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Haemolytic uremic syndrome: diagnosis and management.

ABSTRACT: The thrombotic microangiopathies (TMAs) are a group of diseases characterised by microangiopathic haemolysis, thrombocytopenia, and thrombus formation leading to tissue injury. Traditionally, TMAs have been classified as either thrombotic thrombocytopenic purpura (TTP) or haemolytic uremic syndrome (HUS) based on the clinical presentation, with neurological involvement predominating in the former and acute kidney injury in the latter. However, as our understanding of the pathogenesis of these conditions has increased, it has become clear that this is an over-simplification; there is significant overlap in the clinical presentation of TTP and HUS, there are different forms of HUS, and TMAs can occur in other, diverse clinical scenarios. This review will discuss recent developments in the diagnosis of HUS, focusing on the different forms of HUS and how to diagnose and manage these potentially life-threatening diseases.

SUBMITTER: Sheerin NS

PROVIDER: S-EPMC6764117 | biostudies-literature | 2019

REPOSITORIES: biostudies-literature

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Haemolytic uremic syndrome: diagnosis and management.

Sheerin Neil S NS Glover Emily E

F1000Research 20190925

The thrombotic microangiopathies (TMAs) are a group of diseases characterised by microangiopathic haemolysis, thrombocytopenia, and thrombus formation leading to tissue injury. Traditionally, TMAs have been classified as either thrombotic thrombocytopenic purpura (TTP) or haemolytic uremic syndrome (HUS) based on the clinical presentation, with neurological involvement predominating in the former and acute kidney injury in the latter. However, as our understanding of the pathogenesis of these co ...[more]

PMID: 31598213

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Project description:The aim of this study was to perform a quantitative microbial risk assessment (QMRA) of Shiga toxin-producing Escherichia coli hemolytic uremic syndrome (STEC-HUS) linked to the consumption of Kosher beef produced in Argentina and consumed in Israel in children under 14 years. A probabilistic risk assessment model was developed to characterize STEC prevalence and contamination levels in the beef supply chain (cattle primary production, cattle transport, processing and storage in the abattoir, for export and at retail, and home preparation and consumption). The model was implemented in Microsoft Excel 2016 with the @Risk add-on package. Results of 302 surveys with data collected in Israel were as follows: 92.3% of people consumed beef, mostly at home, and 98.2% preferred levels of cooking that ensured STEC removal from the surface of beef cuts. The preferred degree of ground beef doneness was "well-done" (48.2%). Cooking preference ranged from red to "medium-well done" (51.8%). Median HUS probability from Argentinean beef cut and ground beef consumption in children under 14 years old was <10-15 and 8.57x10-10, respectively. The expected average annual number of HUS cases and deaths due to beef cut and ground beef consumption was zero. Risk of infection and HUS probability correlated with salting effect on E. coli count, processing raw beef before vegetables, ways of storage and refrigeration temperature at home, joint consumption of salad and beef cuts, degree of beef doneness and cutting board washing with detergent after each use with beef and vegetables. The STEC-HUS risk in Israel from consumption of bovine beef produced in Argentina was negligible. The current QMRA results were similar to those of previous beef cut consumption QMRA in Argentina and lower than any of the QMRA performed worldwide in other STEC-HUS linked to ground beef consumption. This study confirms the importance of QMRA to estimate and manage the risk of STEC-HUS from beef consumption. The impact variables identified in the sensitivity analysis allowed us to optimize resources and time management, to focus on accurate actions and to avoid taking measures that would not have an impact on the risk of STEC-HUS.

Project description:BackgroundAtypical haemolytic uraemic syndrome (aHUS) is a rare, potentially life-threatening condition caused by dysregulation of the complement pathway. Eculizumab is currently the only approved treatment for this disorder.ObjectiveOur objective was to investigate the impact of early administration of eculizumab on inpatient resource use and hospitalisation costs in 222 patients with aHUS.MethodsWe conducted a retrospective analysis of the Premier Perspective® Hospital Database, including patients with a diagnosis of aHUS and evidence of eculizumab use for aHUS. Early initiation was defined as having received eculizumab within 7 days of admission, with late initiation defined as starting eculizumab on day 8 or later. This date represents the average time required to obtain a specific diagnostic test to discriminate aHUS from a similar haemolytic syndrome that requires a different treatment. Outcome measures were time from first eculizumab initiation to discharge, discharge status or death, days spent in the intensive care unit (ICU), readmission indicators, dialysis indicators, and total hospital costs. Time from first eculizumab initiation to discharge was analysed using a generalised linear model with a log link and an assumed underlying negative binomial distribution. Logistic regression models were used to test the statistical significance of early versus late initiation as a predictor of the occurrence of readmissions, dialysis, and death. Total hospital costs were analysed using a generalised linear model with a log link and an assumed underlying gamma distribution.ResultsBefore modelling, total length of stay and ICU duration were significantly longer for late initiators than for early initiators, and significantly more late initiators were readmitted within 90 days. Late initiation was associated with significantly higher hospital costs than early initiation. After multivariable analysis, late initiators were 3.2 times more likely to require dialysis. However, there was no significant association between early initiation and time to discharge, readmission, or death for any definition or early initiation after multivariable analysis. Estimated total hospital costs (year 2017 values) were $US103,557 in late initiators and $US85,776 in early initiators (p = 0.0024).ConclusionInitiation of eculizumab within 7 days of hospitalisation is associated with lower dialysis rates, less time in ICU, less plasmapheresis, and lower hospitalisation costs compared with late initiation.

Dataset Information

Haemolytic uremic syndrome: diagnosis and management.

Publications

Haemolytic uremic syndrome: diagnosis and management.

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