Use of protein-C concentrate, heparin, and haemodiafiltration in meningococcus-induced purpura fulminans

@article{Smith1997UseOP,
  title={Use of protein-C concentrate, heparin, and haemodiafiltration in meningococcus-induced purpura fulminans},
  author={Op Smith and Barry White and David Vaughan and Mark Rafferty and Liam Claffey and Barry Lyons and William Casey},
  journal={The Lancet},
  year={1997},
  volume={350},
  pages={1590-1593}
}
BACKGROUND Inflammatory and coagulation processes are both affected in meningococcaemia. Severe acquired protein-C deficiency in meningococcaemia is usually associated with substantial mortality: in survivors, skin grafts, amputation, and end-organ failure are not uncommon. Protein C is a natural anticoagulant and also has important anti-inflammatory activity. We assessed the effects of early replacement therapy with protein-C concentrate together with continuous veno-venous haemodiafiltration… Expand
Protein C substitution in sepsis-associated purpura fulminans
TLDR
Encouraging results are provided on the use of PC substitution in meningococcal purpura and new data is presented on the administration of this drug to patients with septic purPura caused by other bacterial species. Expand
Combined antithrombin and protein C supplementation in meningococcal purpura fulminans: a pharmacokinetic study
TLDR
If AT and PC concentrates are to be given in fulminant meningococcemia, the doses of supplementation should be at least 150  IU/kg AT and 250 IU/kg PC as loading dose and 150 IU / kg AT and 200 IU/, as daily maintenance therapy. Expand
Protein C replacement in severe meningococcemia: rationale and clinical experience.
  • L. Alberio, B. Lämmle, C. Esmon
  • Medicine
  • Clinical infectious diseases : an official publication of the Infectious Diseases Society of America
  • 2001
TLDR
Experimental data showing that activated protein C replacement therapy clearly reduces the mortality rate for fulminant meningococcemia are summarized. Expand
An open-label study of the role of adjuvant hemostatic support with protein C replacement therapy in purpura fulminans-associated meningococcemia.
TLDR
In conclusion, PC replacement therapy in severe meningococcal septicemia was associated with a reduction in predicted morbidity and mortality, and the beneficial effect of PC replacement may reflect both the anticoagulant and anti-inflammatory properties of the PC pathway. Expand
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TLDR
An 18-year-old woman presented with acute deterioration in mental status, with the appearance of more petechiae, and worsening of the thorombocytopenia, and activated protein C at the rate of 1 mg/h was started 8 h after admission and coagulopathy remained a major concern. Expand
Administration of protein C concentrates in patients without congenital deficit: a systematic review of the literature
TLDR
It is concluded that protein C concentrate is an attractive option in septic patients (especially those with meningitis, purpura fulminans, or disseminated intravascular coagulation) and that its cost-benefit ratio must be studied with a large multicenter randomized control trial, possibly including also high risk patients with septic shock and multiple organ failure. Expand
Dysfunction of endothelial protein C activation in severe meningococcal sepsis.
TLDR
In severe meningococcal sepsis, protein C activation is impaired, a finding consistent with down-regulation of the endothelial thrombomodulin-endothelial protein C receptor pathway. Expand
Activation of protein C following infusion of protein C concentrate in children with severe meningococcal sepsis and purpura fulminans: a randomized, double-blinded, placebo-controlled, dose-finding study.
TLDR
Treatment with protein C concentrate is safe in children with purpura fulminans and meningococcal septic shock and leads to dose-related increases of plasma APC and resolution of coagulation imbalances. Expand
Purpura Fulminans: Mechanism and Management of Dysregulated Hemostasis.
TLDR
This review addresses the diagnosis and management of Purpura fulminans with a focus on a rational approach to this condition informed by the available data, and proposed mechanisms underlying the dysregulation of coagulation seen in PF are covered. Expand
Plasma exchange as a source of protein C for acute onset protein C pathway failure
TLDR
A 31‐year‐old patient with pneumococcal septic shock, purpura fulminans (PF) and severe acquired PC deficiency is described, who is successfully treated with conventional therapy and high‐volume plasma exchange as a source of PC. Expand
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