{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,23]],"date-time":"2026-10-23T19:11:37Z","timestamp":1792782697310,"version":"4.5.0"},"reference-count":79,"publisher":"Springer Science and Business Media LLC","issue":"19-20","license":[{"start":{"date-parts":[[2026,4,8]],"date-time":"2026-04-08T00:00:00Z","timestamp":1775606400000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2026,4,8]],"date-time":"2026-04-08T00:00:00Z","timestamp":1775606400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"funder":[{"name":"Marietta Blau Grant"},{"name":"Medical University of Graz","id":[{"id":"https:\/\/ror.org\/02n0bts35","id-type":"ROR","asserted-by":"crossref"}]}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Wien Klin Wochenschr"],"published-print":{"date-parts":[[2026,10]]},"abstract":"<jats:title>Summary<\/jats:title>\n                  <jats:p>Cystic fibrosis (CF) is one of the most common rare, genetic diseases. Newly available, highly effective modulator therapy (HEMT) improves many domains of CF pathophysiology. Thereby, HEMT challenges current standards of care and prediction models of disease outcomes.<\/jats:p>\n                  <jats:p>We established a\u00a0public patient involvement (PPI) group including experts and caregivers of children with CF and collated a\u00a0narrative review on CF management. This review is based on the views of healthcare professionals and caregivers and summarizes important features of the disease, prevention and treatment which are currently considered relevant for caregivers and Austrian healthcare settings.<\/jats:p>","DOI":"10.1007\/s00508-026-02739-3","type":"journal-article","created":{"date-parts":[[2026,4,8]],"date-time":"2026-04-08T16:04:22Z","timestamp":1775664262000},"page":"612-622","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Cystic fibrosis care today\u2014a narrative review based on views of experts, patients and the public"],"prefix":"10.1007","volume":"138","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-3471-5664","authenticated-orcid":false,"given":"Florian","family":"Singer","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anja Maria","family":"Schaffer","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michaela","family":"Auer","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jakob","family":"Auer","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jasmin","family":"Pansy","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Vanessa","family":"Stadlbauer","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ernst","family":"Eber","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2026,4,8]]},"reference":[{"key":"2739_CR1","unstructured":"Verordnung des Eidgen\u00f6ssischen Departements des Innern \u00fcber Geburtsgebrechen (GgV-EDI). XIX. Angeborene Krankheiten, bei denen mehrere Organsysteme betroffen sind 480. Cystische Fibrose (Mucoviszidose). https:\/\/www.fedlex.admin.ch\/eli\/cc\/2021\/708\/de. Accessed 14.10.2025."},{"key":"2739_CR2","doi-asserted-by":"publisher","first-page":"2195","DOI":"10.1016\/S0140-6736(20)32542-3","volume":"397","author":"M Shteinberg","year":"2021","unstructured":"Shteinberg\u00a0M, Haq\u00a0IJ, Polineni\u00a0D, et\u00a0al. Cystic fibrosis. The Lancet. 2021;397:2195\u2013211. https:\/\/doi.org\/10.1016\/S0140-6736(20)32542-3.","journal-title":"The Lancet"},{"key":"2739_CR3","unstructured":"Zolin A, Adamoli A, Bakkeheim E. European Cystic Fibrosis Society Patient Registry (ECFSPR) Annual Report 2023. 2025. https:\/\/www.ecfs.eu. Accessed 9.10.2025."},{"key":"2739_CR4","doi-asserted-by":"publisher","unstructured":"Taylor-Cousar\u00a0JL, Robinson\u00a0PD, Shteinberg\u00a0M, et\u00a0al. CFTR modulator therapy: transforming the landscape of clinical care in cystic fibrosis. The Lancet. 2023;S0140673623016094. https:\/\/doi.org\/10.1016\/S0140-6736(23)01609-4.","DOI":"10.1016\/S0140-6736(23)01609-4"},{"issue":"3","key":"2739_CR5","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.02288-2020","volume":"58","author":"EF Mckone","year":"2021","unstructured":"Mckone\u00a0EF, Ariti\u00a0C, Jackson\u00a0A, et\u00a0al. Original article Survival estimates in European cystic fibrosis patients and the impact of socioeconomic factors: a retrospective registry Cohort study. Eur Respir\u00a0J. 2021;58(3)2002288. https:\/\/doi.org\/10.1183\/13993003.02288-2020.","journal-title":"Eur Respir J"},{"issue":"2","key":"2739_CR6","doi-asserted-by":"publisher","first-page":"243","DOI":"10.1016\/j.jcf.2020.07.017","volume":"20","author":"S Stanojevic","year":"2021","unstructured":"Stanojevic\u00a0S, Vukovojac\u00a0K, Sykes\u00a0J, et\u00a0al. Projecting the impact of delayed access to elexacaftor\/tezacaftor\/ivacaftor for people with Cystic Fibrosis. J\u00a0Cyst Fibros. 