title: Severe Congenital Protein C Deficiency: Practical Aspects of Management.
abstract: Subcutaneous (SC) protein C (PC) was used in a child with purpura fulminans secondary to severe congenital PC deficiency. For maintenance, PC 80-120 IU/kg, given over 60-90 min SC Q48hr, has been successful as a home therapy for more than 3 years. The treatment was monitored by measuring trough PC chromogenic activity (target ≥15%) and D-dimer levels. No change in clinical course was appreciated after discontinuing enoxaparin (and leaving the patient on prophylactic PC replacement alone). A significant discrepancy between clotting-based and chromogenic-based PC activity is shown.
authors.affiliation: Pediatric Hematology, Alberta Children's Hospital, Cumming School of Medicine, University of Calgary, Calgary, Alberta, Canada.
authors.forename: Ravi
authors.initials: R
authors.lastname: Shah
authors.affiliation: Medical Genetics, Alberta Children's Hospital, Calgary, Alberta, Canada.
authors.forename: Patrick
authors.initials: P
authors.lastname: Ferreira
authors.affiliation: Pediatric Hematology, Alberta Children's Hospital, Cumming School of Medicine, University of Calgary, Calgary, Alberta, Canada.
authors.forename: Shelina
authors.initials: S
authors.lastname: Karmali
authors.affiliation: Pediatric Hematology, Alberta Children's Hospital, Cumming School of Medicine, University of Calgary, Calgary, Alberta, Canada.
authors.forename: Doan
authors.initials: D
authors.lastname: Le
mesh_terms: D000925:Anticoagulants / Q000627:therapeutic use; D017984:Enoxaparin / Q000627:therapeutic use; D005260:Female; D006801:Humans; D007231:Infant, Newborn; D016031:Liver Transplantation; D011486:Protein C / Q000008:administration & dosage / Q000235:genetics* / Q000627:therapeutic use*; D020151:Protein C Deficiency / Q000188:drug therapy* / Q000473:pathology*
chemical_list: D000925:Anticoagulants; D017984:Enoxaparin; D011486:Protein C
keywords: congenital protein C deficiency; PROC gene; protein C activity; subcutaneous protein C