ArticlePediatric nephrology (Berlin, Germany)2014
An audit analysis of a guideline for the investigation and initial therapy of diarrhea negative (atypical) hemolytic uremic syndrome.
Article in Pediatric nephrology (Berlin, Germany), 2014. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 60 papers, 6 of them syntheses that pooled it.
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Who cites it
60 citing papers in PubMed, 6 syntheses or guidelines pooled it.
- A systematic review of short-term outcomes in patients with anti-factor H associated atypical HUS managed with plasma exchanges versus eculizumab.Pediatric nephrology (Berlin, Germany) · 2026Pooled it
- Extrarenal manifestations of atypical hemolytic uremic syndrome: a systematic review and meta-analysis.Pediatric research · 2025Pooled it
- Barriers and facilitators to the implementation of guidelines in rare diseases: a systematic review.Orphanet journal of rare diseases · 2023Pooled it
- Systematic review of atypical hemolytic uremic syndrome biomarkers.Pediatric nephrology (Berlin, Germany) · 2022Pooled it
- Clinical Practice Guidelines for the Management of Atypical Hemolytic Uremic Syndrome in Korea.Journal of Korean medical science · 2016Guideline
- Clinical guides for atypical hemolytic uremic syndrome in Japan.Clinical and experimental nephrology · 2016Guideline
- Updates in atypical hemolytic syndrome.Current opinion in pediatrics · 2026Review
- Favorable Outcome With Eculizumab in Hemolytic Uremic Syndrome Presenting With Severe Neurological Complications: A Case Report.Case reports in nephrology · 2026Article
- Comprehensive genetic analysis and genotype-phenotype correlations in pediatric patients with atypical hemolytic uremic syndrome.Pediatric nephrology (Berlin, Germany) · 2025Article
- Indications and recent evidence for apheresis in children and adults with kidney diseases: a comprehensive review.Clinical kidney journal · 2025Review
- Atypical hemolytic uremic syndrome: pathophysiology, clinical presentation, and treatment strategies.Childhood kidney diseases · 2025Review
- Atypical Hemolytic Uremic Syndrome: A Review of Complement Dysregulation, Genetic Susceptibility and Multiorgan Involvement.Journal of clinical medicine · 2025Review
- A Case of Atypical Hemolytic Uremic Syndrome With a Complement Factor I Mutation Triggered by a Femoral Neck Fracture.Nephrology (Carlton, Vic.) · 2025Article
- C3 molecular structural and histopathological analyses in a pediatric case of atypical hemolytic uremic syndrome with life-threatening gastrointestinal bleeding-a case report.Frontiers in pediatrics · 2025Article
- Exploring the Intersection of Atypical Hemolytic Uremic Syndrome and Substance Use: A Comprehensive Narrative Review.Cureus · 2024Review
- Adolescence-onset atypical hemolytic uremic syndrome: is it different from infant-onset?Clinical and experimental nephrology · 2024Article
- Unprecedented Haemorrhagic Stroke: A Rare Manifestation of Atypical Haemolytic Syndrome.Cureus · 2024Article
- Living with Atypical Hemolytic Uremic Syndrome in the Netherlands: Patient and Family Perspective.Kidney international reports · 2024Article
- A case-based narrative review of pregnancy-associated atypical hemolytic uremic syndrome/complement-mediated thrombotic microangiopathy.Kidney international · 2024Article
- Article
Corrections and comments
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Authors and funding
17 authors.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundIn 2009, the European Paediatric Study Group for Haemolytic Uraemic Syndrome (HUS) published a clinical practice guideline for the investigation and initial therapy of diarrhea-negative HUS (now more widely referred to as atypical HUS, aHUS). The therapeutic component of the guideline (comprising early, high-volume plasmapheresis) was derived from anecdotal evidence and expert consensus, and the authors committed to auditing outcome.
methodsQuestionnaires were distributed to pediatric nephrologists across Europe, North America, and the Middle East, who were asked to complete one questionnaire per patient episode of aHUS between July 1, 2009 and December 31, 2010. Comprehensive, anonymous demographic and clinical data were collected.
resultsSeventy-one children were reported with an episode of aHUS during the audit period. Six cases occurred on a background of influenza A H1N1 infection. Of 71 patients, 59 (83 %) received plasma therapy within the first 33 days, of whom ten received plasma infusion only. Complications of central venous catheters occurred in 16 out of 51 patients with a catheter in-situ (31 %). Median time to enter hematological remission was 11.5 days, and eight of 71 (11 %) patients did not enter hematological remission by day 33. Twelve patients (17 %) remained dialysis dependent at day 33.
conclusionsThis audit provides a snapshot of the early outcome of a group of children with aHUS in the months prior to more widespread use of eculizumab.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.