{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,16]],"date-time":"2026-07-16T01:42:21Z","timestamp":1784166141225,"version":"3.55.0"},"reference-count":19,"publisher":"Georg Thieme Verlag KG","issue":"11","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Z Gastroenterol"],"published-print":{"date-parts":[[2020,11]]},"abstract":"<jats:title>Zusammenfassung<\/jats:title><jats:p>\n          Einf\u00fchrung\u2002In diesem Beitrag wird die Pr\u00e4valenz der Erkrankung \u201eMorbus Wilson\u201c in Deutschland auf der Grundlage von bundesweiten Arzneiverordnungsdaten und vertrags\u00e4rztlichen Abrechnungsdaten ermittelt und in ein Verh\u00e4ltnis zu der in Deutschland ermittelten Verordnungsh\u00e4ufigkeit gesetzt.<\/jats:p><jats:p>\nMethodik\u2002F\u00fcr die deskriptive Darstellung der Abrechnungsdaten wird die Datenbank des Zentralinstituts f\u00fcr die kassen\u00e4rztliche Versorgung (Zi) genutzt. Weiterhin liegen Daten des Statistischen Bundesamts \u00fcber die station\u00e4re Behandlung vor.<\/jats:p><jats:p>\nErgebnisse\u2002Auff\u00e4llig ist die Differenz zwischen den Pr\u00e4valenzen der therapierten Patienten und der ermittelten gesicherten Diagnosen. Die Pr\u00e4valenz insgesamt steigt. Die ermittelte Inzidenz und die Betrachtung der Dynamik der Patientenpopulation k\u00f6nnten m\u00f6glicherweise auf eine hohe Fehldiagnostikrate im ersten Erkrankungsjahr schlie\u00dfen lassen. Nach Datenlage ist die hepatische Verlaufsform die h\u00e4ufigere diagnostizierte Verlaufsform. Die humangenetische Diagnose steigt im Durchschnitt am deutlichsten an.<\/jats:p><jats:p>\nSchlagw\u00f6rter\u2002Morbus Wilson, Pr\u00e4valenz, Inzidenz, Trientine, Trientintetrahydrochlorid, D-Penicillamin, Zinkacetat, Zink<\/jats:p>","DOI":"10.1055\/a-1246-3190","type":"journal-article","created":{"date-parts":[[2020,11,16]],"date-time":"2020-11-16T23:39:52Z","timestamp":1605569992000},"page":"1054-1064","source":"Crossref","is-referenced-by-count":3,"title":["Retrospektive Querschnittsstudie von bundesweiten Arzneiverordnungsdaten und vertrags\u00e4rztlichen Abrechnungsdaten von Morbus Wilson"],"prefix":"10.1055","volume":"58","author":[{"given":"Simone-Svea","family":"Janka","sequence":"additional","affiliation":[{"name":"Abteilung Allgemeinmedizin und Versorgungsforschung, Universit\u00e4tsklinikum Heidelberg, Heidelberg, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"J\u00f6rg","family":"B\u00e4tzing","sequence":"additional","affiliation":[{"name":"Zentralinstitut f\u00fcr die kassen\u00e4rztliche Versorgung in der Bundesrepublik Deutschland, Berlin, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gunter","family":"Laux","sequence":"additional","affiliation":[{"name":"Abteilung Allgemeinmedizin und Versorgungsforschung, Universit\u00e4tsklinikum Heidelberg, Heidelberg, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jakob","family":"Holstiege","sequence":"additional","affiliation":[{"name":"Zentralinstitut f\u00fcr die kassen\u00e4rztliche Versorgung in der Bundesrepublik Deutschland, Berlin, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Steffen","family":"Wahler","sequence":"additional","affiliation":[{"name":"Institut f\u00fcr Molekulare Muskuloskelettale Forschung, Klinikum der Universit\u00e4t M\u00fcnchen, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Uta","family":"Merle","sequence":"additional","affiliation":[{"name":"Medizinische Klinik IV, Klinik f\u00fcr Gastroenterologie, Infektionskrankheiten, Vergiftungen, Universit\u00e4tsklinikum Heidelberg, Heidelberg, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Arianeb","family":"Mehrabi","sequence":"additional","affiliation":[{"name":"Klinik f\u00fcr Allgemein-, Viszeral- und Transplantationschirurgie, Chirurgische Universit\u00e4tsklinik Heidelberg, Heidelberg, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Isabelle","family":"Mohr","sequence":"additional","affiliation":[{"name":"Medizinische Klinik IV, Klinik f\u00fcr Gastroenterologie, Infektionskrankheiten, Vergiftungen, Universit\u00e4tsklinikum Heidelberg, Heidelberg, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Karl Heinz","family":"Weiss","sequence":"additional","affiliation":[{"name":"Medizinische Klinik IV, Klinik f\u00fcr Gastroenterologie, Infektionskrankheiten, Vergiftungen, Universit\u00e4tsklinikum Heidelberg, Heidelberg, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Markus","family":"Mieth","sequence":"additional","affiliation":[{"name":"Klinik f\u00fcr Allgemein-, Viszeral- und Transplantationschirurgie, Chirurgische Universit\u00e4tsklinik Heidelberg, Heidelberg, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"194","published-online":{"date-parts":[[2020,11,16]]},"reference":[{"key":"ref1","first-page":"139","article-title":"Diagnosis and phenotypic classification of Wilson disease 1","volume":"23","author":"P