Titre du document / Document title

Von Willebrand factor-cleaving protease in childhood diarrhoea-associated haemolytic uraemic syndrome

Auteur(s) / Author(s)

HUNT Beverley J. ⁽¹⁾ ; LÄMMLE Bernhard ⁽²⁾ ; NEVARD Corinne H. F. ⁽³⁾ ; HAYCOCK George B. ⁽³⁾ ; FURLAN Miha ⁽²⁾ ;

Affiliation(s) du ou des auteurs / Author(s) Affiliation(s)

⁽¹⁾ Department of Haematology, Guy's and St. Thomas'Trust, London, ROYAUME-UNI
⁽²⁾ Central Hematology Laboratory, University of Bern, Inselspital, Bern, SUISSE
⁽³⁾ Department of Paediatric Nephrology, Guy's and St. Thomas'Trust, London, ROYAUME-UNI

Résumé / Abstract

A deficiency of von Willebrand factor (vWF)-cleaving protease, either due to a congenital deficiency or to the presence of a protease inhibitor of vWF-cleaving protease has been associated with thrombotic thrombocytopenic purpura (TTP). We have studied vWF-cleaving protease in diarrhoea-associated haemolytic uraemic syndrome (D+ HUS), ), which shares clinical features with TTP. 29 children with acute D+ HUS and 13 control children were studied. vWF-cleaving protease activity was normal (range 50-150%) in 39 of 42 plasma samples. Levels of protease activity between 25 and 50% were noted in plasma from two D+ HUS patients. One D+HUS patient, who had clinical features of TTP, had a vWF-cleaving protease inhibitor producing a severe deficiency of vWF-cleaving protease. Thus a deficiency of vWF-cleaving protease appears to be atypical in D+HUS. The detection of a vWF-cleaving protease inhibitor in one patient suggests it may be associated with infection such as E. coli 0157.

Revue / Journal Title

Thrombosis and haemostasis   ISSN 0340-6245   CODEN THHADQ 

Source / Source

2001, vol. 85, n^o6, pp. 975-978 (15 ref.)

Langue / Language

Anglais

Editeur / Publisher

Schattauer, Stuttgart, ALLEMAGNE  (1976) (Revue)

Mots-clés anglais / English Keywords

Diarrhea ; Escherichia coli ; Toxin ; Hemolytic uremic syndrome ; Child ; Willebrand factor ; Peptidases ; Protease inhibitor ; Pathophysiology ; Renal failure ; Shiga-like toxin ; Enterobacteriaceae ; Bacteria ; Human ; Hydrolases ; Enzyme ; Urinary system disease ; Hemopathy ; Hemolytic anemia ; Kidney disease ;

Mots-clés français / French Keywords

Diarrhée ; Escherichia coli ; Toxine ; Hémolyse urémie ; Enfant ; Facteur Willebrand ; Peptidases ; Inhibiteur protease ; Physiopathologie ; Insuffisance rénale ; Toxine type Shiga ; Enterobacteriaceae ; Bactérie ; Homme ; Hydrolases ; Enzyme ; Appareil urinaire pathologie ; Hémopathie ; Anémie hémolytique ; Rein pathologie ;

Mots-clés espagnols / Spanish Keywords

Diarrea ; Escherichia coli ; Toxina ; Hemólisis urémica ; Niño ; Factor Willebrand ; Peptidases ; Inhibidor proteasa ; Fisiopatología ; Insuficiencia renal ; Enterobacteriaceae ; Bacteria ; Hombre ; Hydrolases ; Enzima ; Aparato urinario patología ; Hemopatía ; Anemia hemolítica ; Riñón patología ;

Localisation / Location

INIST-CNRS, Cote INIST : 10255, 35400009552069.0050