Outbreak of Shiga toxin-producing Escherichia-coli-associated hemolytic uremic syndrome in Istanbul in 2015: outcome and experience with eculizumab

Ağbaş, Ayşe; Göknar, Nilüfer; Akıncı, Nurver; Yıldırım, Zeynep; Taşdemir, Mehmet; Benzer, Meryem; Gökçe, İbrahim; Candan, Cengiz; Küçük, Nuran; Uzuner, Selçuk; Özçelik, Gül; Demirkol, Demet; Sever, Lale; Çalışkan, Salim

doi:10.1007/s00467-018-4033-0

Cited by 19 publications

(32 citation statements)

References 30 publications

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“…A retrospective case series from Istanbul reported nine paediatric patients with STEC-HUS treated with eculizumab at the discretion of the clinician at a median of 12 days (1-49 days)-in some cases, after the requirement for RRT had resolved [64]. Baseline characteristics between groups were similar but no descriptor for dialysis requirement or maximal creatinine was reported.…”

Section: Eculizumabmentioning

confidence: 99%

“…Baseline characteristics between groups were similar but no descriptor for dialysis requirement or maximal creatinine was reported. Two patients treated with eculizumab were reported to have a reduction in blood product requirements and one child had resolution of neurological involvement and dialysis requirements within 48 h. One child died at day 50 from Gram-negative sepsis following five eculizumab doses [64]. The wide variation and timing of eculizumab administration, loss to follow-up and unclear severity comparators limit the applicability of these data [64].…”

Section: Eculizumabmentioning

confidence: 99%

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Shiga-Toxin E. coli Hemolytic Uremic Syndrome: Review of Management and Long-term Outcome

2020

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Purpose of Review We review the pathophysiology of Shiga-Toxin Enteropathogenic-Hemolytic Uremic Syndrome (STEC-HUS), strategies to ameliorate or prevent evolution of STEC-HUS, management and the improved recognition of long-term adverse outcomes. Recent Findings Following on from the preclinical evidence of a role for the complement system in STEC-HUS, the use of complement blocking agents has been the major focus of most recent clinical research. Novel therapies to prevent or lessen HUS have yet to enter the clinical arena. The long-term outcomes of STEC-HUS, similarly to other causes of AKI, are not as benign as previously thought. Summary Optimizing supportive care in STEC-HUS is the only current recommended treatment. The administration of early isotonic fluids may reduce the severity and duration of STEC-HUS. The role of complement blockade in the management of STEC-HUS remains unclear. The long-term sequelae from STEC-HUS are significant and patients with apparent full renal recovery remain at risk.

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Section: Eculizumabmentioning

confidence: 99%

Section: Eculizumabmentioning

confidence: 99%

Shiga-Toxin E. coli Hemolytic Uremic Syndrome: Review of Management and Long-term Outcome

2020

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“…FFP infusion, TPE, and eculizumab can be given to patients who have severe STEC-HUS accompanying with multiple organ involvement. [12][13][14] The primary rationale behind the ED team's decision to initiate FFP infusion in the patient's first admission was that complement-mediated HUS cases might occasionally be presented with bloody diarrhea. The initiation of FFP was done as a preemptive measure.…”

Section: Discussionmentioning

confidence: 99%

“…Luckily, the serotype O104:H4 had been detected in Turkey earlier, so we had ample experience how to deal with the condition. 13 Though, we still think that the combination of STEC serotype 0157:H7 and enteroaggregative E. coli serotype O104:H4 probably played an active role in regard to increasing the severity of the disease. Unfortunately, we do not have any scientific evidence about this relationship.…”

Section: Discussionmentioning

confidence: 99%

Sudden Cardiac Arrest and Malignant Ventricular Tachycardia in an 8-Year-Old Pediatric Patient Who Has Hemolytic Uremic Syndrome Associated with Shiga Toxin-Producing Escherichia coli

