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Congenital factor VII deficiency

ORPHA:327· ICD-10 D68.2

Definition

A rare, genetic, congenital vitamin K-dependant coagulation factor deficiency disorder characterized by decreased levels or absence of coagulation factor VII (FVII), resulting in bleeding diathesis of variable severity.

Prevalence
1-9 / 1 000 000
Inheritance
Autosomal dominant, Autosomal recessive
Age of onset
All ages