{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,9,25]],"date-time":"2025-09-25T14:08:51Z","timestamp":1758809331999,"version":"3.44.0"},"reference-count":28,"publisher":"Springer Science and Business Media LLC","issue":"10","license":[{"start":{"date-parts":[[2025,9,4]],"date-time":"2025-09-04T00:00:00Z","timestamp":1756944000000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/deed.de"},{"start":{"date-parts":[[2025,9,4]],"date-time":"2025-09-04T00:00:00Z","timestamp":1756944000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/deed.de"}],"funder":[{"DOI":"10.13039\/501100006447","name":"University of Zurich","doi-asserted-by":"crossref","id":[{"id":"10.13039\/501100006447","id-type":"DOI","asserted-by":"crossref"}]}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Radiologie"],"published-print":{"date-parts":[[2025,10]]},"abstract":"<jats:title>Zusammenfassung<\/jats:title>\n          <jats:sec>\n            <jats:title>Hintergrund<\/jats:title>\n            <jats:p>Pulmonale Manifestationen systemischer Erkrankungen stellen ein komplexes und diagnostisch herausforderndes Feld dar. W\u00e4hrend die pulmonale Beteiligung bei immunologischen und h\u00e4matologischen Grunderkrankungen bereits gut beschrieben ist, r\u00fcckt die Lungenbeteiligung bei seltenen genetischen und kongenitalen Systemerkrankungen \u2013\u00a0wie systemischen Speicherkrankheiten, neuromuskul\u00e4ren Erkrankungen und Phakomatosen\u00a0\u2013 erst allm\u00e4hlich st\u00e4rker in den Fokus der radiologischen Diagnostik.<\/jats:p>\n          <\/jats:sec>\n          <jats:sec>\n            <jats:title>Ergebnisse<\/jats:title>\n            <jats:p>H\u00e4ufig manifestiert sich die pulmonale Komponente erst sekund\u00e4r, oft nach langj\u00e4hrigem Fortschreiten und Verschlechterung der Grunderkrankung. Die fr\u00fchzeitige Erkennung pulmonaler Manifestationen ist jedoch entscheidend f\u00fcr das therapeutische Management und den weiteren Verlauf der Erkrankung. In der Bildgebung nimmt die hochaufl\u00f6sende Computertomographie (HRCT) eine Schl\u00fcsselrolle ein. Als technologische Innovation er\u00f6ffnet die Photon-Counting-Detektor-CT (PCD-CT) neue diagnostische M\u00f6glichkeiten durch eine signifikant verbesserte r\u00e4umliche Aufl\u00f6sung sowie ein optimiertes Signal-zu-Rausch-Verh\u00e4ltnis (\u201esignal-to-noise ratio\u201c) im Vergleich zu einer modernen energieintegrierenden Detektor-CT (EID-CT).<\/jats:p>\n          <\/jats:sec>\n          <jats:sec>\n            <jats:title>Schlussfolgerung<\/jats:title>\n            <jats:p>Die gezielte Anwendung dieser modernen CT-Technologien kann somit nicht nur die differenzialdiagnostische Einordnung pulmonaler Manifestationen verbessern, sondern auch eine fr\u00fchzeitige therapeutische Intervention erm\u00f6glichen.<\/jats:p>\n          <\/jats:sec>","DOI":"10.1007\/s00117-025-01498-y","type":"journal-article","created":{"date-parts":[[2025,9,4]],"date-time":"2025-09-04T11:33:20Z","timestamp":1756985600000},"page":"777-787","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Pulmonale Mitbeteiligung bei seltenen systemischen Erkrankungen\u00a0\u2013 Teil\u00a02","Pulmonary involvement in rare systemic diseases\u2014part\u00a02"],"prefix":"10.1007","volume":"65","author":[{"given":"Jasmin","family":"Happe","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Thomas","family":"Frauenfelder","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"297","published-online":{"date-parts":[[2025,9,4]]},"reference":[{"key":"1498_CR1","doi-asserted-by":"publisher","unstructured":"Happe\u00a0J, Frauenfelder\u00a0T (2025) Pulmonale Mitbeteiligung bei seltenen systemischen Erkrankungen. 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