Yeşilbaş

Akıncı

Talebazadeh

et al. 2020

J Pediatr Intensive Care

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Gastrointestinal, neurological, pancreatic, hepatic, and cardiac dysfunction are extrarenal manifestations of hemolytic uremic syndrome associated with Shiga toxin-producing Escherichia coli (STEC-HUS). The most frequent cause of death for STEC-HUS is related to the central nervous system and cardiovascular system. Cardiac-origin deaths are predominantly related to thrombotic microangiopathy-induced ischemia and the immediate development of circulatory collapse. STEC-HUS cardiac related deaths in children are rare with only sporadic cases reported. In our literature search, we did not come across any pediatric case report about STEC-HUS causing sudden cardiac arrest and malignant ventricular tachycardia (VT). Herein, we report the case of an 8-year-old female with a typical clinical manifestation of STEC-HUS. On the seventh day of pediatric intensive care unit admission, the patient had a sudden cardiac arrest, requiring resuscitation for 10 minutes. The patient had return of spontaneous circulation with severe monomorphic pulsed malignant VT. Intravenous treatment with lidocaine, amiodarone and magnesium sulfate were promptly initiated, and we administered multiple synchronized cardioversions, but VT persisted. Furthermore, we were not able to ameliorate her refractory circulation insufficiency by advanced cardiopulmonary resuscitation. Thus, inevitably, the patient lost her life. This case illustrates the need for aggressive management and the dilemma that pediatric critical care specialists, cardiologists, and nephrologists have to face when dealing with STEC-HUS that is worsened by a sudden cardiac arrest accompanied with VT.

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“…Despite all these relevant findings, only a few clinical studies have correlated the complement system activation with the clinical course. Furthermore, they presented conflicting results and most of them reported small series of patients, or even included patients without microbiological diagnosis (20)(21)(22)(23)(24)(25)(26).…”

Section: Introductionmentioning

confidence: 99%

Low C3 Serum Levels Predict Severe Forms of STEC-HUS With Neurologic Involvement

et al. 2020

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Background: The correlation between the severity of hemolytic uremic syndrome related to Shiga toxin-producing Escherichia coli (STEC-HUS) and involvement of the complement system has been examined in a small number of studies, with conflicting results. In the present study, we investigated whether serum C3 levels on admission are associated with neurologic involvement. Methods: To this purpose, 68 consecutive STEC-HUS patients were recruited and main clinical and laboratory variables ad hospital admission were compared between those with or without neurologic involvement. Results: STEC-HUS patients who developed neurologic involvement (NI) showed significant higher leukocyte count, C-reactive protein and hemoglobin, and lower sodium levels as compared with those without. Interestingly, baseline serum levels of C3 were significantly lower in patients with NI as compared with those without (p < 0.001). Moreover, when stratified according to need of Eculizumab rescue therapy due to severe NI, patients treated with this drug showed baseline C3 serum levels significantly lower than those who were not (p < 0.001). Low C3 was independent risk factor for NI in our patients' population when entered as covariate in a multivariate logistic regression analysis including other major variables previously proposed as possible predictors of poor prognosis in STEC-HUS (for instance, leukocyte count, c-reactive protein, sodium levels) (HR 6.401, 95%CI 1.617-25.334, p = 0.008 for C3). To underline the role of complement in the worsening of STEC-HUS patients' clinical conditions and outcomes, all patients were divided into two groups according to the baseline lower vs. normal serum levels of C3 and the main data on care needs were assessed. Interestingly more patients with lower C3 serum levels required renal replacement therapy (p = 0.024), anti-hypertensive therapy (p = 0.011), Intensive Care Unit admission (p = 0.009), and longer hospitalization (p = 0.003), thus displaying Netti et al. C3 and Severe Neurologic STEC-HUS significantly more severe disease features as compared with those with normal C3 serum levels. Conclusions: Our data suggests that children with STEC-HUS with decreased C3 concentrations at admission are more likely to develop neurologic involvement and are at increased risk of having severe clinical complications.

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Outbreak of Shiga toxin-producing Escherichia-coli-associated hemolytic uremic syndrome in Istanbul in 2015: outcome and experience with eculizumab

Abstract: An outbreak of EAEC occurred in a specific region of Istanbul. Livestock markets were suspected as the source. Evidence for beneficial effects of eculizumab on renal outcome was not clear in this cohort.

Cited by 19 publications

References 30 publications

Shiga-Toxin E. coli Hemolytic Uremic Syndrome: Review of Management and Long-term Outcome

Shiga-Toxin E. coli Hemolytic Uremic Syndrome: Review of Management and Long-term Outcome

Sudden Cardiac Arrest and Malignant Ventricular Tachycardia in an 8-Year-Old Pediatric Patient Who Has Hemolytic Uremic Syndrome Associated with Shiga Toxin-Producing Escherichia coli

Low C3 Serum Levels Predict Severe Forms of STEC-HUS With Neurologic Involvement

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