SCPCD - Key Management Information
Key information for the management of
severe congenital protein C deficiency (SCPCD)
Infants with SCPCD usually present within hours of birth with symptoms including:1,4,*
- Purpura fulminans
- Disseminated intravascular coagulation
- Retinal vessel thrombosis
- Venous thrombosis
- Cerebral venous thrombosis
Goldenberg N, Manco-Johnson M. Protein C deficiency. Haemophilia. 2008;14(6):1214–1221.
Price VE, et al. Diagnosis and management of neonatal purpura fulminans. Semin Fetal Neonatal Med. 2011;16(6):318-322.
Patracchini P, et al. Sublocalization of the human protein C gene on chromosome 2q13-q14. Hum Genet. 1989;81(2):191-192.
Chalmers E, et al. Purpura fulminans: recognition, diagnosis and management. Archives of Disease in Childhood. 2011;96(11):1066-1071.
Khor B, et al. Laboratory tests for protein C deficiency. Am. J. Hematol. 2010;85:440–442.
Libourel EJ, et al. Protein C/S ratio, an accurate and simple tool to identify carriers of a protein C gene mutation. British Journal of Haematology. 2002;118:615–618.
Kroiss S, Albisetti M. Use of human protein C concentrates in the treatment of patients with severe congenital protein C deficiency. Biologics: Targets & Therapy. 2010;4:51–60.