ArticleThe Journal of allergy and clinical immunology2021
Severe SARS-CoV-2 disease in the context of a NF-κB2 loss-of-function pathogenic variant.
Article in The Journal of allergy and clinical immunology, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 20 papers, 2 of them syntheses that pooled it.
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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.
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Who cites it
20 citing papers in PubMed, 2 syntheses or guidelines pooled it, 36 citations in OpenAlex.
- Clinical, Immunological, and Genetic Features in Patients with NFKB1 and NFKB2 Mutations: a Systematic Review.Journal of clinical immunology · 2024Pooled it
- COVID-19-related health outcomes in people with primary immunodeficiency: A systematic review.Clinical immunology (Orlando, Fla.) · 2022Pooled it
- Case report: novel NFKB2 variant associated with pediatric eosinophilic granulomatosis with polyangiitis (EGPA) in the COVID-19 pandemic.Pediatric rheumatology online journal · 2025Article
- Intrinsic functional defects in B cells of patients with NFKB2 mutations.Clinical and experimental immunology · 2025Article
- The Scope and Impact of Viral Infections in Common Variable Immunodeficiency (CVID) and CVID-like Disorders: A Literature Review.Journal of clinical medicine · 2024Review
- Severity of SARS-CoV-2 infection in children with inborn errors of immunity (primary immunodeficiencies): a systematic review.Allergy, asthma, and clinical immunology : official journal of the Canadian Society of Allergy and Clinical Immunology · 2023Article
- Struggling with COVID-19 in Adult Inborn Errors of Immunity Patients: A Case Series of Combination Therapy and Multiple Lines of Therapy for Selected Patients.Life (Basel, Switzerland) · 2023Article
- The Importance of Measuring SARS-CoV-2-Specific T-Cell Responses in an Ongoing Pandemic.Pathogens (Basel, Switzerland) · 2023Review
- Impact of SARS-CoV-2 infection and COVID-19 on patients with inborn errors of immunity.The Journal of allergy and clinical immunology · 2023Review
- NFKB2 haploinsufficiency identified via screening for IFN-α2 autoantibodies in children and adolescents hospitalized with SARS-CoV-2-related complications.The Journal of allergy and clinical immunology · 2023Article
- Immunizing the imperfect immune system: Coronavirus disease 2019 vaccination in patients with inborn errors of immunity.Annals of allergy, asthma & immunology : official publication of the American College of Allergy, Asthma, & Immunology · 2022Review
- COVID-19 and Inborn Errors of Immunity.Physiology (Bethesda, Md.) · 2022Review
- Benefit-risk evaluation of COVID-19 vaccination in special population groups of interest.Vaccine · 2022Review
- COMMON VARIABLE IMMUNODEFICIENCY: PREDISPOSING OR PROTECTIVE FACTOR FOR SEVERE COMPLICATIONS OF COVID-19?Acta clinica Croatica · 2022Review
- Review
- Common Variable Immunodeficiency Disorders, T-Cell Responses to SARS-CoV-2 Vaccines, and the Risk of Chronic COVID-19.The journal of allergy and clinical immunology. In practice · 2021Article
- Zoonotic coronavirus epidemics: Severe acute respiratory syndrome, Middle East respiratory syndrome, and coronavirus disease 2019.Annals of allergy, asthma & immunology : official publication of the American College of Allergy, Asthma, & Immunology · 2021Review
- COVID-19 Outcomes in Patients Undergoing B Cell Depletion Therapy and Those with Humoral Immunodeficiency States: A Scoping Review.Pathogens & immunity · 2021Article
- COVID-19 Infection in Patients with Humoral Immunodeficiency: A Case Series and Literature Review.Allergy & rhinology (Providence, R.I.)Article
- Peripheral B Cell Deficiency and Predisposition to Viral Infections: The Paradigm of Immune Deficiencies.Frontiers in immunologyReview
Corrections and comments
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Authors and funding
19 authors at 3 institutions in 1 country.
Funding
Abstract
backgroundSevere acute respiratory syndrome coronavirus 2 (SARS-CoV-2) is a novel coronavirus that emerged recently and has created a global pandemic. Symptomatic SARS-CoV-2 infection, termed coronavirus disease 2019 (COVID-19), has been associated with a host of symptoms affecting numerous organ systems, including the lungs, cardiovascular system, kidney, central nervous system, gastrointestinal tract, and skin, among others.
objectiveAlthough several risk factors have been identified as related to complications from and severity of COVID-19, much about the virus remains unknown. The host immune response appears to affect the outcome of disease. It is not surprising that patients with intrinsic or secondary immune compromise might be particularly susceptible to complications from SARS-CoV-2 infection. Pathogenic loss-of-function or gain-of-function heterozygous variants in nuclear factor-κB2 have been reported to be associated with either a combined immunodeficiency or common variable immunodeficiency phenotype.
methodsWe evaluated the functional consequence and immunologic phenotype of a novel NFKB2 loss of function variant in a 17-year-old male patient and describe the clinical management of SARS-CoV-2 infection in this context.
resultsThis patient required a 2-week hospitalization for SARS-CoV-2 infection, including 7 days of mechanical ventilation. We used biologic therapies to avert potentially fatal acute respiratory distress syndrome and treat hyperinflammatory responses. The patient had an immunologic phenotype of B-cell dysregulation with decreased switched memory B cells. Despite the underlying immune dysfunction, he recovered from the infection with intense management.
conclusionsThis clinical case exemplifies some of the practical challenges in management of patients with SARS-CoV-2 infection, especially in the context of underlying immune dysregulation.
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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.