Ferenci","year":"2003"},{"key":"ref2","first-page":"668","article-title":"Wilson disease in septuagenarian siblings: raising the bar for diagnosis","volume":"41","author":"A Ala","year":"2005"},{"key":"ref3","first-page":"103","article-title":"Wilson\u2019s disease and other neurological copper disorders","volume":"14","author":"O Bandmann","year":"2015"},{"key":"ref4","doi-asserted-by":"crossref","first-page":"21","DOI":"10.1038\/s41572-018-0018-3","article-title":"Wilson disease","volume":"4","author":"A Cz\u0142onkowska","year":"2018","journal-title":"Nat Rev Dis Primers"},{"key":"ref5","doi-asserted-by":"crossref","first-page":"1819","DOI":"10.1073\/pnas.82.6.1819","article-title":"Assignment of the gene for\u00a0Wilson disease to chromosome 13: linkage to the esterase D locus","volume":"82","author":"M Frydman","year":"1985","journal-title":"J\u00a0Proceedings of the National Academy of Sciences"},{"key":"ref6","first-page":"327","article-title":"The Wilson disease gene is a putative copper transporting P\u2013type ATPase similar to the Menkes gene","volume":"5","author":"P C Bull","year":"1993"},{"key":"ref7","volume-title":"Morbus Wilson","author":"P DW Hermann","year":"2012"},{"key":"ref8","volume-title":"I. Wilson\u2019s disease","author":"I S Scheinberg","year":"1984"},{"key":"ref9","doi-asserted-by":"crossref","first-page":"23 S","DOI":"10.1002\/hep.22261","article-title":"Diagnosis and treatment of Wilson disease: An\u00a0update","volume":"47","author":"E A Roberts","year":"2008","journal-title":"Hepatology"},{"key":"ref10","first-page":"151","article-title":"Regional distribution of mutations of the ATP7B gene in patients with Wilson disease: impact on genetic testing","volume":"120","author":"P JHg Ferenci","year":"2006"},{"key":"ref11","first-page":"103","article-title":"Wilson\u2019s disease and other neurological copper disorders","volume":"14","author":"O Bandmann","year":"2015"},{"key":"ref12","doi-asserted-by":"crossref","first-page":"159","DOI":"10.1007\/s00108-017-0378-x","article-title":"Morbus Wilson","volume":"59","author":"D Huster","year":"2018","journal-title":"Der Internist"},{"key":"ref13","doi-asserted-by":"crossref","first-page":"722","DOI":"10.1002\/hep.30911","article-title":"The Prevalence of Wilson disease. An Update","volume":"71","author":"T D Sandahl","year":"2020","journal-title":"Hepatology"},{"key":"ref22","doi-asserted-by":"crossref","first-page":"115","DOI":"10.1136\/gut.2005.087262","article-title":"Clinical presentation, diagnosis and long-term outcome of Wilson\u2019s disease: a cohort study","volume":"56","author":"U Merle","year":"2007","journal-title":"Gut"},{"key":"ref23","doi-asserted-by":"crossref","first-page":"59","DOI":"10.33176\/AACB-18-00014","article-title":"Biochemical Markers for the Diagnosis and Monitoring of Wilson Disease","volume":"40","author":"I Mohr","year":"2019","journal-title":"Clin Biochem Rev"},{"key":"ref24","first-page":"CD012267","article-title":"Biomarkers for diagnosis of Wilson\u2019s disease","volume":"2019","author":"A Ryan","year":"2019","journal-title":"Cochrane Database Syst Rev"},{"key":"ref25","doi-asserted-by":"crossref","first-page":"1028","DOI":"10.1016\/j.cgh.2013.03.012","article-title":"Efficacy and safety of oral chelators in treatment of patients with Wilson disease","volume":"11","author":"K H Weiss","year":"2013","journal-title":"Clin Gastroenterol Hepatol"},{"key":"ref26","doi-asserted-by":"crossref","first-page":"154","DOI":"10.1111\/ene.13171","article-title":"Exchangeable copper: a reflection of the neurological severity in Wilson\u2019s disease","volume":"24","author":"A Poujois","year":"2017","journal-title":"Eur J Neurol"},{"key":"ref27","doi-asserted-by":"crossref","first-page":"671","DOI":"10.1016\/j.jhep.2011.11.007","article-title":"EASL Clinical Practice Guidelines: Wilson\u2019s disease","volume":"56","author":"European Association for Study of Liver","year":"2012","journal-title":"J Hepatol"}],"container-title":["Zeitschrift f\u00fcr Gastroenterologie"],"original-title":[],"language":"de","link":[{"URL":"http:\/\/www.thieme-connect.de\/products\/ejournals\/pdf\/10.1055\/a-1246-3190.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2020,11,16]],"date-time":"2020-11-16T23:40:09Z","timestamp":1605570009000},"score":1,"resource":{"primary":{"URL":"http:\/\/www.thieme-connect.de\/DOI\/DOI?10.1055\/a-1246-3190"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2020,11]]},"references-count":19,"journal-issue":{"issue":"11","published-online":{"date-parts":[[2020,11,16]]},"published-print":{"date-parts":[[2020,11]]}},"URL":"https:\/\/doi.org\/10.1055\/a-1246-3190","relation":{},"ISSN":["0044-2771","1439-7803"],"issn-type":[{"value":"0044-2771","type":"print"},{"value":"1439-7803","type":"electronic"}],"subject":[],"published":{"date-parts":[[2020,11]]}}}