/congenital factor XIII deficiency

congenital factor XIII deficiency

Rare Disease

MONDO:0018029

Also Known As

fibrin-stabilizing factor deficiencydeficiency, Laki-Lorand factorfactor XIII deficiency diseasehereditary factor XIII deficiency diseasefactor XIII deficiencyfibrin stabilising factor deficiencyfibrin stabilizing factor deficiency

Definition

Congenital factor XIII deficiency is an inherited bleeding disorder due to reduced levels and activity of factor XIII (FXIII) and characterized by hemorrhagic diathesis frequently associated with spontaneous abortions and defective wound healing. Factor XIII deficiency is one of the most rare coagulation factor deficiencies.