Hemolytic Uremic Syndrome

Transplant Research Center, Chiara Cucchi de Alessandri e Gilberto Crespi, Villa Camozzi, Via Camozzi, 3 24020, Ranica (BG), Italy.
Journal of the American Society of Nephrology (Impact Factor: 9.47). 05/2005; 16(4):1035-50. DOI: 10.1681/ASN.2004100861
Source: PubMed
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    • "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 [17], an often fatal disease characterized by hemolytic anemia, low platelet count and renal impairment [12]. 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 [13]. "
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    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 · 2.28 Impact Factor
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    • "Microvascular injury with endothelial cell damage is a pathological characteristic of all forms of HUS [49]. The various etiologies of HUS allow classification into infection-induced, genetic, medicationinduced , and HUS associated with systemic diseases characterized by microvascular injury [49]. HUS has been reported in only two patients with KD and AKI [50] [51]. "
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    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; 2013:831834. DOI:10.1155/2013/831834
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    • "We did not encounter any severe complications as a result of TPE in these patients which is similar to other studies [24] [25] though some depict severe complication rates as a consequence of treating patients at the earliest suspicion of the disease [26]. Patients with aHUS (D HUS) often have poor prognosis as compared to D+ HUS [27] [28]. Severe renal insufficiency with the need for renal replacement therapy is often warranted. "
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    ABSTRACT: We present here our experience with therapeutic apheresis (TA) performed for various indications, clinical response and complications in a tertiary care center over last 10 years. Present study is a retrospective analysis of 492 TA procedures performed for 125 patients from January 2000 to December 2009. For each patient: age, gender, weight, clinical indication, pre-procedure hematological profile and ionized calcium levels were recorded. For every procedure following parameters were analyzed: type of venous access (central/peripheral), volume of blood and plasma processed, amount of anticoagulant used, procedure duration, blood flow rate, type of replacement fluid given, response to therapy and adverse reactions. Of 492 TA procedures, 68.8% were performed for neurology, 20.8% hematology-oncology, 9.6% renal and 0.8% for rheumatology patients. Therapeutic plasma exchanges (n=464; 94.3%) and therapeutic cytapheresis (n=28; 6.7%) were performed in 113 and 12 patients, respectively. Majority of patients belonged to ASFA category I and II (n=124; 99.2%). The overall response rate was 84%, with encouraging response in TTP (100%), aHUS (81.8%) and in neurological disorders (88.4%). Adverse events were reported in 52.8% of patients in 14.83% of procedures. Our results of TPE in neurological disorders and in atypical hemolytic uremic syndrome are encouraging and it is a cost effective alternative to IvIg in neurological disorders. Currently, there is a need for establishment of an Indian apheresis registry to understand the scenario of TA across the country and in the expansion of appropriate and applicable indications for TA in our setting.
    Transfusion and Apheresis Science 12/2011; 45(3):239-45. DOI:10.1016/j.transci.2011.09.002 · 1.07 Impact Factor
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