Open Access
Impact of elexacaftor/tezacaftor/ivacaftor on lung function, nutritional status, pulmonary exacerbation frequency and sweat chloride in people with cystic fibrosis: real-world evidence from the German CF Registry
Sivagurunathan Sutharsan
,
Stefanie Dillenhoefer
,
Matthias Welsner
,
Manuel Burkhart
,
Olaf Eickmeier
,
Matthias Kappler
,
Sarah Sieber
,
Susanne Naehrig
,
Lutz Naehrlich
,
Klaus Tenbrock
,
Claus Pfannenstiel
,
Dirk Steffen
,
Jochen Meister
,
Britta Welzenbach
,
Anette Scharschinger
,
Markus Kratz
,
Maike Pincus
,
Tobias Tenenbaum
,
Mirjam Stahl
,
Kerstin Landwehr
,
Stefanie Dillenhöfer
,
Hans Kössel
,
Petra Kaiser
,
Manfred Käding
,
Simone Stolz
,
Stefan Blaas
,
Jutta Hammermann
,
Monika Gappa
,
Antje Schuster
,
Dana Spittel
,
Sabine Zirlik
,
Sabina Schmitt
,
Florian Stehling
,
Sivagurunathan Sutharsan
,
Joachim Bargon
,
Malte Cremer
,
Christina Smaczny
,
Sebastian Fähndrich
,
Andrea Heinzmann
,
Lutz Nährlich
,
Stefan Kuhnert
,
Sebastian Schmidt
,
Bettina Wollschläger
,
Anna Nolde
,
Inka Held
,
Wolfgang Kamin
,
Felix C. Ringshausen
,
Anna-Maria Dittrich
,
Sabine Wege
,
Olaf Sommerburg
,
Norbert Geier
,
Sara Lisa Fleser
,
Heinrike Wilkens
,
Helmut Ellemunter
,
Michael Lorenz
,
Paul Vöhringer
,
Martin Schebek
,
Christian Timke
,
Ingrid Bobis
,
Thomas Nüßlein
,
Doris Dieninghoff
,
Ernst Rietschel
,
Bastian Klinkhammer
,
Freerk Prenzel
,
Alexandra Wald
,
Axel Kempa
,
Folke Brinkmann
,
Eva Lücke
,
Ines Adams
,
Krystyna Poplawska
,
Simone Lehmkühler
,
Monika Bauck
,
Anne Pfülb
,
Rainald Fischer
,
Gudrun Schopper
,
Susanne Nährig
,
Matthias Griese
,
Jörg Grosse
,
Peter Küster
,
Birte KinderHolger Köster
,
Susanne Büsing
,
Margarethe Pohl
,
Carsten Schwarz
,
Andreas Artlich
,
Alexander Kiefer
,
Manfred Ballmann
,
Nikola Gjorgjevski
,
Markus A. Rose
,
Friederike Ruf
,
Rolf Mahlberg
,
Wolfgang Thomas
,
Ute Graepler
,
Sebastian Bode
,
hilipp Meyn
,
Josef Rosenecker
,
Cordula Koerner
,
Klaus-Michael Keller
,
Tina Teßmer
,
Helge Hebestreit
,
Gerhild Lohse
Publication type: Journal Article
Publication date: 2023-09-01
scimago Q1
wos Q1
SJR: 4.088
CiteScore: 22.8
Impact factor: 13.0
ISSN: 26667762
PubMed ID:
37554663
Oncology
Health Policy
Internal Medicine
Abstract
Treatment with elexacaftor/tezacaftor/ivacaftor (ETI) improves multiple clinical outcomes in people with cystic fibrosis (pwCF) with at least one F508del allele. This study evaluated the real-world impact of ETI on lung function, nutritional status, pulmonary exacerbation frequency, and sweat chloride concentrations in a large group of pwCF.This observational cohort study used data from the German CF Registry for pwCF who received ETI therapy and were followed up for a period of 12 months.The study included 2645 pwCF from 67 centres in Germany (mean age 28.0 ± 11.5 years). Over the first year after ETI was initiated, percent predicted forced expiratory volume in 1 s (ppFEV1) increased by 11.3% (95% confidence interval [CI] 10.8-11.8, p < 0.0001), body mass index (BMI) z-score increased by 0.3 (95% CI 0.3-0.4, p < 0.0001) in individuals aged 12 to <18 years and BMI in adults increased by 1.4 kg/m2 (95% CI 1.3-1.4, p < 0.0001), pulmonary exacerbations decreased by 75.9% (p < 0.0001) and mean sweat chloride concentration decreased by 50.9 mmol/L (95% CI -52.6, -49.3, p < 0.0001). Improvements in ppFEV1 over the first year of therapy were greater in pwCF who had not previously received cystic fibrosis transmembrane conductance regulator (CFTR) modulator therapy (12.6% [95% CI 11.9-13.4] vs. 9.7% [95% CI 9.0-10.5] in those with prior CFTR modulator treatment.These real-world data are consistent with the findings of randomised clinical trials, and support the use of ETI as a highly effective treatment option for pwCF who have at least one F508del allele.None.
