{
  "abstract": "Recurrent, sterile inflammatory responses to surgery and trauma in a patient with a heterozygous, truncating NFKB1 variant are described. This case illustrates the autoinflammatory phenotype of truncating NFKB1 variants and highlights the prospect of a targeted, personalized immunomodulatory therapy.",
  "abstract_shown": true,
  "affiliations": [
    "Division of Immunology and Allergy, Children's Hospital of Philadelphia, Philadelphia, PA, USA.",
    "Division of Rheumatology, Children's Hospital of Philadelphia, Philadelphia, PA, USA.",
    "Department of Pediatrics, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, USA."
  ],
  "article_type": "research article",
  "arxiv": null,
  "author_total": 4,
  "authors": [
    {
      "affs": [
        0
      ],
      "name": "Kevin S Ackerman",
      "orcid": "0000-0001-6032-0020"
    },
    {
      "affs": [
        1
      ],
      "name": "Isabel F Armendi",
      "orcid": "0009-0008-5236-9682"
    },
    {
      "affs": [
        1,
        2
      ],
      "name": "Jay J Mehta",
      "orcid": "0000-0003-0364-7531"
    },
    {
      "affs": [
        0,
        2
      ],
      "name": "Neil Romberg",
      "orcid": "0000-0002-1881-5318"
    }
  ],
  "bibtex": "https://research.draconitic.com/paper/c5senjkthj9p1.bib",
  "cited_by": 0,
  "doi": "10.70962/jhi.20260157",
  "fulltext_url": "https://europepmc.org/articles/PMC13693849",
  "id": "c5senjkthj9p1",
  "issns": [
    "3065-8993"
  ],
  "landing_url": "https://europepmc.org/article/MED/42912666",
  "language": "eng",
  "licence": "CC-BY",
  "licence_label": "CC BY",
  "licence_source": "article",
  "licence_url": "https://creativecommons.org/licenses/by/4.0/",
  "metadata_licence": null,
  "native_subjects": [
    [
      "lcc",
      "RC581-607"
    ],
    [
      "openalex",
      "1312|Inflammasome and immune disorders"
    ]
  ],
  "openalex": "W7223414124",
  "pdf_url": "https://europepmc.org/articles/PMC13693849?pdf=render",
  "pmcid": "PMC13693849",
  "pmid": "42912666",
  "published": "2026-10-23",
  "publisher": "Rockefeller University Press",
  "ris": "https://research.draconitic.com/paper/c5senjkthj9p1.ris",
  "sources": [
    {
      "first_seen": "2026-10-30T12:20:03Z",
      "native_id": "MED:42912666",
      "source": "europepmc",
      "url": "https://europepmc.org/article/MED/42912666"
    },
    {
      "first_seen": "2026-10-30T13:44:17Z",
      "native_id": "a48f6c8303614958bed7f5b52320ad24",
      "source": "doaj",
      "url": "https://doi.org/10.70962/jhi.20260157"
    }
  ],
  "subjects": [
    {
      "field": "biochemistry",
      "origin": "openalex:1312|Inflammasome and immune disorders",
      "subfield": "molecular-biology"
    }
  ],
  "title": "NFKB1 truncation-associated recurrent, sterile inflammation effectively treated with IL-1β blockade.",
  "title_text": "NFKB1 truncation-associated recurrent, sterile inflammation effectively treated with IL-1β blockade.",
  "type": "peer-reviewed",
  "updated_at": "2026-10-30T13:44:17Z",
  "url": "https://research.draconitic.com/paper/c5senjkthj9p1",
  "venue": "Journal of human immunity",
  "versions": []
}