PREFUL MRI for Monitoring Perfusion and Ventilation Changes after Elexacaftor-Tezacaftor-Ivacaftor Therapy for Cystic Fibrosis: A Feasibility Study.

Autor: Dohna M; From the Department of Diagnostic and Interventional Radiology (M.D., A.V., F.K., T.F.K., J.G., D.M.R., F.W., J.V.C.), German Center for Lung Research (DZL), Biomedical Research in Endstage and Obstructive Lung Disease (BREATH) (A.V., F.K., T.F.K., J.G., S.T.P., F.C.R., G.H., F.W., A.M.D., J.V.C.), Department for Pediatric Pneumology, Allergology and Neonatology (S.T.P., G.H., A.M.D., J.V.C.), and Department of Respiratory Medicine (F.C.R.), Hannover Medical School, Carl-Neuberg-Str 1, 30625 Hannover, Germany; and European Reference Network on Rare and Complex Respiratory Diseases (ERN-LUNG), Frankfurt, Germany (F.C.R.)., Voskrebenzev A; From the Department of Diagnostic and Interventional Radiology (M.D., A.V., F.K., T.F.K., J.G., D.M.R., F.W., J.V.C.), German Center for Lung Research (DZL), Biomedical Research in Endstage and Obstructive Lung Disease (BREATH) (A.V., F.K., T.F.K., J.G., S.T.P., F.C.R., G.H., F.W., A.M.D., J.V.C.), Department for Pediatric Pneumology, Allergology and Neonatology (S.T.P., G.H., A.M.D., J.V.C.), and Department of Respiratory Medicine (F.C.R.), Hannover Medical School, Carl-Neuberg-Str 1, 30625 Hannover, Germany; and European Reference Network on Rare and Complex Respiratory Diseases (ERN-LUNG), Frankfurt, Germany (F.C.R.)., Klimeš F; From the Department of Diagnostic and Interventional Radiology (M.D., A.V., F.K., T.F.K., J.G., D.M.R., F.W., J.V.C.), German Center for Lung Research (DZL), Biomedical Research in Endstage and Obstructive Lung Disease (BREATH) (A.V., F.K., T.F.K., J.G., S.T.P., F.C.R., G.H., F.W., A.M.D., J.V.C.), Department for Pediatric Pneumology, Allergology and Neonatology (S.T.P., G.H., A.M.D., J.V.C.), and Department of Respiratory Medicine (F.C.R.), Hannover Medical School, Carl-Neuberg-Str 1, 30625 Hannover, Germany; and European Reference Network on Rare and Complex Respiratory Diseases (ERN-LUNG), Frankfurt, Germany (F.C.R.)., Kaireit TF; From the Department of Diagnostic and Interventional Radiology (M.D., A.V., F.K., T.F.K., J.G., D.M.R., F.W., J.V.C.), German Center for Lung Research (DZL), Biomedical Research in Endstage and Obstructive Lung Disease (BREATH) (A.V., F.K., T.F.K., J.G., S.T.P., F.C.R., G.H., F.W., A.M.D., J.V.C.), Department for Pediatric Pneumology, Allergology and Neonatology (S.T.P., G.H., A.M.D., J.V.C.), and Department of Respiratory Medicine (F.C.R.), Hannover Medical School, Carl-Neuberg-Str 1, 30625 Hannover, Germany; and European Reference Network on Rare and Complex Respiratory Diseases (ERN-LUNG), Frankfurt, Germany (F.C.R.)., Glandorf J; From the Department of Diagnostic and Interventional Radiology (M.D., A.V., F.K., T.F.K., J.G., D.M.R., F.W., J.V.C.), German Center for Lung Research (DZL), Biomedical Research in Endstage and Obstructive Lung Disease (BREATH) (A.V., F.K., T.F.K., J.G., S.T.P., F.C.R., G.H., F.W., A.M.D., J.V.C.), Department for Pediatric Pneumology, Allergology and Neonatology (S.T.P., G.H., A.M.D., J.V.C.), and Department of Respiratory Medicine (F.C.R.), Hannover Medical School, Carl-Neuberg-Str 1, 30625 Hannover, Germany; and European Reference Network on Rare and Complex Respiratory Diseases (ERN-LUNG), Frankfurt, Germany (F.C.R.)., Pallenberg ST; From the Department of Diagnostic and Interventional Radiology (M.D., A.V., F.K., T.F.K., J.G., D.M.R., F.W., J.V.C.), German Center for Lung Research (DZL), Biomedical Research in Endstage and Obstructive Lung Disease (BREATH) (A.V., F.K., T.F.K., J.G., S.T.P., F.C.R., G.H., F.W., A.M.D., J.V.C.), Department for Pediatric Pneumology, Allergology and Neonatology (S.T.P., G.H., A.M.D., J.V.C.), and Department of Respiratory Medicine (F.C.R.), Hannover Medical School, Carl-Neuberg-Str 1, 30625 Hannover, Germany; and European Reference Network on Rare and Complex Respiratory Diseases (ERN-LUNG), Frankfurt, Germany (F.C.R.)