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46
Total citations:
46
Citations from 2024:
46
(100%)
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GOST
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Sutharsan S. et al. Impact of elexacaftor/tezacaftor/ivacaftor on lung function, nutritional status, pulmonary exacerbation frequency and sweat chloride in people with cystic fibrosis: real-world evidence from the German CF Registry // The Lancet Regional Health - Europe. 2023. Vol. 32. p. 100690.
GOST all authors (up to 50)
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Sutharsan S. et al. Impact of elexacaftor/tezacaftor/ivacaftor on lung function, nutritional status, pulmonary exacerbation frequency and sweat chloride in people with cystic fibrosis: real-world evidence from the German CF Registry // The Lancet Regional Health - Europe. 2023. Vol. 32. p. 100690.
Cite this
RIS
Copy
TY - JOUR
DO - 10.1016/j.lanepe.2023.100690
UR - https://doi.org/10.1016/j.lanepe.2023.100690
TI - Impact of elexacaftor/tezacaftor/ivacaftor on lung function, nutritional status, pulmonary exacerbation frequency and sweat chloride in people with cystic fibrosis: real-world evidence from the German CF Registry
T2 - The Lancet Regional Health - Europe
AU - Sutharsan, Sivagurunathan
AU - Dillenhoefer, Stefanie
AU - Welsner, Matthias
AU - Burkhart, Manuel
AU - Eickmeier, Olaf
AU - Kappler, Matthias
AU - Sieber, Sarah
AU - Naehrig, Susanne
AU - Naehrlich, Lutz
AU - Tenbrock, Klaus
AU - Pfannenstiel, Claus
AU - Steffen, Dirk
AU - Meister, Jochen
AU - Welzenbach, Britta
AU - Scharschinger, Anette
AU - Kratz, Markus
AU - Pincus, Maike
AU - Tenenbaum, Tobias
AU - Stahl, Mirjam
AU - Landwehr, Kerstin
AU - Dillenhöfer, Stefanie
AU - Kössel, Hans
AU - Kaiser, Petra
AU - Käding, Manfred
AU - Stolz, Simone
AU - Blaas, Stefan
AU - Hammermann, Jutta
AU - Gappa, Monika
AU - Schuster, Antje
AU - Spittel, Dana
AU - Zirlik, Sabine
AU - Schmitt, Sabina
AU - Stehling, Florian
AU - Sutharsan, Sivagurunathan
AU - Bargon, Joachim
AU - Cremer, Malte
AU - Smaczny, Christina
AU - Fähndrich, Sebastian
AU - Heinzmann, Andrea
AU - Nährlich, Lutz
AU - Kuhnert, Stefan
AU - Schmidt, Sebastian
AU - Wollschläger, Bettina
AU - Nolde, Anna
AU - Held, Inka
AU - Kamin, Wolfgang
AU - Ringshausen, Felix C.
