A Study of Factor XIII Concentrate in Subjects With Congenital Factor XIII Deficiency

Inclusion Criteria: * Written informed consent/assent for study participation obtained before undergoing any study-specific procedures * Documented congenital FXIII deficiency which requires prophylactic treatment with a FXIII containing product. * Males and females of any age with congenital FXIII deficiency * Received full hepatitis B vaccination and/or is hepatitis B surface antibody positive Exclusion Criteria: * Diagnosis of acquired FXIII deficiency * Administration of a FXIII-containing product, including blood transfusions or other blood products within 4 weeks prior to the planned Day 0 * Any known congenital or acquired coagulation disorder other than congenital FXIII deficiency * Known or suspected to have antibodies towards FXIII * Use of any other investigational medicinal product within 4 weeks prior to the Baseline Visit (Day 0) * Known Positivity for human immunodeficiency virus (HIV) or a positive result for HIV at the Screening Visit of this study or the FXIII study 2002 (NCT00883090). * Serum aspartate transaminase (AST) or serum alanine transaminase (ALT) concentration \>2.5 times the upper limit of normal at the Screening Visit of this study or at the Day 56 Visit of Factor XIII Study BI71023\_2002 (NCT00883090) * Fibrinogen level less than 85% of the lower limit of normal at the Screening Visit of this study or the Factor XIII Study BI71023\_2002 (NCT00883090) * Active bleeding ≥ Common Terminology Criteria for Adverse Events (CTCAE) Grade 2 and/or ≥ moderate between the Screening and Baseline Visits * Pregnant or breast-feeding * Intention to become pregnant during the course of the study * Female subjects of childbearing potential not using, or not willing to use, a medically reliable method of contraception for the entire duration of the study * Suspected inability (e.g., language problems) or unwillingness to comply with study procedures or history of noncompliance