., Ringshausen FC; From the Department of Diagnostic and Interventional Radiology (M.D., A.V., F.K., T.F.K., J.G., D.M.R., F.W., J.V.C.), German Center for Lung Research (DZL), Biomedical Research in Endstage and Obstructive Lung Disease (BREATH) (A.V., F.K., T.F.K., J.G., S.T.P., F.C.R., G.H., F.W., A.M.D., J.V.C.), Department for Pediatric Pneumology, Allergology and Neonatology (S.T.P., G.H., A.M.D., J.V.C.), and Department of Respiratory Medicine (F.C.R.), Hannover Medical School, Carl-Neuberg-Str 1, 30625 Hannover, Germany; and European Reference Network on Rare and Complex Respiratory Diseases (ERN-LUNG), Frankfurt, Germany (F.C.R.)., Hansen G; From the Department of Diagnostic and Interventional Radiology (M.D., A.V., F.K., T.F.K., J.G., D.M.R., F.W., J.V.C.), German Center for Lung Research (DZL), Biomedical Research in Endstage and Obstructive Lung Disease (BREATH) (A.V., F.K., T.F.K., J.G., S.T.P., F.C.R., G.H., F.W., A.M.D., J.V.C.), Department for Pediatric Pneumology, Allergology and Neonatology (S.T.P., G.H., A.M.D., J.V.C.), and Department of Respiratory Medicine (F.C.R.), Hannover Medical School, Carl-Neuberg-Str 1, 30625 Hannover, Germany; and European Reference Network on Rare and Complex Respiratory Diseases (ERN-LUNG), Frankfurt, Germany (F.C.R.)., Renz DM; From the Department of Diagnostic and Interventional Radiology (M.D., A.V., F.K., T.F.K., J.G., D.M.R., F.W., J.V.C.), German Center for Lung Research (DZL), Biomedical Research in Endstage and Obstructive Lung Disease (BREATH) (A.V., F.K., T.F.K., J.G., S.T.P., F.C.R., G.H., F.W., A.M.D., J.V.C.), Department for Pediatric Pneumology, Allergology and Neonatology (S.T.P., G.H., A.M.D., J.V.C.), and Department of Respiratory Medicine (F.C.R.), Hannover Medical School, Carl-Neuberg-Str 1, 30625 Hannover, Germany; and European Reference Network on Rare and Complex Respiratory Diseases (ERN-LUNG), Frankfurt, Germany (F.C.R.)., Wacker F; From the Department of Diagnostic and Interventional Radiology (M.D., A.V., F.K., T.F.K., J.G., D.M.R., F.W., J.V.C.), German Center for Lung Research (DZL), Biomedical Research in Endstage and Obstructive Lung Disease (BREATH) (A.V., F.K., T.F.K., J.G., S.T.P., F.C.R., G.H., F.W., A.M.D., J.V.C.), Department for Pediatric Pneumology, Allergology and Neonatology (S.T.P., G.H., A.M.D., J.V.C.), and Department of Respiratory Medicine (F.C.R.), Hannover Medical School, Carl-Neuberg-Str 1, 30625 Hannover, Germany; and European Reference Network on Rare and Complex Respiratory Diseases (ERN-LUNG), Frankfurt, Germany (F.C.R.)., Dittrich AM; From the Department of Diagnostic and Interventional Radiology (M.D., A.V., F.K., T.F.K., J.G., D.M.R., F.W., J.V.C.), German Center for Lung Research (DZL), Biomedical Research in Endstage and Obstructive Lung Disease (BREATH) (A.V., F.K., T.F.K., J.G., S.T.P., F.C.R., G.H., F.W., A.M.D., J.V.C.), Department for Pediatric Pneumology, Allergology and Neonatology (S.T.P., G.H., A.M.D., J.V.C.), and Department of Respiratory Medicine (F.C.R.), Hannover Medical School, Carl-Neuberg-Str 1, 30625 Hannover, Germany; and European Reference Network on Rare and Complex Respiratory Diseases (ERN-LUNG), Frankfurt, Germany (F.C.R.)., Vogel-Claussen J; From the Department of Diagnostic and Interventional Radiology (M.D., A.V., F.K., T.F.K., J.G., D.M.R., F.W., J.V.C.), German Center for Lung Research (DZL), Biomedical Research in Endstage and Obstructive Lung Disease (BREATH) (A.V., F.K., T.F.K., J.G., S.T.P., F.C.R., G.H., F.W., A.M.D., J.V.C.), Department for Pediatric Pneumology, Allergology and Neonatology (S.T.P., G.H., A.M.D., J.V.C.), and Department of Respiratory Medicine (F.C.R.), Hannover Medical School, Carl-Neuberg-Str 1, 30625 Hannover, Germany; and European Reference Network on Rare and Complex Respiratory Diseases (ERN-LUNG), Frankfurt, Germany (F.C.R.).

