Literature DB >> 26676648

Smith-Magenis Syndrome: Face Speaks.

Rekha Gupta1, Neerja Gupta2, Sheela Nampoothiri3, Kausik Mandal4, Yougal Kishore4, Pankaj Sharma2, Madhulika Kabra2, Shubha R Phadke5.   

Abstract

Smith-Magenis syndrome is a well delineated microdeletion syndrome with characteristic facial and behavioral phenotype. With the availability of the multi-targeted molecular cytogenetic techniques like Multiplex Ligation Probe Amplification and cytogenetic microarray, the cases are diagnosed even without clinical suspicion. Here, the authors present clinical features of nine Indian cases of Smith-Magenis syndrome. Characteristic facial phenotype including tented upper lip, broad forehead, midface hypoplasia, short philtrum and upslant of palpebral fissure is obvious in the photographs. The behavioral variations were seen in some of the cases but were not the presenting features. The characteristic facial phenotype can be an important clinical guide to the diagnosis.

Entities:  

Keywords:  Cytogenetic microarray (CMA); Developmental delay (DD); Fluorescent in situ hybridization (FISH); Intellectual disability (ID); Multiplex ligation dependant probe amplification (MLPA); Smith-Magenis syndrome (SMS)

Mesh:

Year:  2015        PMID: 26676648     DOI: 10.1007/s12098-015-1940-y

Source DB:  PubMed          Journal:  Indian J Pediatr        ISSN: 0019-5456            Impact factor:   1.967


  13 in total

1.  Distinctiveness and correlates of maladaptive behaviour in children and adolescents with Smith-Magenis syndrome.

Authors:  E M Dykens; A C Smith
Journal:  J Intellect Disabil Res       Date:  1998-12

2.  Homologous recombination of a flanking repeat gene cluster is a mechanism for a common contiguous gene deletion syndrome.

Authors:  K S Chen; P Manian; T Koeuth; L Potocki; Q Zhao; A C Chinault; C C Lee; J R Lupski
Journal:  Nat Genet       Date:  1997-10       Impact factor: 38.330

3.  Characterization of self-injurious behaviors in children and adults with Smith-Magenis syndrome.

Authors:  B Finucane; K H Dirrigl; E W Simon
Journal:  Am J Ment Retard       Date:  2001-01

4.  The face of Smith-Magenis syndrome: a subjective and objective study.

Authors:  J E Allanson; F Greenberg; A C Smith
Journal:  J Med Genet       Date:  1999-05       Impact factor: 6.318

5.  Multi-disciplinary clinical study of Smith-Magenis syndrome (deletion 17p11.2)

Authors:  F Greenberg; R A Lewis; L Potocki; D Glaze; J Parke; J Killian; M A Murphy; D Williamson; F Brown; R Dutton; C McCluggage; E Friedman; M Sulek; J R Lupski
Journal:  Am J Med Genet       Date:  1996-03-29

6.  Smith-Magenis syndrome deletion: a case with equivocal cytogenetic findings resolved by fluorescence in situ hybridization.

Authors:  R C Juyal; F Greenberg; G A Mengden; J R Lupski; B J Trask; G van den Engh; E A Lindsay; H Christy; K S Chen; A Baldini
Journal:  Am J Med Genet       Date:  1995-09-11

Review 7.  Ophthalmic manifestations of Smith-Magenis syndrome.

Authors:  R M Chen; J R Lupski; F Greenberg; R A Lewis
Journal:  Ophthalmology       Date:  1996-07       Impact factor: 12.079

8.  Gender, genotype, and phenotype differences in Smith-Magenis syndrome: a meta-analysis of 105 cases.

Authors:  E A Edelman; S Girirajan; B Finucane; P I Patel; J R Lupski; A C M Smith; S H Elsea
Journal:  Clin Genet       Date:  2007-06       Impact factor: 4.438

9.  Molecular analysis of the Smith-Magenis syndrome: a possible contiguous-gene syndrome associated with del(17)(p11.2).

Authors:  F Greenberg; V Guzzetta; R Montes de Oca-Luna; R E Magenis; A C Smith; S F Richter; I Kondo; W B Dobyns; P I Patel; J R Lupski
Journal:  Am J Hum Genet       Date:  1991-12       Impact factor: 11.025

10.  Genotype-phenotype correlation in Smith-Magenis syndrome: evidence that multiple genes in 17p11.2 contribute to the clinical spectrum.

Authors:  Santhosh Girirajan; Christopher N Vlangos; Barbara B Szomju; Emily Edelman; Christopher D Trevors; Lucie Dupuis; Marjan Nezarati; David J Bunyan; Sarah H Elsea
Journal:  Genet Med       Date:  2006-07       Impact factor: 8.822

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  2 in total

1.  Prenatal Diagnosis of 17p11.2 Copy Number Abnormalities Associated With Smith-Magenis and Potocki-Lupski Syndromes in Fetuses.

Authors:  Meiying Cai; Xianguo Fu; Liangpu Xu; Na Lin; Hailong Huang
Journal:  Front Genet       Date:  2021-12-21       Impact factor: 4.599

2.  Acoustic Analysis of Phonation in Children With Smith-Magenis Syndrome.

Authors:  Irene Hidalgo-De la Guía; Elena Garayzábal-Heinze; Pedro Gómez-Vilda; Rafael Martínez-Olalla; Daniel Palacios-Alonso
Journal:  Front Hum Neurosci       Date:  2021-06-03       Impact factor: 3.169

  2 in total

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