Literature DB >> 28691455

ADULT syndrome: dental features of a very rare condition.

L Azzi1, V Maurino1, R Vinci2, F Croveri1, A Boggio1, A Tagliabue1, J Silvestre-Rangil3, L Tettamanti1.   

Abstract

The Acro-Dermato-Ungual-Lacrimal-Tooth syndrome (ADULT syndrome) is one of the rarest ectodermal dysplasias and it is associated with several malformations involving especially the limbs. The most clinical features are the presence of ectrodactyly, syndactyly, hypermelanosis or multiple lentigines, onhycodysplasia, abnormalities in the lacrimal duct, recurrent conjuntivitis, photophobia, mammarian hypoplasia, hypotrichosis and frontal alopecia, hypohydrosis, cutaneous photosensitivity, nasal bridge prominence, exfoliative dermatitis and xerosis. The ectodermal dysfunction expresses itself with conoid teeth, enamel hypoplasia, dentinal dysplasia and especially hypodontia, with following functional and aesthetic defects. We report the case of an 11-year-old Caucasian girl affected by ADULT syndrome.

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Year:  2017        PMID: 28691455

Source DB:  PubMed          Journal:  J Biol Regul Homeost Agents        ISSN: 0393-974X            Impact factor:   1.711


  1 in total

1.  Tooth defects of EEC and AEC syndrome caused by heterozygous TP63 mutations in three Chinese families and genotype-phenotype correlation analyses of TP63-related disorders.

Authors:  Jinglei Zheng; Haochen Liu; Yuan Zhan; Yang Liu; Sing-Wai Wong; Tao Cai; Hailan Feng; Dong Han
Journal:  Mol Genet Genomic Med       Date:  2019-05-02       Impact factor: 2.183

  1 in total

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