{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2022,4,3]],"date-time":"2022-04-03T14:05:29Z","timestamp":1648994729661},"reference-count":15,"publisher":"Georg Thieme Verlag KG","issue":"10","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Pneumologie"],"published-print":{"date-parts":[[2020,10]]},"abstract":"<jats:title>Zusammenfassung<\/jats:title><jats:p>Die septische Granulomatose (engl.: chronic granulomatous disease, CGD) ist bei Kindern und Jugendlichen mit h\u00e4ufigen Infektionen durch bestimmte Erreger differenzialdiagnostisch zu ber\u00fccksichtigen.<\/jats:p><jats:p>In vorliegender Kasuistik berichten wir \u00fcber eine 64-j\u00e4hrige Patientin mit \u00fcber Jahre zunehmenden bronchopulmonalen Infektionen, teilweise durch seltene Erreger, Autoimmunph\u00e4nomenen, Malignomen und im weiteren Verlauf rezidivierenden organisierenden Pneumonien (OP) mit Nachweis von Granulomen. Urs\u00e4chlich wurde bei der Patientin eine septische Granulomatose, Unterform p47phox-Defizienz diagnostiziert. Ein \u00dcberleben von Patienten mit einem prim\u00e4ren Immundefekt (PID) bis ins hohe Erwachsenenalter wird trotz wiederholter Komplikationen in Einzelf\u00e4llen beobachtet, insbesondere wenn die defekte Zellstruktur eine Restaktivit\u00e4t aufweist. Bei rezidivierenden bronchopulmonalen Infektionen insbesondere durch bestimmte seltene Erreger und in Kombination mit organisierend-granulomat\u00f6sen Lungenreaktionen sowie Autoimmunph\u00e4nomenen ist auch bei \u00e4lteren Erwachsenen an eine CGD zu denken. Durch eine Diagnoseverz\u00f6gerung kommt es oft zu einem erheblichen Anstieg der Morbidit\u00e4t und Mortalit\u00e4t.<\/jats:p>","DOI":"10.1055\/a-1175-4122","type":"journal-article","created":{"date-parts":[[2020,7,9]],"date-time":"2020-07-09T22:53:50Z","timestamp":1594335230000},"page":"678-683","source":"Crossref","is-referenced-by-count":0,"title":["Septische Granulomatose als seltene Differenzialdiagnose rezidivierender pulmonaler Infektionen bei Erwachsenen"],"prefix":"10.1055","volume":"74","author":[{"given":"J.","family":"Kleymann","sequence":"additional","affiliation":[{"name":"Medizinische Klinik 1, Bereich Pneumologie, Universit\u00e4tsklinikum Carl Gustav Carus, Dresden"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"C.","family":"Sch\u00fctz","sequence":"additional","affiliation":[{"name":"Klinik f\u00fcr Kinder- und Jugendmedizin, Universit\u00e4tsklinikum Carl Gustav Carus, 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