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The Journal of Bone and Joint Surgery (American) 84:1613-1618 (2002)
© 2002 The Journal of Bone and Joint Surgery, Inc.

Scientific Article

Prospective Reevaluation of the Association Between Thrombotic Diathesis and Legg-Perthes Disease

M. Timothy Hresko, MD, Patricia A. McDougall, MD, Jed B. Gorlin, MD, Eleftherios C. Vamvakas, MD, PhD, James R. Kasser, MD and Ellis J. Neufeld, MD, PhD

Investigation performed at the Department of Orthopaedic Surgery and the Division of Hematology, Children's Hospital, Harvard Medical School, Boston, Massachusetts

M. Timothy Hresko, MD
James R. Kasser, MD
Ellis J. Neufeld, MD, PhD
Department of Orthopaedic Surgery (M.T.H. and J.R.K.) and Division of Hematology (E.J.N.), Children's Hospital, 300 Longwood Avenue, Boston, MA 02115. E-mail address for M.T. Hresko: timothy.hresko{at}tch.harvard.edu E-mail address for J.R. Kasser: james.kasser@tch.harvard.edu. E-mail address for E.J. Neufeld: ellis.neufeld@tch.harvard.edu

Patricia A. McDougall, MD
Department of Orthopaedic Surgery, Prevea Clinic, 900 South Webster Avenue, Green Bay, WI 54301

Jed B. Gorlin, MD
Memorial Blood Centers of Minnesota, 2304 Park Avenue, Minneapolis, MN 55404. E-mail address: jed@mbcm.org

Eleftherios C. Vamvakas, MD, PhD
New York University Medical Center Blood Bank, RRG-17, 400 East 34th Street, New York, NY 10016. E-mail address: stephen.vamvakas@med.nyu. edu

In support of the research or preparation of this manuscript, E.J. Neufeld received grant K24HL04184 from the National Institutes of Health. None of the authors received payments or other benefits or a commitment or agreement to provide such benefits from a commercial entity. No commercial entity paid or directed, or agreed to pay or direct, any benefits to any research fund, foundation, educational institution, or other charitable or nonprofit organization with which the authors are affiliated or associated

Background: Legg-Perthes disease is associated with ischemia of the capital femoral epiphysis in children. Thrombophilia has been implicated as a potential cause of the condition, and screening of patients with Legg-Perthes disease for thrombophilia has been recommended. We analyzed the value of screening for inherited thrombophilia in patients with Legg-Perthes disease by examining the association between Legg-Perthes disease and abnormalities in the thrombotic pathway.

Methods: A random series of consecutive patients with Legg-Perthes disease were prospectively enrolled in this study. Assays for the detection of factor-V Leiden mutation and the plasma concentrations of protein C, protein S, antithrombin III, and lipoprotein (a) were performed on plasma samples from children with Legg-Perthes disease, and the results were compared with those for pooled plasma from normal controls. Plasma concentrations below the 95% midrange of the control values were classified as protein deficiencies. The estimated population frequency of each coagulation abnormality was compared with the proportion of the study group with the corresponding abnormality.

Results: The proportion of abnormalities observed in the study group did not differ from the estimated population frequency for protein C, protein S, antithrombin III, or factor-V Leiden mutation. A lipoprotein (a) level of >30 mg/dL (>1.07 µmol/L) was found in 16% of the study group.

Conclusions: Our data do not suggest that thrombotic diatheses due to deficiency of protein C, protein S, or antithrombin III or due to factor-V Leiden mutation are major causes of Legg-Perthes disease. The elevated levels of lipoprotein (a) in children with Legg-Perthes disease suggest that they may be at risk for atherosclerosis as adults.


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