Evaluation of anticoagulant system in Turkish children with Perthes disease

BACKGROUND: Perthes Disease (PD) is generally a self-limiting disease of childhood but it causes severe pain and may lead to deformity of the femoral head. Intravascular thrombosis seems to form the main mechanism in the pathogenesis of the disease. The aim of this study was to determine hereditary thrombotic risk factors in Turkish children with PD. METHODS: In 46 Perthes patients (35 male, 11 female), family history of thrombotic events was investigated, Protein C (PC), free-Protein S (f-PS), antithrombin (AT) activities, fibrinogen level, and resistance to activated Protein C (APC) were measured. The results were compared with a healthy control group of 79 children matched by age and sex. The relationship between the severity of disease and coagulation system abnormalities was evaluated. RESULTS: While the mean PC and AT activities were significantly lower in the patients than those of the controls, the proportions of patients with low AT activity, resistance to APC, and a history of hereditary thrombophilia were significantly higher than those of the controls. No difference was observed in coagulation system disorders relative to severity of the disease and bilateral or unilateral disease involvement. CONCLUSIONS: This study shows that a possible association between PD and inherited hypercoagulability. Determination of thrombotic risk factors in these patients may bring a new approach to the treatment. Most importantly, this may be a stimulant to take precautions for other thrombotic events, which patients may face later in life.