Predictors of Fatality in Postdiarrheal Hemolytic Uremic Syndrome

Oakes, Robert S.; Siegler, Richard L.; McReynolds, Markham A; Pysher, Theodore J.; Pavia, Andrew T.

doi:10.1542/peds.2005-0785

Cited by 120 publications

(110 citation statements)

References 26 publications

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“…One potential strength of this study is that we present data on a large number of consecutive patients from a single institution with inclusion criteria of referral for plasma exchange and a blood picture consistent with thrombotic microangiopathy (regardless of presumed underlying cause). Our patient population of 65 cases was comparable with that of other series published in the literature [20][21][22]. Our consistent approach over the years has previously allowed us to aid identification of significant predisposing factors to TMA [2].…”

Section: Discussionsupporting

confidence: 64%

Infection frequently triggers thrombotic microangiopathy in patients with preexisting risk factors: A single‐institution experience

Douglas

Pollock

Young

et al. 2010

J of Clinical Apheresis

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Thrombotic microangiopathies are rare conditions characterized by microangiopathic hemolytic anemia, microthrombi, and multiorgan insult. The disorders, which include hemolytic uremic syndrome and thrombotic thrombocytopenic purpura, are often acute and life threatening. We report a retrospective analysis of 65 patients presenting to our institution from 1997 to 2008 with all forms of thrombotic microangiopathy. Therapeutic plasma exchange was a requirement for analysis and 65 patients were referred to our institution; 66% of patients were female and median age at presentation was 52 years. Bacterial infection was the most commonly identified etiologic factor and in the multivariate model was the only significant variable associated with survival outcome (odds ratio 5.1, 95% confidence interval, 1.2-21.7). As infection can be considered a common trigger event for thrombotic microangiopathy, patients with hepatobiliary sepsis may benefit from elective cholecystectomy. We conclude that bacterial infection frequently triggers TTP and other thrombotic microangiopathies in patients with preexisting risk factors and propose a model for the development of these syndromes. J. Clin. Apheresis 25:47-53, 2010. V V C 2010 Wiley-Liss, Inc.

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Section: Discussionsupporting

confidence: 64%

Infection frequently triggers thrombotic microangiopathy in patients with preexisting risk factors: A single‐institution experience

Douglas

Pollock

Young

et al. 2010

J of Clinical Apheresis

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“…Generally, the prognosis of STEC-HUS is favourable in the majority of children, although serious systemic complications, such as haemorrhagic colitis and CNS involvement, can occur and mortality is between 1% and 5% [40][41][42]. In patients aged over 65 years, a lethal outcome occurs in up to 50% of cases [43].…”

Section: Hus: Stec-hus and Ahusmentioning

confidence: 99%

“…In patients aged over 65 years, a lethal outcome occurs in up to 50% of cases [43]. Long-term consequences affect up to 20% of children with STEC-HUS and include arterial hypertension, neurological impairment, chronic kidney disease, or diabetes mellitus [40,41,44].…”

Section: Hus: Stec-hus and Ahusmentioning

confidence: 99%

“…While aHUS and STEC-HUS almost always have renal involvement and while severe renal injury requiring haemodialysis is less common in patients with severe ADAMTS13 deficiency, acute renal failure has been reported in up to 10% of TTP patients [50,53]. CNS involvement has been considered a hallmark of TTP, with neurologic injury reported in 25%-79% of patients at presentation, it is also the most frequent extra-renal symptom in aHUS, occurring in 10%-48% of patients [49] and can occur in patients with STEC-HUS [41]. Thus the type of organ involvement cannot be used as a reliable diagnostic tool.…”

Section: Diagnosismentioning

confidence: 99%

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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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“…Previous studies have demonstrated a significant association between hemoconcentration and severe bowel injury, the need for and longer duration of dialysis, and acute phase death [1,2]. In their recent paper published in Pediatric Nephrology, Ardissino and colleagues found in a cohort of 61 patients that hemoconcentration at admission also correlated with major neurological involvement [3].…”

mentioning

confidence: 99%

Hemoconcentration in hemolytic uremic syndrome: time to review the standard case definition?

2014

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Predictors of Fatality in Postdiarrheal Hemolytic Uremic Syndrome

Abstract: Diarrheal HUS patients presenting with oligoanuria, dehydration, WBC >20 x 10(9)/L, and hematocrit >23% are at substantial risk for fatal hemolytic uremic syndrome. Such individuals should be referred to pediatric tertiary care centers.

Cited by 120 publications

References 26 publications

Infection frequently triggers thrombotic microangiopathy in patients with preexisting risk factors: A single‐institution experience

Infection frequently triggers thrombotic microangiopathy in patients with preexisting risk factors: A single‐institution experience

Atypical hemolytic uremic syndrome: from diagnosis to treatment

Hemoconcentration in hemolytic uremic syndrome: time to review the standard case definition?

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