ArticlePediatric nephrology (Berlin, Germany)2019
A pediatric neurologic assessment score may drive the eculizumab-based treatment of Escherichia coli-related hemolytic uremic syndrome with neurological involvement.
Article in Pediatric nephrology (Berlin, Germany), 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 25 papers, 2 of them syntheses that pooled it.
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Who cites it
25 citing papers in PubMed, 2 syntheses or guidelines pooled it, 34 citations in OpenAlex.
- Eculizumab in severe pediatric STEC-HUS and its impact on neurological prognosis-a systematic review and meta-analysis.European journal of pediatrics · 2025Pooled it
- Eculizumab in Shiga toxin-producing Escherichia coli hemolytic uremic syndrome: a systematic review.Pediatric nephrology (Berlin, Germany) · 2024Pooled it
- Oxidative Stress and Mitochondrial Dysfunction in Chronic Kidney Disease: From Molecular Mechanisms to Biomarkers and Targeted Therapies.Antioxidants (Basel, Switzerland) · 2026Review
- Inter-society consensus on the management of acute bloody diarrhea and shiga toxin-producing Escherichia coli infection in the molecular microbiology era.Italian journal of pediatrics · 2026Review
- Favorable Outcome With Eculizumab in Hemolytic Uremic Syndrome Presenting With Severe Neurological Complications: A Case Report.Case reports in nephrology · 2026Article
- Eculizumab is efficacious and safe in pediatric patients with various forms of hemolytic uremic syndrome: a retrospective clinical experience of a tertiary center.Frontiers in pharmacology · 2025Article
- Diagnostic and Therapeutic Aspects of Monoclonal Gammopathies of Renal Significance (MGRS): An Update.Diagnostics (Basel, Switzerland) · 2024Review
- Eculizumab in STEC-HUS with severe neurological involvement: a New Zealand perspective.Pediatric nephrology (Berlin, Germany) · 2024Article
- Gastrointestinal involvement in STEC-associated hemolytic uremic syndrome: 10 years in a pediatric center.Pediatric nephrology (Berlin, Germany) · 2024Observational
- Efficacy and Safety of Eculizumab in EnteroaggregativePediatric reports · 2024Article
- Factors affecting dialysis duration in children with Shiga toxin-producing Escherichia coli-associated hemolytic uremic syndrome.Pediatric nephrology (Berlin, Germany) · 2023Article
- Hemolytic uremic syndrome with central nervous system manifestations, a case report and literature review.Radiology case reports · 2023Article
- Review
- Neurological involvement in children with hemolytic uremic syndrome.European journal of pediatrics · 2022Article
- Review
- Peripheral nervous system manifestations of Shiga toxin-producing E. coli-induced haemolytic uremic syndrome in children.Italian journal of pediatrics · 2021Article
- Hemolytic uremic syndrome related to Shiga-like toxin-producing Escherichia coli with encephalitis hiding a human herpesvirus-6 infection: a case report.Journal of medical case reports · 2021Article
- Utilization Pattern for Eculizumab Among Children With Hemolytic Uremic Syndrome.Frontiers in pediatrics · 2021Article
- SARS-CoV-2 and Viral Sepsis: Immune Dysfunction and Implications in Kidney Failure.Journal of clinical medicine · 2020Review
- Recurrent Glomerulonephritis after Renal Transplantation: The Clinical Problem.International journal of molecular sciences · 2020Review
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Authors and funding
17 authors at 3 institutions in 1 country.
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No grant is acknowledged in the PubMed record.
Abstract
backgroundThrombotic microangiopathy (TMA) is a clinical syndrome encompassing a large group of rare but severe disorders including thrombotic thrombocytopenic purpura (TTP) and both typical and atypical forms of hemolytic uremic syndrome (HUS). The key role of the complement system is well known in TTP and atypical HUS, but recent reports describe its involvement in the pathogenesis of HUS secondary to gastrointestinal infections due to Shiga toxin-producing Escherichia coli (STEC).
methodsTMA mainly affects the kidney, but extra-renal complications are frequently described. The involvement of the central nervous system (CNS) represents often a life-threatening condition and it can result in serious long-term disability in HUS patients who overcome the acute phase of illness. In the present study, we retrospectively analyzed a pediatric cohort of a single tertiary pediatric hospital in Southern Italy, in which this complication occurred in 12/54 children (22% of cases), of whom five with severe neurological involvement had been successfully treated with eculizumab.
resultsThe great clinical variability of brain injury in our cohort has led us to retrospectively build a "neurological score" useful to assess the clinical severity of neurologic involvement. Subjects with higher neurologic score due to the most severe CNS involvement resulted in the group of patients early treated with eculizumab, obtaining a good clinical response (four out five patients). In conclusion, the early treatment with eculizumab in children with severe neurological involvement during STEC-HUS was associated with complete regression of both acute kidney injury (AKI) and neurological lesions observed at magnetic resonance imaging (MRI).
conclusionsA "neurological score" may be a useful tool to drive the early treatment of CNS complications in STEC-HUS with eculizumab, although future perspective controlled studies are urgently needed to validate this therapeutic approach.
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