Evaluation of Recombinant Factor XIII for Prevention of Bleeding in Patients With FXIII Inherited Deficiency

Inclusion Criteria: * Diagnosis of congenital FXIII A-subunit deficiency (confirmed by genotyping at screening visit) * Treatment with regular FXIII replacement therapy initiated at least 6 months prior to screening and one of the following : a documented history of at least one 1 treatment-requiring bleeding episode prior to initiation of regular replacement therapy or a documented family history of FXIII congenital deficiency (only for subjects on regular replacement therapy prior to screening) * Documented history of at least two 2 bleeding episodes requiring treatment with FXIII containing blood products within the last 12 months prior to screening (only for subjects receiving on-demand treatment prior to screening) Exclusion Criteria: * Known neutralizing antibodies (inhibitors) towards FXIII * Any known congenital or acquired coagulation disorder other than congenital FXIII deficiency * Documented history of at least 2 treatment-requiring bleeding episodes per year during previous regular replacement therapy with FXIII containing blood products (fresh frozen plasma (FFP), plasma-derived FXIII (pd FXIII) and cryoprecipitate) * Known or suspected allergy to trial product(s) or related products * Planned major surgery during the trial period. Catheter, ports and dental extractions do not count as surgeries and will not exclude the subject * Renal insufficiency defined as current dialysis therapy * Any history of confirmed venous or arterial thrombo-embolic events