Literature DB >> 30643948

Apert syndrome without craniosynostosis.

Diego de Ângelis Ramos1, Hamilton Matushita2, Daniel Dante Cardeal2, Clarissa Nóbrega Gambarra Nascimento2, Manoel Jacobsen Teixeira2.   

Abstract

BACKGROUND: Apert syndrome is a rare form of syndromic craniosynostosis, also known as acrocephalosyndactyly, which is a disorder characterized by a unique set of craniofacial, hand, and foot abnormalities. Diagnosis is made through a genetic analysis, where the mutation of FGFR2, Ser252Trp, and Pro253Arg confirms the diagnosis. CASE
PRESENTATION: Although craniosynostosis is the most common characteristic in clinical presentation, we present an atypical case of a one-and-a-half-year-old girl with Apert syndrome confirmed by genetic testing but without craniosynostosis.

Entities:  

Keywords:  Apert syndrome; Atypical case; Craniosynostosis; Fibroblast growth factor receptors (FGFR)2

Year:  2019        PMID: 30643948     DOI: 10.1007/s00381-019-04050-1

Source DB:  PubMed          Journal:  Childs Nerv Syst        ISSN: 0256-7040            Impact factor:   1.475


  14 in total

Review 1.  Apert's syndrome: cephalometric evaluation and considerations on pathogenesis.

Authors:  A Avantaggiato; F Carinci; C Curioni
Journal:  J Craniofac Surg       Date:  1996-01       Impact factor: 1.046

Review 2.  Management of craniosynostoses.

Authors:  D Renier; E Lajeunie; E Arnaud; D Marchac
Journal:  Childs Nerv Syst       Date:  2000-11       Impact factor: 1.475

Review 3.  Clinical genetics of craniosynostosis.

Authors:  Andrew O M Wilkie; David Johnson; Steven A Wall
Journal:  Curr Opin Pediatr       Date:  2017-12       Impact factor: 2.856

4.  Exclusive paternal origin of new mutations in Apert syndrome.

Authors:  D M Moloney; S F Slaney; M Oldridge; S A Wall; P Sahlin; G Stenman; A O Wilkie
Journal:  Nat Genet       Date:  1996-05       Impact factor: 38.330

5.  Clinical variability in patients with Apert's syndrome.

Authors:  E Lajeunie; R Cameron; V El Ghouzzi; N de Parseval; P Journeau; M Gonzales; A L Delezoide; J Bonaventure; M Le Merrer; D Renier
Journal:  J Neurosurg       Date:  1999-03       Impact factor: 5.115

6.  Birth prevalence study of the Apert syndrome.

Authors:  M M Cohen; S Kreiborg; E J Lammer; J F Cordero; P Mastroiacovo; J D Erickson; P Roeper; M L Martínez-Frías
Journal:  Am J Med Genet       Date:  1992-03-01

7.  Mutations in the transmembrane domain of FGFR3 cause the most common genetic form of dwarfism, achondroplasia.

Authors:  R Shiang; L M Thompson; Y Z Zhu; D M Church; T J Fielder; M Bocian; S T Winokur; J J Wasmuth
Journal:  Cell       Date:  1994-07-29       Impact factor: 41.582

8.  Progressive postnatal craniosynostosis and increased intracranial pressure.

Authors:  John P Connolly; Joseph Gruss; Marianne L Seto; Michael F Whelan; Richard Ellenbogen; Avery Weiss; Steven R Buchman; Michael L Cunningham
Journal:  Plast Reconstr Surg       Date:  2004-04-15       Impact factor: 4.730

9.  Mutations in the gene encoding fibroblast growth factor receptor-3 in achondroplasia.

Authors:  F Rousseau; J Bonaventure; L Legeai-Mallet; A Pelet; J M Rozet; P Maroteaux; M Le Merrer; A Munnich
Journal:  Nature       Date:  1994-09-15       Impact factor: 49.962

10.  Developmental expression of two murine fibroblast growth factor receptors, flg and bek.

Authors:  A Orr-Urtreger; D Givol; A Yayon; Y Yarden; P Lonai
Journal:  Development       Date:  1991-12       Impact factor: 6.868

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