Literature DB >> 8486865

Rapid recovery of acquired purpura fulminans in a patient with familial C4bBP deficiency.

F G Lemesle1, J C Gris, J F Schwed, C Arich.   

Abstract

A 32-year-old pregnant woman developed meningococcemia associated purpura fulminans and quickly improved with therapy. After this disease C4b-Binding Protein (C4bBP) plasma levels remained very low while protein S activity was in the normal range. Familial investigation proved a hereditary C4bBP deficiency. This observation points out the role of the protein C-protein S system during acquired purpura fulminans.

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Year:  1993        PMID： 8486865     DOI： 10.1007/bf01708373

Source DB:  PubMed          Journal:  Intensive Care Med        ISSN： 0342-4642            Impact factor:   17.440


  11 in total

1.  Neonatal purpura fulminans associated with homozygous protein S deficiency.

Authors:  C Mahasandana; V Suvatte; R A Marlar; M J Manco-Johnson; L J Jacobson; W E Hathaway
Journal:  Lancet       Date:  1990-01-06       Impact factor: 79.321

2.  Free protein S levels are elevated in familial C4b-binding protein deficiency.

Authors:  P C Comp; J Forristall; C D West; R G Trapp
Journal:  Blood       Date:  1990-12-15       Impact factor: 22.113

3.  Complement deficiencies in patients over ten years old with meningococcal disease due to uncommon serogroups.

Authors:  C A Fijen; E J Kuijper; A J Hannema; A G Sjöholm; J P van Putten
Journal:  Lancet       Date:  1989-09-09       Impact factor: 79.321

4.  Functional and immunologic protein S levels are decreased during pregnancy.

Authors:  P C Comp; G R Thurnau; J Welsh; C T Esmon
Journal:  Blood       Date:  1986-10       Impact factor: 22.113

5.  Coumarin necrosis associated with hereditary protein C deficiency.

Authors:  W G McGehee; T A Klotz; D J Epstein; S I Rapaport
Journal:  Ann Intern Med       Date:  1984-07       Impact factor: 25.391

6.  Meningococcemia and purpura fulminans in adults: acute deficiencies of proteins C and S and early treatment with antithrombin III concentrates.

Authors:  F Fourrier; P Lestavel; C Chopin; A Marey; J Goudemand; A Rime; J Mangalaboyi
Journal:  Intensive Care Med       Date:  1990       Impact factor: 17.440

Review 7.  Bacterial meningitis: recent advances in pathophysiology and treatment.

Authors:  A R Tunkel; B Wispelwey; W M Scheld
Journal:  Ann Intern Med       Date:  1990-04-15       Impact factor: 25.391

8.  The beneficial effects of early dexamethasone administration in infants and children with bacterial meningitis.

Authors:  C M Odio; I Faingezicht; M Paris; M Nassar; A Baltodano; J Rogers; X Sáez-Llorens; K D Olsen; G H McCracken
Journal:  N Engl J Med       Date:  1991-05-30       Impact factor: 91.245

Review 9.  Diagnosis and treatment of homozygous protein C deficiency. Report of the Working Party on Homozygous Protein C Deficiency of the Subcommittee on Protein C and Protein S, International Committee on Thrombosis and Haemostasis.

Authors:  R A Marlar; R R Montgomery; A W Broekmans
Journal:  J Pediatr       Date:  1989-04       Impact factor: 4.406

10.  Changes in protein C and free protein S during pregnancy and following hysterectomy.

Authors:  R Warwick; R A Hutton; L Goff; E Letsky; M Heard
Journal:  J R Soc Med       Date:  1989-10       Impact factor: 18.000
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