ArticleClinical and experimental nephrology2018
Clinical characteristics and genetic backgrounds of Japanese patients with atypical hemolytic uremic syndrome.
Article in Clinical and experimental nephrology, 2018. The graph could read no effect estimate from its abstract, so it casts no vote on the map. An erratum has been issued. Cited by 41 papers, 2 of them syntheses that pooled it.
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Who cites it
41 citing papers in PubMed, 2 syntheses or guidelines pooled 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
- Pregnancy-Associated Atypical Hemolytic Uremic Syndrome: A Systematic Review.Obstetrics and gynecology · 2020Pooled it
- Article
- Poststreptococcal Glomerulonephritis Secondary to Bacterial Vaginosis, Complicated by Hemolytic Anemia and Thrombocytopenia.Internal medicine (Tokyo, Japan) · 2026Article
- C3 p.Asp1115Asn Variant-associated Atypical Hemolytic Uremic Syndrome with Spontaneous Remission Triggered by Influenza and COVID-19.Internal medicine (Tokyo, Japan) · 2026Article
- Article
- Comprehensive genetic analysis and genotype-phenotype correlations in pediatric patients with atypical hemolytic uremic syndrome.Pediatric nephrology (Berlin, Germany) · 2025Article
- Atypical hemolytic uremic syndrome treated with anti-C5 antibody agent eculizumab, without genetic complement abnormalities.Nagoya journal of medical science · 2025Article
- Atypical hemolytic uremic syndrome: pathophysiology, clinical presentation, and treatment strategies.Childhood kidney diseases · 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
- Atypical hemolytic uremic syndrome with aFrontiers in pediatrics · 2025Article
- Terminal complement inhibition in atypical haemolytic uremic syndrome: a single-centre experience.Frontiers in pharmacology · 2025Article
- Outcomes from the International Society of Nephrology Hemolytic Uremic Syndromes International Forum.Kidney international · 2024Article
- Managing anti-factor H antibody-associated hemolytic uremic syndrome: time for consensus.Pediatric nephrology (Berlin, Germany) · 2024Article
- Eculizumab for adult patients with atypical haemolytic-uraemic syndrome: full dataset analysis of Japanese post-marketing surveillance.Journal of nephrology · 2024Article
- Atypical Hemolytic Uremic Syndrome Triggered by Acute Pancreatitis in a Patient with a Membrane Cofactor Protein (CD46) Genetic Variant.Internal medicine (Tokyo, Japan) · 2024Article
- Annual trends in atypical haemolytic uremic syndrome management in Japan and factors influencing early diagnosis and treatment: a retrospective study.Scientific reports · 2024Article
- Practical approach to thrombocytopenia in patients with sepsis: a narrative review.Thrombosis journal · 2024Review
- Anti-C5 monoclonal antibody treatment showing pathological resolution of complement-mediated atypical hemolytic uremic syndrome: a case report.BMC nephrology · 2024Article
Corrections and comments
- Erratum issued
Authors and funding
13 authors.
Funding
Abstract
backgroundAtypical hemolytic uremic syndrome (aHUS) is caused by complement overactivation, and its presentation and prognosis differ according to the underlying molecular defects. The aim of this study was to characterize the genetic backgrounds of aHUS patients in Japan and to elucidate the associations between their genetic backgrounds, clinical findings, and outcomes.
methodsWe conducted a nationwide epidemiological survey of clinically diagnosed aHUS patients and examined 118 patients enrolled from 1998 to 2016 in Japan. We screened variants of seven genes related to complement and coagulation, as well as positivity for anti-CFH antibodies, and assessed clinical manifestations, laboratory findings, and clinical course.
resultsThe most frequent genetic abnormalities were in C3 (31%) and the frequency of CFH variants was relatively low (10%) compared to Western countries. The predominant variant in this cohort was C3 p.I1157T (23%), which was related to favorable outcomes despite frequent relapses. A total of 72% of patients received plasma therapy, while 42% were treated with eculizumab. The prognosis of Japanese aHUS patients was relatively favorable, with a total mortality rate of 5.4% and a renal mortality rate of 15%.
conclusionsThe common occurrence of genotype C3, especially the p.I1157T variant was the characteristic of the genetic backgrounds of Japanese aHUS patients that differed from those of Caucasian patients. In addition, the favorable prognosis of patients with the unique C3 p.I1157T variant indicates that understanding the clinical characteristics of individual gene alterations is important for predicting prognosis and determining therapeutic strategies in aHUS.
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