Document Detail


COFS syndrome with familial 1;16 translocation.
MedLine Citation:
PMID:  9001808     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
We report on an Egyptian girl with phenotypic abnormalities of cerebro-oculofacio-skeletal syndrome. She had microcephaly, bilateral congenital cataract, nystagmus, long ear pinnae, camptodactyly, prominent heels, coxa valga, kyphosis and flexure contracture of the elbows and knees. CT scan showed bilateral symmetrical intracranial calcifications. In addition, she had an apparently balanced translocation: 46,XX,t(1;16)(q23;q13) in all cells transmitted from a phenotypically normal mother with a similar balanced translocation mosaicism. We suggest that genes for COFS syndrome could be located on chromosome 1q23 or 16q13. We recommend chromosomal analysis and DNA studies in cases with COFS manifestations.
Authors:
S A Temtamy; N A Meguid; A Mahmoud; H H Afifi; A Gerzawy; M S Zaki
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Clinical genetics     Volume:  50     ISSN:  0009-9163     ISO Abbreviation:  Clin. Genet.     Publication Date:  1996 Oct 
Date Detail:
Created Date:  1997-03-26     Completed Date:  1997-03-26     Revised Date:  2004-11-17    
Medline Journal Info:
Nlm Unique ID:  0253664     Medline TA:  Clin Genet     Country:  DENMARK    
Other Details:
Languages:  eng     Pagination:  240-3     Citation Subset:  IM    
Affiliation:
Human Genetics Department, National Research Centre, Cairo, Egypt.
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MeSH Terms
Descriptor/Qualifier:
Bone and Bones / abnormalities
Child, Preschool
Chromosome Aberrations / genetics*,  radiography
Chromosome Disorders
Consanguinity
Craniofacial Abnormalities / genetics*,  radiography
Developmental Disabilities
Female
Humans
Karyotyping
Skull / radiography
Syndrome
Translocation, Genetic / genetics*

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