"Diarrhoea-associated HUS is the most severe clinical manifestation of infection with Shiga toxin-producing
Escherichia coli and is more common in children
1. Pathogenesis of the syndrome is based on the reaction of the innate immune system to toxemia
3. Characteristic features of the syndrome are hemorrhagic enterocolitis, hemolytic anemia, thrombocytopenia and acute renal failure, but some patients develop more unusual manifestations that potentially lead to MOF and increase mortality
[Show abstract][Hide abstract] ABSTRACT: Introduction: Hemolytic-uremic syndrome (HUS) is a leading cause of acute renal failure in infants and young children. It is traditionally defined as a triad of acute renal failure, hemolytic anemia and thrombocytopenia that occur within a week after prodromal hemorrhagic enterocolitis. Severe cases can also be presented by acute respiratory distress syndrome (ARDS), toxic megacolon with ileus, pancreatitis, central nervous system (CNS) disorders and multiple organ failure (MOF).
Case presentation: A previously healthy 4-year old Caucasian girl developed acute renal failure, thrombocytopenia and hemolytic anemia following a short episode of abdominal pain and bloody diarrhea. By the end of the first week the diagnosis of the typical HUS was established. During the second week the disease progressed into MOF that included ileus, pancreatitis, hepatitis, coma and ARDS, accompanied by hemodynamic instability and extreme leukocytosis. Nonetheless, the girl made a complete recovery after one month of the disease. She was successfully treated in the intensive care unit and significant improvement was noticed after plasmapheresis and continuous veno-venous hemodialysis.
Conclusions: Early start of plasmapheresis and meticulous supportive treatment in the intensive care unit, including renal placement therapy, may be the therapy of choice in severe cases of HUS presented by MOF. Monitoring of prognostic factors is important for early performance of appropriate diagnostic and therapeutical interventions.
F1000 Research 06/2014; 3:79. DOI:10.12688/f1000research.2546.1
"The toxicity of MOA was first tested in vivo on mice, showing how parenterally administered MOA causes symptoms comparable to the human Hemolytic Uremic Syndrome , an often fatal disease characterized by hemolytic anemia, low platelet count and renal impairment . At the same time, the lectin was tested on mouse-derived endothelial and dermal primary cell cultures, showing a cytotoxic effect of MOA leading to cell death and detachment . "
[Show abstract][Hide abstract] ABSTRACT: The Marasmius oreades mushroom agglutinin (MOA) is a blood group B-specific lectin carrying an active proteolytic domain. Its enzymatic activity has recently been shown to be critical for toxicity of MOA toward the fungivorous soil nematode Caenorhabditis elegans. Here we present evidence that MOA also induces cytotoxicity in a cellular model system (murine NIH/3T3 cells), by inhibiting protein synthesis, and that cytotoxicity correlates, at least in part, with proteolytic activity. A peptide-array screen identified the apoptosis mediator BAX as a potential proteolytic substrate and further suggests a variety of bacterial and fungal peptides as potential substrates. These findings are in line with the suggestion that MOA and related proteases may play a role for host defense.
Biochemical and Biophysical Research Communications 05/2014; 447(4):586–589. DOI:10.1016/j.bbrc.2014.04.043 · 2.30 Impact Factor
"Microvascular injury with endothelial cell damage is a pathological characteristic of all forms of HUS . The various etiologies of HUS allow classification into infection-induced, genetic, medicationinduced , and HUS associated with systemic diseases characterized by microvascular injury . HUS has been reported in only two patients with KD and AKI  . "
[Show abstract][Hide abstract] ABSTRACT: Kawasaki disease (KD) is a systemic vasculitis and can develop multiple organ injuries including kidney and urinary tract involvement. These disorders include pyuria, prerenal acute kidney injury (AKI), renal AKI caused by tubulointerstitial nephritis (TIN), hemolytic uremic syndrome (HUS), and immune-complex mediated nephropathy, renal AKI associated with either Kawasaki disease shock syndrome or unknown causes, acute nephritic syndrome (ANS), nephrotic syndrome (NS), renal tubular abnormalities, renal abnormalities in imaging studies, and renal artery lesions (aneurysms and stenosis). Pyuria is common in KD and originates from the urethra and/or the kidney. TIN with AKI and renal tubular abnormalities probably result from renal parenchymal inflammation caused by T-cell activation. HUS and renal artery lesions are caused by vascular endothelial injuries resulting from vasculitis. Some patients with ANS have immunological abnormalities associated with immune-complex formation. Nephromegaly and renal parenchymal inflammatory foci are detected frequently in patients with KD by renal ultrasonography and renal scintigraphy, respectively. Although the precise pathogenesis of KD is not completely understood, renal vasculitis, immune-complex mediated kidney injuries, or T-cell immune-regulatory abnormalities have been proposed as possible mechanisms for the development of kidney and urinary tract injuries.
International Journal of Pediatrics 10/2013; 2013(3):831834. DOI:10.1155/2013/831834
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