Human protein C concentrate in the treatment of purpura fulminans: A retrospective analysis of safety and outcome in 94 pediatric patients

Alex Veldman, Doris Fischer, Flora Wong, Wolfhart Kreuz, Michael Sasse, Bruno Eberspacher, Ulrich Mansmann, Rudolf Schosser

Research output: Contribution to journalArticleResearchpeer-review

Abstract

INTRODUCTION: Purpura fulminans (PF) is a devastating complication of uncontrolled systemic inflammation, associated with high incidence of amputations, skin grafts and death. In this study, we aimed to clarify the clinical profile of pediatric patients with PF who improved with protein C (PC) treatment, explore treatment effects and safety, and to refine the prognostic significance of protein C plasma levels. METHODS: In Germany, patients receiving protein C concentrate (Ceprotin, Baxter AG, Vienna, Austria) are registered. The database was used to locate all pediatric patients with PF treated with PC from 2002 to 2005 for this national, retrospective, multi-centered study. RESULTS: Complete datasets were acquired in 94 patients, treated in 46 centers with human, non-activated protein C concentrate for purpura fulminans. PC was given for 2 days (median, range 1-24 days) with a median daily dose of 100 IU/kg. Plasma protein C levels increased from a median of 27 to a median of 71 under treatment. 22.3 of patients died, 77.7 survived to discharge. Skin grafts were required in 9.6 , amputations in 5.3 . PF recovered or improved in 79.8 , remained unchanged in 13.8 and deteriorated in 6.4 . Four adverse events occurred in 3 patients, none classified as severe. Non-survivors had lower protein C plasma levels (P <0.05) and higher prevalence of coagulopathy at admission (P <0.01). Time between admission and start of PC substitution was longer in patients who died compared to survivors (P = 0.03).
Original languageEnglish
Pages (from-to)1 - 7
Number of pages7
JournalCritical Care
Volume14
Issue number4 (R156)
DOIs
Publication statusPublished - 2010

Cite this

Veldman, Alex ; Fischer, Doris ; Wong, Flora ; Kreuz, Wolfhart ; Sasse, Michael ; Eberspacher, Bruno ; Mansmann, Ulrich ; Schosser, Rudolf. / Human protein C concentrate in the treatment of purpura fulminans: A retrospective analysis of safety and outcome in 94 pediatric patients. In: Critical Care. 2010 ; Vol. 14, No. 4 (R156). pp. 1 - 7.
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abstract = "INTRODUCTION: Purpura fulminans (PF) is a devastating complication of uncontrolled systemic inflammation, associated with high incidence of amputations, skin grafts and death. In this study, we aimed to clarify the clinical profile of pediatric patients with PF who improved with protein C (PC) treatment, explore treatment effects and safety, and to refine the prognostic significance of protein C plasma levels. METHODS: In Germany, patients receiving protein C concentrate (Ceprotin, Baxter AG, Vienna, Austria) are registered. The database was used to locate all pediatric patients with PF treated with PC from 2002 to 2005 for this national, retrospective, multi-centered study. RESULTS: Complete datasets were acquired in 94 patients, treated in 46 centers with human, non-activated protein C concentrate for purpura fulminans. PC was given for 2 days (median, range 1-24 days) with a median daily dose of 100 IU/kg. Plasma protein C levels increased from a median of 27 to a median of 71 under treatment. 22.3 of patients died, 77.7 survived to discharge. Skin grafts were required in 9.6 , amputations in 5.3 . PF recovered or improved in 79.8 , remained unchanged in 13.8 and deteriorated in 6.4 . Four adverse events occurred in 3 patients, none classified as severe. Non-survivors had lower protein C plasma levels (P <0.05) and higher prevalence of coagulopathy at admission (P <0.01). Time between admission and start of PC substitution was longer in patients who died compared to survivors (P = 0.03).",
author = "Alex Veldman and Doris Fischer and Flora Wong and Wolfhart Kreuz and Michael Sasse and Bruno Eberspacher and Ulrich Mansmann and Rudolf Schosser",
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Human protein C concentrate in the treatment of purpura fulminans: A retrospective analysis of safety and outcome in 94 pediatric patients. / Veldman, Alex; Fischer, Doris; Wong, Flora; Kreuz, Wolfhart; Sasse, Michael; Eberspacher, Bruno; Mansmann, Ulrich; Schosser, Rudolf.

