Very rare. Autosomal recessive transmission of a mutation of the F13B (1q31.3) or the F13A gene (6p25.1). Factor XIII is necessary for the stabilisation of the clot formed by fibrin. The clinical consequences are similar to severe hemophilia: bleeding of the umbilical stump after the fall of the umbilical cord, cerebral hemorrhage, hemarthroses, deep bruises. The classic coagulation tests (INR, APTT and TT) are normal.

Anesthetic implications: 

seek the advice of a specialist in hemostasis to improve hemostasis using concentrated factors from human plasma or recombinant factor XIII (NovoThirteen®)

References : 

-         Inbal A, Oldenburg J, Carcao M et al. 
Recombinant factor XIII : safe and novel treatment for congenital factor XIII deficiency. 
Blood 2012 ; 119 : 5111-7.

-        Kumar R, Carcao M. 
Inherited abnormalities of coagulation. 
Pediatr Clin N Am 2013; 60: 1419-41.

Updated: May 2019