2021;20(2):243\u20139. https:\/\/doi.org\/10.1016\/j.jcf.2020.07.017.","journal-title":"J Cyst Fibros"},{"issue":"4","key":"2739_CR7","doi-asserted-by":"publisher","first-page":"607","DOI":"10.1016\/j.jcf.2023.02.004","volume":"22","author":"A Lopez","year":"2023","unstructured":"Lopez\u00a0A, Daly\u00a0C, Vega-Hernandez\u00a0G, et\u00a0al. Elexacaftor\/tezacaftor\/ivacaftor projected survival and long-term health outcomes in people with cystic fibrosis homozygous for F508del. J\u00a0Cyst Fibros. 2023;22(4):607\u201314. https:\/\/doi.org\/10.1016\/j.jcf.2023.02.004.","journal-title":"J Cyst Fibros"},{"key":"2739_CR8","doi-asserted-by":"publisher","DOI":"10.1136\/bmj.j3453","volume":"358","author":"S Staniszewska","year":"2017","unstructured":"Staniszewska\u00a0S, Brett\u00a0J, Simera\u00a0I, et\u00a0al. GRIPP2 reporting checklists: tools to improve reporting of patient and public involvement in research. BMJ. 2017;358:j3453. https:\/\/doi.org\/10.1136\/bmj.j3453.","journal-title":"BMJ"},{"key":"2739_CR9","doi-asserted-by":"publisher","DOI":"10.12688\/f1000research.15162.1","volume":"7","author":"P Hoddinott","year":"2018","unstructured":"Hoddinott\u00a0P, Pollock\u00a0A, O\u2019Cathain\u00a0A, et\u00a0al. How to incorporate patient and public perspectives into the design and conduct of research. F1000Research. 2018;7:752. https:\/\/doi.org\/10.12688\/f1000research.15162.1.","journal-title":"F1000Research"},{"key":"2739_CR10","doi-asserted-by":"publisher","first-page":"1922","DOI":"10.1038\/s41591-023-02445-x","volume":"29","author":"OL Aiyegbusi","year":"2023","unstructured":"Aiyegbusi\u00a0OL, McMullan\u00a0C, Hughes\u00a0SE, et\u00a0al. Considerations for patient and public involvement and engagement in health research. Nat Med. 2023;29:1922\u20139. https:\/\/doi.org\/10.1038\/s41591-023-02445-x.","journal-title":"Nat Med"},{"key":"2739_CR11","doi-asserted-by":"publisher","first-page":"45","DOI":"10.1038\/nrg3849","volume":"16","author":"GR Cutting","year":"2015","unstructured":"Cutting\u00a0GR. Cystic fibrosis genetics: from molecular understanding to clinical application. Nat Rev Genet. 2015;16:45\u201356. https:\/\/doi.org\/10.1038\/nrg3849.","journal-title":"Nat Rev Genet"},{"key":"2739_CR12","doi-asserted-by":"publisher","first-page":"966","DOI":"10.1002\/ppul.22519","volume":"47","author":"SA McColley","year":"2012","unstructured":"McColley\u00a0SA, Ren\u00a0CL, Schechter\u00a0MS, et\u00a0al. Risk factors for onset of persistent respiratory symptoms in children with cystic fibrosis. Pediatr Pulmonol. 2012;47:966\u201372. https:\/\/doi.org\/10.1002\/ppul.22519.","journal-title":"Pediatr Pulmonol"},{"key":"2739_CR13","doi-asserted-by":"publisher","first-page":"580","DOI":"10.1183\/13993003.00580-2017","volume":"50","author":"E Kieninger","year":"2017","unstructured":"Kieninger\u00a0E, Yammine\u00a0S, Korten\u00a0I, et\u00a0al. Elevated lung clearance index in infants with cystic fibrosis shortly after birth. Eur Respir\u00a0J. 2017;50:580\u20132017. https:\/\/doi.org\/10.1183\/13993003.00580-2017.","journal-title":"Eur Respir J"},{"key":"2739_CR14","doi-asserted-by":"publisher","first-page":"146","DOI":"10.1164\/rccm.200901-0069OC","volume":"180","author":"PD Sly","year":"2009","unstructured":"Sly\u00a0PD, Brennan\u00a0S, Gangell\u00a0C, et\u00a0al. Lung Disease at Diagnosis in Infants with Cystic Fibrosis Detected by Newborn Screening. Am J Respir Crit Care Med. 2009;180:146\u201352. https:\/\/doi.org\/10.1164\/rccm.200901-0069OC.","journal-title":"Am J Respir Crit Care Med"},{"key":"2739_CR15","doi-asserted-by":"publisher","first-page":"943","DOI":"10.1164\/rccm.202102-0278oc","volume":"204","author":"M Stahl","year":"2021","unstructured":"Stahl\u00a0M, Steinke\u00a0E, Graeber\u00a0SY, et\u00a0al. Magnetic Resonance Imaging Detects Progression of Lung Disease and Impact of Newborn Screening in Preschool Children with Cystic Fibrosis. Am J Respir Crit Care Med. 2021;204:943\u201353. https:\/\/doi.org\/10.1164\/rccm.202102-0278oc.","journal-title":"Am J Respir Crit Care Med"},{"key":"2739_CR16","doi-asserted-by":"publisher","unstructured":"Sanders\u00a0DB, Mayer-Hamblett\u00a0N, Rosenfeld\u00a0M, et\u00a0al. Characteristics of individuals with cystic fibrosis in the United States ineligible for ivacaftor and elexacaftor\/tezacaftor\/ivacaftor. J\u00a0Cyst Fibros. 2024;1569-1993(24)00799\u20119. https:\/\/doi.org\/10.1016\/j.jcf.2024.07.015.","DOI":"10.1016\/j.jcf.2024.07.015"},{"issue":"9","key":"2739_CR17","doi-asserted-by":"publisher","first-page":"917","DOI":"10.1164\/rccm.202308-1317OC","volume":"208","author":"P McNally","year":"2023","unstructured":"McNally\u00a0P, Lester\u00a0K, Stone\u00a0G, et\u00a0al. Improvement in Lung Clearance Index and Chest Computed Tomography Scores with Elexacaftor\/Tezacaftor\/Ivacaftor Treatment in People with Cystic Fibrosis Aged 12 Years and Older \u2013 The RECOVER Trial. Am J Respir Crit Care Med. 2023;208(9):917\u201329. https:\/\/doi.org\/10.1164\/rccm.202308-1317OC.","journal-title":"Am J Respir Crit Care Med"},{"key":"2739_CR18","doi-asserted-by":"publisher","first-page":"153","DOI":"10.1016\/j.jcf.2018.02.006","volume":"17","author":"C Castellani","year":"2018","unstructured":"Castellani\u00a0C, Duff\u00a0AJA, Bell\u00a0SC, et\u00a0al. ECFS best practice guidelines: the 2018 revision. J\u00a0Cyst Fibros. 