In: Critical Care, Vol. 14, No. 4 (R156), 2010, p. 1 - 7.

Research output: Contribution to journalArticleResearchpeer-review

TY - JOUR

T1 - Human protein C concentrate in the treatment of purpura fulminans: A retrospective analysis of safety and outcome in 94 pediatric patients

AU - Veldman, Alex

AU - Fischer, Doris

AU - Wong, Flora

AU - Kreuz, Wolfhart

AU - Sasse, Michael

AU - Eberspacher, Bruno

AU - Mansmann, Ulrich

AU - Schosser, Rudolf

PY - 2010

Y1 - 2010

N2 - INTRODUCTION: Purpura fulminans (PF) is a devastating complication of uncontrolled systemic inflammation, associated with high incidence of amputations, skin grafts and death. In this study, we aimed to clarify the clinical profile of pediatric patients with PF who improved with protein C (PC) treatment, explore treatment effects and safety, and to refine the prognostic significance of protein C plasma levels. METHODS: In Germany, patients receiving protein C concentrate (Ceprotin, Baxter AG, Vienna, Austria) are registered. The database was used to locate all pediatric patients with PF treated with PC from 2002 to 2005 for this national, retrospective, multi-centered study. RESULTS: Complete datasets were acquired in 94 patients, treated in 46 centers with human, non-activated protein C concentrate for purpura fulminans. PC was given for 2 days (median, range 1-24 days) with a median daily dose of 100 IU/kg. Plasma protein C levels increased from a median of 27 to a median of 71 under treatment. 22.3 of patients died, 77.7 survived to discharge. Skin grafts were required in 9.6 , amputations in 5.3 . PF recovered or improved in 79.8 , remained unchanged in 13.8 and deteriorated in 6.4 . Four adverse events occurred in 3 patients, none classified as severe. Non-survivors had lower protein C plasma levels (P <0.05) and higher prevalence of coagulopathy at admission (P <0.01). Time between admission and start of PC substitution was longer in patients who died compared to survivors (P = 0.03).

AB - INTRODUCTION: Purpura fulminans (PF) is a devastating complication of uncontrolled systemic inflammation, associated with high incidence of amputations, skin grafts and death. In this study, we aimed to clarify the clinical profile of pediatric patients with PF who improved with protein C (PC) treatment, explore treatment effects and safety, and to refine the prognostic significance of protein C plasma levels. METHODS: In Germany, patients receiving protein C concentrate (Ceprotin, Baxter AG, Vienna, Austria) are registered. The database was used to locate all pediatric patients with PF treated with PC from 2002 to 2005 for this national, retrospective, multi-centered study. RESULTS: Complete datasets were acquired in 94 patients, treated in 46 centers with human, non-activated protein C concentrate for purpura fulminans. PC was given for 2 days (median, range 1-24 days) with a median daily dose of 100 IU/kg. Plasma protein C levels increased from a median of 27 to a median of 71 under treatment. 22.3 of patients died, 77.7 survived to discharge. Skin grafts were required in 9.6 , amputations in 5.3 . PF recovered or improved in 79.8 , remained unchanged in 13.8 and deteriorated in 6.4 . Four adverse events occurred in 3 patients, none classified as severe. Non-survivors had lower protein C plasma levels (P <0.05) and higher prevalence of coagulopathy at admission (P <0.01). Time between admission and start of PC substitution was longer in patients who died compared to survivors (P = 0.03).

UR - http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2945140/pdf/cc9226.pdf

U2 - 10.1186/cc9226

DO - 10.1186/cc9226

M3 - Article

VL - 14

SP - 1

EP - 7

JO - Critical Care

JF - Critical Care

SN - 1364-8535

IS - 4 (R156)

ER -