Papillon-Leage and psaume syndrome patient with multiple dental and orofacial anomalies

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dc.authorid0000-0001-9009-6218
dc.contributor.authorKılınç, Delal Dara
dc.contributor.authorÖzsarp, Ecem
dc.date.accessioned10.07.201910:49:13
dc.date.accessioned2019-07-10T19:49:32Z
dc.date.available10.07.201910:49:13
dc.date.available2019-07-10T19:49:32Z
dc.date.issued2019
dc.departmentİstanbul Medipol Üniversitesi, Diş Hekimliği Fakültesi, Ortodonti Ana Bilim Dalı
dc.descriptionWOS: 000471973600021
dc.descriptionPubMed ID: 31187776
dc.description.abstractPapillon-Leage-Psaume Syndrome, also known as oral-facial-digital syndrome (OFDS) type I, describes a group of neurodevelopmental disorders that are characterized by anomalies of the oral cavity, facial features, and the digits. Central nervous system (CNS) anomalies and visceral organ abnormalities such as kidney, pancreas, and ovarian cysts can also be seen in these patients. Among 13 potential types, female-inherited OFDS type I is the most common and it has been reported to be lethal in males. After the identification of the genetic relation of OFDS in 2001, it is now known that, except X-linked OFDS Type I and VIII, generally all types of OFDSs are autosomal recessive. The dentist's knowledge about the syndrome can reduce the development of physical and dental anomalies by facilitating early diagnosis. This article presents a patient with Papillon-Leage-Psaume Syndrome (Oral-Facial-Digital Syndrome Type I).
dc.identifier.citationKılınç, D. D. ve Özsarp, E. (2019). Papillon-Leage and psaume syndrome patient with multiple dental and orofacial anomalies. Nigerian Journal of Clinical Practice, 22(6), 872-876. https://dx.doi.org/10.4103/njcp.njcp_451_18
dc.identifier.doi10.4103/njcp.njcp_451_18
dc.identifier.endpage876
dc.identifier.issn1119-3077
dc.identifier.issue6
dc.identifier.scopusqualityQ3
dc.identifier.startpage872
dc.identifier.urihttps://dx.doi.org/10.4103/njcp.njcp_451_18
dc.identifier.urihttps://hdl.handle.net/20.500.12511/1628
dc.identifier.volume22
dc.identifier.wosqualityQ4
dc.indekslendigikaynakWeb of Science
dc.indekslendigikaynakScopus
dc.indekslendigikaynakPubMed
dc.language.isoen
dc.publisherWolters Kluwer Medknow Publications
dc.relation.ispartofNigerian Journal of Clinical Practiceen_US
dc.relation.publicationcategoryMakale - Uluslararası Hakemli Dergi - Kurum Öğretim Elemanı
dc.rightsinfo:eu-repo/semantics/embargoedAccess
dc.subjectCleft Palate
dc.subjectOral-Facial-Digital Syndrome Type I
dc.subjectPapillon-Leage Psaume Syndrome
dc.titlePapillon-Leage and psaume syndrome patient with multiple dental and orofacial anomalies
dc.typeArticle

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