2018;17:153\u201378. https:\/\/doi.org\/10.1016\/j.jcf.2018.02.006.","journal-title":"J Cyst Fibros"},{"key":"2739_CR19","doi-asserted-by":"publisher","first-page":"100","DOI":"10.1016\/j.smrv.2018.07.002","volume":"42","author":"A Shakkottai","year":"2018","unstructured":"Shakkottai\u00a0A, O\u2019Brien\u00a0LM, Nasr\u00a0SZ, et\u00a0al. Sleep disturbances and their impact in pediatric cystic fibrosis. Sleep Med Rev. 2018;42:100\u201310. https:\/\/doi.org\/10.1016\/j.smrv.2018.07.002.","journal-title":"Sleep Med Rev"},{"key":"2739_CR20","doi-asserted-by":"publisher","unstructured":"Schaupp\u00a0L, Addante\u00a0A, V\u00f6ller\u00a0M, et\u00a0al. Longitudinal Effects of Elexacaftor\/Tezacaftor\/Ivacaftor on Sputum Viscoelastic Properties, Airway Infection and Inflammation in Patients with Cystic Fibrosis. Eur Respir\u00a0J. 2023;2202153. https:\/\/doi.org\/10.1183\/13993003.02153-2022.","DOI":"10.1183\/13993003.02153-2022"},{"key":"2739_CR21","doi-asserted-by":"publisher","DOI":"10.1128\/mbio.00519-24","volume":"15","author":"CR Armbruster","year":"2024","unstructured":"Armbruster\u00a0CR, Hilliam\u00a0YK, Zemke\u00a0AC, et\u00a0al. Persistence and evolution of Pseudomonas aeruginosa following initiation of highly effective modulator therapy in cystic fibrosis. mBio. 2024;15:e00519-24. https:\/\/doi.org\/10.1128\/mbio.00519-24.","journal-title":"mBio"},{"key":"2739_CR22","doi-asserted-by":"publisher","first-page":"724","DOI":"10.1016\/j.jcf.2016.08.004","volume":"15","author":"SJ Schwarzenberg","year":"2016","unstructured":"Schwarzenberg\u00a0SJ, Hempstead\u00a0SE, McDonald\u00a0CM, et\u00a0al. Enteral tube feeding for individuals with cystic fibrosis: Cystic Fibrosis Foundation evidence-informed guidelines. J\u00a0Cyst Fibros. 2016;15:724\u201335. https:\/\/doi.org\/10.1016\/j.jcf.2016.08.004.","journal-title":"J Cyst Fibros"},{"key":"2739_CR23","doi-asserted-by":"publisher","first-page":"24","DOI":"10.1016\/S1569-1993(11)60005-2","volume":"10","author":"C Colombo","year":"2011","unstructured":"Colombo\u00a0C, Ellemunter\u00a0H, Houwen\u00a0R, et\u00a0al. Guidelines for the diagnosis and management of distal intestinal obstruction syndrome in cystic fibrosis patients. J\u00a0Cyst Fibros. 2011;10:24\u20138. https:\/\/doi.org\/10.1016\/S1569-1993(11)60005-2.","journal-title":"J Cyst Fibros"},{"issue":"3","key":"2739_CR24","doi-asserted-by":"publisher","first-page":"474","DOI":"10.1016\/j.jcf.2023.10.001","volume":"23","author":"JG Mainz","year":"2024","unstructured":"Mainz\u00a0JG, Lester\u00a0K, Elnazir\u00a0B, et\u00a0al. Reduction in abdominal symptoms (CFAbd-Score), faecal M2-pyruvate-kinase and Calprotectin over one year of treatment with Elexacaftor-Tezacaftor-Ivacaftor in people with CF aged \u2265\u202f12\u00a0years \u2013 The RECOVER study. J\u00a0Cyst Fibros. 2024;23(3):474\u201380. https:\/\/doi.org\/10.1016\/j.jcf.2023.10.001.","journal-title":"J Cyst Fibros"},{"key":"2739_CR25","doi-asserted-by":"publisher","DOI":"10.1038\/s41572-024-00538-6","volume":"10","author":"MA Mall","year":"2024","unstructured":"Mall\u00a0MA, Burgel\u00a0PR, Castellani\u00a0C, et\u00a0al. Cystic fibrosis. Nat Rev Dis Primers. 2024;10:53. https:\/\/doi.org\/10.1038\/s41572-024-00538-6.","journal-title":"Nat Rev Dis Primers"},{"key":"2739_CR26","doi-asserted-by":"publisher","first-page":"2697","DOI":"10.2337\/dc10-1768","volume":"33","author":"A Moran","year":"2010","unstructured":"Moran\u00a0A, Brunzell\u00a0C, Cohen\u00a0RC, et\u00a0al. Clinical care guidelines for cystic fibrosis-related diabetes: A position statement of the American Diabetes Association and a clinical practice guideline of the Cystic Fibrosis Foundation, endorsed by the Pediatric Endocrine Society. Diabetes Care. 2010;33:2697\u2013708. https:\/\/doi.org\/10.2337\/dc10-1768.","journal-title":"Diabetes Care"},{"issue":"6","key":"2739_CR27","doi-asserted-by":"publisher","first-page":"838","DOI":"10.1016\/j.jcf.2019.03.009","volume":"18","author":"M Rosenfeld","year":"2019","unstructured":"Rosenfeld\u00a0M, Cunningham\u00a0S, Harris\u00a0WT, et\u00a0al. An open-label extension study of ivacaftor in children with CF and a CFTR gating mutation initiating treatment at age 2\u20135 years (KLIMB). J\u00a0Cyst Fibros. 2019;18(6):838\u201343. https:\/\/doi.org\/10.1016\/j.jcf.2019.03.009.","journal-title":"J Cyst Fibros"},{"issue":"2","key":"2739_CR28","doi-asserted-by":"publisher","first-page":"263","DOI":"10.1016\/j.jcf.2023.01.012","volume":"22","author":"C Colombo","year":"2023","unstructured":"Colombo\u00a0C, Ramm\u00a0GA, Lindblad\u00a0A, et\u00a0al. Characterization of CFTR mutations in people with cystic fibrosis and severe liver disease who are not eligible for CFTR modulators. J\u00a0Cyst Fibros. 