Familial deficiency of vitamin K-dependent clotting factors

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Familial deficiency of vitamin K-dependent clotting factors.

Combined deficiency of vitamin K-dependent clotting factors II, VII, IX and X (and proteins C, S, and Z) is usually an acquired clinical problem, often resulting from liver disease, malabsorption, or warfarin overdose. A rare inherited form of defective gamma-carboxylation resulting in early onset of bleeding was first described by McMillan and Roberts in 1966 and subsequently has been termed '...

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Hereditary combined deficiency of the vitamin K-dependent clotting factors

Hereditary combined vitamin K-dependent clotting factors deficiency (VKCFD) is a rare congenital bleeding disorder resulting from variably decreased levels of coagulation factors II, VII, IX and X as well as natural anticoagulants protein C, protein S and protein Z. The spectrum of bleeding symptoms ranges from mild to severe with onset in the neonatal period in severe cases. The bleeding sympt...

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A case report of temporary and acquired vitamin K-dependent clotting factor deficiency without any response to administrated vitamin K

Abstract Background and Objectives Vitamin K-dependent clotting factor deficiency (VKCFD) is usually an acquired problem due to liver disease, malabsorption, and overdose of warfarin.  In the present paper the significance and role of vitamin K-dependent coagulation factors in menorrhagia were evaluated.   Case We present a rare case of 43 year woman with acquired vitamin K deficiency and se...

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Procoagulant Vitamin K-dependent Clotting Factors

We have evaluated the contribution of depression of individual procoagulant vitamin K-dependent clotting factors to the ability of warfarin to protect rabbits against tissue factor-induced coagulation. Mean activities of individual procoagulant factors were determined, in assays with rabbit substrates, for a group of rabbits achieving a protective degree of anticoagulation with warfarin. Values...

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Structure-function relationships of epidermal growth factor modules in vitamin K-dependent clotting factors.

ODULES* homologous to the epidermal growth M factor (EGF) precursor have been found in a large number of proteins with diverse functions. Among these proteins are the vitamin K-dependent clotting factors VII, IX, and X, proteins C and S of the protein C anticoagulant system, and the nonvitamin-K-dependent proteins urokinase (uPA), tissue-type plasminogen activator (tPA), and factor XI1 (Fig l)....

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ژورنال

عنوان ژورنال: Haemophilia

سال: 2008

ISSN: 1351-8216

DOI: 10.1111/j.1365-2516.2008.01853.x