Efficacy and Safety Study of Protein C Concentrate in Subjects With Severe Congenital Protein C Deficiency

Inclusion Criteria:

- Newborn subjects <= 6 months of age: diagnosis of severe congenital protein C
deficiency, with documented functional protein C level < 20%. If a genetic diagnosis
is not available prior to initiation of Protein C Concentrate treatment, a documented
family history of protein C deficiency is required.

- Subjects > 6 months of age: confirmed diagnosis of severe congenital protein C
deficiency, i.e., by

- a genetic analysis of severe congenital protein C deficiency (i.e., homozygous or
double heterozygous) OR

- a documented family history of protein C deficiency AND a documented functional
protein C level < 20% while the subject is in an asymptomatic state and not receiving
oral anticoagulation therapy

- Signed and dated informed consent from either the subject or the subject's legally
authorized representative prior to enrollment. Informed consent includes consent for
conducting a genetic analysis (if the genetic defect is unknown).

Exclusion Criteria:

- Subjects with a history of allergic reactions to Protein C Concentrate. In the case of
allergic reactions occurring at the Protein C Concentrate injection site; exclusion
from the study is at the discretion of the investigator but should be reported as an
AE regardless of the subject's inclusion in or exclusion from the study.

- Participation in any clinical study in which another investigational agent is used
within 30 days prior to enrollment or its use is expected at any time for the duration
of study participation.