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Coagulation abnormalities in Legg-Calvé-Perthes disease

A Vosmaer1, R Rodrigues Pereira, J S Koenderman

  • 1Department of Orthopaedics, Ikazia Hospital, P.O. Box 5009, 3008 AA Rotterdam, the Netherlands. a.vosmaer@ikazia.nl

The Journal of Bone and Joint Surgery. American Volume
|January 6, 2010
PubMed

Insights

Thrombophilia, or a tendency to clot, is linked to Legg-Calvé-Perthes disease in children. Specific genetic mutations and protein levels increase the risk, particularly in males, suggesting a thrombotic component in its cause.

Area of Science:

  • Pediatric Orthopedics
  • Hematology
  • Genetics

Background:

  • Legg-Calvé-Perthes disease involves osteonecrosis of the proximal femoral epiphysis in children.
  • The exact cause is unknown, but vascular occlusions and hypercoagulable disorders are suspected.
  • This study investigates the role of thrombophilia in the etiology of Legg-Calvé-Perthes disease.

Purpose of the Study:

  • To evaluate the etiologic role of thrombophilia in pediatric Legg-Calvé-Perthes disease.
  • To determine the association between specific thrombophilic factors and the risk of developing the condition.

Main Methods:

  • A case-control study involving 169 pediatric patients diagnosed with Legg-Calvé-Perthes disease.
  • Inclusion of two control groups: 474 subjects from a venous thrombosis study and 38 children treated for asthma.
  • Analysis of protein C, protein S, factor VIII, fibrinogen levels, and factor V Leiden and prothrombin G20210A mutations.

Main Results:

  • Increased risk of Legg-Calvé-Perthes disease associated with factor V Leiden mutation (OR, 3.3), prothrombin G20210A mutation (OR, 2.6), elevated factor VIII (OR, 7.5), and protein S deficiency (OR, 2.8).
  • No significant association found between high fibrinogen or protein C deficiency and the disease.
  • Males exhibited a higher risk (OR, 2.4) and stronger effects of certain thrombophilic factors, with risk increasing with more coagulation abnormalities.

Conclusions:

  • The findings suggest a significant thrombotic component in the etiology of Legg-Calvé-Perthes disease.
  • Specific thrombophilic factors contribute to the risk, especially in males.
  • Further research into coagulation disorders is warranted for understanding and potentially managing the disease.
Abstract

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