2023;22(2):263\u20135. https:\/\/doi.org\/10.1016\/j.jcf.2023.01.012.","journal-title":"J Cyst Fibros"},{"key":"2739_CR29","doi-asserted-by":"publisher","first-page":"1220","DOI":"10.1097\/HEP.0000000000000646","volume":"79","author":"ZM Sellers","year":"2024","unstructured":"Sellers\u00a0ZM, Assis\u00a0DN, Paranjape\u00a0SM, et\u00a0al. Cystic fibrosis screening, evaluation, and management of hepatobiliary disease consensus recommendations. Hepatology. 2024;79:1220\u201338. https:\/\/doi.org\/10.1097\/HEP.0000000000000646.","journal-title":"Hepatology"},{"key":"2739_CR30","doi-asserted-by":"publisher","DOI":"10.1016\/j.jhepr.2025.101512","volume":"7","author":"C Mouliade","year":"2025","unstructured":"Mouliade\u00a0C, Parlati\u00a0L, Tzedakis\u00a0S, et\u00a0al. Cystic fibrosis liver disease progression in the era of elexacaftor\u2013tezacaftor\u2013ivacaftor. JHEP Rep. 2025;7:101512. https:\/\/doi.org\/10.1016\/j.jhepr.2025.101512.","journal-title":"JHEP Rep"},{"issue":"2","key":"2739_CR31","doi-asserted-by":"publisher","first-page":"349","DOI":"10.1016\/j.jcf.2023.09.006","volume":"23","author":"DH Tewkesbury","year":"2024","unstructured":"Tewkesbury\u00a0DH, Scott\u00a0J, Barry\u00a0PJ, et\u00a0al. Effects of elexacaftor\/tezacaftor\/ivacaftor on liver fibrosis markers in adults with cystic fibrosis. J\u00a0Cyst Fibros. 2024;23(2):349\u201353. https:\/\/doi.org\/10.1016\/j.jcf.2023.09.006.","journal-title":"J Cyst Fibros"},{"issue":"6","key":"2739_CR32","doi-asserted-by":"publisher","first-page":"1122","DOI":"10.1016\/j.jcf.2024.03.011","volume":"23","author":"HE Protich","year":"2024","unstructured":"Protich\u00a0HE, Molleston\u00a0JP, Bozic\u00a0M, et\u00a0al. Elexacaftor\/Tezacaftor\/Ivacaftor use in Pediatric Cystic Fibrosis Patients with Advanced Liver Disease. J\u00a0Cyst Fibros. 2024;23(6):1122\u20138. https:\/\/doi.org\/10.1016\/j.jcf.2024.03.011.","journal-title":"J Cyst Fibros"},{"issue":"9","key":"2739_CR33","doi-asserted-by":"publisher","first-page":"2469","DOI":"10.1002\/ppul.26527","volume":"58","author":"CJ Bathgate","year":"2023","unstructured":"Bathgate\u00a0CJ, Muther\u00a0E, Georgiopoulos\u00a0AM, et\u00a0al. Positive and negative impacts of elexacaftor\/tezacaftor\/ivacaftor: Healthcare providers\u2019 observations across US centers. Pediatr Pulmonol. 2023;58(9):2469\u201377. https:\/\/doi.org\/10.1002\/ppul.26527.","journal-title":"Pediatr Pulmonol"},{"issue":"5","key":"2739_CR34","doi-asserted-by":"publisher","first-page":"951","DOI":"10.1016\/j.pcl.2022.05.008","volume":"69","author":"M Ischander","year":"2022","unstructured":"Ischander\u00a0M, Lozowski-Sullivan\u00a0S. Psychologist\u2019s Unique Role in Improving Quality of Life of Children with Chronic Lung Diseases and Their Families. Pediatr Clin North Am. 2022;69(5):951\u201363. https:\/\/doi.org\/10.1016\/j.pcl.2022.05.008.","journal-title":"Pediatr Clin North Am"},{"issue":"1","key":"2739_CR35","doi-asserted-by":"publisher","first-page":"26","DOI":"10.1136\/thoraxjnl-2015-207488","volume":"71","author":"AL Quittner","year":"2016","unstructured":"Quittner\u00a0AL, Abbott\u00a0J, Georgiopoulos\u00a0AM, et\u00a0al. International Committee on Mental Health in Cystic Fibrosis: Cystic Fibrosis Foundation and European Cystic Fibrosis Society consensus statements for screening and treating depression and anxiety. Thorax. 2016;71(1):26\u201334. https:\/\/doi.org\/10.1136\/thoraxjnl-2015-207488.","journal-title":"Thorax"},{"key":"2739_CR36","doi-asserted-by":"publisher","first-page":"244","DOI":"10.1002\/ppul.23091","volume":"50","author":"LS Blackwell","year":"2015","unstructured":"Blackwell\u00a0LS, Quittner\u00a0AL. Daily pain in adolescents with CF: Effects on adherence, psychological symptoms, and health-related quality of life. Pediatr Pulmonol. 2015;50:244\u201351. https:\/\/doi.org\/10.1002\/ppul.23091.","journal-title":"Pediatr Pulmonol"},{"issue":"4","key":"2739_CR37","doi-asserted-by":"publisher","first-page":"1136","DOI":"10.1002\/ppul.26304","volume":"58","author":"C Chadwick","year":"2023","unstructured":"Chadwick\u00a0C, Arcinas\u00a0R, Ham\u00a0M, et\u00a0al. The Use of DXA for Early Detection of Pediatric Cystic Fibrosis related Bone Disease. Pediatr Pulmonol. 2023;58(4):1136\u201344. https:\/\/doi.org\/10.1002\/ppul.26304.","journal-title":"Pediatr Pulmonol"},{"issue":"6","key":"2739_CR38","doi-asserted-by":"publisher","first-page":"980","DOI":"10.1016\/j.jcf.2023.04.011","volume":"22","author":"F Holz","year":"2023","unstructured":"Holz\u00a0F, Can\u00a0E, Grehn\u00a0C, et\u00a0al. Manifestation and staging of arthropathy in cystic fibrosis. Defining different stages of cystic fibrosis arthropathy using ultrasound imaging and clinical scoring. J\u00a0Cyst Fibros. 