{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,27]],"date-time":"2026-02-27T00:04:31Z","timestamp":1772150671501,"version":"3.50.1"},"reference-count":17,"publisher":"Georg Thieme Verlag KG","issue":"02","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Klin Padiatr"],"published-print":{"date-parts":[[2026,3]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>Rare pediatric pulmonary diseases, such as childhood interstitial lung\n                        disease and congenital thoracic malformations, pose diagnostic and\n                        therapeutic challenges due to their low prevalence and clinical\n                        heterogeneity. In contrast to cystic fibrosis and primary ciliary\n                        dyskinesia, which are supported by dedicated care networks, many other rare\n                        pediatric pulmonary diseases lack structured management pathways. This study\n                        aimed to assess pediatric pulmonologists\u2019 clinical exposure, confidence, and\n                        educational needs related to rare pediatric pulmonary diseases.<\/jats:p>\n                  <jats:p>A web-based survey was distributed to all 914 members of the German Society\n                        for Pediatric Pulmonology. The questionnaire evaluated clinical experience,\n                        diagnostic confidence, and preferences for educational contents and formats.\n                        Responses were analyzed using descriptive statistics and group comparisons.<\/jats:p>\n                  <jats:p>Among 209 respondents (22.9% response rate), clinical exposure was low\n                        (median: 3 patients\/y; interquartile range: 1\u20135), with 22.5% treating none.\n                        Only 36.7% of respondents felt confident in suspecting a rare pediatric\n                        pulmonary disease, 22.0% in diagnosis, and 13.3% in treatment. Educational\n                        interest was high (68.8%), particularly in clinical presentation (70.7%),\n                        treatment (69.8%), and imaging (59.0%). Clinicians with&lt;10 years of\n                        experience reported greater interest in clinical presentation than those\n                        with\u226510 years (84.5% vs. 57.3%, p=0.0002). Workshops, webinars, and\n                        online discussions were the most preferred learning formats.<\/jats:p>\n                  <jats:p>Despite limited exposure and low reported confidence, pediatric\n                        pulmonologists express strong interest in further education on rare\n                        pediatric pulmonary diseases. Tailored, accessible educational strategies\n                        are essential to improve awareness, diagnosis, and care for children with\n                        rare pulmonary conditions.<\/jats:p>","DOI":"10.1055\/a-2713-3667","type":"journal-article","created":{"date-parts":[[2025,10,31]],"date-time":"2025-10-31T23:46:10Z","timestamp":1761954370000},"page":"86-92","source":"Crossref","is-referenced-by-count":0,"title":["Rare Pediatric Pulmonary Diseases: Insights from a Survey of\n                    Pediatric Pulmonologists in German-Speaking Countries"],"prefix":"10.1055","volume":"238","author":[{"given":"Julia","family":"Carlens","sequence":"additional","affiliation":[{"name":"Dept of Paediatric Pneumology, Allergology and Neonatology, Hannover\n                    Medical School, Hannover, Germany, Hannover, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Srdjan","family":"Micic","sequence":"additional","affiliation":[{"name":"Department of Respiratory Medicine, University\n                    Children\u2019s Hospital Zurich Emergency Department, Z\u00fcrich,\n                    Switzerland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Nicolaus","family":"Schwerk","sequence":"additional","affiliation":[{"name":"Dept of Paediatric Pneumology, Allergology and Neonatology, Hannover\n                    Medical School, Hannover, Germany, Hannover, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Matthias","family":"Griese","sequence":"additional","affiliation":[{"name":"Department of Paediatrics, Children\u2019s University Hospital Munich,\n                    