{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,1,15]],"date-time":"2026-01-15T12:54:52Z","timestamp":1768481692107,"version":"3.49.0"},"reference-count":52,"publisher":"Georg Thieme Verlag KG","issue":"05","license":[{"start":{"date-parts":[[2024,3,8]],"date-time":"2024-03-08T00:00:00Z","timestamp":1709856000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Klin Padiatr"],"published-print":{"date-parts":[[2024,9]]},"abstract":"<jats:title>Zusammenfassung<\/jats:title><jats:p>Das Bardet-Biedl-Syndrom (BBS) ist eine seltene, autosomal-rezessiv vererbte\n                    Multisystemerkrankung. Pathophysiologisch liegt eine Funktionsst\u00f6rung des\n                    Prim\u00e4rziliums vor. Die Klinik ist heterogen und variabel und zeigt sich\n                    insbesondere in einer Retinadystrophie, Adipositas, Polydaktylie,\n                    Nierenauff\u00e4lligkeiten, Hypogonadismus und Entwicklungsverz\u00f6gerungen. Mit der\n                    Zulassung des Melanocortin 4-Rezeptor-Agonisten Setmelanotid kann erstmals eine\n                    medikament\u00f6se Therapie der BBS-assoziierten Hyperphagie und der Adipositas\n                    angeboten werden. Hyperphagie und das starke \u00dcbergewicht bereits im Kindesalter\n                    stellen eine erhebliche Krankheitslast dar und gehen zudem mit einem\n                    Komorbidit\u00e4ts- und erh\u00f6htem Mortalit\u00e4tsrisiko einher. Aufgrund der limitierten\n                    Erfahrungen mit Setmelanotid bei BBS soll hiermit ein tragf\u00e4higes umfassendes\n                    Therapiekonzept vorgelegt werden. Die Therapie sollte nach genetisch gesicherter\n                    Diagnose und individueller Indikationsstellung in Zentren mit spezieller\n                    Expertise erfolgen. Voraussetzungen f\u00fcr den bestm\u00f6glichen Therapieeffekt mit\n                    Setmelanotid ist die ad\u00e4quate Aufkl\u00e4rung des Patienten \u00fcber die Modalit\u00e4ten der\n                    Therapie (t\u00e4gliche subkutane Injektion) und m\u00f6gliche unerw\u00fcnschte\n                    Arzneimittelwirkungen. Des Weiteren ist gemeinsam mit dem Patienten die\n                    Einbeziehung von Psychologen, Ern\u00e4hrungsberatern und Pflegediensten (Support bei\n                    der Applikation) zu erw\u00e4gen. Die Beurteilung des Therapieerfolgs sollte mit\n                    geeigneten Outcome-Measurements erfolgen und zur Evidenzf\u00f6rderung zentralisiert\n                    mittels etablierter Register-Infrastrukturen erfasst werden.<\/jats:p>","DOI":"10.1055\/a-2251-5382","type":"journal-article","created":{"date-parts":[[2024,3,8]],"date-time":"2024-03-08T23:45:00Z","timestamp":1709941500000},"page":"269-279","source":"Crossref","is-referenced-by-count":4,"title":["Verbesserte Versorgungs-und Behandlungsoptionen f\u00fcr Patienten mit\n                    Hyperphagie-assoziierter Adipositas bei Bardet-Biedl-Syndrom"],"prefix":"10.1055","volume":"236","author":[{"given":"Metin","family":"Cetiner","sequence":"additional","affiliation":[{"name":"Department of Pediatrics II, University Hospital Essen, Essen,\n                    Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Carsten","family":"Bergmann","sequence":"additional","affiliation":[{"name":"Human genetic diagnostics, Medical Genetics Mainz, Mainz,\n                    Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Markus","family":"Bettendorf","sequence":"additional","affiliation":[{"name":"Pediatric Endocrinology and Diabetes, Heidelberg University Hospital\n                    Department of General Pediatrics Pediatric Neurology Metabolic Diseases\n                    Gastroenterology and Nephrology, Heidelberg, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Johanna","family":"Faust","sequence":"additional","affiliation":[{"name":"Psychiatry and psychotherapy, Max-Planck-Institute for Psychiatry,\n                    M\u00fcnchen, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Anja","family":"G\u00e4ckler","sequence":"additional","affiliation":[{"name":"Department of Nephrology, University Hospital Essen, Essen,\n                    Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Bernarda","family":"Gillissen","sequence":"additional","affiliation":[{"name":"Bardet Biedl syndrome Working Group, PRO RETINA Deutschland e V, Bonn,\n                    Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Matthias","family":"Hansen","sequence":"additional","affiliation":[{"name":"KFH Kidney Center for Children and Adolescents, Clementine Children\u2019s\n                    Hospital - Dr Christ\u2019sche Foundation, Frankfurt am Main, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Maximilian","family":"Kerber","sequence":"additional","affiliation":[{"name":"Bardet Biedl syndrome Working Group, PRO RETINA Deutschland e V, Bonn,\n                    Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"G\u00fcnter","family":"Klaus","sequence":"additional","affiliation":[{"name":"KFH Kidney Center for Children and Adolescents, University Hospitals\n                    Giessen and Marburg Campus Giessen, Marburg, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jens","family":"K\u00f6nig","sequence":"additional","affiliation":[{"name":"Department of General Pediatrics, University Hospital M\u00fcnster, M\u00fcnster,\n                    Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1906-8681","authenticated-orcid":false,"given":"Laura","family":"K\u00fchlewein","sequence":"additional","affiliation":[{"name":"Department of Ophthalmology, University Hospital T\u00fcbingen Clinic of\n                    Ophthalmology, T\u00fcbingen, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jun","family":"Oh","sequence":"additional","affiliation":[{"name":"Pediatric Nephrology, University Medical Center Hamburg-Eppendorf\n                    Department of Pediatrics, Hamburg, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Annette","family":"Richter-Unruh","sequence":"additional","affiliation":[{"name":"Department of Pediatric Endocrinology and Diabetology, University\n                    Hospital of the Ruhr University Bochum, Bochum, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Julia","family":"von Schnurbein","sequence":"additional","affiliation":[{"name":"Department of Pediatrics and Adolescent Medicine, Ulm University\n                    Hospital, Ulm, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Martin","family":"Wabitsch","sequence":"additional","affiliation":[{"name":"Department of Pediatrics and Adolescent Medicine, Ulm University\n                    Hospital, Ulm, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Susann","family":"Weihrauch-Bl\u00fcher","sequence":"additional","affiliation":[{"name":"Department of Pediatrics I, University Hospital Halle, Halle,\n                    Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Lars","family":"Pape","sequence":"additional","affiliation":[{"name":"Department of Pediatrics II, University Hospital Essen, Essen,\n                    Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"194","published-online":{"date-parts":[[2024,3,8]]},"reference":[{"key":"ref1","doi-asserted-by":"crossref","first-page":"115","DOI":"10.2147\/TCRM.S338653","article-title":"Bardet-Biedl Syndrome: Current Perspectives and Clinical Outlook","volume":"19","author":"A Melluso","year":"2023","journal-title":"Ther Clin Risk Manag"},{"key":"ref2","volume-title":"Sur un syndrome d\u2019ob\u00e9sit\u00e9 cong\u00e9nitale avec polydactylie et r\u00e9tinite pigmentaire\n                    (contribution \u00e0 l\u2019\u00e9tude des formes cliniques de l\u2019ob\u00e9sit\u00e9 hypophysaire)","author":"G 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