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Warsaw Breakage Syndrome--A further report, emphasising cutaneous findings.
Bailey, Claire; Fryer, Alan E; Greenslade, Mark.
Afiliação
  • Bailey C; Department of Clinical Genetics, Liverpool Women's Hospital, Crown Street, Liverpool, L8 7SS, UK.
  • Fryer AE; Department of Clinical Genetics, Liverpool Women's Hospital, Crown Street, Liverpool, L8 7SS, UK. Electronic address: alan.fryer@lwh.nhs.uk.
  • Greenslade M; Bristol Genetics Laboratory, Southmead Hospital, Bristol, BS10 5NB.
Eur J Med Genet ; 58(4): 235-7, 2015 Apr.
Article em En | MEDLINE | ID: mdl-25701697
We report a new case of Warsaw Breakage syndrome (WABS) with 2 confirmed mutations in DDX11. Like the previous reported cases [Capo-Chichi et al., 2012; Van der Lelij et al., 2010], there was evidence of pre- and postnatal growth retardation, severe microcephaly, intellectual disability and facial dysmorphism. The patient had sensorineural hearing loss with evidence of bilateral hypoplastic cochleas on imaging, another feature which has been reported in the previous cases of WABS. In our case the patient exhibited a chronic rash of livedo reticularis with telangiectasia on her legs. Abnormally pigmented lesions and cutis mamorata were reported in the original WABS case.
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Texto completo: 1 Base de dados: MEDLINE Assunto principal: Anormalidades Múltiplas / DNA Helicases / RNA Helicases DEAD-box Tipo de estudo: Diagnostic_studies Limite: Adolescent / Female / Humans País/Região como assunto: Europa Idioma: En Revista: Eur J Med Genet Assunto da revista: GENETICA MEDICA Ano de publicação: 2015 Tipo de documento: Article

Texto completo: 1 Base de dados: MEDLINE Assunto principal: Anormalidades Múltiplas / DNA Helicases / RNA Helicases DEAD-box Tipo de estudo: Diagnostic_studies Limite: Adolescent / Female / Humans País/Região como assunto: Europa Idioma: En Revista: Eur J Med Genet Assunto da revista: GENETICA MEDICA Ano de publicação: 2015 Tipo de documento: Article