---
title: "Influenza A(H1N1)–triggered aHUS in a child with homozygous CD46 variant treated with ravulizumab"
id: "pubmed-42579038"
canonical_url: "https://medichelpline.com/clinical-feed/pubmed-42579038"
content_type: "clinical_feed_article"
specialty: "Critical Care"
source_name: "PubMed / NCBI"
source_url: "https://pubmed.ncbi.nlm.nih.gov/42579038/"
doi: "10.1007/s13730-026-01171-w"
published_at: "2026-08-11T00:00:00.000Z"
evidence_level: "Journal Article"
license: "CC-BY-NC-4.0 / Informational Use"
---
# Influenza A(H1N1)–triggered aHUS in a child with homozygous CD46 variant treated with ravulizumab
## Provenance & Clinical Metadata
- **Canonical URL:** https://medichelpline.com/clinical-feed/pubmed-42579038
- **Specialty:** [Critical Care](https://medichelpline.com/clinical-feed/critical-care.md)
- **Primary Source:** PubMed / NCBI
- **Source URL:** [Original Journal Publication](https://pubmed.ncbi.nlm.nih.gov/42579038/)
- **DOI:** [10.1007/s13730-026-01171-w](https://doi.org/10.1007%2Fs13730-026-01171-w)
- **Published At:** 2026-08-11T00:00:00.000Z
- **Evidence Rating:** Journal Article
## Executive GIST (TL;DR)
- A previously healthy 12-year-old girl developed fever, diarrhea, abdominal pain, mucosal bleeding, jaundice, and acute kidney injury after four days of symptoms, with laboratory evidence of **microangiopathic hemolytic anemia** and thrombocytopenia. - ADAMTS13 activity was preserved, supporting a diagnosis outside thrombotic thrombocytopenic purpura; nasopharyngeal PCR detected **influenza A(H1N1)** and a gastrointestinal multiplex PCR identified enteropathogenic Escherichia coli without Shiga toxin. - Initial management included **therapeutic plasma exchange** and hemodialysis, but hematologic and renal parameters did not improve and multiorgan involvement progressed (respiratory failure, elevated pancreatic enzymes). - Treatment was changed to terminal complement blockade with **ravulizumab**, after which there was rapid hematologic normalization and eventual full renal recovery by day 106. - Genetic testing identified a homozygous splice-site variant in **CD46** (c.286 + 1G > C), consistent with **atypical hemolytic uremic syndrome (aHUS)** caused by defects in membrane-bound complement regulation. - The case emphasizes that infections can unmask genetic complement defects, that plasma exchange may be ineffective in membrane regulator–related aHUS, and that early **C5 inhibition** can produce favorable hematologic and renal outcomes. - Reported details such as clinical timeline, detected pathogens, preserved ADAMTS13 activity, genetic variant, and recovery milestones are those provided in the source; no additional unreported clinical or laboratory specifics were included in the original report.
## Clinical Analysis & Structured Key Points
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Affiliations Expand ### Affiliations * 1 Children's Hospital Los Angeles, Pasto, Nariño, Colombia. * 2 Children's Hospital Los Angeles, Pasto, Nariño, Colombia. andreacamilamz13@gmail.com. * PMID: **42579038** * DOI: [ 10.1007/s13730-026-01171-w ](https://doi.org/10.1007/s13730-026-01171-w) Item in Clipboard Case Reports # Influenza A(H1N1) triggered atypical hemolytic uremic syndrome in a child with homozygous CD46 variant successfully treated with ravulizumab: a case report Liliana Mazzillo Vega et al. CEN Case Rep. 2026. Show details Display options Display options Format Abstract PubMed PMID CEN Case Rep Actions * [ Search in PubMed ](https://pubmed.ncbi.nlm.nih.gov/?term=%22CEN+Case+Rep%22%5Bjour%5D&sort=date&sort_order=desc) * [ Search in NLM Catalog ](https://www.ncbi.nlm.nih.gov/nlmcatalog?term=%22CEN+Case+Rep%22%5BTitle+Abbreviation%5D) * [ Add to Search ](https://pubmed.ncbi.nlm.nih.gov/42579038/) . 2026 Aug 11;15(5):131. doi: 10.1007/s13730-026-01171-w. ### Authors [Liliana Mazzillo Vega](https://pubmed.ncbi.nlm.nih.gov/?term=Mazzillo+Vega+L&cauthor_id=42579038)[ 1 ](https://pubmed.ncbi.nlm.nih.gov/42579038/#short-view-affiliation-1 "Children's Hospital Los Angeles, Pasto, Nariño, Colombia."), [Andrea Camila Montero Zambrano](https://pubmed.ncbi.nlm.nih.gov/?term=Montero+Zambrano+AC&cauthor_id=42579038)[ 2 ](https://pubmed.ncbi.nlm.nih.gov/42579038/#short-view-affiliation-2 "Children's Hospital Los Angeles, Pasto, Nariño, Colombia. andreacamilamz13@gmail.com."), [Alejandro Luna Mazzillo](https://pubmed.ncbi.nlm.nih.gov/?term=Luna+Mazzillo+A&cauthor_id=42579038)[ 1 ](https://pubmed.ncbi.nlm.nih.gov/42579038/#short-view-affiliation-1 "Children's Hospital Los Angeles, Pasto, Nariño, Colombia.") ### Affiliations * 1 Children's Hospital Los Angeles, Pasto, Nariño, Colombia. * 2 Children's Hospital Los Angeles, Pasto, Nariño, Colombia. andreacamilamz13@gmail.com. * PMID: **42579038** * DOI: [ 10.1007/s13730-026-01171-w ](https://doi.org/10.1007/s13730-026-01171-w) Item