Safety and Efficacy of Monthly Replacement Therapy With Recombinant Factor XIII (rFXIII) in Paediatric Subjects With Congenital Factor XIII A-subunit Deficiency: An Extension to Trial F13CD-3760/Mentor™4 (mentor™5)

• Frequency of development of anti-rFXIII antibodies, including inhibitors [ Time Frame: every 4th week, from week 0 to week 56 ] [ Designated as safety issue: No ]
• Clinical laboratory assessments: Biochemistry, haematology [ Time Frame: every 6th month, from 0 to week 56 ] [ Designated as safety issue: No ]
• Physical examinations [ Time Frame: every 4th week, from 0 to week 52 ] [ Designated as safety issue: No ]
• Vital signs (Blood Pressure) [ Time Frame: every 4th week, from week 0 to week 56 ] [ Designated as safety issue: No ]
• Vital signs (Pulse) [ Time Frame: every 4th week, from week 0 to week 56 ] [ Designated as safety issue: No ]
• Rate (number per subject year) of all bleeding episodes requiring treatment with a FXIII containing product other than recombinant factor XIII [ Time Frame: weeks 0-52 ] [ Designated as safety issue: No ]

• Frequency of development of anti-rFXIII antibodies, including inhibitors [ Time Frame: every 4th week, from week 0 to week 56 ] [ Designated as safety issue: No ]
• Clinical laboratory assessments: Biochemistry, haematology [ Time Frame: every 6th month, from 0 to week 56 ] [ Designated as safety issue: No ]
• Physical examinations [ Time Frame: every 4th week, from 0 to week 52 ] [ Designated as safety issue: No ]
• Vital signs (Blood Pressure) [ Time Frame: every 4th week, from week 0 to week 56 ] [ Designated as safety issue: No ]
• Vital signs (Pulse) [ Time Frame: every 4th week, from week 0 to week 56 ] [ Designated as safety issue: No ]
• Rate (number per subject year) of all bleeding episodes requiring treatment with a FXIII containing product other than recombiant factor XIII [ Time Frame: weeks 0-52 ] [ Designated as safety issue: No ]

This trial will be conducted in Asia, Europe and the United States of America (USA).

The aim of this clinical trial is to investigate long-term safety of rFXIII when administered for prevention of bleeding episodes in children aged between 1 and 6 years with congenital FXIII A-subunit deficiency. This trial is an extension to trial F13CD-3760 (NCT01230021). If applicable the trial will be extended up to maximum 3 years dependent on when recombinant factor XIII will be commercially available in subject's respective country for use in children of 1-6 years of age

• Congenital Bleeding Disorder
• Congenital FXIII Deficiency

Inclusion Criteria:

• Completed participation in trial F13CD-3760 (NCT01230021)

Exclusion Criteria:

• Known or suspected hypersensitivity to trial product or related products
• Known history of development of inhibitors against FXIII (factor XIII)
• Hereditary or acquired coagulation disorder other than FXIII congenital deficiency
• Platelet count (thrombocytes) less than 50X10e9 / L
• Previous history of autoimmune disorder involving autoantibodies e.g., systemic lupus erythematosus
• Previous history of arterial or venous thromboembolic events e.g., cerebrovascular accident or deep vein thrombosis
• Any disease or condition which, judged by the trial physician, could imply a potential hazard to the subject, interfere with the trial participation or trial outcome including renal and/or liver dysfunction