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  • 1990-1994  (2)
  • 1965-1969
  • Haemolytic uraemic syndrome  (2)
  • 1
    Electronic Resource
    Electronic Resource
    Springer
    Pediatric nephrology 7 (1993), S. 515-519 
    ISSN: 1432-198X
    Keywords: Haemolytic uraemic syndrome ; Prostacyclin
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The role of prostacyclin (PGI2) in the pathogenesis of haemolytic uraemic syndrome (HUS) is controversial. In part, confusion has been caused by failure to distinguish between two main sub-types of the syndrome: extrinsic, diarrhoea-associated HUS (D+ HUS), usually caused by infection with verocytotoxin-producingEscherichia coli orShigella dysenteriae, and the heterogeneous group of non-prodromal forms where intrinsic factors predominate (D− HUS). This paper critically reviews data confined to D+ HUS. Two methods have been used to assess PGI2 synthesis; the generation of PGI2 from endothelium in the presence of HUS plasma in vitro and the measurement of stable metabolites in body fluids. No concensus could be reached with regard to the former. The reported increase of PGI2 stable metabolites in plasma may represent reduced clearance or increased carriage by plasma lipids. Apparent differences between studies of urinary excretion of PGI2 metabolites may reflect the way excretion was expressed. If the metabolite concentration is factored for urinary creatinine, it appears that renal excretion and thus renal synthesis of PGI2 is reduced. However, these are insufficient data on which to attribute the pathogenesis of D+ HUS to disordered PGI2 metabolism.
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1432-198X
    Keywords: Blood group P1 ; Haemolytic uraemic syndrome ; Verotoxin
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Blood group P1 expression was scored by direct agglutination in 32 patients who had previously developed post-enteropathic haemolytic uraemic syndrome (HUS). Sixty-six children of similar ages undergoing venepuncture for other renal disorders acted as controls. The expression of P1 in controls was that expected from the normal caucasian population, 23% being negative. By contrast, there was an excess of HUS patients with weak or absent expression of P1 (χ2 for linear trend 5.45,P〈0.02), and this was particularly evident in those with a poor outcome. Verotoxin (VT), which is associated with HUS, requires the terminal disaccharide of the P1 antigen to bind to cells, and after internalization disrupts the transcription of ribonucleic acid. Mature erythrocytes do not synthesize protein and may be toxin resistant. We postulate that strong expression of P1 antigen may promote the binding of VT to red cells and thus reduce the dose to vulnerable nucleated cleated endothelial cells. P1 positivity may be protective, and P1 negativity a risk factor in HUS.
    Type of Medium: Electronic Resource
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