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      Orthodontic Management of Silver-Russell Syndrome. A Case Report

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          There is no author summary for this article yet. Authors can add summaries to their articles on ScienceOpen to make them more accessible to a non-specialist audience.

          Abstract

          This case report describes the orthodontic management of a 10-year-old female with Silver-Russell syndrome by means of gradual expansion of the mandibular dental arch using removable appliances and subsequent comprehensive treatment of malocclusion by means of fixed appliances.

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          Three-generation dominant transmission of the Silver-Russell syndrome.

          Rita Shapiro, B Vibert, Rebecca Duncan … (1990)
          We report on 7 patients with the Silver-Russell syndrome (SRS) in two 3-generation families. Three patients in each of the families had an undergrowth of the left side of the body when compared with the normal right side. The clinical courses were mild as compared to the severity sometimes described in sporadic cases. These patients and a review of 190 SRS cases from the literature showed that there were 23 families in which 38 patients had completely expressed SRS. In 17 of the families, multiple maternal relatives had complete or partial expressions of the SRS. Most SRS patients have been reported to occur sporadically; however, of the 197 propositi analyzed, 19% had more than one affected individual in a family and several different modes of inheritance could have been responsible. Two families (8.7%) had spontaneous dominant mutations (twins) and possible autosomal recessive transmission was present in 4 families (17.4%). Because no male-to-male transmission has yet been documented in the 21 families in the literature and the two families reported here, X-linked dominant inheritance is a possibility in 17 families (74%). Thus, although sporadic occurrences and genetic heterogeneity appear to be involved in the SRS, dominant inheritance may be a major causal factor.
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            The Russell-Silver syndrome: a case report and brief review of the literature.

            Robert Perkins, Maj Tuan A Hoang-Xuan (2015)
            The Russell-Silver syndrome's phenotypic features consist of musculoskeletal abnormalities, genitourinary malformations, craniofacial dysmorphy and cutaneous dyschromia, which is usually reported as café au lait spots. We present the first instances of a large, unilateral, achromic patch as an additional cutaneous manifestation of the Russell-Silver syndrome.
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              Silver-Russell dwarfism and cystic fibrosis in a twin. Endocrine, chromosomal, dermatoglyphic, and craniofacial studies.

              Jeffrey Christiansen, L Taussig, G Braunstein … (1973)
              Article 0 comments Cited 3 times – based on 0 reviews     Review now
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                Author and article information

                Journal
                Journal ID (nlm-ta): Open Dent J
                Journal ID (iso-abbrev): Open Dent J
                Journal ID (publisher-id): TODENTJ
                Title: The Open Dentistry Journal
                Publisher: Bentham Open
                ISSN (Electronic): 1874-2106
                Publication date (Electronic): 10 August 2012
                Publication date Collection: 2012
                Volume: 6
                Pages: 131-136
                Affiliations
                Department of Orthodontics, School of Dentistry, Aristotle University of Thessaloniki, Greece
                Author notes
                [* ]Address correspondence to this author at the Ioulia Ioannidou-Marathiotou, Associate Professor, Department of Orthodontics, School of Dentistry, Aristotle University of Thessaloniki, Agiou Dimitriou Street, 54124 Thessaloniki, Greece; Tel.: +30 2310 999483; Fax: +30 2310 999549; E-mail: ioannidou@ 123456dent.auth.gr
                Article
                Publisher ID: TODENTJ-6-131
                DOI: 10.2174/1874210601206010131
                PMC ID: 3426784
                PubMed ID: 22927891
                SO-VID: 929dcbc9-c64e-454d-b2cc-3aa894c68068
                Copyright © © Ioannidou-Marathiotou et al.; Licensee Bentham Open.
                License:

                This is an open access article licensed under the terms of the Creative Commons Attribution Non-Commercial License ( http://creativecommons.org/licenses/by-nc/3.0/) which permits unrestricted, non-commercial use, distribution and reproduction in any medium, provided the work is properly cited.

                History
                Date received : 7 June 2012
                Date revision received : 03 July 2012
                Date accepted : 04 July 2012
                Categories
                Subject: Case Report

                ScienceOpen disciplines: Dentistry
                Keywords: orthodontics.,silver-russell syndrome
                Data availability:
                ScienceOpen disciplines: Dentistry
                Keywords: orthodontics., silver-russell syndrome

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