CASE REPORT: Inherited Factor XIII Deficiency ; Significance of Diagnosis

Abstract

Abstract: Factor XIII deficiency is a rare disorder characterized by difficult to control bleeding following trauma or occurring spontaneously. The defect is either inherited or acquired. Autosomal recessive inherited deficiency of Factor XIII is more common in Pediatrics. The condition is diagnosed on high index of suspicion in the presence of a typical history and a normal coagulation profile. Incidence of this rare condition is 1 in 2-3 million live births. A case of an eight year old boy is presented here, admitted in our hospital with a history of difficult to control scalp bleeding following trivial trauma.

Key words: Factor XIII deficiency, umbilical stump bleeding (omphalorrhagia), intracranial bleed