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Blood, 1 April 2004, Vol. 103, No. 7, pp. 2467-2473.
Prepublished online as a Blood First Edition Paper on November 13, 2003; DOI 10.1182/blood-2003-05-1457.


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CLINICAL OBSERVATIONS, INTERVENTIONS, AND THERAPEUTIC TRIALS

Joint range-of-motion limitations among young males with hemophilia: prevalence and risk factors

J. Michael Soucie, Christy Cianfrini, Robert L. Janco, Roshni Kulkarni, Julie Hambleton, Bruce Evatt, Angela Forsyth, Sue Geraghty, Keith Hoots, Tom Abshire, Randall Curtis, Ann Forsberg, Heather Huszti, Margaret Wagner, and Gilbert C. White, II

From the Hematologic Diseases Branch, Division of AIDS, STD, and TB Laboratory Research, National Center for Infectious Disease, Centers for Disease Control and Prevention, Atlanta, GA; Vanderbilt University School of Medicine, Nashville, TN; Michigan State University, East Lansing, MI; the University of California, San Francisco, CA; Presbyterian Medical Center, Philadelphia, PA; Mountain States Regional Hemophilia and Thrombosis Center, Aurora, CO; the University of Texas Health Sciences Center, Houston, TX; Emory University School of Medicine, Atlanta, GA; the Department of Health Services, Berkeley, CA; Umass Memorial Health Care, Worcester, MA; Children's Hospital of Orange County, Orange, CA; Christiana Care Health System, Newark, DE; and the University of North Carolina School of Medicine, Chapel Hill, NC.

Chronic joint disease from repeated bleeding into joints is a serious complication of hemophilia. To measure the extent of and to identify risk factors for deviations from normal in joint range of motion (ROM), we used cross-sectional data collected from 4343 males with hemophilia aged 2 to 19 years who received care at 136 US hemophilia treatment centers (HTCs). Factors examined included age, race/ethnicity, family history, insurance status, age at diagnosis and first HTC visit, frequency of HTC visits, hemophilia type, bleeding frequency, prophylaxis use, inhibitor status, body mass index (BMI), and recent orthopedic procedures. Trained personnel using a standard protocol obtained ROM measurements on 10 joints (hips, knees, shoulders, elbows, and ankles). Analyses used multiple linear regression to model overall ROM limitation separately by disease severity. For persons in all severity groups, joint ROM limitation was positively associated with older age, nonwhite race, and increased BMI. For those with severe disease, ROM limitation was also positively associated with number of bleeds and was greater for those with inhibitors or recent orthopedic procedures. We conclude that ROM limitations begin at an early age, especially for those with severe and moderate disease, and that BMI is an important, potentially modifiable risk factor.


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