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

doi:10.1084/jem.20180628

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Article

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

The Journal of experimental medicine - 1 Oct 2018

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

Abstract excerpt

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-stimulated gene factor 3 (ISGF3) trimers (STAT1/STAT2/IRF9) in response to IFN-α2b. The transcriptome induced by...

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PMID: 30143481
DOI: 10.1084/jem.20180628
PMCID: PMC6170168

Genes

STAT1
STAT2

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-08.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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