Publikováno v: Radiology. Cardiothoracic imaging [Radiol Cardiothorac Imaging] 2024 Apr; Vol. 6 (2), pp. e230104.

Spirometric and anthropometric improvements in response to elexacaftor/tezacaftor/ivacaftor depending on age and lung disease severity.

Autor: Schütz K; Department of Pediatric Pneumology, Allergology and Neonatology, Hannover Medical School, Hannover, Germany.; Biomedical Research in Endstage and Obstructive Lung Disease Hannover (BREATH), German Center for Lung Research, Hannover Medical School, Hannover, Germany., Pallenberg ST; Department of Pediatric Pneumology, Allergology and Neonatology, Hannover Medical School, Hannover, Germany.; Biomedical Research in Endstage and Obstructive Lung Disease Hannover (BREATH), German Center for Lung Research, Hannover Medical School, Hannover, Germany., Kontsendorn J; Department of Pediatric Pneumology, Allergology and Neonatology, Hannover Medical School, Hannover, Germany., DeLuca D; Department of Pediatric Pneumology, Allergology and Neonatology, Hannover Medical School, Hannover, Germany.; Biomedical Research in Endstage and Obstructive Lung Disease Hannover (BREATH), German Center for Lung Research, Hannover Medical School, Hannover, Germany., Sukdolak C; Department of Pediatric Pneumology, Allergology and Neonatology, Hannover Medical School, Hannover, Germany.; Biomedical Research in Endstage and Obstructive Lung Disease Hannover (BREATH), German Center for Lung Research, Hannover Medical School, Hannover, Germany., Minso R; Department of Pediatric Pneumology, Allergology and Neonatology, Hannover Medical School, Hannover, Germany.; Biomedical Research in Endstage and Obstructive Lung Disease Hannover (BREATH), German Center for Lung Research, Hannover Medical School, Hannover, Germany., Büttner T; Department of Pediatric Pneumology, Allergology and Neonatology, Hannover Medical School, Hannover, Germany.; Biomedical Research in Endstage and Obstructive Lung Disease Hannover (BREATH), German Center for Lung Research, Hannover Medical School, Hannover, Germany.; Department of Respiratory Medicine, Hannover Medical School, Hannover, Germany., Wetzke M; Department of Pediatric Pneumology, Allergology and Neonatology, Hannover Medical School, Hannover, Germany., Dopfer C; Department of Pediatric Pneumology, Allergology and Neonatology, Hannover Medical School, Hannover, Germany., Sauer-Heilborn A; Department of Respiratory Medicine, Hannover Medical School, Hannover, Germany., Ringshausen FC; Biomedical Research in Endstage and Obstructive Lung Disease Hannover (BREATH), German Center for Lung Research, Hannover Medical School, Hannover, Germany.; Department of Respiratory Medicine, Hannover Medical School, Hannover, Germany.; European Reference Network on Rare and Complex Respiratory Diseases (ERN-LUNG), Frankfurt, Germany., Junge S; Department of Pediatric Pneumology, Allergology and Neonatology, Hannover Medical School, Hannover, Germany., Tümmler B; Department of Pediatric Pneumology, Allergology and Neonatology, Hannover Medical School, Hannover, Germany.; Biomedical Research in Endstage and Obstructive Lung Disease Hannover (BREATH), German Center for Lung Research, Hannover Medical School, Hannover, Germany., Hansen G; Department of Pediatric Pneumology, Allergology and Neonatology, Hannover Medical School, Hannover, Germany.; Biomedical Research in Endstage and Obstructive Lung Disease Hannover (BREATH), German Center for Lung Research, Hannover Medical School, Hannover, Germany., Dittrich AM; Department of Pediatric Pneumology, Allergology and Neonatology, Hannover Medical School, Hannover, Germany.; Biomedical Research in Endstage and Obstructive Lung Disease Hannover (BREATH), German Center for Lung Research, Hannover Medical School, Hannover, Germany.