2023;22(6):980\u20138. https:\/\/doi.org\/10.1016\/j.jcf.2023.04.011.","journal-title":"J Cyst Fibros"},{"issue":"5","key":"2739_CR39","doi-asserted-by":"publisher","first-page":"944","DOI":"10.1016\/j.jcf.2023.04.002","volume":"22","author":"J Madaule","year":"2023","unstructured":"Madaule\u00a0J, Valenzuela\u00a0F, Mittaine\u00a0M, et\u00a0al. Exploration of the relationship between cumulative exposure to tobramycin and ototoxicity in patients with cystic fibrosis. J\u00a0Cyst Fibros. 2023;22(5):944\u20138. https:\/\/doi.org\/10.1016\/j.jcf.2023.04.002.","journal-title":"J Cyst Fibros"},{"issue":"1","key":"2739_CR40","doi-asserted-by":"publisher","DOI":"10.1183\/13993003","volume":"66","author":"MA Mall","year":"2025","unstructured":"Mall\u00a0MA, Wainwright\u00a0CE, Legg\u00a0J, et\u00a0al. Elexacaftor\/tezacaftor\/ivacaftor in children aged \u2265\u202f6\u00a0years with cystic fibrosis heterozygous for F508del and a\u00a0minimal function mutation: results from a\u00a096-week open-label extension study. Eur Respir\u00a0J. 2025;66(1)2402435. https:\/\/doi.org\/10.1183\/13993003.","journal-title":"Eur Respir J"},{"key":"2739_CR41","unstructured":"Royal Brompton Hospital. Clinical guidelines for the care of children with cystic fibrosis 2023. https:\/\/www.rbht.nhs.uk\/childrencf. Accessed 15.10.2025."},{"key":"2739_CR42","doi-asserted-by":"publisher","first-page":"43","DOI":"10.1016\/j.jcf.2014.03.011","volume":"13","author":"M Stern","year":"2014","unstructured":"Stern\u00a0M, Bertrand\u00a0DP, Bignamini\u00a0E, et\u00a0al. European Cystic Fibrosis Society Standards of Care: Quality Management in cystic fibrosis. J\u00a0Cyst Fibros. 2014;13:43\u201359. https:\/\/doi.org\/10.1016\/j.jcf.2014.03.011.","journal-title":"J Cyst Fibros"},{"key":"2739_CR43","doi-asserted-by":"publisher","unstructured":"Noordhoek JJ, Jeyaratnam J, Zomer D et al. Development and outcomes of a patient driven cystic fibrosis quality of care improvement project. J Cyst Fibros 2023;22(1):172\u2013178. https:\/\/doi.org\/10.1016\/j.jcf.2022.07.004.","DOI":"10.1016\/j.jcf.2022.07.004."},{"key":"2739_CR44","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1086\/676882","volume":"35","author":"L Saiman","year":"2014","unstructured":"Saiman\u00a0L, Siegel\u00a0JD, LiPuma\u00a0JJ, et\u00a0al. Infection Prevention and Control Guideline for Cystic Fibrosis: 2013 Update. Infect Control Hosp Epidemiol. 2014;35:1\u201367. https:\/\/doi.org\/10.1086\/676882.","journal-title":"Infect Control Hosp Epidemiol"},{"key":"2739_CR45","unstructured":"Cystische Fibrose Schweiz (CFCH). Hygieneempfehlungen bei Cystischer Fibrose. 2020. https:\/\/cystischefibroseschweiz.ch. Accessed 9.10.2025."},{"issue":"3","key":"2739_CR46","doi-asserted-by":"publisher","first-page":"376","DOI":"10.1016\/j.jcf.2019.08.006","volume":"19","author":"E Hatziagorou","year":"2020","unstructured":"Hatziagorou\u00a0E, Orenti\u00a0A, Drevinek\u00a0P, et\u00a0al. Changing epidemiology of the respiratory bacteriology of patients with cystic fibrosis\u2013data from the European cystic fibrosis society patient registry. J\u00a0Cyst Fibros. 2020;19(3):376\u201383. https:\/\/doi.org\/10.1016\/j.jcf.2019.08.006.","journal-title":"J Cyst Fibros"},{"issue":"1","key":"2739_CR47","doi-asserted-by":"publisher","first-page":"12","DOI":"10.1016\/j.jcf.2023.12.002","volume":"23","author":"K Southern","year":"2024","unstructured":"Southern\u00a0K, Addy\u00a0C, Bell\u00a0S, et\u00a0al. Standards for the care of people with cystic fibrosis; establishing and maintaining health. J\u00a0Cyst Fibros. 2024;23(1):12\u201328. https:\/\/doi.org\/10.1016\/j.jcf.2023.12.002.","journal-title":"J Cyst Fibros"},{"key":"2739_CR48","volume-title":"The early Cystic fibrosis years","year":"2018","unstructured":"De Boeck\u00a0K, Southern\u00a0KW, editors. The early Cystic fibrosis years. European Cystic Fibrosis Society; 2018."},{"issue":"4","key":"2739_CR49","doi-asserted-by":"publisher","first-page":"533","DOI":"10.1016\/j.jcf.2014.12.015","volume":"14","author":"J Abbott","year":"2015","unstructured":"Abbott\u00a0J, Elborn\u00a0JS, Georgiopoulos\u00a0AM, et\u00a0al. Cystic Fibrosis Foundation and European Cystic Fibrosis Society Survey of cystic fibrosis mental health care delivery. J\u00a0Cyst Fibros. 2015;14(4):533\u20139. https:\/\/doi.org\/10.1016\/j.jcf.2014.12.015.","journal-title":"J Cyst Fibros"},{"issue":"1","key":"2739_CR50","doi-asserted-by":"publisher","first-page":"91","DOI":"10.1016\/j.jcf.2020.08.010","volume":"20","author":"DJ Conrad","year":"2021","unstructured":"Conrad\u00a0DJ, Billings\u00a0J, Teneback\u00a0C, et\u00a0al. Multi-dimensional clinical phenotyping of a national cohort of adult cystic fibrosis patients. J\u00a0Cyst Fibros. 