Prevalence of clinical hip abnormalities in haemophilia A and B: an analysis of the UDC database

Authors

  • D. Kelly,

    Corresponding authorCurrent affiliation:
    1. Division of Critical Care Medicine, Department of Anesthesia, Perioperative and Pain Medicine at Boston Children's Hospital
    • Weill Cornell Medical College, New York, NY,, USA
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  • Q. C. Zhang,

    1. Division of Blood Disorders, National Center on Birth Defects and Developmental Disabilities, CDC, Atlanta, GA, USA
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  • J. M. Soucie,

    1. Division of Blood Disorders, National Center on Birth Defects and Developmental Disabilities, CDC, Atlanta, GA, USA
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  • M. Manco-Johnson,

    1. Mountain States Regional Hemophilia and Thrombosis Center, University of Colorado, Denver and Children's Hospital, Aurora, CO, USA
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  • D. DiMichele,

    1. Weill Cornell Medical College, New York, NY,, USA
    Current affiliation:
    1. Division of Blood Diseases and Resources at the National Heart, Lung and Blood Institute
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  • the Joint Outcome Subcommittee of the Coordinating Committee for the Universal Data Collection Database and the Hemophilia Treatment Center Network Investigators


  • The findings and conclusions in this report are those of the authors and do not necessarily represent the official position of the Centers for Disease Control and Prevention.

Correspondence: Daniel Kelly, MD, Division of Critical Care Medicine, Department of Anesthesia, Perioperative and Pain Medicine, Boston Children's Hospital, 300 Longwood Avenue, Bader 620, Boston, MA 02115, USA.

Tel.: +(617) 355 7327; fax: (617) 730 0453;

e-mail: daniel.kelly@childrens.harvard.edu

Summary

Clinical hip abnormalities, secondary to recurrent joint and/or muscle bleeding in persons with haemophilia, have not been well characterized and have the potential for significant morbidity. We aimed to examine the prevalence of clinical hip abnormalities in the US haemophilia population and to explore associations between these findings and putative risk factors. We conducted a study of hip abnormalities of 8192 subjects aged 2–69 years with haemophilia A and haemophilia B (54% of haemophilia A and haemophilia B are severe) currently enrolled in the Universal Data Collection (UDC) database. Associations between hip abnormality and type/severity of haemophilia A/B, current age, history of high-titre (≥5 BU) inhibitor (HTinh), concomitant ankle (AA) and knee arthropathy (KA), overweight and obesity and prophylaxis were examined using logistic regression. Overall prevalence of hip abnormality at the last recorded UDC visit for all subjects was 16.7%. Haemophilia A (aOR = 1.3, 1.0–1.4), severe haemophilia (aOR = 1.3, 1.0–1.5), a history of HTinh (aOR = 1.4, 1.1–1.7), and concomitant AA (aOR = 1.7, 1.4–1.9) were each independently associated with hip abnormality. Older age (45–69 years) was significantly associated with hip abnormality prevalence only in subjects with KA (aOR = 3.4, 1.9–5.9). The presence of overweight (aOR = 1.4, 1.1–1.8) and obesity (aOR = 2.1, 1.6–2.8) was associated with hip abnormality only among subjects without KA. Hip abnormality prevalence was not influenced by prophylaxis (aOR = 0.9, 0.8–1.1). These data suggest that hip abnormalities in US patients with haemophilia are associated with haemophilia severity and type, HTinh, concomitant AA and, depending on the presence or absence of KA, advancing age and obesity.

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