Publikováno v: Frontiers in pharmacology [Front Pharmacol] 2023 Jul 04; Vol. 14, pp. 1171544. Date of Electronic Publication: 2023 Jul 04 (Print Publication: 2023).

Changes in cystic fibrosis transmembrane conductance regulator protein expression prior to and during elexacaftor-tezacaftor-ivacaftor therapy.

Autor: Stanke F; Department of Pediatric Pneumology, Allergology and Neonatology, Hannover Medical School, Hannover, Germany.; Biomedical Research in Endstage and Obstructive Lung Disease Hannover (BREATH), German Center for Lung Research, Hannover Medical School, Hannover, Germany., Pallenberg ST; Department of Pediatric Pneumology, Allergology and Neonatology, Hannover Medical School, Hannover, Germany., Tamm S; Department of Pediatric Pneumology, Allergology and Neonatology, Hannover Medical School, Hannover, Germany.; Biomedical Research in Endstage and Obstructive Lung Disease Hannover (BREATH), German Center for Lung Research, Hannover Medical School, Hannover, Germany., Hedtfeld S; Department of Pediatric Pneumology, Allergology and Neonatology, Hannover Medical School, Hannover, Germany., Eichhorn EM; Department of Pediatric Pneumology, Allergology and Neonatology, Hannover Medical School, Hannover, Germany., Minso R; Department of Pediatric Pneumology, Allergology and Neonatology, Hannover Medical School, Hannover, Germany., Hansen G; Department of Pediatric Pneumology, Allergology and Neonatology, Hannover Medical School, Hannover, Germany.; Biomedical Research in Endstage and Obstructive Lung Disease Hannover (BREATH), German Center for Lung Research, Hannover Medical School, Hannover, Germany., Welte T; Biomedical Research in Endstage and Obstructive Lung Disease Hannover (BREATH), German Center for Lung Research, Hannover Medical School, Hannover, Germany.; Department of Respiratory Medicine, Hannover Medical School, Hannover, Germany., Sauer-Heilborn A; Department of Respiratory Medicine, Hannover Medical School, Hannover, Germany., Ringshausen FC; Biomedical Research in Endstage and Obstructive Lung Disease Hannover (BREATH), German Center for Lung Research, Hannover Medical School, Hannover, Germany.; Department of Respiratory Medicine, Hannover Medical School, Hannover, Germany., Junge S; Department of Pediatric Pneumology, Allergology and Neonatology, Hannover Medical School, Hannover, Germany., Tümmler B; Department of Pediatric Pneumology, Allergology and Neonatology, Hannover Medical School, Hannover, Germany.; Biomedical Research in Endstage and Obstructive Lung Disease Hannover (BREATH), German Center for Lung Research, Hannover Medical School, Hannover, Germany., Dittrich AM; Department of Pediatric Pneumology, Allergology and Neonatology, Hannover Medical School, Hannover, Germany.; Biomedical Research in Endstage and Obstructive Lung Disease Hannover (BREATH), German Center for Lung Research, Hannover Medical School, Hannover, Germany.