2021;20(1):91\u20136. https:\/\/doi.org\/10.1016\/j.jcf.2020.08.010.","journal-title":"J Cyst Fibros"},{"key":"2739_CR51","doi-asserted-by":"publisher","first-page":"00411","DOI":"10.1183\/23120541.00411-2021","volume":"7","author":"A Jung","year":"2021","unstructured":"Jung\u00a0A, Orenti\u00a0A, Dunlevy\u00a0F, et\u00a0al. Factors for severe outcomes following SARS-CoV\u20112 infection in people with cystic fibrosis in Europe. ERJ Open Res. 2021;7:411\u20132021. https:\/\/doi.org\/10.1183\/23120541.00411-2021.","journal-title":"ERJ Open Res"},{"key":"2739_CR52","doi-asserted-by":"publisher","first-page":"408","DOI":"10.1152\/ajplung.00225.2020","volume":"319","author":"BA Stanton","year":"2020","unstructured":"Stanton\u00a0BA, Hampton\u00a0TH, Ashare\u00a0A. SARS-CoV\u20112 (COVID-19) and cystic fibrosis. Am J Physiol-Lung Cell Mol Physiol. 2020;319:408\u201315. https:\/\/doi.org\/10.1152\/ajplung.00225.2020.","journal-title":"Am J Physiol-Lung Cell Mol Physiol"},{"issue":"4","key":"2739_CR53","doi-asserted-by":"publisher","first-page":"327","DOI":"10.1136\/thoraxjnl-2016-208450","volume":"72","author":"S Stanojevic","year":"2017","unstructured":"Stanojevic\u00a0S, McDonald\u00a0A, Waters\u00a0V, et\u00a0al. Effect of pulmonary exacerbations treated with oral antibiotics on clinical outcomes in cystic fibrosis. Thorax. 2017;72(4):327\u201332. https:\/\/doi.org\/10.1136\/thoraxjnl-2016-208450.","journal-title":"Thorax"},{"key":"2739_CR54","doi-asserted-by":"publisher","first-page":"483","DOI":"10.1002\/ppul.10100","volume":"33","author":"C Schaedel","year":"2002","unstructured":"Schaedel\u00a0C, De Monestrol\u00a0I, Hjelte\u00a0L, et\u00a0al. Predictors of deterioration of lung function in cystic fibrosis. Pediatr Pulmonol. 2002;33:483\u201391. https:\/\/doi.org\/10.1002\/ppul.10100.","journal-title":"Pediatr Pulmonol"},{"key":"2739_CR55","doi-asserted-by":"publisher","first-page":"857","DOI":"10.1016\/j.jcf.2023.05.004","volume":"22","author":"M Rosenfeld","year":"2023","unstructured":"Rosenfeld\u00a0M, Faino\u00a0AV, Qu\u00a0P, et\u00a0al. Association of Pseudomonas aeruginosa infection stage with lung function trajectory in children with cystic fibrosis. J\u00a0Cyst Fibros. 2023;22:857\u201363. https:\/\/doi.org\/10.1016\/j.jcf.2023.05.004.","journal-title":"J Cyst Fibros"},{"key":"2739_CR56","doi-asserted-by":"publisher","first-page":"321","DOI":"10.1016\/j.jcf.2019.03.002","volume":"18","author":"KJ Ramos","year":"2019","unstructured":"Ramos\u00a0KJ, Smith\u00a0PJ, McKone\u00a0EF, et\u00a0al. Lung transplant referral for individuals with cystic fibrosis: Cystic Fibrosis Foundation consensus guidelines. J\u00a0Cyst Fibros. 2019;18:321\u201333. https:\/\/doi.org\/10.1016\/j.jcf.2019.03.002.","journal-title":"J Cyst Fibros"},{"issue":"2","key":"2739_CR57","doi-asserted-by":"publisher","first-page":"137","DOI":"10.1016\/S2213-2600(12)70058-9","volume":"1","author":"S McCuaig","year":"2013","unstructured":"McCuaig\u00a0S, Martin\u00a0JG. How the airway smooth muscle in cystic fibrosis reacts in proinflammatory conditions: implications for airway hyper-responsiveness and asthma in cystic fibrosis. Lancet Respir Med. 2013;1(2):137\u201347. https:\/\/doi.org\/10.1016\/S2213-2600(12)70058-9.","journal-title":"Lancet Respir Med"},{"key":"2739_CR58","doi-asserted-by":"publisher","first-page":"360","DOI":"10.1378\/chest.94.2.360","volume":"94","author":"PA Eggleston","year":"1988","unstructured":"Eggleston\u00a0PA, Rosenstein\u00a0BJ, Stackhouse\u00a0CM, et\u00a0al. Airway Hyperreactivity in Cystic Fibrosis. Chest. 1988;94:360\u20135. https:\/\/doi.org\/10.1378\/chest.94.2.360.","journal-title":"Chest"},{"issue":"1","key":"2739_CR59","doi-asserted-by":"publisher","first-page":"202","DOI":"10.1016\/j.chest.2018.07.025","volume":"155","author":"CS King","year":"2019","unstructured":"King\u00a0CS, Brown\u00a0AW, Aryal\u00a0S, et\u00a0al. Critical Care of the Adult Patient With Cystic Fibrosis. Chest. 2019;155(1):202\u201314. https:\/\/doi.org\/10.1016\/j.chest.2018.07.025.","journal-title":"Chest"},{"key":"2739_CR60","doi-asserted-by":"publisher","first-page":"298","DOI":"10.1164\/rccm.201002-0157OC","volume":"182","author":"PA Flume","year":"2010","unstructured":"Flume\u00a0PA, Mogayzel\u00a0PJ, Robinson\u00a0KA, et\u00a0al. Cystic Fibrosis Pulmonary Guidelines. Am J Respir Crit Care Med. 2010;182:298\u2013306. https:\/\/doi.org\/10.1164\/rccm.201002-0157OC.","journal-title":"Am J Respir Crit Care Med"},{"key":"2739_CR61","doi-asserted-by":"publisher","first-page":"680","DOI":"10.1164\/rccm.201207-1160OE","volume":"187","author":"PJ Mogayzel","year":"2013","unstructured":"Mogayzel PJ Jr, Naureckas ET, Robinson KA, et al.