Recent studies have demonstrated that protein C deficiency is associated with recurrent familial thrombosis. In plasma, activated protein C functions as an anticoagulant. This anticoagulant response requires a vitamin K-dependent plasma protein cofactor, referred to as protein S. Since the anticoagulant activity of activated protein C is dependent on protein S, we hypothesized that patients lac...

Stroke in young is a major health problem in developing countries along with CAD, according to various Indian studies its prevalence is 25-34%. Thrombophilic disorders constitute aetiology in 60% cases of stroke of undetermined aetiology. A 20 yrs old young female presented with symptoms of left PCA thrombosis (P2 syndrome), on evaluation--Isolated Protein--S deficiency is noticed. In this case...

Vitamin K dependent protein S (ProS) is a plasma glycoprotein with anticoagulant properties [1]. Its anticoagulant function is well established in the literature based on clinical data, its genetics and on laboratory assays. ProS is able to prolong the clotting time acting as a cofactor for activated protein C. Furthermore, since the 1980’ies it is known that hereditary ProS deficiency (PSD) in...

A 23-year-old female presented with dural sinus thrombosis caused by protein S deficiency. She suffered superior sagittal sinus thrombosis 6 days after delivering her first child. Past history showed deep vein thrombosis at the age of 20. While conservative management was initiated because of the potential risk of increasing intracranial hemorrhage, several hours later she deteriorated rapidly ...

BACKGROUND Only a few mutations associated with qualitative protein S deficiency have already been described. Sensitivity and specificity for type II PROS1 mutations of commercially available reagents for measuring Protein S (PS) activity are not well established. Whether these mutations are significant risk factors for thrombosis remains an unresolved question. METHODS In order to address th...

We report a case of a 6-year-old girl with severe protein S deficiency due to a homozygous mutation and recurrent episodes of skin necrosis. She developed purpura fulminans at birth and a catheter-related venous thrombosis complicated by massive pulmonary embolism at the sixth day of life. Long-term oral anticoagulant therapy with a vitamin K-antagonist was started with a therapeutic range of t...