Pneumococcal-related Hemolytic Uremic Syndrome in the United Kingdom: National Surveillance, 2006–2016

Background: children
Source: The Pediatric Infectious Disease Journal - Category: Infectious Diseases Tags: Original Studies Source Type: research

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AbstractWith the introduction of the complement C5-inhibitor eculizumab, a new era was entered for patients with atypical hemolytic uremic syndrome (aHUS). Eculizumab therapy very effectively reversed thrombotic microangiopathy and reduced mortality and morbidity. Initial guidelines suggested lifelong treatment and recommended prophylactic use of eculizumab in aHUS patients receiving a kidney transplant. However, there is little evidence to support lifelong therapy or prophylactic treatment in kidney transplant recipients. Worldwide, there is an ongoing debate regarding the optimal dose and duration of treatment, particula...
Source: Pediatric Nephrology - Category: Urology & Nephrology Source Type: research
;oz A Abstract The prospective Chronic Kidney Disease in Children cohort with data collected from 2003 to 2018 provided the first opportunity to characterize the incidence of renal replacement therapy (RRT) initiation over the life course of pediatric kidney diseases. Parametric generalized gamma models were fit and extrapolated for overall RRT, and by specific modality (dialysis or preemptive transplant). Children were stratified by diagnosis: n=650 with non-glomerular (mostly congenital), n=49 glomerular-hemolytic uremic syndrome, and n=216 with glomerular-non-HUS (heterogeneous childhood onset). Estimated years...
Source: Am J Epidemiol - Category: Epidemiology Authors: Tags: Am J Epidemiol Source Type: research
Abstract Thrombotic thrombocytopenic purpura (TTP) is a rare thrombotic microangiopathy (TMA) characterized by the severe deficiency of a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13 (ADAMTS13) activity (
Source: Thrombosis and Haemostasis - Category: Hematology Authors: Tags: Thromb Haemost Source Type: research
Authors: Raina R, Grewal MK, Radhakrishnan Y, Tatineni V, DeCoy M, Burke LL, Bagga A Abstract Atypical hemolytic uremic syndrome (aHUS) is a chronic life threatening condition that arises from genetic abnormalities resulting in uncontrolled complement amplifying activity. The introduction of eculizumab, the humanized monoclonal antibody, has brought about a paradigm shift in the management of aHUS. However, there are many knowledge gaps, diagnostic issues, access and cost issues, and patient or physician challenges associated with the use of this agent. Limited data on the natural history of aHUS along with the und...
Source: International Journal of Nephrology and Renovascular Disease - Category: Urology & Nephrology Tags: Int J Nephrol Renovasc Dis Source Type: research
We reported here clinical cases where eculizumab use was probably not appropriate once the correct diagnosis of typical HUS was made and cases where it would have been useful because of the late diagnosis of aHUS.
Source: Journal of Pediatric Hematology Oncology - Category: Hematology Tags: Online Articles: Original Articles Source Type: research
ConclusionStimulation of ciGEnC with TNF α + Stx2a is associated with profound metabolic changes indicative of increased inflammation, oxidative stress and energy turnover.
Source: Metabolomics - Category: Biology Source Type: research
Defective complement action is a cause of several human glomerular diseases including atypical hemolytic uremic syndrome (aHUS), anti-neutrophil cytoplasmic antibody mediated vasculitis (ANCA), C3 glomerulopathy, IgA nephropathy, immune complex membranoproliferative glomerulonephritis, ischemic reperfusion injury, lupus nephritis, membranous nephropathy, and chronic transplant mediated glomerulopathy. Here we summarize ongoing clinical trials of complement inhibitors in nine glomerular diseases and show which inhibitors are used in trials for these renal disorders (http://clinicaltrials.gov).
Source: Frontiers in Immunology - Category: Allergy & Immunology Source Type: research
Severe deficiency of plasma ADAMTS13 activity is the primary cause of thrombotic thrombocytopenic purpura (TTP) whereas overwhelming activation of complement via an alternative pathway results in atypical hemolytic uremic syndrome (aHUS), the prototypes of thrombotic microangiopathy (TMA). However, clinical and pathogenic distinctions between TTP and aHUS are often quite challenging. Clinical reports have suggested that complement activation may play a role in the development of TTP, which is caused by severe deficiency of plasma ADAMTS13 activity. However, the experimental evidence to support this hypothesis is still lack...
Source: Blood - Category: Hematology Authors: Tags: Thrombocytopenia, Platelets and Thrombopoiesis, Thrombosis and Hemostasis Source Type: research
ConclusionsSTEC prevalence among young children with diarrhea in Romania was high and the risk of HUS is emerging. The establishment of a systematic laboratory-based surveillance program including identification of the circulating STEC strains coupled with epidemiological investigation of HUS patients is warranted to determine the source and mode of transmission of STEC and prevent of STEC-associated diarrhea and HUS.
Source: Gut Pathogens - Category: Microbiology Source Type: research
In conclusion, WGS is a promising tool for gaining serovar and virulence gene information, especially from a public health perspective.
Source: Journal of Clinical Microbiology - Category: Microbiology Authors: Tags: Epidemiology Source Type: research
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