\u00a0Cystic Fibrosis Pulmonary Guidelines Chronic Medications for Maintenance of Lung Health. Am J Respir Crit Care Med. 2013;187:680\u20139. https:\/\/doi.org\/10.1164\/rccm.201207-1160OE.","journal-title":"Am J Respir Crit Care Med"},{"issue":"11","key":"2739_CR62","doi-asserted-by":"publisher","first-page":"1507","DOI":"10.1513\/AnnalsATS.202404-366OC","volume":"21","author":"N Mayer-Hamblett","year":"2024","unstructured":"Mayer-Hamblett\u00a0N, Gifford\u00a0AH, Kloster\u00a0M, et\u00a0al. Impact of Discontinuing Both Hypertonic Saline and Dornase Alfa After Elexacaftor\/Tezacaftor\/Ivacaftor in Cystic Fibrosis. Ann Am Thorac Soc. 2024;21(11):1507\u201315. https:\/\/doi.org\/10.1513\/AnnalsATS.202404-366OC.","journal-title":"Ann Am Thorac Soc"},{"issue":"18","key":"2739_CR63","doi-asserted-by":"publisher","first-page":"1693","DOI":"10.1056\/NEJMra2216474","volume":"389","author":"H Grasemann","year":"2023","unstructured":"Grasemann\u00a0H, Ratjen\u00a0F. Cystic Fibrosis. N\u00a0Engl J Med. 2023;389(18):1693\u2013707. https:\/\/doi.org\/10.1056\/NEJMra2216474.","journal-title":"N Engl J Med"},{"key":"2739_CR64","doi-asserted-by":"publisher","first-page":"788","DOI":"10.1016\/j.jcf.2023.05.010","volume":"22","author":"A Leonard","year":"2023","unstructured":"Leonard\u00a0A, Bailey\u00a0J, Bruce\u00a0A, et\u00a0al. Nutritional considerations for a new era: A CF foundation position paper. J\u00a0Cyst Fibros. 2023;22:788\u201395. https:\/\/doi.org\/10.1016\/j.jcf.2023.05.010.","journal-title":"J Cyst Fibros"},{"key":"2739_CR65","doi-asserted-by":"publisher","first-page":"9","DOI":"10.1016\/j.prrv.2020.12.001","volume":"42","author":"J Konrad","year":"2022","unstructured":"Konrad\u00a0J, Eber\u00a0E, Stadlbauer\u00a0V. Changing paradigms in the treatment of gastrointestinal complications of cystic fibrosis in the era of cystic fibrosis transmembrane conductance regulator modulators. Paediatr Respir Rev. 2022;42:9\u201316. https:\/\/doi.org\/10.1016\/j.prrv.2020.12.001.","journal-title":"Paediatr Respir Rev"},{"key":"2739_CR66","doi-asserted-by":"publisher","first-page":"413","DOI":"10.1016\/j.clnu.2023.12.017","volume":"43","author":"M Wilschanski","year":"2024","unstructured":"Wilschanski\u00a0M, Munck\u00a0A, Carrion\u00a0E, et\u00a0al. ESPEN-ESPGHAN-ECFS guideline on nutrition care for cystic fibrosis. Clin Nutr. 2024;43:413\u201345. https:\/\/doi.org\/10.1016\/j.clnu.2023.12.017.","journal-title":"Clin Nutr"},{"key":"2739_CR67","unstructured":"Fachabteilung 6E \u2013 Elementare und musikalische Bildung Kinderbildungs- und -betreuungsreferat. Amt der Steierm\u00e4rkischen Landesregierung. Information zur Verabreichung von Medikamenten in Kinderbildungs- und \u2013betreuungseinrichtungen. 2025. https:\/\/www.verwaltung.steiermark.at. Accessed 15.10.2025."},{"key":"2739_CR68","doi-asserted-by":"publisher","first-page":"17","DOI":"10.1016\/j.prrv.2022.10.002","volume":"46","author":"M Sheppard","year":"2023","unstructured":"Sheppard\u00a0M, Selvadurai\u00a0H, Robinson\u00a0PD, et\u00a0al. Approaches to the management of haemoptysis in young people with cystic fibrosis. Paediatr Respir Rev. 2023;46:17\u201322. https:\/\/doi.org\/10.1016\/j.prrv.2022.10.002.","journal-title":"Paediatr Respir Rev"},{"key":"2739_CR69","doi-asserted-by":"publisher","first-page":"1818","DOI":"10.1513\/AnnalsATS.202111-1266OC","volume":"19","author":"SC Carter","year":"2022","unstructured":"Carter\u00a0SC, Franciosi\u00a0AN, O\u2019Shea\u00a0KM, et\u00a0al. Acute Pulmonary Exacerbation Phenotypes in Patients with Cystic Fibrosis. Ann Am Thorac Soc. 2022;19:1818\u201326. https:\/\/doi.org\/10.1513\/AnnalsATS.202111-1266OC.","journal-title":"Ann Am Thorac Soc"},{"key":"2739_CR70","doi-asserted-by":"publisher","DOI":"10.1186\/s12931-024-02778-2","volume":"25","author":"D Drummond","year":"2024","unstructured":"Drummond\u00a0D, Roy\u00a0C, Cornet\u00a0M, et\u00a0al. Acute respiratory failure due to pulmonary exacerbation in children with cystic fibrosis admitted in a pediatric intensive care unit: outcomes and factors associated with mortality. Respir Res. 2024;25:190. https:\/\/doi.org\/10.1186\/s12931-024-02778-2.","journal-title":"Respir Res"},{"key":"2739_CR71","doi-asserted-by":"publisher","first-page":"514","DOI":"10.1016\/j.jcf.2021.09.013","volume":"21","author":"DA Aziz","year":"2022","unstructured":"Aziz\u00a0DA, Siddiqui\u00a0F, Abbasi\u00a0Q, et\u00a0al. Characteristics of electrolyte imbalance and pseudo-bartter syndrome in hospitalized cystic fibrosis children and adolescents. J\u00a0Cyst Fibros. 2022;21:514\u20138. https:\/\/doi.org\/10.1016\/j.jcf.2021.09.013.","journal-title":"J Cyst Fibros"},{"key":"2739_CR72","doi-asserted-by":"publisher","first-page":"53","DOI":"10.1002\/ppul.26958","volume":"59","author":"RV Milano","year":"2024","unstructured":"Milano\u00a0RV, Morneault-Gill\u00a0K, Kamal\u00a0HY, et\u00a0al. Pancreatitis in cystic fibrosis: Presentation, medical and surgical management, and the impact of modulator therapies. Pediatr Pulmonol. 2024;59:53\u2013S60. https:\/\/doi.org\/10.1002\/ppul.26958.","journal-title":"Pediatr Pulmonol"},{"issue":"4","key":"2739_CR73","doi-asserted-by":"publisher","first-page":"309","DOI":"10.1016\/j.jcf.2013.03.005","volume":"12","author":"D Nazareth","year":"2013","unstructured":"Nazareth\u00a0D, Walshaw\u00a0M. A review of renal disease in cystic fibrosis. J\u00a0Cyst Fibros. 