Coagulation and fibrinolytic disturbances are related to carotid intima thickness and arterial blood pressure in Turner syndrome.

OBJECTIVE

Turner syndrome (TS) is characterized by growth retardation, hypogonadism and a high risk of cardiovascular complications and atherosclerosis; case reports suggest that thrombo-embolic complications may be present.

DESIGN

Cross-sectional study.

PATIENTS: Sixty women with TS.

MEASUREMENTS: We characterized the activities of the haemostatic system, elucidated by the assessment of a panel of clotting factors and thrombosis risk factors and related these findings to carotid intima thickness (CIMT) and blood pressure.

RESULTS

Most (81%) received hormone replacement therapy. The medians of all measured factors and inflammatory parameters were not different from normative data, but many cases displayed values of C-reactive protein (CRP) (40%), fibrinogen (15%), fibrin D-dimer (15%), factor VIII (25%), von Willebrand factor (vWF) (15%), cholesterol and liver parameters that were greater than normative limits. CRP, fibrinogen, vWF, factor VIII and liver parameters were highly and positively correlated. Haemostatic variables were positively related to both CIMT and blood pressure. The Factor V Leiden G1691A gene polymorphism heterozygosity was detected in 12·5%.

CONCLUSION

We describe a significant proportion of individual TS females having high levels of vWF, factor VIII, fibrinogen and CRP (15-40%) and an increased frequency of the Leiden mutation, with important associations with CIMT and blood pressure, suggesting that a subset of TS may have an unfavourable haemostatic balance, which may contribute to the increased risk of premature ischaemic heart disease and possibly increase the risk of deep venous and portal vein thrombosis.