Joint status and related risk factors in patients with severe hemophilia A: a single-center cross-sectional study.

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  • Additional Information
    • Source:
      Publisher: Taylor & Francis Country of Publication: England NLM ID: 9708388 Publication Model: Print Cited Medium: Internet ISSN: 1607-8454 (Electronic) Linking ISSN: 10245332 NLM ISO Abbreviation: Hematology Subsets: MEDLINE
    • Publication Information:
      Publication: 2016- : Abingdon : Taylor & Francis
      Original Publication: [Amsterdam] : Newark, NJ : Harwood Academic Publishers ; International Publishers Distributor,
    • Subject Terms:
      Hemarthrosis/*epidemiology ; Hemarthrosis/*etiology ; Hemophilia A/*complications ; Hemophilia A/*epidemiology; Adolescent ; Adult ; Child ; Child, Preschool ; Cross-Sectional Studies ; Delayed Diagnosis ; Disease Management ; Disease Susceptibility ; Hemarthrosis/therapy ; Hemophilia A/diagnosis ; Hemophilia A/therapy ; Humans ; Male ; Risk Assessment ; Risk Factors ; Young Adult
    • Abstract:
      Objectives: Hemophilic arthropathy is the most common complication of severe hemophilia A. This study aims to investigated joint status and related risk factors in patients with severe hemophilia A (PWSHA).
      Methods: This single-center study included 31 patients. Six index joints (both elbows, knees, and ankles) were evaluated using the Hemophilia Early Arthropathy Detection with UltraSound in China (HEAD-US-C) and Hemophilia Joint Health Score (HJHS). Treatment adherence was measured using the Validated Hemophilia Regimen Treatment Adherence Scale-Prophylaxis (VERITAS-Pro). We analyzed the influence of age, treatment delay (the interval between diagnosis and the initiation of treatment), prophylaxis, and treatment adherence on joint outcomes.
      Results: All patients were male (median age, 22 years). The median age at diagnosis was 1 year; that at initial treatment was 5 years. All patients experienced joint bleeding. HEAD-US-C and HJHS scores were positively correlated (R = 0.70, P  < 0.0001). Median [range] HEAD-US-C and HJHS scores were 15 [0-36] and 32 [2-49], respectively. Age was positively correlated with both HEAD-US-C ( P  = 0.002) and HJHS scores ( P  < 0.0001). The difference of HEAD-US-C scores between groups with ≤1 year and >1 year treatment delay was close to significant ( P  = 0.055). HJHS scores were significantly different between these two groups ( P  = 0.03). Joint assessment scores were not significantly different between on-demand and low-dose prophylaxis groups. VERITAS-Pro scores were correlated with both HEAD-US-C and HJHS scores ( P  = 0.046 and P  = 0.005, respectively).
      Conclusions: Hemophilic arthropathy was pervasive in PWSHA. Age and poor adherence were significantly correlated with joint damage. Prompt treatment and adherence improvement may reduce severity.
    • Contributed Indexing:
      Keywords: Hemophilia A; hemarthrosis; joint diseases; medication adherence; prophylaxis; quality of life
    • Publication Date:
      Date Created: 20211229 Date Completed: 20220201 Latest Revision: 20220429
    • Publication Date:
      20240829
    • Accession Number:
      10.1080/16078454.2021.2019892
    • Accession Number:
      34964431