Subcutaneous administration of protein C concentrate.
Children born with severe homozygous protein C deficiency do not survive beyond the neonatal period unless they receive protein C replacement. Protein C concentrate (Baxter Biosciences, Vienna) is usually given intravenously via a central venous catheter The authors describe 2 children in whom protein C concentrate was successfully administered by subcutaneous infusion after they had had recurrent central line infections. Alternate daily doses of 250-350 IU/kg resulted in trough protein C levels of > 25 IU/dL. In the follow-up period of 1-2 years neither child has had a thrombotic episode or purpuric skin lesions, and infusions are managed in the home by their parents.
Mathias
M
M
Great Ormond Street Hospital for Children NHS Trust, London, UK. liesnr@gosh.uhs.uk
Khair
K
K
Burgess
C
C
Liesner
R
R
D005260:Female; D006801:Humans; D007231:Infant, Newborn; D011486:Protein C / Q000008:administration & dosage*; D020151:Protein C Deficiency / Q000188:drug therapy*; D040521:Subcutaneous Tissue
D011486:Protein C