Warsaw Breakage Syndrome--A further report, emphasising cutaneous findings.
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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Base de dados:
MEDLINE
Assunto principal:
Anormalidades Múltiplas
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DNA Helicases
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RNA Helicases DEAD-box
Tipo de estudo:
Diagnostic_studies
Limite:
Adolescent
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Female
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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