Vitamin K Dependent Clotting Factors Deficiency

Combined deficiency of vitamin k dependent clotting factors.

Vitamin k dependent clotting factors deficiency. 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. Acquired forms of the disorder can be caused by intestinal malabsorption of vitamin k. Hereditary combined deficiency of the vitamin k dependent coagulation factors is a rare bleeding disorder. Familial multiple coagulation factor deficiency is rare.

Clinical symptoms of the disease include episodes of intracranial hemorrhage in the first weeks of life. Hereditary combined vitamin k dependent clotting factors deficiency vkcfd is a rare inherited coagulation defect that forms part of a wider group of rare disorders named familial multiple coagulation factor deficiencies fmcfds. Vitamin k independent factors are in the normal range. 1 this reaction requires normal cycling of vitamin k from its reduced form to the oxidized state followed by regeneration of reduced vitamin k.

2 3 these reactions are accomplished via. Without the activation step certain biochemical reactions cannot be completed. Acquired forms of the disorder can be caused by intestinal malabsorption of vitamin k. The spectrum of bleeding symptoms ranges from mild to severe with onset in the neonatal period in severe cases.

Vitamin k plays a role in your blood clot formation and bone health. Many types of food contain vitamin k and it is rare to have a deficiency. Fmcfds are characterized by the simultaneous decrease in the levels of two or more coagulation factors. A rare inherited form of defective gamma carboxylation resulting in early onset o.

In order to continue the chain reaction of the coagulation cascade these four factors. Deficiency of vitamin k leads to decreased activity of vitamin k dependent coagulation factors ii vii ix x. Deficiency of all vitamin k dependent clotting factors leads to a bleeding tendency that is usually reversed by oral administration of vitamin k. Vitamin k is an essential cofactor in the activation of certain proteins within your body 1.

The function of each of the vitamin k dependent proteins vkdp depends upon normal gamma ƴ carboxylation of a number of glutamic acid residues in the amino terminal region of the proteins the gla domain. Inherited combined deficiency of the vitamin k dependent clotting factors vkcfd is a very rare inherited bleeding disorder that is caused by a problem with clotting factors ii vii ix and x. 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. Familial multiple coagulation factor deficiency is rare.

To date only eleven families have been reported in the literature.