{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,4]],"date-time":"2026-06-04T14:08:39Z","timestamp":1780582119930,"version":"3.54.1"},"reference-count":23,"publisher":"Springer Science and Business Media LLC","issue":"1","license":[{"start":{"date-parts":[[2024,3,26]],"date-time":"2024-03-26T00:00:00Z","timestamp":1711411200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2024,3,26]],"date-time":"2024-03-26T00:00:00Z","timestamp":1711411200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Orphanet J Rare Dis"],"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>Low-dose pharmacokinetic (PK)-guided extended half-life (EHL) factor VIII (FVIII) prophylaxis can reduce the bleeding risk in hemophilia A (HA) patients. An increase in physical activities for promoting musculoskeletal health may enhance the benefits of prophylactic therapy.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Objectives<\/jats:title>\n                    <jats:p>To determine the clinical impact of moderate- to vigorous-intensity physical activities in HA patients during low-dose PK-guided EHL FVIII prophylaxis.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Patients\/Methods<\/jats:title>\n                    <jats:p>This prospective study enrolled patients with moderate\/severe HA (baseline FVIII levels\u2009\u2264\u20095 IU\/dL) who had received low-dose PK-guided EHL FVIII prophylaxis for \u2265\u20096 months. An individualized exercise protocol was introduced to each participant, targeting a 65% increase in the maximum predicted heart rate for \u2265\u2009150\u00a0min\/week, while continuing low-dose PK-guided EHL FVIII prophylaxis for 6 months. Before and after implementing the intervention, annualized bleeding rates (ABR), annualized joint bleeding rates (AJBR), Hemophilia Joint Health Scores (HJHS), skeletal muscle mass, hemophilia-specific quality-of-life (QoL) scores and annualized FVIII consumption were compared.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      Of 13 participants (mean age\u2009\u00b1\u2009standard deviation [SD]: 20.1\u2009\u00b1\u20096.8 years), ABR, AJBR, and HJHS were significantly reduced (mean differences [MD]\u2009\u00b1\u2009SD: \u22125.7\u2009\u00b1\u20092.6 bleeds\/year, \u22124.2\u2009\u00b1\u20092.6 joint bleeds\/year, and \u22124.3\u2009\u00b1\u20093.2 marks, respectively;\n                      <jats:italic>P<\/jats:italic>\n                      \u2009&lt;\u20090.05) after applying the 6-month exercise protocol. Skeletal muscle mass and QoL scores had also improved (\n                      <jats:italic>P<\/jats:italic>\n                      \u2009=\u20090.001), while FVIII usage had decreased (MD\u2009\u00b1\u2009SD: \u2212129.1\u2009\u00b1\u2009208.7 IU\/kg\/year;\n                      <jats:italic>P<\/jats:italic>\n                      \u2009&lt;\u20090.05).\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>The combination of moderate- to vigorous-intensity physical activities with low-dose PK-guided EHL FVIII prophylaxis improves bleeding prevention, musculoskeletal status and QoL in patients with moderate\/severe HA. By minimizing FVIII consumption, this strategy helps optimize hemophilia care in countries with budget constraints. ClinicalTrials.gov NCT05728528.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1186\/s13023-024-03092-2","type":"journal-article","created":{"date-parts":[[2024,3,26]],"date-time":"2024-03-26T09:04:09Z","timestamp":1711443849000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":5,"title":["Moderate- to vigorous-intensity physical activities for hemophilia A patients during low-dose pharmacokinetic-guided extended half-life factor VIII prophylaxis"],"prefix":"10.1186","volume":"19","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-1951-4365","authenticated-orcid":false,"given":"Chonlatis","family":"Srichumpuang","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Arunothai","family":"Rakmanotham","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6257-0867","authenticated-orcid":false,"given":"Chatphatai","family":"Moonla","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1097-3482","authenticated-orcid":false,"given":"Darintr","family":"Sosothikul","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2024,3,26]]},"reference":[{"issue":"9","key":"3092_CR1","doi-asserted-by":"publisher","first-page":"2112","DOI":"10.1111\/jth.15444","volume":"19","author":"R Gualtierotti","year":"2021","unstructured":"Gualtierotti R, Solimeno LP, Peyvandi F. 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