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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

 

SEVERITY
NO DEFICIENCY
MILD
MODERATE
SEVERE
PROTEIN C
ACTIVITY
LEVEL (IU/dL)*
65-135
21-64
1-20
<1
*Adult levels

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.


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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.

References:

  1. Goldenberg N, Manco-Johnson M. Protein C deficiency. Haemophilia. 2008;14(6):1214–1221.

  2. Chalmers E, et al. Purpura fulminans: recognition, diagnosis and management. Archives of Disease in Childhood. 2011;96(11):1066-1071.

  3. Price VE, et al. Diagnosis and management of neonatal purpura fulminans. Semin Fetal Neonatal Med. 2011;16(6):318-22.

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