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Inborn errors of immunity: Recent progress.
Seth, Neha; Tuano, Karen S; Chinen, Javier.
  • Seth N; Division of Immunology, Allergy and Retrovirology, Department of Pediatrics, Baylor College of Medicine, Texas Children's Hospital, The Woodlands, Tex.
  • Tuano KS; Division of Immunology, Allergy and Retrovirology, Department of Pediatrics, Baylor College of Medicine, Texas Children's Hospital, The Woodlands, Tex.
  • Chinen J; Division of Immunology, Allergy and Retrovirology, Department of Pediatrics, Baylor College of Medicine, Texas Children's Hospital, The Woodlands, Tex. Electronic address: jchinen@bcm.edu.
J Allergy Clin Immunol ; 148(6): 1442-1450, 2021 12.
Article in English | MEDLINE | ID: covidwho-1474659
ABSTRACT
Recent advances in the field of inborn errors of immunity (IEIs) have been wide in scope, including progress in mechanisms of disease, diagnosis, and management. New gene defects affecting the immune response continue to be reported, as many as 26 in the year 2020. It was noted that the presentation of IEIs might not include recurrent infections in 9% of cases, and that current diagnostic methods can identify molecular causes in 92% of patients with severe combined immunodeficiency. Progress in immunopathogenesis explained mechanisms leading to symptoms of autosomal-recessive hyper-IgE syndrome. There was an emphasis on research in primary antibody deficiencies. The benefit of antibiotic prophylaxis to reduce the frequency of infections was demonstrated in these patients. The regimen of rituximab and azathioprine or mycophenolate was proven effective for chronic granulocytic interstitial pneumonia. The efficacy and adverse events of hematopoietic stem cell transplant in different IEI conditions were reported, as well as different strategies to improve outcomes, supporting its use in immunodeficiency and immunodysregulatory syndromes. The recent pandemic of coronavirus disease 2019 affected patients with IEIs, in particular those with deficiency in the interferon-mediated activation of the immune response. Initial data suggest that coronavirus disease 2019 vaccines might elicit anti-coronavirus disease 2019-neutralizing antibody responses in some patients with IEI conditions.
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Full text: Available Collection: International databases Database: MEDLINE Main subject: Primary Immunodeficiency Diseases / COVID-19 / Genetic Diseases, Inborn / Immunologic Deficiency Syndromes Type of study: Diagnostic study Topics: Vaccines Limits: Humans Language: English Journal: J Allergy Clin Immunol Year: 2021 Document Type: Article

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Full text: Available Collection: International databases Database: MEDLINE Main subject: Primary Immunodeficiency Diseases / COVID-19 / Genetic Diseases, Inborn / Immunologic Deficiency Syndromes Type of study: Diagnostic study Topics: Vaccines Limits: Humans Language: English Journal: J Allergy Clin Immunol Year: 2021 Document Type: Article