ArticleClinical journal of the American Society of Nephrology : CJASN2013
Genetics and outcome of atypical hemolytic uremic syndrome: a nationwide French series comparing children and adults.
Article in Clinical journal of the American Society of Nephrology : CJASN, 2013. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 371 papers, 7 of them syntheses that pooled it.
What it found
Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.
The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.
Who cites it
371 citing papers in PubMed, 7 syntheses or guidelines pooled it, 703 citations in OpenAlex.
- Extrarenal manifestations of atypical hemolytic uremic syndrome: a systematic review and meta-analysis.Pediatric research · 2025Pooled it
- Kidney and pregnancy outcomes in pregnancy-associated atypical hemolytic uremic syndrome: A systematic review and meta-analysis.Medicine · 2025Pooled it
- Variants in complement genes are uncommon in patients with anti-factor H autoantibody-associated atypical hemolytic uremic syndrome.Pediatric nephrology (Berlin, Germany) · 2023Pooled it
- Systematic review of atypical hemolytic uremic syndrome biomarkers.Pediatric nephrology (Berlin, Germany) · 2022Pooled it
- Interventions for atypical haemolytic uraemic syndrome.The Cochrane database of systematic reviews · 2021Pooled it
- Anti-Factor B Antibodies and Acute Postinfectious GN in Children.Journal of the American Society of Nephrology : JASN · 2020Pooled it
- Pregnancy-Associated Atypical Hemolytic Uremic Syndrome: A Systematic Review.Obstetrics and gynecology · 2020Pooled it
- IPNA clinical practice recommendations for the management of aHUS and secondary TMA in children.Pediatric nephrology (Berlin, Germany) · 2026Article
- Real-time, Multidisciplinary Approach of Women With Pregnancy-Associated Thrombotic Microangiopathy.Kidney international reports · 2026Article
- Atypical Hemolytic Uremic Syndrome in Children and Adults With the Hot Spot C3 Gene Variant p.Arg161Trp.Kidney international reports · 2026Article
- Atypical Hemolytic Uremic Syndrome Associated With Malignant Hypertension Presenting With Pulmonary-Renal Syndrome-like Symptoms.Kidney medicine · 2026Article
- Article
- Complement inhibitor therapy in atypical hemolytic uremic syndrome (aHUS): evaluating the economic impact of introducing eculizumab biosimilars in Germany.BMC nephrology · 2026Article
- Analysis of causes and outcomes of principal secondary thrombotic microangiopathy: a 9-year cohort study from a tertiary pediatric center in China.Research and practice in thrombosis and haemostasis · 2026Article
- Association of Complement Genetics with Outcomes in IgA Nephropathy.Clinical journal of the American Society of Nephrology : CJASN · 2026Article
- Article
- C3 p.Asp1115Asn Variant-associated Atypical Hemolytic Uremic Syndrome with Spontaneous Remission Triggered by Influenza and COVID-19.Internal medicine (Tokyo, Japan) · 2026Article
- Hemoglobinuria-associated acute kidney injury in hemolytic uremic syndrome without renal thrombotic microangiopathy.BMC nephrology · 2026Article
- Comprehensive gene profiling by Next-Generation sequencing in a cohort of Egyptian pediatric Atypical HUS.Journal, genetic engineering & biotechnology · 2026Article
- Thrombotic microangiopathy after kidney transplantation: diagnosis and management strategies.Clinical kidney journal · 2026Review
311 more citing papers are in PubMed but not listed here.
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
16 authors at 2 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
BACKGROUND AND
objectivesAtypical hemolytic uremic syndrome (aHUS) is a rare complement-mediated kidney disease that was first recognized in children but also affects adults. This study assessed the disease presentation and outcome in a nationwide cohort of patients with aHUS according to the age at onset and the underlying complement abnormalities. DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: A total of 214 patients with aHUS were enrolled between 2000 and 2008 and screened for mutations in the six susceptibility factors for aHUS and for anti-factor H antibodies.
resultsOnset of aHUS occurred as frequently during adulthood (58.4%) as during childhood (41.6%). The percentages of patients who developed the disease were 23%, 40%, 70%, and 98% by age 2, 18, 40, and 60 years, respectively. Mortality was higher in children than in adults (6.7% versus 0.8% at 1 year) (P=0.02), but progression to ESRD after the first aHUS episode was more frequent in adults (46% versus 16%; P<0.001). Sixty-one percent of patients had mutations in their complement genes. The renal outcome was not significantly different in adults regardless of genetic background. Only membrane cofactor protein (MCP) and undetermined aHUS were less severe in children than adults. The frequency of relapse after 1 year was 92% in children with MCP-associated HUS and approximately 30% in all other subgroups.
conclusionMortality rate was higher in children than adults with aHUS, but renal prognosis was worse in adults than children. In children, the prognosis strongly depends on the genetic background.
Indexed as
Identifiers
What OpenQuestion holds
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.