Mandibulofacial Dysostosis, Guion-Almeida Type

Alternative Names

  • MFDGA
  • Mandibulofacial Dysostosis with Microcephaly
  • MFDM
  • Growth and Mental Retardation, Mandibulofacial Dysostosis, Microcephaly, and Cleft Palate
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WHO-ICD-10 version:2010

Congenital malformations, deformations and chromosomal abnormalities

Other congenital malformations

OMIM Number

610536

Gene Map Locus

17q21.31

Description

Mandibulofacial dysostosis with microcephaly is a rare syndrome comprising progressive microcephaly, midface and malar hypoplasia, micrognathia, microtia, dysplastic ears, preauricular skin tags, significant developmental delay, and speech delay. Many patients have major sequelae, including choanal atresia that results in respiratory difficulties, conductive hearing loss, and cleft palate. [From OMIM]

Epidemiology in the Arab World

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Subject IDCountrySexFamily HistoryParental ConsanguinityHPO TermsVariantZygosityMode of InheritanceReferenceRemarks
610536.1.1LebanonFemaleYesNo Intellectual disability; Microcephaly; M...NM_004247.4:c.2622dupTHeterozygousAutosomal, DominantMégarbané et al., 2005; Voigt et al. 2013 Germline mosaicism i...
610536.1.2LebanonFemaleYesNo Intellectual disability; Microcephaly; M...NM_004247.4:c.2622dupTHeterozygousAutosomal, DominantMégarbané et al., 2005; Voigt et al. 2013 Sister of 610536.1.1...
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