in Clipboard Cite Display options Display options Format Abstract PubMed PMID ## Abstract To describe a pediatric case of aHUS triggered by influenza A(H1N1) infection in a patient with a homozygous pathogenic variant in CD46, successfully treated with ravulizumab. A previously healthy 12-year-old girl presented with a four-day history of fever, diarrhea, abdominal pain, mucosal bleeding, jaundice, and acute kidney injury. Initial evaluation confirmed microangiopathic hemolytic anemia and thrombocytopenia. ADAMTS13 activity was preserved. Nasopharyngeal polymerase chain reaction testing detected influenza A virus subtype H1N1, and a multiplex gastrointestinal polymerase chain reaction panel identified enteropathogenic Escherichia coli; Shiga toxin was not detected. Despite therapeutic plasma exchange and hemodialysis, there was no hematologic or renal improvement, and multi-organ involvement persisted, including respiratory failure and elevated pancreatic enzyme levels. Terminal complement inhibition with ravulizumab was initiated, leading to rapid hematologic normalization and full renal recovery by day 106. Genetic testing revealed a homozygous splice-site variant in CD46 (c.286 + 1G > C), consistent with atypical hemolytic uremic syndrome. This case underscores the importance of early C5 inhibition in complement-mediated thrombotic microangiopathy associated with defects in membrane-bound complement regulators, in which therapeutic plasma exchange may have limited efficacy. It also highlights how infections can act as triggers that unmask underlying genetic susceptibility. **Keywords:** Antibodies, monoclonal, humanized; Atypical hemolytic uremic syndrome; Complement system proteins; Genetic predisposition to disease; Influenza A virus, H1N1 subtype. © 2026. The Author(s), under exclusive licence to Japanese Society of Nephrology. [PubMed Disclaimer](https://pubmed.ncbi.nlm.nih.gov/disclaimer/) ## Conflict of interest statement Declarations. Conflict of interest: The authors have declared that no Conflict of interest exists. Ethical approval: This article does not contain any studies with human participants or animals performed by any of the authors. Informed consent: Informed consent was obtained from the legal guardians of the participant included in the study. ## References #### Bibliography 1. 1. Boyer O, Niaudet P. Hemolytic-uremic syndrome in children. Pediatr Clin North Am. 2022;69(6):1181–97. . - [DOI](https://doi.org/10.1016/j.pcl.2022.07.006) - [PubMed](https://pubmed.ncbi.nlm.nih.gov/36880929/) 2. 1. Bogdan RG, Anderco P, Ichim C, Cimpean AM, Todor SB, Glaja-Iliescu M, et al. Atypical hemolytic uremic syndrome: a review of complement dysregulation, genetic susceptibility and multiorgan involvement. JCM. 2025;14(7):2527. . - [DOI](https://doi.org/10.3390/jcm14072527) - [PubMed](https://pubmed.ncbi.nlm.nih.gov/40217974/) - [PMC](https://pmc.ncbi.nlm.nih.gov/articles/11989465/) 3. 1. Ardissino G, Cresseri D, Mancuso MC, Capone V, Porcaro L, Amico V, et al. Outcome of atypical hemolytic uremic syndrome: role of triggers and complement abnormalities in the response to C5 inhibition. J Nephrol. 2024;37(4):1017–26. . - [DOI](https://doi.org/10.1007/s40620-023-01873-9) - [PubMed](https://pubmed.ncbi.nlm.nih.gov/38280096/) 4. 1. Meyer BJ, Kunz N, Seki S, Higgins R, Ghosh A, Hupfer R, et al. Immunologic and genetic contributors to CD46-dependent immune dysregulation. J Clin Immunol. 2023;43(8):1840–56. . - [DOI](https://doi.org/10.1007/s10875-023-01547-y) - [PubMed](https://pubmed.ncbi.nlm.nih.gov/37477760/) - [PMC](https://pmc.ncbi.nlm.nih.gov/articles/10661731/) 5. 1. Fakhouri F, Fila M, Hummel A, Ribes D, Sellier-Leclerc AL, Ville S, et al. Eculizumab discontinuation in children and adults with atypical hemolytic-uremic syndrome: a prospective multicenter study. Blood. 2021;137(18):2438–49. . - [DOI](https://doi.org/10.1182/blood.2020009280) - [PubMed](https://pubmed.ncbi.nlm.nih.gov/33270832/) Show all 32 references ## Publication types * Case Reports Actions * [ Search in PubMed ](https://pubmed.ncbi.nlm.nih.gov/?term=%22Case+Reports%22%5Bpt%5D&sort=date&sort_order=desc) * [ Search in MeSH ](https://www.ncbi.nlm.nih.gov/mesh?term=Case+Reports) * [ Add to Search ](https://pubmed.ncbi.nlm.nih.gov/42579038/) ## MeSH terms * Antibodies, Monoclonal, Humanized* / therapeutic use Actions * [ Search in PubMed ](https://pubmed.ncbi.nlm.nih.gov/?term=%22Antibodies%2C+Monoclonal%2C+Humanized%2Ftherapeutic+use%22%5BMAJR%5D&sort=date&sort_order=desc) * [ Search in MeSH ](https://www.ncbi.nlm.nih.gov/mesh?term=Antibodies%2C+Monoclonal%2C+Humanized) * [ Add to Search ](https://pubmed.ncbi.nlm.nih.gov/42579038/) * Atypical Hemolytic Uremic Syndrome* / diagnosis Actions * [ Search in PubMed 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