{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,27]],"date-time":"2026-03-27T00:32:28Z","timestamp":1774571548022,"version":"3.50.1"},"reference-count":19,"publisher":"Georg Thieme Verlag KG","issue":"17","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Dtsch Med Wochenschr"],"published-print":{"date-parts":[[2025,8]]},"abstract":"<jats:title>Was ist neu?<\/jats:title><jats:p>Zur Diagnosesicherung der autoimmunen Hepatitis (AIH) ist eine Leberbiopsie notwendig. F\u00fcr die Remissionsinduktion der AIH ist bei adulten Patienten in der Regel eine Prednisolondosis von 0,5mg\/kg KG\/Tag ausreichend. Das Therapieansprechen sollte sp\u00e4testens nach 6 Monaten beurteilt werden. Obetichols\u00e4ure wurde als bisher zugelassene Zweitlinientherapie der prim\u00e4r bili\u00e4ren Cholangitis (PBC) die europ\u00e4ische Marktzulassung entzogen. Dennoch sollte grunds\u00e4tzlich eine Zweitlinientherapie der PBC bei unzureichendem UDCA-Ansprechen (UDCA: Ursodeoxychols\u00e4ure) und bei Vorliegen von Risiken der Krankheitsprogression erfolgen. Die neue Nomenklatur der Gallengangstenosen bei der prim\u00e4r sklerosierenden Cholangitis (PSC) umfasst die \u201ehochgradige Striktur\u201c (&gt;75%ige Reduktion des Gangdurchmessers) und die \u201eklinisch relevante Striktur\u201c (hochgradige Striktur mit zus\u00e4tzlichen Symptomen wie bakterielle Cholangitis). Die Erstlinientherapie von Pruritus bei adulter PSC umfasst Bezafibrat oder Rifampicin (beide Off-Label).<\/jats:p><jats:p>Bei klinischem Verdacht auf eine genetisch-cholestatische Lebererkrankung im Kindesalter soll fr\u00fchzeitig eine molekulargenetische Abkl\u00e4rung mittels \u201enext generation sequencing\u201c erfolgen. F\u00fcr die Behandlung des cholestatischen Pruritus sind bei Patienten mit Alagille-Syndrom (ALGS) und progressiver intrahepatischer famili\u00e4rer Cholestase (PFIC) die neuen, selektiven Inhibitoren des ilealen Gallens\u00e4uretransporters (IBAT), Odevixibat und Maralixibat, zugelassen. Neben der Verbesserung des Pruritus senken IBAT-Inhibitoren die Serumgallens\u00e4urespiegel. Inwieweit damit auch eine Reduktion der Gallens\u00e4uretoxizit\u00e4t und eine Verl\u00e4ngerung des transplantationsfreien \u00dcberlebens erzielt werden, ist derzeit noch unklar. Die erkrankungsspezifische, spezialisierte Versorgung soll durch eine strukturierte Transition von Jugendlichen mit genetisch-cholestatischen Lebererkrankungen erhalten werden.<\/jats:p>","DOI":"10.1055\/a-2462-8656","type":"journal-article","created":{"date-parts":[[2025,8,7]],"date-time":"2025-08-07T22:52:30Z","timestamp":1754607150000},"page":"1006-1012","source":"Crossref","is-referenced-by-count":1,"title":["Autoimmune und genetisch-cholestatische Lebererkrankungen: die neuen Leitlinien"],"prefix":"10.1055","volume":"150","author":[{"given":"Marcial","family":"Sebode","sequence":"first","affiliation":[{"name":"I. Medizinische Klinik und Poliklinik, Universit\u00e4tsklinikum Hamburg-Eppendorf, Hamburg, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Christian","family":"Hudert","sequence":"first","affiliation":[{"name":"Klinik f\u00fcr P\u00e4diatrie m. S. 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