Literature DB >> 25387514

Syndrome in question. Costello syndrome.

Isy Lima Peixoto1, Ana Maria Carreno1, Vania Mesquita Gadelha Prazeres1, Caroline Albuquerque Rodrigues Chirano1, Gabriel Maroja Ihara1, Patricia Bandeira de Melo Akel1.   

Abstract

Costello syndrome (CS) is a rare genetic disorder, first described by Costello in 1971, caused by mutations in the HRAS proto-oncogene. Clinical findings include facial dysmorphism, skin disorders, cognitive impairment, cardiac and musculoskeletal defects. There is an increased risk of malignancies in these patients, due to the proto-oncogene mutation, and also sudden death secondary to heart disease. We report a case with characteristic phenotype, highlighting the peculiar skin changes.

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Year:  2014        PMID: 25387514      PMCID: PMC4230678          DOI: 10.1590/abd1806-4841.20143062

Source DB:  PubMed          Journal:  An Bras Dermatol        ISSN: 0365-0596            Impact factor:   1.896


  5 in total

Review 1.  Noonan, Costello and cardio-facio-cutaneous syndromes: dysregulation of the Ras-MAPK pathway.

Authors:  William E Tidyman; Katherine A Rauen
Journal:  Expert Rev Mol Med       Date:  2008-12-09       Impact factor: 5.600

2.  Costello syndrome: update on the original cases and commentary.

Authors:  J M Costello
Journal:  Am J Med Genet       Date:  1996-03-15

3.  Dermatological phenotype in Costello syndrome: consequences of Ras dysregulation in development.

Authors:  D H Siegel; J A Mann; A L Krol; K A Rauen
Journal:  Br J Dermatol       Date:  2012-03       Impact factor: 9.302

Review 4.  Costello syndrome: an overview.

Authors:  Raoul C M Hennekam
Journal:  Am J Med Genet C Semin Med Genet       Date:  2003-02-15       Impact factor: 3.908

Review 5.  Costello syndrome: a Ras/mitogen activated protein kinase pathway syndrome (rasopathy) resulting from HRAS germline mutations.

Authors:  Karen W Gripp; Angela E Lin
Journal:  Genet Med       Date:  2012-03       Impact factor: 8.822

  5 in total

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