Congenital factor XIII deficiency is an autosomal recessive disorder.

Caused by mutations in the genes that code for either FXIII subunit A or B (rarer).

Most common bleeding sites include umbilical stump, soft tissue, surgical and intracranial.

Poor wound healing is often present.

Measure FXIII activity.

PT, aPTT, and thrombin time are usually normal.
Genetic analysis to identify gene mutation.

Prophylaxis recommended especially with levels <10%