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  • 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: Haemolytic uraemic syndrome ; Endothelium ; Soluble vascular cell adhesion molecule-1 ; Soluble intercellular adhesion molecule-1
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Plasma concentrations of soluble vascular cell adhesion molecule-1 (sVCAM-1), E-selectin (sE-selectin) and intercellular adhesion molecule-1 (sICAM-1) were measured by enzyme-linked immunosorbent assay in four groups of children. Group 1 consisted of 20 patients with acute diarrhoea-associated haemolytic uraemic syndrome (D+HUS), the aetiology of HUS being verocytotoxin-producingEscherichia coli infection in each case. Controls consisted of 11 patients who had previously had D+HUS (group 2), 12 with chronic renal failure (group 3) and 8 healthy controls (group 4). When compared with healthy controls, the acute D+HUS group had higher sVCAM-1 (median 1,875 ng/ml, range 1,200–6,450 ng/ml vs. 1,200 ng/ml, range 975–2,125 ng/ml), von Willebrand factor antigen, (1.9 U/ml, range 0.85–5.1 U/ml vs. 0.55 U/ml, range 0.3–1.57 U/ml), white cell count (WBC, 14.5×109/l, range 7.8–43.1 109/l vs. 8.9 109/l, range 5.7–10.8 109/l) and neutrophil count (PMN, 10.1×109/l, range 4.3–26.5 109/l vs. 4.3 109/l, range 3.7–6.6 109/l), allP〈0.005, and sICAM-1 was reduced (230 ng/ml, range 130–340 ng/ml vs. 400 ng/ml, range 260–690 ng/ml),P〈0.05. Within the acute D+HUS group there was a significant correlation between sICAM-1 and PMN (r=0.56,P〈0.01). There was no correlation between any adhesion molecule and plasma creatinine or von Willebrand factor. Comparing the acute HUS group with children with chronic renal failure, WBC (P〈0.001), PMN (P〈0.01) and sVCAM-1 (P〈0.01) were significantly elevated, but there was no difference between the von Willebrand factor (P=0.08) or the sICAM-1 (P〉0.1). sVCAM-1 is elevated and sICAM-1 decreased in acute D+HUS. This pattern of altered adhesion molecule concentration is unlike that in adults with vasculitis and suggests that different endothelial regulatory factors are at play.
    Type of Medium: Electronic Resource
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  • 3
    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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