FVIII inhibitors will be determined based on clinical observations and confirmed by FVIII inhibitor testing in the laboratory.

FVIII inhibitor testing could be carried out at any time at the physician's discretion. Patients were checked for clinical symptoms suggesting FVIII inhibitor development, any suspicion of inhibitor formation was to be investigated by FVIII inhibitor testing. No symptoms led to suspicion of inhibitor formation in any patient treated with Nuwiq®. Within the FAS population, inhibitor levels were tested in 40 patients at screening, 44 between screening and completion and 21 at study completion.

Adverse drug reactions (ADRs) including hypersensitivity reactions will be recorded by patients in treatment diaries which will be reviewed at each Follow-up Visit.

Total number of bleeding episodes under prophylaxis treatment divided by the duration of prophylactic phase (in years)

Of the 77 participants within the prophylactic treatment group, 74 patients had at least 3 months under a prophylactic regimen and had at least one bleeding episode and were analyzed.

At the end of a BE, treatment efficacy was to be assessed either by the patient (or the patient's parent or legal guardian) or by the treating physician in case of on-site treatment using a 4-point scale including the four items 'excellent,' 'good,' moderate,' and 'none.' Excellent result was defined as abrupt pain relief and/or unequivocal improvement in objective signs of bleeding within approximately 8 hours after a single injection. Good was definite pain relief and/or improvement in signs of bleeding within approximately 8-12 hours after an injection requiring up to 2 injections for complete resolution. Moderate was probable or slight beneficial effect within approximately 12 hours after the first injection requiring more than two injections for complete resolution. None was no improvement after 12 hours, or worsening of symptoms, requiring more than 2 injections for complete resolution.

At the end of the postoperative period, an overall assessment of the efficacy of treatment in the pre-, peri-, and postoperative periods using the 'excellent,' 'good,' moderate,' and 'none' scale will be done jointly by the surgeon and the hematologist. Based on this assessment, efficacy ratings assessed as either 'excellent' or 'good' will be considered 'successfully treated'.

A total of 4 patients had 6 surgeries that were treated with Nuwiq®. Two of these surgeries were minor and 4 were major. Five surgeries had an overall efficacy assessment performed jointly by the hematologist and surgeon. One of the six surgeries was treated pre-op with another FVIII product and post-op with Nuwiq® and therefore an assessment of Nuwiq® efficacy could not be performed

At the end of a BE, treatment efficacy was to be assessed either by the patient (or the patient's parent or legal guardian) or by the treating physician in case of on-site treatment using a 4-point scale including the four items 'excellent', 'good', 'moderate', and 'none.'

Excellent result was defined as abrupt pain relief and/or unequivocal improvement in objective signs of bleeding within approximately 8 hours after a single injection. Good was definite pain relief and/or improvement in signs of bleeding within approximately 8-12 hours after an injection requiring up to 2 injections for complete resolution. Moderate was probable or slight beneficial effect within approximately 12 hours after the first injection requiring more than two injections for complete resolution. None was no improvement after 12 hours, or worsening of symptoms, requiring more than 2 injections for complete resolution.

Analysis was only performed on patients in the prophylactic treatment group who experienced bleeding episode that required treatment with Nuwiq. Of the 77 patients in the prophylactic treatment group, 48 patients experienced a BE that required treatment with Nuwiq and were assessed using the 4-point efficacy scale