M\u00fcnchen, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Alexander","family":"Moeller","sequence":"additional","affiliation":[{"name":"Department of Respiratory Medicine, University\n                    Children\u2019s Hospital Zurich Emergency Department, Z\u00fcrich,\n                    Switzerland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Elias","family":"Seidl","sequence":"additional","affiliation":[{"name":"Department of Respiratory Medicine, University\n                    Children\u2019s Hospital Zurich Emergency Department, Z\u00fcrich,\n                    Switzerland"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"194","published-online":{"date-parts":[[2025,10,31]]},"reference":[{"key":"ref2","doi-asserted-by":"publisher","first-page":"18825","DOI":"10.1073\/pnas.0904709106","article-title":"Rescue of CF airway epithelial cell function in vitro by a CFTR potentiator,\n                    VX-770","volume":"106","author":"F Van Goor","year":"2009","journal-title":"Proc Natl Acad Sci"},{"key":"ref3","doi-asserted-by":"publisher","first-page":"529","DOI":"10.1164\/rccm.202108-1986OC","article-title":"Clinical Effectiveness of Elexacaftor\/Tezacaftor\/Ivacaftor in People with Cystic\n                    Fibrosis: A Clinical Trial","volume":"205","author":"D P Nichols","year":"2022","journal-title":"Am J Respir Crit Care Med"},{"key":"ref4","doi-asserted-by":"crossref","first-page":"361","DOI":"10.1159\/000538606","article-title":"Improved nutritional outcomes and gastrointestinal symptoms in adult cystic\n                    fibrosis patients treated with elexacaftor\/tezacaftor\/ivacaftor","volume":"42","author":"N Stastna","year":"2024","journal-title":"Dig Dis"},{"key":"ref5","doi-asserted-by":"crossref","first-page":"1176815","DOI":"10.3389\/fphar.2023.1176815","article-title":"Real-life impact of highly effective CFTR modulator therapy in children with\n                    cystic fibrosis","volume":"14","author":"M Olivier","year":"2023","journal-title":"Front Pharmacol"},{"key":"ref6","doi-asserted-by":"publisher","first-page":"1522","DOI":"10.1164\/rccm.202102-0509OC","article-title":"A Phase 3 Open-Label Study of Elexacaftor\/Tezacaftor\/Ivacaftor in Children 6\n                    through 11 Years of Age with Cystic Fibrosis and at Least One F508del\n                    Allele","volume":"203","author":"E T Zemanick","year":"2021","journal-title":"Am J Respir Crit Care Med"},{"key":"ref7","doi-asserted-by":"crossref","first-page":"00989-2023","DOI":"10.1183\/23120541.00989-2023","article-title":"Estimates of primary ciliary dyskinesia prevalence: a scoping review","volume":"10","author":"W B Wee","year":"2024","journal-title":"ERJ Open Res"},{"key":"ref8","doi-asserted-by":"publisher","first-page":"e24","DOI":"10.1164\/rccm.201805-0819ST","article-title":"Diagnosis of Primary Ciliary Dyskinesia. 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Perspectives\n                    from the ERN-LUNG educational programme survey","volume":"15","author":"P Powell","year":"2019","journal-title":"Breathe"},{"key":"ref18","doi-asserted-by":"publisher","first-page":"160","DOI":"10.1186\/1472-6963-9-160","article-title":"Response rates in postal surveys of healthcare professionals between 1996 and\n                    2005: An observational study","volume":"9","author":"J V Cook","year":"2009","journal-title":"BMC Health Serv Res"}],"container-title":["Klinische P\u00e4diatrie"],"original-title":[],"language":"de","link":[{"URL":"http:\/\/www.thieme-connect.de\/products\/ejournals\/pdf\/10.1055\/a-2713-3667.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,2,26]],"date-time":"2026-02-26T23:48:30Z","timestamp":1772149710000},"score":1,"resource":{"primary":{"URL":"http:\/\/www.thieme-connect.de\/DOI\/DOI?10.1055\/a-2713-3667"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,10,31]]},"references-count":17,"journal-issue":{"issue":"02","published-online":{"date-parts":[[2026,2,26]]},"published-print":{"date-parts":[[2026,2]]}},"URL":"https:\/\/doi.org\/10.1055\/a-2713-3667","archive":["Portico","CLOCKSS"],"relation":{},"ISSN":["0300-8630","1439-3824"],"issn-type":[{"value":"0300-8630","type":"print"},{"value":"1439-3824","type":"electronic"}],"subject":[],"published":{"date-parts":[[2025,10,31]]}}}