{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,20]],"date-time":"2026-10-20T19:36:54Z","timestamp":1792525014394,"version":"4.5.0"},"reference-count":31,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2013,8,22]],"date-time":"2013-08-22T00:00:00Z","timestamp":1377129600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/100008484","name":"BioMarin Pharmaceutical","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100008484","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100003330","name":"Travis Roy Foundation","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100003330","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100002842","name":"Chiang Mai University","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100002842","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["J of Inher Metab Disea"],"published-print":{"date-parts":[[2014,3]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Objective<\/jats:title>\n                    <jats:p>To assess oral manifestations of 17 patients with mucopolysaccharidosis type VI (MPS VI) or Maroteaux\u2010Lamy syndrome.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>We performed comprehensive oral examinations in 17 patients with MPS VI. Panoramic radiographs was performed only in 14 patients. All patients were of Thai, Turkish, and Indian origins. Ten of 17 patients had enzyme replacement therapy (ERT) (Naglazyme). Most Turkish patients (10\/11) were on ERT. The Thai and Indian patients have never had ERT.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>Oral and radiographic examinations showed that hypoplastic mandibular condyles (93.3 %), malposition of unerupted teeth (92.9 %), large dental follicles (92.3 %), anterior open bite (86.7 %), maxillary constriction (56.3 %), and taurodontism (53.8 %) were common among patients with MPS VI. Newly recognized oral findings found in our study included taurodontism, long tooth roots, abnormal frenum, missing teeth, supernumerary teeth, and microdontia. Two patients who started ERT prior to 3 years old did not develope anterior open bite and one of them had mildly affected mandibular condyles.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>Our study provides the most comprehensive study of oral manifestations in patients with MPS VI. Receiving ERT at very young ages may lessen craniofacial malformations including hypoplasic mandibular condyles and anterior open bite. Oral manifestations can be used as diagnostic features for MPS VI prior to assessing leukocyte ARSB activity or urinary excretion of dermatan sulfate.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1007\/s10545-013-9645-8","type":"journal-article","created":{"date-parts":[[2013,8,21]],"date-time":"2013-08-21T09:01:24Z","timestamp":1377075684000},"page":"263-268","source":"Crossref","is-referenced-by-count":34,"title":["Oral manifestations of 17 patients affected with mucopolysaccharidosis type VI"],"prefix":"10.1002","volume":"37","author":[{"given":"Piranit Nik","family":"Kantaputra","sequence":"first","affiliation":[{"name":"Division of Pediatric Dentistry, Department of Orthodontics and Pediatric Dentistry; Craniofacial Genetics Laboratory, Faculty of Dentistry Chiang Mai University Chiang Mai Thailand"},{"name":"Dentaland Clinic Chiang Mai Thailand"},{"name":"Division of Pediatric Dentistry, Department of Orthodontics and Pediatric Dentistry; Craniofacial Genetics Laboratory, Faculty of Dentistry Chiang Mai University; DENTALAND CLINIC Chiang Mai 50200 Thailand"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"H\u00fclya","family":"Kayserili","sequence":"additional","affiliation":[{"name":"Medical Genetics Department, Istanbul Medical Faculty Istanbul University Istanbul 34093 Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yeliz","family":"G\u00fcven","sequence":"additional","affiliation":[{"name":"Department of Pedodontics, Faculty of Dentistry Istanbul University Istanbul 34093 Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Warissara","family":"Kantaputra","sequence":"additional","affiliation":[{"name":"Dentaland Clinic Chiang Mai Thailand"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mehmet C.","family":"Balci","sequence":"additional","affiliation":[{"name":"Division of Nutrition and Metabolism, Department of Pediatrics, Istanbul Medical Faculty Istanbul University 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