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The role of coagulation abnormalities in the development of Perthes' disease
W D Kealey1, E E Mayne, W McDonald
1Department of Orthopaedic Surgery, Musgrave Park Hospital, Belfast, Northern Ireland, UK.
Insights
Perthes
Area of Science:
- Pediatric Orthopedics
- Hematology
Background:
- Perthes' disease is a hip condition in children.
- Previous studies suggested a link between Perthes' disease and thrombophilia or hypofibrinolysis.
Purpose of the Study:
- To investigate the association between thrombophilia and Perthes' disease in a Northern Ireland cohort.
- To determine if antithrombotic factor deficiencies or activated protein C resistance are etiological factors.
Main Methods:
- A case-control study comparing 139 children with Perthes' disease to 220 healthy children.
- Assays for antithrombotic factors (protein C, protein S, antithrombin III) and activated protein C resistance were performed.
- Activated partial thromboplastin time was measured in both groups.
Main Results:
- No significant deficiencies in antithrombotic factors or activated protein C resistance were found in children with Perthes' disease.
- A significantly higher proportion (38.1%) of children with Perthes' disease had a prolonged activated partial thromboplastin time compared to controls (5.9%).
- This suggests thrombophilia due to factor deficiency is not the primary cause of Perthes' disease.
Conclusions:
- Thrombophilia linked to antithrombotic factor deficiency or activated protein C resistance is unlikely to be an etiological factor for Perthes' disease.
- The cause of the prolonged activated partial thromboplastin time in Perthes' disease patients requires further investigation.
Abstract:
Recent reports have suggested an association between Perthes' disease and an underlying thrombophilic or hypofibrinolytic tendency. In Northern Ireland there is a high incidence of Perthes' disease (11.7 per 100,000 or 1 in 607 children) in a stable paediatric population. We reviewed 139 children with Perthes' disease and compared them with a control group of 220 aged- and gender-matched healthy primary schoolchildren with similar racial and ethnic backgrounds. There were no significant deficiencies of antithrombotic factors protein C, protein S, antithrombin III or resistance to activated protein C. A total of 53 (38.1%) of the children with Perthes' disease had a prolonged activated partial thromboplastin time (>38) compared with 13 (5.9%) of the control group (p < 0.001). Our findings have shown that using standard assays, thrombophilia secondary to antithrombotic factor deficiency or resistance to activated protein does not appear to be an aetiological factor for Perthes' disease. The cause of the prolonged activated partial thromboplastin time, usually associated with a clotting factor deficiency, is under further investigation.
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