Document Detail


Imperforate anus is a rare associated finding in blepharocheilodontic syndrome.
MedLine Citation:
PMID:  20101698     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Blepharocheilodontic (BCD) syndrome is a rare autosomal-dominant condition that is characterized by lower eyelid ectropion, upper eyelid distichiasis, euryblepharon, bilateral cleft lip and palate, and conical teeth. It exhibits considerable phenotypic variability among affected individuals. An additional rare associated manifestation is imperforate a.u. (IA), which has been reported in three cases [Guyuron et al. (1995); J Craniofac Surg 6:392-394; Gorlin et al. (1996); Am J Med Genet 65:109-112; da Silva Lopes et al. (2003); Am J Med Genet Part A 121A:266-270]. Here we report on a family with BCD that includes IA, confirming that anorectal anomalies are a part of BCD syndrome.
Authors:
K Nicole Weaver; Katherine D Rutledge; John H Grant; Nathaniel H Robin
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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 Feb 
Date Detail:
Created Date:  2010-02-01     Completed Date:  2010-04-02     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  101235741     Medline TA:  Am J Med Genet A     Country:  United States    
Other Details:
Languages:  eng     Pagination:  438-40     Citation Subset:  IM    
Copyright Information:
Copyright 2010 Wiley-Liss, Inc.
Affiliation:
University of Alabama School of Medicine, Birmingham, Alabama, USA.
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MeSH Terms
Descriptor/Qualifier:
Abnormalities, Multiple / diagnosis*,  genetics*
Anus, Imperforate / genetics*
Child, Preschool
Cleft Lip / genetics
Cleft Palate / genetics
Ectropion / genetics*
Eyelids / abnormalities*
Female
Genes, Dominant
Humans
Infant
Pedigree
Phenotype
Syndrome*
Tooth Abnormalities / genetics*

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


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