Efficacity and safety of CFTR modulators in patients with interstitial lung disease caused by ABCA3 transporter deficiency

New approaches in rare interstitial lung diseases

Chair(s): Cormac McCarthy, Elisabetta Renzoni

Session type: Poster session

Mathilde Le Brun

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Efficacy of Jak inhibitors in children with intra-alveolar haemorrhage not related to COPA gene mutations

Anais Le

Poster

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Flow cytometric evaluation of whole lung lavage (WLL) fluid from patients with pulmonary alveolar proteinosis (PAP): preliminary findings

Lutz-bernhard Jehn

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Hypomorphic mutation in SFTPB leads to adult pulmonary fibrosis

Tifenn Desroziers

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Determining the diagnostic yield of genomic testing in interstitial lung disease in Ireland

Jisha Jasmin

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Myeloid-dependent immunosuppressive features drive IPF progression

Dmytro Sirokha

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WT SP-A restores abnormal oligomerization and secretion of mutant SFTPA1 and SFTPA2

Tifenn Desroziers

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Cystatin C is associated with interstitial lung abnormalities features

Yuchen Zhang

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Altered regulation and pro-proliferative role of RNA binding proteins in idiopathic pulmonary fibrosis

Poornima Mahavadi

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Cyclic AMP-dependent up-regulation of surfactant protein B in differentiating alveolar basal cells

Petra Khan

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Correlation between Quantitative 3D HRCT analysis and Lung Function in Diffuse Cystic Lung Diseases

Yasuhito Sekimoto

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DIAGNOSTIC VALUE OF KL-6 AND GDF-15 IN LYMPHANGIOLEIOMYOMATOSIS.

Marta Garcia Moyano

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Implication of the complement cascade in the establishment of the pre-metastatic niche through the induction of the epithelial-to-mesenchymal transition by circulating exosomes in lymphangioleiomyomatosis (LAM)

Amina Jouida

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Correlation between childhood interstitial lung disease (chILD) phenotype and ABCA3 variants

Simona Taverna

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Characterization of fibrodysplasia ossificans progressiva (FOP) by respiratory oscillometry (Osc)

Anastasiia Vasileva

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Interleukin-6 and krebs von den lungen-6 in lymphangioleiomyomatosis patients

Beatrice Perea

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Impact of familial character and/or the existence of a mutation in telomere-related genes in patients with pleuroparenchymal fibroelastosis

Alice Camboulive

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Efficacity and safety of CFTR modulators in patients with interstitial lung disease caused by ABCA3 transporter deficiency

Mathilde Le Brun

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Induced pluripotent stem cell derived type 2 alveolar epithelial cells: A novel platform for exploring telomere biology in pulmonary fibrosis

Mari Ozaki

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Machine learned decision tree for diagnosis of ASMD among patients with unexplained ILD

Francesco Bonella

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Necroptosis study in an epithelial in vitro model expressing surfactant protein A1

Ioannis Tomos

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The ERS International Congress 2023, Milan

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