years at Enrolment into PROTECT VIII.Acta haematologica · 2025Trial
- Real-world adherence to and persistence with emicizumab in people with hemophilia A using a national claims database.Journal of managed care & specialty pharmacy · 2026Observational
- Final data from the German AHEAD study: effectiveness and safety of octocog alfa and rurioctocog alfa pegol for hemophilia A in a real-world setting.Therapeutic advances in hematology · 2026Article
- Disease burden and unmet medical need in severe hemophilia in Greece: insights from clinicians and patients.Frontiers in public health · 2026Article
- Assessment of Clinical Characteristics and Sociocontextual Factors on Medication Adherence in Children and Adolescents With Hemophilia.Pediatric blood & cancer · 2025Article
- The haemophilia joint health score for the assessment of joint health in patients with haemophilia.Haemophilia : the official journal of the World Federation of Hemophilia · 2024Review
- Noninterventional study assessing joint health in persons with hemophilia A after switching to turoctocog alfa pegol: design of pathfinderReal.Research and practice in thrombosis and haemostasis · 2024Article
- The role of point-of-Care Musculoskeletal Ultrasound for Routine Joint evaluation and management in the Hemophilia Clinic - A Real World Experience.BMC musculoskeletal disorders · 2022Article
- Current therapeutic approaches in the management of hemophilia-a consensus view by the Romanian Society of Hematology.Annals of translational medicine · 2021Review
- A Delphi Consensus Approach for Difficult-to-Treat Patients with Severe Hemophilia A without Inhibitors.Journal of blood medicine · 2021Article
- Asymptomatic Joint Bleeding and Joint Health in Hemophilia: A Review of Variables, Methods, and Biomarkers.Journal of blood medicine · 2021Review
Corrections and comments
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Authors and funding
18 authors at 2 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundPhysical activity in people with haemophilia (PWH) reduces the development of severe arthropathy, but it must be performed after regular, proper prophylaxis. Strict adherence to treatment is crucial to achieving effectiveness and established outcomes. The primary aim of this study was to collect prospective data on adherence to prophylaxis for over 36 months. A secondary aim was to verify whether adherence correlates with physical activity. MATERIALS AND
methodsItalian patients with severe haemophilia A treated on prophylaxis with octocog alfa were included in the study. Physical findings were assessed by the Haemophilia and Exercise Project (HEP)-Test-Q and the Early Prophylaxis Immunologic Challenge (EPIC)-Norfolk Physical Activity Questionnaire; orthopaedic status was assessed by the Hemophilia Joint Health Score (HJHS). Adherence was measured as percentage of empty vials returned with respect to the prescribed amount.
resultsForty-two PWH were enrolled: 31% children, 21.4% adolescents, and 47.6% adults. Type, frequency and impact of physical activities differed among the three groups. The HEP-Test-Q showed the highest impairments in the domains "endurance" and "strength/co-ordination". Eight percent of patients were classified as adherent to prophylaxis. Among them, 50% had at least one bleeding episode in the year before enrolment; this percentage dropped during the three years of the study. While remaining stable in the "non-adherent" group, the HJHS score decreased in the "adherent" patients. The mean number of school/work days lost was lower in adherent patients (from 3.4±6.8 to 0.2±0.9) than in non-adherent ones. DISCUSSION: PWH with better orthopaedic scores reported better physical performance. Adherence to long-term prophylaxis proved to be high and correlated with a reduction in bleeds, target joints, school/work days lost, and with a performance improvement in endurance sports activities over time.
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