Publikováno v: Frontiers in pharmacology [Front Pharmacol] 2023 Jan 27; Vol. 14, pp. 1114584. Date of Electronic Publication: 2023 Jan 27 (Print Publication: 2023).

Effects of Elexacaftor/Tezacaftor/Ivacaftor Therapy on Lung Clearance Index and Magnetic Resonance Imaging in Patients with Cystic Fibrosis and One or Two F508del Alleles.

Autor: Graeber SY; Department of Pediatric Respiratory Medicine, Immunology and Critical Care Medicine, and Cystic Fibrosis Center, and.; Berlin Institute of Health at Charité - Universitätsmedizin Berlin, Berlin, Germany.; German Center for Lung Research (DZL), associated partner site, Berlin, Germany., Renz DM; Department for Radiology., Stahl M; Department of Pediatric Respiratory Medicine, Immunology and Critical Care Medicine, and Cystic Fibrosis Center, and.; Berlin Institute of Health at Charité - Universitätsmedizin Berlin, Berlin, Germany.; German Center for Lung Research (DZL), associated partner site, Berlin, Germany., Pallenberg ST; Department for Pediatric Pneumology, Allergology, and Neonatology.; German Center for Lung Research, Biomedical Research in Endstage and Obstructive Lung Disease (BREATH)., Sommerburg O; Division of Pediatric Pulmonology and Allergy and Cystic Fibrosis Center, Department of Pediatrics.; Department of Translational Pulmonology, Translational Lung Research Center Heidelberg (TLRC), German Center for Lung Research (DZL), and., Naehrlich L; Department of Pediatrics, Justus-Liebig-University Giessen, Giessen, Germany; and.; Universities of Giessen and Marburg Lung Center (UGMLC), German Center for Lung Research (DZL), Giessen, Germany., Berges J; Division of Pediatric Pulmonology and Allergy and Cystic Fibrosis Center, Department of Pediatrics.; Department of Translational Pulmonology, Translational Lung Research Center Heidelberg (TLRC), German Center for Lung Research (DZL), and., Dohna M; Department for Radiology., Ringshausen FC; German Center for Lung Research, Biomedical Research in Endstage and Obstructive Lung Disease (BREATH).; Department for Pneumology, and., Doellinger F; Department of Radiology, Charité - Universitätsmedizin Berlin, corporate member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Berlin, Germany., Vitzthum C; Department of Pediatric Respiratory Medicine, Immunology and Critical Care Medicine, and Cystic Fibrosis Center, and.; German Center for Lung Research (DZL), associated partner site, Berlin, Germany., Röhmel J; Department of Pediatric Respiratory Medicine, Immunology and Critical Care Medicine, and Cystic Fibrosis Center, and.; German Center for Lung Research (DZL), associated partner site, Berlin, Germany., Allomba C; Department of Pediatric Respiratory Medicine, Immunology and Critical Care Medicine, and Cystic Fibrosis Center, and.; German Center for Lung Research (DZL), associated partner site, Berlin, Germany., Hämmerling S; Division of Pediatric Pulmonology and Allergy and Cystic Fibrosis Center, Department of Pediatrics., Barth S; Department of Pediatrics, Justus-Liebig-University Giessen, Giessen, Germany; and.; Universities of Giessen and Marburg Lung Center (UGMLC), German Center for Lung Research (DZL), Giessen, Germany., Rückes-Nilges C; Department of Pediatrics, Justus-Liebig-University Giessen, Giessen, Germany; and.; Universities of Giessen and Marburg Lung Center (UGMLC), German Center for Lung Research (DZL), Giessen, Germany., Wielpütz MO; Department of Translational Pulmonology, Translational Lung Research Center Heidelberg (TLRC), German Center for Lung Research (DZL), and.; Department of Diagnostic and Interventional Radiology, University of Heidelberg, Heidelberg, Germany., Hansen G; Department for Pediatric Pneumology, Allergology, and Neonatology.; German Center for Lung Research, Biomedical Research in Endstage and Obstructive Lung Disease (BREATH).; Cluster of Excellence RESIST (EXC 2155), German Research Foundation (DFG), Hannover Medical School, Hannover, Germany., Vogel-Claussen J; Department for Radiology.; Department for Pediatric Pneumology, Allergology, and Neonatology., Tümmler B; Department for Pediatric Pneumology, Allergology, and Neonatology.; German Center for Lung Research, Biomedical Research in Endstage and Obstructive Lung Disease (BREATH)., Mall MA; Department of Pediatric Respiratory Medicine, Immunology and Critical Care Medicine, and Cystic Fibrosis Center, and.; Berlin Institute of Health at Charité - Universitätsmedizin Berlin, Berlin, Germany.; German Center for Lung Research (DZL), associated partner site, Berlin, Germany., Dittrich AM; Department for Pediatric Pneumology, Allergology, and Neonatology.; German Center for Lung Research, Biomedical Research in Endstage and Obstructive Lung Disease (BREATH).

