title: Non Activated Protein C Supplementation in Septic Pediatric Hematological Patients.
abstract: The purpose of the study was to examine safety and efficacy of non-activated Protein C (PC) supplementation in our cohort of septic pediatric hematological patients. We conducted a retrospective study of 22 septic patients receiving human plasma-derived PC concentrate from 2008 to 2015 at our Pediatric Oncology Center (Bari, Italy). The Surviving sepsis campaign definitions for sepsis, severe sepsis and septic shock were used to define the patients' septic status. For each patient, we calculated Lansky performance status scale (LPSS) and a risk score defined the Hematologic risk score (HRS) that we created in 2007. Patients were defined as High risk for severe sepsis/septic shock in case of HRS>3. HRS<3 identified low risk patients. Baseline serum PC levels, PC administration dosage and duration and days until a 20% improvement in LPSS. Observed baseline serum PC levels (bPC) blood concentrations ranged from 31 to 80%. Patients received PC supplementation in case of low age-related bPC levels or >10% PC concentration decrease within 12 hours from the first evaluation. All patients received 80 U/kg/day PC, intravenously, every twenty-four hours. No drug-related adverse event was observed. The observed sepsis-related mortality rate in our cohort was 9%. PC supplementation in our cohort appeared to be safe, and, probably due to prompt PC administration, we observed an overall mortality that was much lower than expected mortality in cancer severe septic patients.
authors.affiliation: Division of Pediatric Hematology-Oncology, Department of Pediatrics, University of Bari , Italy.
authors.forename: Teresa
authors.initials: T
authors.lastname: Perillo
authors.affiliation: Division of Pediatric Hematology-Oncology, Department of Pediatrics, University of Bari , Italy.
authors.forename: Paola
authors.initials: P
authors.lastname: Muggeo
authors.affiliation: Division of Pediatric Hematology-Oncology, Department of Pediatrics, University of Bari , Italy.
authors.forename: Giampaolo
authors.initials: G
authors.lastname: Arcamone
authors.affiliation: Division of Pediatric Hematology-Oncology, Department of Pediatrics, University of Bari , Italy.
authors.forename: Francesco De
authors.initials: FD
authors.lastname: Leonardis
authors.affiliation: Division of Pediatric Hematology-Oncology, Department of Pediatrics, University of Bari , Italy.
authors.forename: Nicola
authors.initials: N
authors.lastname: Santoro
keywords: Protein C; leukemia; pediatrics; sepsis; supplementation