ArticleCritical care (London, England)2010
Human protein C concentrate in the treatment of purpura fulminans: a retrospective analysis of safety and outcome in 94 pediatric patients.
Article in Critical care (London, England), 2010. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 17 papers.
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Who cites it
17 citing papers in PubMed, 56 citations in OpenAlex.
- Article
- Purpura Fulminans in a 22-Month-Old Child: Early Recognition and Favorable Outcome Without Limb Loss.Cureus · 2026Article
- Article
- Protein C in adult patients with sepsis: from pathophysiology to monitoring and supplementation.Journal of anesthesia, analgesia and critical care · 2025Review
- Infectious purpura fulminans associated with pneumococcal septicaemia in a patient with unacknowledged functional asplenia.BMJ case reports · 2024Article
- A Rare Cause of a Rare Disorder:Case reports in critical care · 2022Article
- Factors leading to neonatal mortality due to neonatal purpura fulminans through the lens of a child death review.BMJ case reports · 2021Article
- Protein C Pathway in Paediatric and Neonatal Sepsis.Frontiers in pediatrics · 2021Review
- Persistence of endothelial thrombomodulin in a patient with infectious purpura fulminans treated with protein C concentrate.Blood advances · 2018Article
- An ADAM-10 dependent EPCR shedding links meningococcal interaction with endothelial cells to purpura fulminans.PLoS pathogens · 2018Article
- Coagulation and Bleeding Management in Pediatric Extracorporeal Membrane Oxygenation: Clinical Scenarios and Review.Frontiers in medicine · 2018Review
- Refining anti-inflammatory therapy strategies for bronchopulmonary dysplasia.Journal of cellular and molecular medicine · 2017Article
- Non Activated Protein C Supplementation in Septic Pediatric Hematological Patients.Pediatric reports · 2016Article
- Article
- [Sepsis-associated Purpura Fulminans International Registry--Europe (SAPFIRE)].Medizinische Klinik, Intensivmedizin und Notfallmedizin · 2014Article
- Purpura Fulminans following Thermal Injury.Case reports in emergency medicine · 2013Article
- Purpura Fulminans fromCase reports in oncologyArticle
Corrections and comments
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Authors and funding
8 authors at 5 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
introductionPurpura 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.
methodsIn 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.
resultsComplete 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).
conclusionsThis retrospective dataset shows that, compared to historic controls, only few pediatric patients with PF under PC substitution needed dermatoplasty and/or amputations. Apart from epistaxis, no bleeding was observed. Although the data comes from a retrospective study, the evidence we present suggests that PC had a beneficial impact on the need for dermatoplasty and amputations, pointing to the potential value of carrying out a prospective randomised controlled trial.
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