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Article History

Received: 3 December 2024

Revised: 20 February 2025

Accepted: 14 March 2025

First Online: 16 April 2025

Compliance with ethical standards

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: The scientific guarantor of this publication is Mark O. Wielpütz.

: The authors of this manuscript declare relationships with the following companies: M.O.W.: Vertex Pharmaceuticals: Participation in clinical intervention trials with payment to the institution during the last 36 months. Honoraria for lectures from Vertex Pharmaceuticals paid to the institution. Boehringer Ingelheim: Study grants and honoraria for lectures paid to the institution. O.S.: Vertex Pharmaceuticals: Participation in 6 official clinical intervention trials with regular payment to the institution during the last 36 months. Honoraria for lectures of approximately (but not more than) 10,000 € during the last 36 months. M.E.: Vertex Pharmaceuticals: Honoraria for lectures from Vertex Pharmaceuticals. S.Y.G.: Vertex Pharmaceuticals: Personal fees for presentations and for advisory board. Grant payments made to the institution. Mukoviszidose Institut gGmbH, Bonn (German CF Foundation): Grants or contracts, payments made to the institution. H.U.K.: Siemens: Research support, payments to the institution. M.St.: Vertex Pharmaceuticals: Independent RIA grant (payment to institution), various clinical studies (payment to institution), honoraria for lectures and personal fees for advisory board. M.A.M.: Vertex Pharmaceuticals: Payments to the institution for independent medical grant. Personal fees for consultancy, lectures, travel reimbursement and advisory board membership. Boehringer Ingelheim: Personal fees for consultancy, lectures, travel reimbursement and advisory board membership. Arrowhead Pharmaceuticals: Personal fees for consultancy, lectures and advisory board membership. Santhera: Personal fees for consultancy and advisory board membership. Sterna Biologicals: Personal fees for consultancy. Enterprise Therapeutics: Personal fees for consultancy and advisory board membership. Kither Biotech: Personal fees for consultancy and advisory board membership. Antabio: Personal fees for consultancy and advisory board membership.

: No complex statistical methods were necessary for this paper.

: Written informed consent was obtained from all patients or their parents or legal guardians.

: This study is part of an ongoing prospective observational study (clinicaltrials.gov identifiers NCT00760071, NCT02270476 and DRKS00031784) and was approved by the responsible ethics committee of the coordinating center (Institutional review board of the University of Heidelberg, Germany).

: The study subjects have been previously reported in the following two publications: [, ]. However, the quantitative analysis of the perfusion measurements proposed in this study was never performed before.

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