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Article

Progressive Disease With Low Survival in Adult Patients With Pulmonary Fibrosis Carrying Surfactant-Related Gene Mutations: An Observational Study.

Chest - 1 Apr 2023

Klay Dymph, Grutters Jan C, van der Vis Joanne J, Platenburg Mark G J P, Kelder Johannes C, Tromp Ellen, van Moorsel Coline H M

Abstract excerpt

BACKGROUND: In some patients with progressive fibrosing interstitial lung disease (ILD), disease is caused by carriage of a mutation in a surfactant-related gene (SRG) such as SFTPC, SFTPA2, or ABCA3. However, no aggregated data on disease evolution and treatment outcome have been presented for these patients. RESEARCH QUESTION: In adult patients with ILD with an SRG mutation, what is the course of lung function...

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Identifiers

PMID: 36370864
DOI: 10.1016/j.chest.2022.11.002

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-07.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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