Severe Congenital Protein C Deficiency: Practical Aspects of Management. Issue 8 (2nd May 2016)

• Record Type: Journal Article
• Authors: Shah, Ravi; Ferreira, Patrick; Karmali, Shelina; Le, Doan
• Is Part Of: Pediatric blood & cancer. Volume 63:Issue 8(2016)
• Page Start: 1488
• Page End: 1490
• Publication Date: 2016-05-02
• Subjects: congenital protein C deficiency; PROC gene -- protein C activity -- subcutaneous protein C; Tumors in children -- Periodicals; Blood -- Diseases -- Periodicals; Cancer in children -- Periodicals; 618.92
• DOI: 10.1002/pbc.25997
• Languages: English
• ISSNs: 1545-5009

Abstract

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.