Deficiency of Factor XIII is rare and results in a bleeding disorder with a number of findings that may suggest the diagnosis.

Inheritance of hereditary deficiency: autosomal recessive


Clinical findings:

(1) bleeding from the umbilicus in the neonatal period

(2) delayed separation of the umbilical cord

(3) delayed bleeding 12-36 hours after trauma

(4) poor wound healing

(5) unexplained and repeated intracranial hemorrhage

(6) hemarthrosis

(7) recurrent fetal loss in females


Laboratory findings:

(1) normal PT and PTT

(2) abnormal clot solubility in 5 M urea or a weak organic acid

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