Literature DB >> 19681142

Oral findings in ankyloblepharon-ectodermal dysplasia-cleft lip/palate (AEC) syndrome.

Frank Farrington1, Leonard Lausten.   

Abstract

This article outlines the dental conditions and treatment findings of individuals ranging in age from 4 months to 30 years of age diagnosed with ankyloblepharon-ectodermal dysplasia-cleft lip/palate (AEC) syndrome. The average number of permanent teeth present is 4.75 with a range of 0-12 teeth. The most frequently present permanent teeth are first molars, canines and maxillary incisors. The dentition of AEC-affected individuals in our study is similar to reports in other ectodermal dysplasia syndromes. The dental treatment findings, including those necessitated by the cleft palate defect seen in the syndrome, are also similar to age appropriate treatment seen in other cases of ectodermal dysplasia syndromes. Only 22% of subjects had prosthetic replacement of missing teeth. History indicated that issues related to treatment of the palatal cleft and lack of recognition of the oral defects as a part of the underlying medical problem were factors in receiving comprehensive tooth replacement care. (c) 2009 Wiley-Liss, Inc.

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Year:  2009        PMID: 19681142     DOI: 10.1002/ajmg.a.32790

Source DB:  PubMed          Journal:  Am J Med Genet A        ISSN: 1552-4825            Impact factor:   2.802


  4 in total

1.  Case report: unusual dental morphology in a child with ankyloblepharon-ectodermal defects-cleft lip/palate (AEC) syndrome.

Authors:  K Fitzgerald; S A Lynch; E McKiernan
Journal:  Eur Arch Paediatr Dent       Date:  2011-08

2.  Gene p63: In ectrodactyly-ectodermal dysplasia clefting, ankyloblepharon-ectodermal dysplasia, Rapp-Hodgkin syndrome.

Authors:  Cornelia van Straten; Kurt-W Butow
Journal:  Ann Maxillofac Surg       Date:  2013-01

3.  Mutant p63 causes defective expansion of ectodermal progenitor cells and impaired FGF signalling in AEC syndrome.

Authors:  Giustina Ferone; Helen A Thomason; Dario Antonini; Laura De Rosa; Bing Hu; Marica Gemei; Huiqing Zhou; Raffaele Ambrosio; David P Rice; Dario Acampora; Hans van Bokhoven; Luigi Del Vecchio; Maranke I Koster; Gianluca Tadini; Bradley Spencer-Dene; Michael Dixon; Jill Dixon; Caterina Missero
Journal:  EMBO Mol Med       Date:  2012-01-13       Impact factor: 12.137

4.  Novel missense mutation of the TP63 gene in a newborn with Hay-Wells/Ankyloblepharon-Ectodermal defects-Cleft lip/palate (AEC) syndrome: clinical report and follow-up.

Authors:  Gregorio Serra; Vincenzo Antona; Mario Giuffré; Federica Li Pomi; Lucia Lo Scalzo; Ettore Piro; Ingrid Anne Mandy Schierz; Giovanni Corsello
Journal:  Ital J Pediatr       Date:  2021-09-28       Impact factor: 2.638

  4 in total

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