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To compare clinical outcomes before and after using combination between moderate to vigorous-intensity physical activities and pharmacokinetic-guided extended half-life FVIII concentrates prophylaxis in moderate to severe hemophilia A patients
This is a prospective cohort study of severe hemophilia A patients receiving low-dose EHL FVIII concentrates with PK-guided EHL FVIII concentrates prophylaxis alone for 6 months. Additional exercise workshops from sport scientists were introduced individually to this group of patients along with using the same prophylaxis method for another period of 6 months. The annualized bleeding rates (ABR), the annualized joint bleeding rates (AJBR), the haemophilia-specific quality-of-life (Haemo-QoL-A) scores, the hemophilia Joint Health Scores (HJHS) and skeletal muscle mass were compared between these two periods
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| PK-guided EHL FVIII concentrates prophylaxis with moderate intensity physical activities | Experimental | PK-guided EHL FVIII concentrates prophylaxis with moderate intensity physical activities |
|
| PK-guided EHL FVIII concentrates prophylaxis alone | Active Comparator | PK-guided EHL FVIII concentrates prophylaxis alone |
|
| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| combination between moderate to vigorous-intensity physical activities and pharmacokinetic-guided extended half-life FVIII concentrates prophylaxis | Drug | combination between moderate to vigorous-intensity physical activities and pharmacokinetic-guided extended half-life FVIII concentrates prophylaxis |
| Measure | Description | Time Frame |
|---|---|---|
| annualized bleeding rates (ABR) | annualized bleeding rates (ABR) | 6 months |
| annualized joint bleeding rates (AJBR) | annualized joint bleeding rates (AJBR) | 6 months |
| haemophilia-specific quality-of-life (Haemo-QoL-A) scores | haemophilia-specific quality-of-life (Haemo-QoL-A) scores | 6 months |
| hemophilia Joint Health Scores (HJHS) | hemophilia Joint Health Scores (HJHS) | 6 months |
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Inclusion Criteria:
Exclusion Criteria:
-
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| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| King Chulalongkorn Memorial Hospital | Bangkok | 10330 | Thailand |
| PubMed Identifier | Type | Citation | Retractions |
|---|---|---|---|
| 38532451 | Derived | Srichumpuang C, Rakmanotham A, Moonla C, Sosothikul D. Moderate- to vigorous-intensity physical activities for hemophilia A patients during low-dose pharmacokinetic-guided extended half-life factor VIII prophylaxis. Orphanet J Rare Dis. 2024 Mar 26;19(1):135. doi: 10.1186/s13023-024-03092-2. |
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|
| pharmacokinetic-guided extended half-life FVIII concentrates prophylaxis alone | Drug | pharmacokinetic-guided extended half-life FVIII concentrates prophylaxis alone |
|
| ID | Term |
|---|---|
| D006467 | Hemophilia A |
| ID | Term |
|---|---|
| D025861 | Blood Coagulation Disorders, Inherited |
| D001778 | Blood Coagulation Disorders |
| D006402 | Hematologic Diseases |
| D006425 | Hemic and Lymphatic Diseases |
| D020147 | Coagulation Protein Disorders |
| D006474 | Hemorrhagic Disorders |
| D030342 | Genetic Diseases, Inborn |
| D009358 | Congenital, Hereditary, and Neonatal Diseases and Abnormalities |
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