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Besonders der vordere Augenabschnitt ist MPS-typspezifisch von Hornhaut- und Kammerwinkelver\u00e4nderungen betroffen. Ziel dieser Studie war die Charakterisierung von Vorderabschnittsver\u00e4nderungen bei verschiedenen MPS-Typen anhand klinischer, bildgebender und funktioneller Parameter.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methoden<\/jats:title>\n                    <jats:p>In einer klinischen Querschnittsuntersuchung wurden Patienten mit MPS (Typen\u00a0I, II, III, IV, VI) mit einer alters\u00e4hnlichen Kontrollgruppe verglichen. Erhoben wurden allgemeine ophthalmologische und orthoptische Untersuchungen, Spaltlampenbefunde, Vorderabschnitts-Scheimpflug-Messungen (Oculus Pentacam\u00ae HR; Oculus Optikger\u00e4te GmbH, Wetzlar, Deutschland) und die optische Biometrie mittels IOLMaster 700 (Carl Zeiss Meditec AG, Jena, Deutschland). Zudem wurde das Anterior-Segment-Modul der optischen Koh\u00e4renztomographie (AS-OCT; SPECTRALIS\u00ae OCT, Heidelberg Engineering GmbH, Heidelberg, Deutschland) zur Beurteilung des Kammerwinkels eingesetzt.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Ergebnisse<\/jats:title>\n                    <jats:p>\n                      Es wurden 58\u00a0Augen von 29\u00a0MPS-Patienten und 58\u00a0Augen gesunder Kontrollen eingeschlossen. Die MPS-Gruppe umfasste \u00fcberwiegend m\u00e4nnliche Patienten (72,4\u202f%), die Kontrollgruppe \u00fcberwiegend Frauen (65,5\u202f%); das Medianalter betrug\u00a021 (IQR\u00a014\u201332) bzw. 23\u00a0Jahre (IQR\u00a013\u201334). Der bestkorrigierte Visus war bei MPS signifikant schlechter (Median:\u00a00,3\u00a0logMAR vs. \u22120,1\u00a0logMAR;\n                      <jats:italic>p<\/jats:italic>\n                      \u202f&lt;\u20090,001). Stromale Hornhauttr\u00fcbungen traten in 44,8\u202f% der MPS-Augen auf, v.\u202fa. bei Typ\u00a0I, IV und\u00a0VI. Der Augeninnendruck war erh\u00f6ht (Median: 16\u202fmm\u202fHg vs. 12\u202fmm\u202fHg;\n                      <jats:italic>p<\/jats:italic>\n                      \u202f&lt;\u20090,001), ohne glaukomtypische Gesichtsfeld- oder Papillenver\u00e4nderungen. Die Vorderkammertiefe war signifikant reduziert (Median: 3,0\u202fmm vs. 3,5\u202fmm;\n                      <jats:italic>p<\/jats:italic>\n                      \u202f&lt;\u20090,001), ebenso die Kammerwinkelweite (ACA\/AOD (ACA; engl. Anterior Chamber Angle, AOD; engl. Angle Opening Distance); jeweils\n                      <jats:italic>p<\/jats:italic>\n                      \u202f&lt;\u20090,001), insbesondere bei MPS\u00a0I, II und\u00a0VI.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Schlussfolgerung<\/jats:title>\n                    <jats:p>Der vordere Augenabschnitt ist bei MPS-Patienten typspezifisch ver\u00e4ndert. Pentacam, IOLMaster und AS-OCT eignen sich zur Detektion struktureller Ver\u00e4nderungen. Strukturierte ophthalmologische Untersuchungen k\u00f6nnen typische Ver\u00e4nderungen bei Patienten mit MPS detektieren und Ver\u00e4nderungen fr\u00fchzeitig erkennen lassen.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1007\/s00347-026-02529-2","type":"journal-article","created":{"date-parts":[[2026,9,21]],"date-time":"2026-09-21T04:50:45Z","timestamp":1789966245000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Ver\u00e4nderungen des vorderen Augenabschnitts bei Mukopolysaccharidosen","Anterior segment changes in mucopolysaccharidoses"],"prefix":"10.1007","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-2057-2191","authenticated-orcid":false,"given":"Juliane","family":"Matlach","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anna J.","family":"Schmelmer","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lucas","family":"Wipprecht","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Julia V.","family":"Stingl","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Susanne","family":"Hopf","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Norbert","family":"Pfeiffer","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Susanne","family":"Pitz","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lisa","family":"Maiurano","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Julia B.","family":"Hennermann","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Heike M.","family":"Elflein","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2026,9,21]]},"reference":[{"issue":"2","key":"2529_CR1","doi-asserted-by":"publisher","DOI":"10.3390\/diagnostics11020273","volume":"11","author":"B \u00c7elik","year":"2021","unstructured":"\u00c7elik\u00a0B, Tomatsu\u00a0SC, Tomatsu\u00a0S, Khan\u00a0SA (2021) Epidemiology of Mucopolysaccharidoses Update. 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Au\u00dferdem erhielt J. Matlach Honorare f\u00fcr das Schreiben von wissenschaftlichen Artikeln f\u00fcr Omni med.  S.\u00a0Pitz erhielt Reise- und Vortragshonorare von Sanofi, ArgenX und Amgen. J.B.\u00a0Hennermann erhielt Reiseerstattungen und\/oder Honorare von Amicus, Chiesi, Sanofi und Takeda. H.M.\u00a0Elflein erhielt Vortragshonorare von AMGEN und Chiesi. A.J.\u00a0Schmelmer, L.\u00a0Wipprecht, J.V.\u00a0Stingl, S.\u00a0Hopf, N.\u00a0Pfeiffer und L.\u00a0Maiurano geben an, dass kein Interessenkonflikt besteht.","order":1,"name":"Ethics","label":"Interessenkonflikt","group":{"name":"EthicsHeading","label":"Einhaltung ethischer Richtlinien"}},{"value":"F\u00fcr diesen Beitrag wurden von den Autor\/-innen keine Studien an Menschen oder Tieren durchgef\u00fchrt. F\u00fcr die aufgef\u00fchrten Studien gelten die jeweils dort angegebenen ethischen Richtlinien.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Einhaltung ethischer Richtlinien"}}]}}