Premium
Severe Congenital Protein C Deficiency: Practical Aspects of Management
Author(s) -
Shah Ravi,
Ferreira Patrick,
Karmali Shelina,
Le Doan
Publication year - 2016
Publication title -
pediatric blood and cancer
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.116
H-Index - 105
eISSN - 1545-5017
pISSN - 1545-5009
DOI - 10.1002/pbc.25997
Subject(s) - medicine , purpura fulminans , protein c , pediatrics , surgery
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.