Publikováno v: American journal of respiratory and critical care medicine [Am J Respir Crit Care Med] 2022 Aug 01; Vol. 206 (3), pp. 311-320.

CFTR modulation with elexacaftor-tezacaftor-ivacaftor in people with cystic fibrosis assessed by the β-adrenergic sweat rate assay.

Autor: Pallenberg ST; Department of Pediatric Pneumology, Allergology and Neonatology, Hannover Medical School, Hannover, Germany., Junge S; Department of Pediatric Pneumology, Allergology and Neonatology, Hannover Medical School, Hannover, Germany., Ringshausen FC; Department of Respiratory Medicine, Hannover Medical School, Hannover, Germany; Biomedical Research in Endstage and Obstructive Lung Disease Hannover (BREATH), German Center for Lung Research, Hannover Medical School, Hannover, Germany., Sauer-Heilborn A; Department of Respiratory Medicine, Hannover Medical School, Hannover, Germany., Hansen G; Department of Pediatric Pneumology, Allergology and Neonatology, Hannover Medical School, Hannover, Germany; Biomedical Research in Endstage and Obstructive Lung Disease Hannover (BREATH), German Center for Lung Research, Hannover Medical School, Hannover, Germany., Dittrich AM; Department of Pediatric Pneumology, Allergology and Neonatology, Hannover Medical School, Hannover, Germany; Biomedical Research in Endstage and Obstructive Lung Disease Hannover (BREATH), German Center for Lung Research, Hannover Medical School, Hannover, Germany., Tümmler B; Department of Pediatric Pneumology, Allergology and Neonatology, Hannover Medical School, Hannover, Germany; Biomedical Research in Endstage and Obstructive Lung Disease Hannover (BREATH), German Center for Lung Research, Hannover Medical School, Hannover, Germany., Nietert M; Department of Medical Bioinformatics, University Medical Center Göttingen, Göttingen, Germany. Electronic address: manuel.nietert@med.uni-goettingen.de.

Publikováno v: Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society [J Cyst Fibros] 2022 May; Vol. 21 (3), pp. 442-447. Date of Electronic Publication: 2021 Oct 29.