AU - Dittrich, Anna-Maria
AU - Wege, Sabine
AU - Sommerburg, Olaf
AU - Geier, Norbert
AU - Fleser, Sara Lisa
AU - Wilkens, Heinrike
AU - Ellemunter, Helmut
AU - Lorenz, Michael
AU - Vöhringer, Paul
AU - Schebek, Martin
AU - Timke, Christian
AU - Bobis, Ingrid
AU - Nüßlein, Thomas
AU - Dieninghoff, Doris
AU - Rietschel, Ernst
AU - Klinkhammer, Bastian
AU - Prenzel, Freerk
AU - Wald, Alexandra
AU - Kempa, Axel
AU - Brinkmann, Folke
AU - Lücke, Eva
AU - Adams, Ines
AU - Poplawska, Krystyna
AU - Lehmkühler, Simone
AU - Bauck, Monika
AU - Pfülb, Anne
AU - Fischer, Rainald
AU - Schopper, Gudrun
AU - Nährig, Susanne
AU - Griese, Matthias
AU - Grosse, Jörg
AU - Küster, Peter
AU - KinderHolger Köster, Birte
AU - Büsing, Susanne
AU - Pohl, Margarethe
AU - Schwarz, Carsten
AU - Artlich, Andreas
AU - Kiefer, Alexander
AU - Ballmann, Manfred
AU - Gjorgjevski, Nikola
AU - Rose, Markus A.
AU - Ruf, Friederike
AU - Mahlberg, Rolf
AU - Thomas, Wolfgang
AU - Graepler, Ute
AU - Bode, Sebastian
AU - hilipp Meyn
AU - Rosenecker, Josef
AU - Koerner, Cordula
AU - Keller, Klaus-Michael
AU - Teßmer, Tina
AU - Hebestreit, Helge
AU - Lohse, Gerhild
PY - 2023
DA - 2023/09/01
PB - Elsevier
SP - 100690
VL - 32
PMID - 37554663
SN - 2666-7762
ER -
Cite this
BibTex (up to 50 authors)
Copy
@article{2023_Sutharsan,
author = {Sivagurunathan Sutharsan and Stefanie Dillenhoefer and Matthias Welsner and Manuel Burkhart and Olaf Eickmeier and Matthias Kappler and Sarah Sieber and Susanne Naehrig and Lutz Naehrlich and Klaus Tenbrock and Claus Pfannenstiel and Dirk Steffen and Jochen Meister and Britta Welzenbach and Anette Scharschinger and Markus Kratz and Maike Pincus and Tobias Tenenbaum and Mirjam Stahl and Kerstin Landwehr and Stefanie Dillenhöfer and Hans Kössel and Petra Kaiser and Manfred Käding and Simone Stolz and Stefan Blaas and Jutta Hammermann and Monika Gappa and Antje Schuster and Dana Spittel and Sabine Zirlik and Sabina Schmitt and Florian Stehling and Sivagurunathan Sutharsan and Joachim Bargon and Malte Cremer and Christina Smaczny and Sebastian Fähndrich and Andrea Heinzmann and Lutz Nährlich and Stefan Kuhnert and Sebastian Schmidt and Bettina Wollschläger and Anna Nolde and Inka Held and Wolfgang Kamin and Felix C. Ringshausen and Anna-Maria Dittrich and Sabine Wege and Olaf Sommerburg and others},
title = {Impact of elexacaftor/tezacaftor/ivacaftor on lung function, nutritional status, pulmonary exacerbation frequency and sweat chloride in people with cystic fibrosis: real-world evidence from the German CF Registry},
journal = {The Lancet Regional Health - Europe},
year = {2023},
volume = {32},
publisher = {Elsevier},
month = {sep},
url = {https://doi.org/10.1016/j.lanepe.2023.100690},
pages = {100690},
doi = {10.1016/j.lanepe.2023.100690}
}