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Prospective reevaluation of the association between thrombotic diathesis and legg-perthes disease

M Timothy Hresko1, Patricia A McDougall, Jed B Gorlin

  • 1Department of Orthopaedic Surgery, Harvard Medical School, Boston, Massachusetts 02115, USA. timothy.hresko@tch.harvard.edu

The Journal of Bone and Joint Surgery. American Volume
|September 5, 2002
PubMed

Insights

Screening for inherited thrombophilia in children with Legg-Perthes disease is not recommended, as common clotting pathway abnormalities are not associated with the condition. Elevated lipoprotein (a) may indicate future atherosclerosis risk.

Area of Science:

  • Pediatric Orthopedics
  • Hematology
  • Genetics

Background:

  • Legg-Calvé-Perthes disease involves childhood hip ischemia.
  • Thrombophilia is a suspected, yet unproven, cause.
  • Screening for inherited thrombophilia in affected children has been advised.

Purpose of the Study:

  • To evaluate the diagnostic value of screening for inherited thrombophilia in pediatric Legg-Perthes disease patients.
  • To investigate the association between Legg-Perthes disease and thrombotic pathway abnormalities.

Main Methods:

  • Prospective enrollment of consecutive pediatric Legg-Perthes disease patients.
  • Assays for factor-V Leiden mutation, protein C, protein S, antithrombin III, and lipoprotein (a) levels.
  • Comparison of patient results with pooled normal control plasma.

Main Results:

  • No significant difference in the prevalence of protein C, protein S, antithrombin III deficiencies, or factor-V Leiden mutation compared to population frequencies.
  • Elevated lipoprotein (a) levels (>30 mg/dL) were observed in 16% of pediatric patients.

Conclusions:

  • Deficiencies in protein C, protein S, antithrombin III, and factor-V Leiden mutation are unlikely to be primary causes of Legg-Perthes disease.
  • Elevated lipoprotein (a) in children with Legg-Perthes disease may signal an increased risk for adult-onset atherosclerosis.
Abstract

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