Literature DB >> 32888391

Alazami syndrome: Phenotypic expansion and clinical resemblance to Smith-Lemli-Opitz syndrome.

Simone Gana1, Massimo Plumari1, Elena Rossi1,2, Annalisa Saracino3, Melanie Iorio3, Martina Paola Zanaboni4, Simona Orcesi3,4, Enza Maria Valente1,2.   

Abstract

Biallelic mutations in the LARP7 gene have been recently shown to cause Alazami syndrome, a rare condition characterized by short stature, intellectual disability, and peculiar facial dysmorphisms. To date, only 24 cases have been reported. Here, we describe two brothers initially suspected to have Smith-Lemli-Opitz syndrome, in whom clinical exome sequencing detected a novel homozygous truncating variant in LARP7. These cases expand the phenotypic spectrum of Alazami syndrome to include toes syndactyly and adaptive behavior, and confirm the power of "genotype first" approach in patients with syndromic presentations overlapping distinct rare conditions.
© 2020 Wiley Periodicals LLC.

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Year:  2020        PMID: 32888391     DOI: 10.1002/ajmg.a.61832

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


  1 in total

1.  Patient with Phenylketonuria and Intellectual Disability-Problem Not Always Caused Exclusively by Insufficient Metabolic Control (Coexistence of PKU and Alazami Syndrome).

Authors:  Michał Patalan; Alicja Leśniak; Krzysztof Bernatowicz; Hanna Romanowska; Elżbieta Krzywińska-Zdeb; Mieczysław Walczak; Maria Giżewska
Journal:  Int J Environ Res Public Health       Date:  2022-02-24       Impact factor: 3.390

  1 in total

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