2013;12(4):309\u201317. https:\/\/doi.org\/10.1016\/j.jcf.2013.03.005.","journal-title":"J Cyst Fibros"},{"key":"2739_CR74","doi-asserted-by":"publisher","first-page":"598","DOI":"10.1016\/j.jcf.2023.05.012","volume":"22","author":"T Vagg","year":"2023","unstructured":"Vagg\u00a0T, Shanthikumar\u00a0S, Ibrahim\u00a0H, et\u00a0al. Telehealth in Cystic Fibrosis. A systematic review incorporating a novel scoring system and expert weighting to identify a \u2018top 10 manuscripts\u2019 to inform future best practices implementation. J\u00a0Cyst Fibros. 2023;22:598\u2013606. https:\/\/doi.org\/10.1016\/j.jcf.2023.05.012.","journal-title":"J Cyst Fibros"},{"key":"2739_CR75","doi-asserted-by":"publisher","first-page":"704","DOI":"10.1016\/j.jcf.2025.03.670","volume":"24","author":"M Svedberg","year":"2025","unstructured":"Svedberg\u00a0M, Michelsen\u00a0J, Roberts\u00a0E, et\u00a0al. Remote monitoring of cystic fibrosis lung disease in children and young adults. J\u00a0Cyst Fibros. 2025;24:704\u20139. https:\/\/doi.org\/10.1016\/j.jcf.2025.03.670.","journal-title":"J Cyst Fibros"},{"key":"2739_CR76","unstructured":"European Cystic Fibrosis Society (ECFS). Telehealth in cystic fibrosis. 2025. https:\/\/www.ecfs.eu\/telehealth. Accessed 14.10.2025."},{"key":"2739_CR77","doi-asserted-by":"publisher","first-page":"1263","DOI":"10.1111\/hex.13258","volume":"24","author":"K Milley","year":"2021","unstructured":"Milley\u00a0K, Chima\u00a0S, McIntosh\u00a0JG, et\u00a0al. Long-term consumer involvement in cancer research: Working towards partnership. Health Expect Int J Public Particip Health Care Health Policy. 2021;24:1263\u20139. https:\/\/doi.org\/10.1111\/hex.13258.","journal-title":"Health Expect Int J Public Particip Health Care Health Policy"},{"key":"2739_CR78","doi-asserted-by":"publisher","first-page":"359","DOI":"10.1016\/j.jcf.2024.08.004","volume":"24","author":"NJ Rowbotham","year":"2025","unstructured":"Rowbotham\u00a0NJ, Smith\u00a0S, Jahnke\u00a0N, et\u00a0al. Infection prevention and control in cystic fibrosis: An update of a systematic review of interventions. J\u00a0Cyst Fibros. 2025;24:359\u201363. https:\/\/doi.org\/10.1016\/j.jcf.2024.08.004.","journal-title":"J Cyst Fibros"},{"key":"2739_CR79","doi-asserted-by":"crossref","unstructured":"Hisert KB, Birket SE, Clancy JP et\u00a0al. Understanding and Addressing the Needs of People with Cystic Fibrosis in the Era of CFTR Modulator Therapy. The Lancet Respiratory Medicine 2023;11(10): 916\u201331. https:\/\/doi.org\/10.1016\/S2213-2600(23)00324\u20117.","DOI":"10.1016\/S2213-2600(23)00324-7"}],"container-title":["Wiener klinische Wochenschrift"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s00508-026-02739-3.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/article\/10.1007\/s00508-026-02739-3","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s00508-026-02739-3.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,10,15]],"date-time":"2026-10-15T22:28:27Z","timestamp":1792103307000},"score":1,"resource":{"primary":{"URL":"https:\/\/link.springer.com\/article\/10.1007\/s00508-026-02739-3"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,4,8]]},"references-count":79,"journal-issue":{"issue":"19-20","published-print":{"date-parts":[[2026,10]]}},"alternative-id":["2739"],"URL":"https:\/\/doi.org\/10.1007\/s00508-026-02739-3","relation":{},"ISSN":["0043-5325","1613-7671"],"issn-type":[{"value":"0043-5325","type":"print"},{"value":"1613-7671","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,4,8]]},"assertion":[{"value":"16 June 2025","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"12 March 2026","order":2,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"8 April 2026","order":3,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"F.\u00a0Singer reports grants from Medical University of Graz; payments from Novartis Pharma, Vertex Pharmaceuticals; travel fees from Chiesi Pharmaceuticals, Sanofi-Aventis, outside the submitted work. A.M.\u00a0Schaffer reports travel fees from Viatris outside the submitted work. V.\u00a0Stadlbauer reports research grants, other research support, and\/or speaker honoraria from Gilead, Merz Therapeutics, Lactosan, Institute Allergosan, Winclove Probiotics, Immundiagnostik GmbH, Alnylam, Sanofi, Tillotts outside the submitted work. E.\u00a0Eber reports research support and\/or speaker honoraria from Chiesi Pharmaceuticals, Gilead Sciences, Insmed, Vertex Pharmaceuticals, and Vifor Pharma outside the submitted work. M.\u00a0Auer, J.\u00a0Auer and J.\u00a0Pansy declare that they have no competing interests.","order":1,"name":"Ethics","label":"Conflict of interest","group":{"name":"EthicsHeading","label":"Ethics"}}]}}