Literature DB >> 1204988

Cerebro-facio-thoracic dysplasia: report of three cases.

I Pascual-Castroviejo, J M Santolaya, V L Martin, T Rodriguez-Costa, A Tendero, F Mulas.   

Abstract

Three children, two girls and one boy, are described as suffering from a new syndrome, 'cerebro-facio-thoracic dysplasia'. The characteristic findings are mental retardation, characteristic facies, narrow forehead, bushy eyebrows with synophrys, hypertelorism, broad nose, wide philtrum, triangular-shaped mouth, short neck, marked maxillary hypoplasia, a low hairline (especially posteriorly in the midline on the neck), brachycephaly, calcified clinoid ligements, and multiple bony abnormalities in the upper thoracic vertebrae and sometimes in the cervical region, together with a variety of deformities of the upper ribs. It seems possible that the condition is hereditary and that it may be inherited as a result of an autosomal recessive trait.

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Year:  1975        PMID: 1204988     DOI: 10.1111/j.1469-8749.1975.tb04673.x

Source DB:  PubMed          Journal:  Dev Med Child Neurol        ISSN: 0012-1622            Impact factor:   5.449


  8 in total

1.  Cerebrofaciothoracic dysplasia: a new family.

Authors:  N Philip; A Guala; A Moncla; M Monlouis; S Aymé; F Giraud
Journal:  J Med Genet       Date:  1992-07       Impact factor: 6.318

2.  Hereditary multiple exostoses.

Authors:  R C Hennekam
Journal:  J Med Genet       Date:  1991-04       Impact factor: 6.318

3.  Pascual-Castroviejo type II syndrome (P-CIIS). Importance of the presence of persistent embryonic arteries.

Authors:  Ignacio Pascual-Castroviejo; Juan Alvarez-Linera; Juan Coya; Juan Viaño; Samuel-Ignacio Pascual-Pascual; Ramón Velázquez-Fragua; Juan-Carlos López-Gutiérrez
Journal:  Childs Nerv Syst       Date:  2010-07-31       Impact factor: 1.475

4.  Whole-exome sequencing links TMCO1 defect syndrome with cerebro-facio-thoracic dysplasia.

Authors:  Davut Pehlivan; Ender Karaca; Hatip Aydin; Christine R Beck; Tomasz Gambin; Donna M Muzny; B Bilge Geckinli; Ali Karaman; Shalini N Jhangiani; Richard A Gibbs; James R Lupski
Journal:  Eur J Hum Genet       Date:  2014-01-15       Impact factor: 4.246

5.  Evidence for variable gene expression in a large inbred kindred with autosomal recessive spondylocostal dysostosis.

Authors:  P D Turnpenny; R J Thwaites; F N Boulos
Journal:  J Med Genet       Date:  1991-01       Impact factor: 6.318

6.  Cerebro-facio-thoracic dysplasia (Pascual-Castroviejo syndrome): Identification of a novel mutation, use of facial recognition analysis, and review of the literature.

Authors:  Jennifer A F Tender; Carlos R Ferreira
Journal:  Transl Sci Rare Dis       Date:  2018-04-13

7.  Ca2+ homeostasis maintained by TMCO1 underlies corpus callosum development via ERK signaling.

Authors:  Ke-Yan Yang; Song Zhao; Haiping Feng; Jiaqi Shen; Yuwei Chen; Si-Tong Wang; Si-Jia Wang; Yu-Xin Zhang; Yun Wang; Caixia Guo; Hongmei Liu; Tie-Shan Tang
Journal:  Cell Death Dis       Date:  2022-08-04       Impact factor: 9.685

Review 8.  From Disease Description and Gene Discovery to Functional Cell Pathway: A Decade-Long Journey for TMCO1.

Authors:  Helen Batchelor-Regan; Baozhong Xin; Aimin Zhou; Heng Wang
Journal:  Front Genet       Date:  2021-05-20       Impact factor: 4.599

  8 in total

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