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Life-threatening influenza pneumonitis in a child with inherited IRF9 deficiency

Life-threatening pulmonary influenza can be caused by inborn errors of type I and III IFN immunity. We report a 5-yr-old child with severe pulmonary influenza at 2 yr. She is homozygous for a loss-of-function IRF9 allele. Her cells activate gamma-activated factor (GAF) STAT1 homodimers but not IFN-s...

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Detalles Bibliográficos
Autores principales: Hernandez, Nicholas, Melki, Isabelle, Jing, Huie, Habib, Tanwir, Huang, Susie S.Y., Danielson, Jeffrey, Kula, Tomasz, Drutman, Scott, Belkaya, Serkan, Rattina, Vimel, Lorenzo-Diaz, Lazaro, Boulai, Anais, Rose, Yoann, Kitabayashi, Naoki, Rodero, Mathieu P., Dumaine, Cecile, Blanche, Stéphane, Lebras, Marie-Noëlle, Leung, Man Chun, Mathew, Lisa Sara, Boisson, Bertrand, Zhang, Shen-Ying, Boisson-Dupuis, Stephanie, Giliani, Silvia, Chaussabel, Damien, Notarangelo, Luigi D., Elledge, Stephen J., Ciancanelli, Michael J., Abel, Laurent, Zhang, Qian, Marr, Nico, Crow, Yanick J., Su, Helen C., Casanova, Jean-Laurent
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Rockefeller University Press 2018
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6170168/
https://www.ncbi.nlm.nih.gov/pubmed/30143481
http://dx.doi.org/10.1084/jem.20180628