Article
ABCA3-related interstitial lung disease beyond infancy.
Thorax - 1 Jun 2023
Li Yang, Seidl Elias, Knoflach Katrin, Gothe Florian, Forstner Maria Elisabeth, Michel Katarzyna, Pawlita Ingo, Gesenhues Florian, Sattler Franziska, Yang Xiaohua, Kroener Carolin, Reu-Hofer Simone, Ley-Zaporozhan Julia, Kammer Birgit, Krüger-Stollfuß Ingrid, Dinkel Julien, Carlens Julia, Wetzke Martin, Moreno-Galdó Antonio, Torrent-Vernetta Alba, Lange Joanna, Krenke Katarzyna, Rumman Nisreen, Mayell Sarah, Sismanlar Tugba, Aslan Ayse, Regamey Nicolas, Proesmans Marijke, Stehling Florian, Naehrlich Lutz, Ayse Kilinc, Becker Sebastian, Koerner-Rettberg Cordula, Plattner Erika, Manali Effrosyni D, Papiris Spyridon A, Campo Ilaria, Kappler Matthias, Schwerk Nicolaus, Griese Matthias
Abstract excerpt
BACKGROUND: The majority of patients with childhood interstitial lung disease (chILD) caused by pathogenic variants in ATP binding cassette subfamily A member 3 (ABCA3) develop severe respiratory insufficiency within their first year of life and succumb to disease if not lung transplanted. This register-based cohort study reviews patients with ABCA3 lung disease who survived beyond the age of 1 year. METHOD: Over...
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