Article
Lethal Influenza in Two Related Adults with Inherited GATA2 Deficiency.
Journal of clinical immunology - 1 May 2018
Sologuren Ithaisa, Martínez-Saavedra María Teresa, Solé-Violán Jordi, de Borges de Oliveira Edgar, Betancor Eva, Casas Inmaculada, Oleaga-Quintas Carmen, Martínez-Gallo Mónica, Zhang Shen-Ying, Pestano Jose, Colobran Roger, Herrera-Ramos Estefanía, Pérez Carmen, López-Rodríguez Marta, Ruiz-Hernández José Juan, Franco Nieves, Ferrer José María, Bilbao Cristina, Andújar-Sánchez Miguel, Álvarez Fernández Mercedes, Ciancanelli Michael J, Rodríguez de Castro Felipe, Casanova Jean-Laurent, Bustamante Jacinta, Rodríguez-Gallego Carlos
Abstract excerpt
The pathogenesis of life-threatening influenza A virus (IAV) disease remains elusive, as infection is benign in most individuals. We studied two relatives who died from influenza. We Sanger sequenced GATA2 and evaluated the mutation by gene transfer, measured serum cytokine levels, and analyzed circulating T- and B-cells. Both patients (father and son, P1 and P2) died in 2011 of H1N1pdm IAV infection at the ages...
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