Co-Existent Pseudoxanthoma Elasticum and Vitamin K-Dependent Coagulation Factor Deficiency

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...

Pseudoxanthoma elasticum

Pseudoxanthoma elasticum (PXE) is a genetic metabolic disease with autosomal recessive inheritance caused by mutations in the ABCC6 gene. The lack of functional ABCC6 protein leads to ectopic mineralization that is most apparent in the elastic tissues of the skin, eyes and blood vessels. The clinical prevalence of PXE has been estimated at between 1 per 100,000 and 1 per 25,000, with slight fem...

Pseudoxanthoma elasticum

Treatment of vitamin K-dependent coagulation factor deficiency and subarachnoid hemorrhage.

BACKGROUND In adults, vitamin K-dependent coagulation factor deficiency (VKCFD) increases in the recent years. We treated a VKCFD patient with subarachnoid hemorrhage, with favorable outcomes. METHODS A 19-year-old male student with VKCFD was treated at our hospital. The initial treatment was injection of a large dose of vitamin K and fresh plasma, and then with oral high dose of vitamin K4. ...

Vitamin K does not prevent soft tissue mineralization in a mouse model of pseudoxanthoma elasticum.

Pseudoxanthoma elasticum (PXE) is a heritable disease characterized by calcified elastic fibers in cutaneous, ocular, and vascular tissues. PXE is caused by mutations in ABCC6, which encodes a protein of the ATP-driven organic anion transporter family. The inability of this transporter to secrete its substrate into the circulation is the likely cause of PXE. Vitamin K plays a role in the regula...