Article
GATA2 deficiency in children and adults with severe pulmonary alveolar proteinosis and hematologic disorders.
BMC pulmonary medicine - 12 Aug 2015
Griese Matthias, Zarbock Ralf, Costabel Ulrich, Hildebrandt Jenna, Theegarten Dirk, Albert Michael, Thiel Antonia, Schams Andrea, Lange Joanna, Krenke Katazyrna, Wesselak Traudl, Schön Carola, Kappler Matthias, Blum Helmut, Krebs Stefan, Jung Andreas, Kröner Carolin, Klein Christoph, Campo Ilaria, Luisetti Maurizio, Bonella Francesco
Abstract excerpt
BACKGROUND: The majority of cases with severe pulmonary alveolar proteinosis (PAP) are caused by auto-antibodies against GM-CSF. A multitude of genetic and exogenous causes are responsible for few other cases. Goal of this study was to determine the prevalence of GATA2 deficiency in children and adults with PAP and hematologic disorders. METHODS: Of 21 patients with GM-CSF-autoantibody negative PAP, 13 had no...
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