Document Detail

Use of protein-C concentrate, heparin, and haemodiafiltration in meningococcus-induced purpura fulminans.
MedLine Citation:
PMID:  9393338     Owner:  NLM     Status:  MEDLINE    
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 and conventional treatment in meningococcaemia. METHODS: 12 patients aged between 3 months and 27 years with meningococcaemia and severe acquired protein-C deficiency (mean 0.20 IU/mL) were studied. All patients had septic shock, widespread purpura, skin necrosis, and disseminated intravascular coagulopathy. After a test dose of protein-C concentrate, patients received a continuous infusion with the dose adjusted daily to keep the plasma concentration between 0.8 and 1.2 IU/mL. 11 patients were given unfractionated intravenous heparin (10-15 IU kg-1 h-1). Nine patients had haemodiafiltration and one had peritoneal dialysis. The Glasgow meningococcal septicaemia prognostic score and the paediatric risk of mortality score predicted a minimum mortality of 80% and 57%, respectively. FINDINGS: No patient died. No adverse reactions to the treatment were seen. Two patients had lower-limb amputations, one of whom had a thrombotic cerebrovascular accident; both patients had received the protein-C concentrate and heparin later than the rest of the group (60 h [16.97] vs 12 h [3.13]). One patient developed chronic renal failure despite receiving protein-C infusion 15 h after admission. INTERPRETATION: The acquired severe deficiency of protein C in meningococcaemia contributes to the pathogenesis of the thrombotic necrotic lesions in the skin and other organs and probably has an important role in the inflammatory response. Protein-C therapy is merely one approach to improve the host response in this syndrome. We suggest that a double-blind, randomised, controlled multicentre trial is needed to confirm our results.
O P Smith; B White; D Vaughan; M Rafferty; L Claffey; B Lyons; W Casey
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Publication Detail:
Type:  Clinical Trial; Journal Article    
Journal Detail:
Title:  Lancet     Volume:  350     ISSN:  0140-6736     ISO Abbreviation:  Lancet     Publication Date:  1997 Nov 
Date Detail:
Created Date:  1998-01-06     Completed Date:  1998-01-06     Revised Date:  2004-11-17    
Medline Journal Info:
Nlm Unique ID:  2985213R     Medline TA:  Lancet     Country:  ENGLAND    
Other Details:
Languages:  eng     Pagination:  1590-3     Citation Subset:  AIM; IM    
Department of Paediatric Haematology, National Children's Hospital, Dublin, Ireland.
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MeSH Terms
Anticoagulants / therapeutic use*
Child, Preschool
Heparin / therapeutic use*
Meningitis, Meningococcal / complications,  therapy*
Protein C / therapeutic use*
Purpura, Schoenlein-Henoch / etiology,  therapy*
Treatment Outcome
Reg. No./Substance:
0/Anticoagulants; 0/Protein C; 9005-49-6/Heparin
Comment In:
Lancet. 1998 Mar 28;351(9107):988-9   [PMID:  9734970 ]
Lancet. 1998 Mar 28;351(9107):987-8   [PMID:  9734969 ]
Lancet. 1998 Mar 28;351(9107):987; author reply 988   [PMID:  9734968 ]
Lancet. 1998 Mar 28;351(9107):986-7; author reply 988   [PMID:  9734967 ]
Lancet. 1997 Nov 29;350(9091):1565-6   [PMID:  9393331 ]

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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