Prognostic Markers in Diarrhoea-Associated Haemolytic-Uraemic Syndrome: Initial Neutrophil Count, Human Neutrophil Elastase and Von Willebrand Factor Antigen

Abstract

The observed increase in plasma von Willebrand factor antigen (vWF:Ag) in patients with the haemolytic-uraemic syndrome is presumed to be secondary to endothelial damage, which is a central event in these diseases. As the prognostic value of such changes has not been previously evaluated, vWF:Ag has been measured in plasma from children reported to a national survey of haemolytic-uraemic syndrome. Human neutrophil elastase was also measured, as an initial neutrophilia has been shown to have prognostic value in diarrhoea-associated haemolytic-uraemic syndrome. Despite a significant elevation of plasma vWF:Ag concentration in these patients at presentation, the values did not correlate with the period of thrombocytopenia, the need for dialysis, or outcome. However, children with a poor outcome had significantly greater plasma elastase concentrations compared to those who had a good outcome.

Induction of Neutrophil Extracellular Traps in Shiga Toxin-Associated Hemolytic Uremic Syndrome

“…Besides the cytotoxic effects of Stx, the inflammatory response mediated by neutrophils (PMN) also contributes to HUS evolution [3]. HUS patients show a high peripheral PMN count, which correlates with a poor prognosis [4]. Moreover, these cells have shown signs of previous activation, indicated by a reduced expression of different membrane molecules and intracellular components, as well as impaired cytokine-induced responses [5].…”

Shiga Toxin Translocation across Intestinal Epithelial Cells Is Enhanced by Neutrophil Transmigration

“…In addition, histological studies of gastrointestinal tissue of patients infected with STEC show neutrophil infiltration in the lamina propria and crypts (18,23,24,42). High IL-8 levels have been noted in HUS patients, and it was suggested that IL-8 expression was, at least in part, responsible for neutrophil recruitment in the disease (13,14,15,32,50). Rabbit models of STEC infection have implicated intestinal neutrophils as contributing to pathogenesis (12,46).…”

Haemolytic Uraemic Syndrome (HUS): Clinical Medicine Versus Clinical Anatomy

“…Severe thrombocytopenia is uncommon but prolonged thrombocytopenia for more than 10 days is associated with long-term renal picture. Factor VIII levels do not correlate with clinical outcome [12]. Table 5 summarizes laboratory tests/ investigations according to Haematology specialty.…”