Hemolytic Uremic Syndrome is associated with dysregulation of Chemokine Receptor expression in Circulating Monocytes.

Conclusions: The increase in CCRs expression correlates with HUS severity suggesting that the dysregulation of these receptors might contributes to an increased risk for renal damage. Activated Mo could be recruited by chemokines and then receptors could be dysregulated. The dysregulation of CCRs and their ligands observed during acute period suggest that chemokine pathway would participate in HUS development.
Source: Clinical Science - Category: Biomedical Science Authors: Source Type: research

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Abstract Typical hemolytic uremic syndrome is caused by the Shiga toxin (Stx2) and lipopolysaccharide (LPS) of Escherichia coli and leads to acute kidney injury. The role of innate immunity in this pathogenesis is unclear. We analyzed the role of high-mobility group box 1 (HMGB1) at the onset of disease in a murine model. C57BL/6 mice were intraperitoneally administered with saline (Group A); anti-HMGB1 monoclonal antibody (Group B); Stx2 and LPS, to elicit severe disease (Group C); or Stx2, LPS, and anti-HMGB1 antibody (Group D). While all Group C mice died by day 5 of the experiment, all Group D mice survived. A...
Source: American Journal of Physiology. Renal Physiology - Category: Physiology Authors: Tags: Am J Physiol Renal Physiol Source Type: research
Mauro Nicola Laforgia Shiga toxin-producing Escherichia Coli (STEC) infections routinely run as a common gastroenteritis, but in many cases they may evolve towards hemolytic uremic syndrome (HUS). HUS is a rare disease characterized by microangiopathic hemolytic anemia, thrombocytopenia, and acute renal failure. Gut microorganisms have a fundamental impact on human physiology, because they modulate normal intestinal functions and play a pivotal role in influencing the local and systemic immune responses. Despite surveillance established in many countries and major progresses in the understanding of STEC-HUS mechanism...
Source: International Journal of Environmental Research and Public Health - Category: Environmental Health Authors: Tags: Review Source Type: research
Purpose of review Atypical hemolytic uremic syndrome (aHUS) is a diagnosis that has captured the interest of specialists across multiple fields. The hallmark features of aHUS are microangiopathic hemolysis and thrombocytopenia, which creates a diagnostic dilemma because of the occurrence of these findings in a wide variety of clinical disorders. Recent findings In most of the instances, aHUS is a diagnosis of exclusion after ruling out causes such as Shigella toxin, acquired or genetic a disintegrin and metalloproteinase thrombospondin motif 13 deficiency (thrombotic thrombocytopenic purpura), and vitamin B12 deficien...
Source: Current Opinion in Nephrology and Hypertension - Category: Urology & Nephrology Tags: SPECIAL COMMENTARY Source Type: research
A 6-month-old boy presented with acute renal failure, thrombocytopenia, and severe non-immune hemolytic anemia. Infection by Shiga-like toxin-producingEscherichia coli and other causes of microangiopathic hemolysis were ruled out, leading to a diagnosis of atypical hemolytic uremic syndrome (aHUS). Neither pathogenic variants in HUS-associated genes nor anti-factor H antibodies were identified. Copy number variation analysis uncovered 4 copies of complement factor H related genes,CFHR1-CFHR4, conceivably leading to higher than normal levels of the corresponding proteins. However, this abnormality was also found in the heal...
Source: Nephron - Category: Urology & Nephrology Source Type: research
AbstractShiga toxin (Stx)-producingEscherichia coli (STEC) is the offending agent in post-diarrhea-associated hemolytic uremic syndrome (HUS), a disorder characterized by thrombocytopenia, microangiopathic hemolytic anemia, and acute kidney failure, with thrombi occluding the renal microvasculature. Endothelial dysfunction has been recognized as the trigger event in the development of microangiopathic processes. Glomerular endothelial cells are susceptible to the toxic effects of Stxs that, via nuclear factor kappa B (NF- κB) activation, induce the expression of genes encoding for adhesion molecules and chemokines, c...
Source: Pediatric Nephrology - Category: Urology & Nephrology Source Type: research
Conclusions:While disease-directed therapy in atypical HUS (i.e. eculizumab) is required to effectively manage acute, complement-mediated hemolytic anemia and renal failure, correction of the calculated iron deficit in co-existing iron deficiency anemia, if present, can result in gradual but marked improvement in hemoglobin. Conversely, patients with atypical HUS who are actively hemolyzing with a normocytic anemia should still be evaluated for iron deficiency and undergo iron transfusions for any calculated deficit.DisclosuresNo relevant conflicts of interest to declare.
Source: Blood - Category: Hematology Authors: Tags: 311. Disorders of Platelet Number or Function Source Type: research
Complement-mediated thrombotic microangiopathy (TMA), also known as atypical hemolytic uremic syndrome (aHUS) is a rare, hereditary, progressive, life-threatening disorder caused by a disruption in regulation of the alternative pathway of the complement system. Eculizumab, a terminal complement inhibitor, has emerged as a first-line therapy, however data are limited to small case series (Brocklebank et al., 2017). Here, we present a diagnostically challenging case of complement-mediated TMA, who received eculizumab therapy with excellent hematologic response.A 68-year-old female with history of possible Sjogren's syndrome,...
Source: Blood - Category: Hematology Authors: Tags: 311. Disorders of Platelet Number or Function Source Type: research
Hemolytic uremic syndrome (HUS) is the clinical triad of thrombocytopenia, anemia, and acute kidney injury. Classically associated with enterocolitis from Shiga toxin –producing Escherichia coli, HUS is also associated with Streptococcus pneumoniae infections; genetic dysregulation of the alternative complement pathway or coagulation cascade; and, rarely, a hereditary disorder of cobalamin C metabolism. These share a common final pathway of a prothrombotic and proinflammatory state on the endothelial cell surface, with fibrin and platelet deposition. Much work has been done to distinguish between the different mechan...
Source: Pediatric Clinics of North America - Category: Pediatrics Authors: Source Type: research
AbstractDiarrhea-associated hemolytic uremic syndrome is characterized by hemolytic anemia, thrombocytopenia, and acute kidney injury secondary to enteric infection, typically Shiga toxin-producingEscherichia coli. Shiga toxin 2 is able to activate alternative complement pathways; therefore, the aim of the study was to analyze C3 as a predictor of clinical courses in patients with diarrhea-associated hemolytic uremic syndrome. We hypothesized that the patients with increased complement activation at admission suffered from a more severe course. We retrospectively analyzed data of 33 pediatric patients between 1999 and 2015...
Source: European Journal of Pediatrics - Category: Pediatrics Source Type: research
We report the case of a 81-year-old female presenting with gastroenteritic symptoms and laboratory findings of a hemolytic anemia, thrombocytopenia and acute renal failure with fatal outcome. A hemolytic-uremic syndrome caused by an infection with EHEC was diagnosed. As a further complication, a urinary tract infection with K. pneumoniae was diagnosed and treated. PMID: 30278845 [PubMed - in process]
Source: Praxis - Category: General Medicine Authors: Tags: Praxis (Bern 1994) Source Type: research
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