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Evaluation of anticoagulant system in Turkish children with Perthes disease
Deniz Yilmaz1, Levent Karapinar, Bulent Karapinar
1Ege University Faculty of Medicine, Department of Pediatrics, Izmir, Turkey. dyilmazk@yahoo.com
Insights
Pediatric Perthes disease (PD) is linked to inherited clotting disorders. Identifying these thrombotic risk factors may improve treatment and prevent future blood clots in affected children.
Area of Science:
- Pediatric Orthopedics
- Hematology
- Genetics
Background:
- Perthes disease (PD) is a childhood condition causing hip pain and potential femoral head deformity.
- Intravascular thrombosis is implicated in the pathogenesis of Perthes disease.
- Hereditary thrombotic risk factors are investigated in Turkish children with PD.
Purpose of the Study:
- To determine hereditary thrombotic risk factors in Turkish children diagnosed with Perthes disease.
- To explore the association between coagulation system abnormalities and PD.
- To evaluate the relationship between disease severity and coagulation parameters.
Main Methods:
- Investigated family history of thrombotic events in 46 PD patients.
- Measured Protein C (PC), free-Protein S (f-PS), antithrombin (AT) activities, fibrinogen, and activated Protein C (APC) resistance.
- Compared results with 79 age- and sex-matched healthy controls.
Main Results:
- PD patients showed significantly lower mean PC and AT activities compared to controls.
- Higher proportions of PD patients had low AT activity, APC resistance, and a history of hereditary thrombophilia.
- No correlation found between coagulation disorders and disease severity or laterality.
Conclusions:
- A potential association exists between Perthes disease and inherited hypercoagulability.
- Identifying thrombotic risk factors may offer novel treatment approaches for PD.
- Early detection can prompt precautions against future thrombotic events in PD patients.
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.
