Search for authorsSearch for similar articles
547
views
0
recommends
+1 Recommend
1 collections
    0
    shares

      To submit to Bentham Journals, please click here

      • Record: found
      • Abstract: found
      • Article: found
      Is Open Access

      Orthodontic Management of Silver-Russell Syndrome. A Case Report

      case-report

      Read this article at

      Bookmark
          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.

          Related collections

          Most cited references23

          • Record: found
          • Abstract: not found
          • Article: not found

          Syndrome of congenital hemihypertrophy, shortness of stature, and elevated urinary gonadotropins.

            Bookmark
            • Record: found
            • Abstract: found
            • Article: not found

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

            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.
              Bookmark
              • Record: found
              • Abstract: found
              • Article: not found

              Growth hormone therapy in Silver Russell syndrome: 5 years experience of the Australian and New Zealand Growth database (OZGROW).

              Data were analysed on 33 children (22 males) with Silver Russell syndrome treated with growth hormone for periods up to 5 years. Baseline data (medians) at commencement of growth hormone (GH) therapy were age 6.7 years, bone age delay 1.7 years, height standard deviation score (SDS)-3.2, weight SDS -3.1, and growth velocity 5.7 cm/ year. All were prepubertal. Median birth weight SDS for gestational age was -3.2. GH was commenced at 14 IU/m2 per week and subsequently adjusted according to response. Growth velocity and growth velocity SDS for chronological age (CA) improved over baseline and gains in height SDS for CA were 1.0, 1.5 and 1.8 SD over 3, 4 and 5 years respectively (P < 0.001). No significant increase in height SDS for bone age was observed. Increased GH doses were required after the 1st year to maintain growth rates. Mean bone age advancement was 3.1 years after 3 years of treatment, and 6.0 years after 5 years treatment. Younger age was a predictor of the growth response over the 1st year. Predictors of response after 3 years were catch up growth, low weight SDS at birth and low height SDS for CA. Age at onset of puberty was normal, but height at onset of puberty was lower than normal means. We have demonstrated significant improvement in growth in Silver Russell syndrome after 3 years of GH therapy, however data on estimated mature height and final height are insufficient to conclude final outcomes. Further follow up is required to assess the long-term benefit.
                Bookmark

                Author and article information

                Journal
                Open Dent J
                Open Dent J
                TODENTJ
                The Open Dentistry Journal
                Bentham Open
                1874-2106
                10 August 2012
                2012
                : 6
                : 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
                TODENTJ-6-131
                10.2174/1874210601206010131
                3426784
                22927891
                929dcbc9-c64e-454d-b2cc-3aa894c68068
                © Ioannidou-Marathiotou et al.; Licensee Bentham Open.

                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
                : 7 June 2012
                : 03 July 2012
                : 04 July 2012
                Categories
                Case Report

                Dentistry
                orthodontics.,silver-russell syndrome
                Dentistry
                orthodontics., silver-russell syndrome

                Comments

                Comment on this article