What is protein C deficiency?
Protein C deficiency can be caused by mutation of the protein C gene (congenital protein C deficiency) or as a result of other conditions (acquired protein C deficiency).1,2 The severity of the deficiency is determined by the remaining plasma activity of protein C.1
Severe Congenital Protein C Deficiency (SCPCD) is an autosomal recessive, rare disorder that leads to high initial mortality and long-term morbidity in survivors2. In neonates, SCPCD can manifest, within hours after birth, as purpura fulminans with necrosis of the skin and venous thrombosis.1-3
Which tests confirm SCPCD?Resources for your patients
The severity of protein C deficiency is based on protein C activity1
The mean plasma concentration of protein C in a healthy term infant is 40 IU dL, with a lower limit of normal of 25 IU dL.
Protein C concentration increases from birth until 6 months of age. Protein C concentration remains slightly low through childhood and achieves the adult range after puberty.
Warnings and Precautions
Hypersensivity: CEPROTIN may contain trace amounts of mouse protein and/or heparin as a result of the manufacturing process. Allergic reactions to mouse protein and/or heparin cannot be ruled out. If symptoms of hypersensitivity/allergic reaction occur, discontinue the injection/infusion. In case of anaphylactic shock, the current medical standards for treatment are to be observed.
Transmission of infectious agents: Because CEPROTIN is made from human plasma, it may carry a risk of transmitting infectious agents, e.g. viruses, the variant Creutzfeldt-Jakob disease (vCJD) agent, and theoretically, the Creutzfeld-Jakob disease (CJD) agent.
Bleeding episodes: Several bleeding episodes have been observed in clinical studies. Concurrent anticoagulant medication may have been responsible for these bleeding episodes. However, it cannot be completely ruled out that the administration of CEPROTIN further contributed to these bleeding events. Simultaneous administration of CEPROTIN and tissue plasminogen activator (tPA) may further increase the risk of bleeding from tPA.
Heparin-induced thrombocytopenia (HIT): CEPROTIN contains trace amounts of heparin which may lead to HIT, which can be associated with a rapid decrease of the number of thombocytes. If HIT is suspected, determine the platelet count immediately and consider discontinuation of CEPROTIN.
Low sodium diet/Renal impairment: Patients on a low sodium diet or who have renal impairment should be informed that the quantity of sodium in the maximum daily dose of CEPROTIN exceeds 200 mg. Monitor patients with renal impairment closely for sodium overload.
Adverse Reactions
Common adverse reactions related to CEPROTIN observed in clinical trials were hypersensitivity or allergic reactions: lightheadedness, itching and rash.
INDICATION
CEPROTIN [Protein C Concentrate (Human)] is indicated for neonates, pediatric and adult patients with severe congenital Protein C deficiency for the prevention and treatment of venous thrombosis and purpura fulminans.
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References:
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Goldenberg N, Manco-Johnson M. Protein C deficiency. Haemophilia. 2008;14(6):1214–1221.
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Chalmers E, et al. Purpura fulminans: recognition, diagnosis and management. Archives of Disease in Childhood. 2011;96(11):1066-1071.
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Price VE, et al. Diagnosis and management of neonatal purpura fulminans. Semin Fetal Neonatal Med. 2011;16(6):318-22.