Registry Study of COAGADEX® Patients With Moderate or Severe Hereditary Factor X Deficiency Undergoing Major Surgery

The primary objective is to collect additional surgical data on the clinical effectiveness of
Coagadex, in a post-marketing environment, for peri-operative hemostatic cover during major
surgery in patients with moderate or severe hereditary factor X (FX) deficiency.

The secondary objective is to review safety data on Coagadex through the collection of any
Adverse Drug Reactions (ADRs) from the first dose of Coagadex in the hospital prior to the
surgical procedure until the first follow-up after discharge (i.e., follow-up completed) and
Serious ADRs up until the post-operative care follow-up has been completed. Data on any
deaths and pregnancies reported within this time period will be collected.

Inclusion Criteria:

- Patients >12 years of age with severe or moderate hereditary FX deficiency. Severe is
defined as <1 IU/dL and moderate as 1 to 5 IU/dL (3)

- Patients who have given written informed consent (for sites in the USA this will
include the Health Insurance Portability and Accountability Act (HIPAA) authorization
statement), OR, in the case of 12-18 year olds, written informed consent from the
parents/ guardians and where appropriate assent from the child

- Patients requiring major surgery. Major surgery is defined as procedures typically
requiring:

- full anesthesia or regional anesthesia, e.g. epidural or spinal and

- involving the opening of major cavities such as thoracic, abdominal surgery;
orthopedic surgery, and Caesarean section (C-section) and

- requiring at least one overnight stay in hospital (16)

Exclusion Criteria:

- Patients known to be pregnant, unless the surgery is C-section.

- Patients who participated in a clinical study trial in the last 30 days prior to study
enrolment, except if they have been involved in another study involving Coagadex.

- Patients with a known history of inhibitor development to FX.

- Patients who are required or expected to take other factor X-containing medications
during or after surgery.

- Patients with existing known thrombocytopenia (platelets < 50 x 109/L).

- Patients with existing known clinically significant renal disease (creatinine >200
µmol/L).

- Patients with existing known clinically significant liver disease (ALT levels greater
than three times the upper limit of normal).

- Patients with existing known other coagulopathy or thrombophilia.

- Patients with a known intolerance or allergy to Coagadex or its excipients.

- Patients known to have abused chemicals or drugs within the past 12 months.

- Patients with a history of unreliability or non-cooperation.