Long-term Renal Prognosis of Diarrhea-Associated Hemolytic Uremic Syndrome

Garg, Amit X.; Suri, Rita S.; Barrowman, Nick; Rehman, Faisal; Matsell, Douglas G.; Rosas‐Arellano, M. Patricia; Salvadori, M; Haynes, R. Brian; Clark, William F.

doi:10.1001/jama.290.10.1360

Cited by 457 publications

(349 citation statements)

References 55 publications

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“…The observed mortality rate in the more recent period is the lowest ever reported in a series of this size, although the difference was not statistically significant compared with the mortality rate in the historical cohort (17). The prevalence of long-term renal complications observed in the recent period is comparable with previous observational studies of D+HUS (17).…”

Section: Discussionsupporting

confidence: 65%

Diarrhea-associated hemolytic uremic syndrome in southern Alberta: A long-term single-centre experience

Grisaru

Midgley²,

Hamiwka³

et al. 2011

Paediatrics &Amp; Child Health

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Section: Discussionsupporting

confidence: 65%

Diarrhea-associated hemolytic uremic syndrome in southern Alberta: A long-term single-centre experience

Grisaru

Midgley²,

Hamiwka³

et al. 2011

Paediatrics &Amp; Child Health

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“…HUS lacks specific treatment; HUS patients are generally given supportive care of electrolytes for water imbalance, anaemia, hypertension and renal failure (Mele et al, 2014). The mortality rate has decreased due to improved diagnosis and treatment, yet 1-2 % of patients die during the acute phase of the disease (Loirat, 2013;Mele et al, 2014) and 30 % of patients evidence long-term renal damage (Garg et al, 2003;Spinale et al, 2013).…”

Section: Introductionmentioning

confidence: 99%

Shiga toxins and stx phages: highly diverse entities

2015

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Shiga toxins are the main virulence factors of a group of Escherichia coli strains [Shiga toxinproducing E. coli (STEC)] that cause severe human diseases, such as haemorrhagic colitis and haemolytic-uraemic syndrome. The Shiga toxin family comprises several toxin subtypes, which have been differentially related to clinical manifestations. In addition, the phages that carry the Shiga toxin genes (stx phages) are also diverse. These phages play an important role not only in the dissemination of Shiga toxin genes and the emergence of new STEC strains, but also in the regulation of Shiga toxin production. Consequently, differences in stx phages may affect the dissemination of stx genes as well as the virulence of STEC strains. In addition to presenting an overview of Shiga toxins and stx phages, in this review we highlight current knowledge about the diversity of stx phages, with emphasis on its impact on STEC virulence. We consider that this diversity should be taken into account when developing STEC infection treatments and diagnostic approaches, and when conducting STEC control in reservoirs.

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“…Damage of endothelial cells would in itself activate complement [10]. Together the platelet and complement activation and endothelial damage leads to platelet aggregation, small vessel obstruction, mechanical haemolysis and TMA, mainly in the kidney but also in other organs, notably the central nervous system (CNS) and gastrointestinal tract [9,11,12]. The alternative pathway involves the constitutive low level activation of C3 and the subsequent deposit of C3b on cell membranes, ultimately leading to the generation of the membrane attack complex, (MAC), C5b-9.…”

Section: Haemolytic Uraemic Syndromementioning

confidence: 99%

“…For example, the outbreak in Germany in 2011 resulted in almost 4000 people being infected with a particularly virulent enteroaggregative E. coli strain, leading to 854 cases of HUS of which 54 (6.3%) died [24]. A pooled analysis of almost 3500 patients from 49 studies of STEC-HUS found that overall the incidence of death or end-stage renal disease (ESRD) was 12% and clinically significant renal damage occurred in 25% of patients [11].…”

Section: Hus: Stec-hus and Ahusmentioning

confidence: 99%

Atypical hemolytic uremic syndrome: from diagnosis to treatment

Franchini¹

2015

Clinical Chemistry and Laboratory Medicine (CCLM)

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Thrombotic microangiopathy (TMA) is a relatively rare condition but a medical urgency requiring immediate intervention to avoid irreversible organ damage or death. Symptoms on presentation include microangiopathic haemolytic anaemia, thrombocytopenia and organ damage. The most frequent direct causes of TMA are thrombotic thrombocytopenic purpura (TTP) and haemolytic uremic syndrome (HUS). The most common form of HUS is related to Shiga toxin producing Escherichia coli (STEC) infection while approximately 10% of cases are due to dysregulation of the complement pathway (atypical haemolytic uremic syndrome, aHUS). Optimal treatment regimens differ depending on the underlying cause; however, differential diagnosis may be difficult. The most accurate method of diagnosis is based on exclusion and should consider, beyond the symptoms common to TMA, ADAMTS13 activity levels and STEC infection status. For the management of TTP, plasma exchange (PE) is the most important acute intervention and is associated with lower mortality and better outcomes than plasma infusion. In most patients with STEC-HUS, the course of disease is selflimiting although management of acute kidney injury is often required. Until recently, the management of aHUS consisted of early and intensive PE, although this was mostly ineffective in protecting from subsequent organ damage. Eculizumab, an inhibitor of the alternative complement pathway, produces a rapid and sustained inhibition of the TMA process, with significant improvements in long-term clinical outcomes. Due to the significant improvement achieved, eculizumab has subsequently been approved as first-line therapy when an unequivocal diagnosis of aHUS has been made.

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Long-term Renal Prognosis of Diarrhea-Associated Hemolytic Uremic Syndrome

Cited by 457 publications

References 55 publications

Diarrhea-associated hemolytic uremic syndrome in southern Alberta: A long-term single-centre experience

Diarrhea-associated hemolytic uremic syndrome in southern Alberta: A long-term single-centre experience

Shiga toxins and stx phages: highly diverse entities

Atypical hemolytic uremic syndrome: from diagnosis to treatment

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