Document Detail


Fourth case of cerebral, ocular, dental, auricular, skeletal syndrome (CODAS), description of new features and molecular analysis.
MedLine Citation:
PMID:  20503327     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Cerebral, ocular, dental, auricular, skeletal syndrome (CODAS, OMIM 600373) is a very rare congenital malformation syndrome. This clinical entity is highly distinctive and associates mental retardation, cataract, enamel abnormalities, malformations of the helix, epiphyseal and vertebral malformations, and characteristic dysmorphic features. Since 1991, only three affected children have been reported. The etiology and pattern of inheritance of CODAS syndrome still remain unknown. We describe a new sporadic case presenting with all the characteristic features of CODAS syndrome associated with previously unreported malformations of the heart, larynx, and liver. All investigations such as karyotype, metabolic screening and array CGH were normal.
Authors:
S Marlin; H Ducou Le Pointe; M Le Merrer; M F Portnoi; S Chantot; L Jonard; A Mantel-Guiochon; J P Siffroi; E N Garabedian; F Denoyelle
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  American journal of medical genetics. Part A     Volume:  152A     ISSN:  1552-4833     ISO Abbreviation:  Am. J. Med. Genet. A     Publication Date:  2010 Jun 
Date Detail:
Created Date:  2010-05-26     Completed Date:  2010-08-30     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  101235741     Medline TA:  Am J Med Genet A     Country:  United States    
Other Details:
Languages:  eng     Pagination:  1510-4     Citation Subset:  IM    
Copyright Information:
(c) 2010 Wiley-Liss, Inc.
Affiliation:
Service de Génétique, Hôpital Trousseau, APHP, Paris, France. sandrine.marlin@trs.aphp.fr
Data Bank Information
Bank Name/Acc. No.:
OMIM/600373
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MeSH Terms
Descriptor/Qualifier:
Abnormalities, Multiple / diagnosis*,  genetics
Bone and Bones / abnormalities*
Cataract / congenital,  diagnosis*,  genetics
Cerebral Cortex / abnormalities*
Child, Preschool
Heart Atria / abnormalities*
Humans
Male
Mental Retardation / diagnosis*,  genetics
Muscle, Skeletal / abnormalities*
Syndrome
Tooth Abnormalities / diagnosis*,  genetics

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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