Document Detail


Characterization of a de novo complex chromosomal rearrangement in a patient with cri-du-chat and trisomy 5p syndromes.
MedLine Citation:
PMID:  19842199     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Two syndromes with abnormalities of the short arm of chromosome 5 have been described: cri-du-chat (resulting from 5p deletion) and trisomy 5p. We report for the first time a patient with both syndromes, resulting from a complex chromosomal rearrangement with an inverted duplication of 5p13.1-p14.2, a deletion of 5p14.2-pter, and a duplication of 5p12, characterized by array-CGH and BAC clones. The patient showed phenotypic characteristics of both syndromes and died at 3 months of age as a result of cardiorespiratory failure, probably associated with the clinical severity of the trisomy 5p syndrome. We propose a potential causative mechanism for this rearrangement.
Authors:
Ascensi??n Vera-Carbonell; Juan Antonio Bafalliu; Encarna Guill??n-Navarro; Ariadna Escalona; Mar??a J Ballesta-Mart??nez; Carme Fuster; Asunci??n Fern??ndez; Isabel L??pez-Exp??sito
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Publication Detail:
Type:  Case Reports; Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  American journal of medical genetics. Part A     Volume:  149A     ISSN:  1552-4833     ISO Abbreviation:  Am. J. Med. Genet. A     Publication Date:  2009 Nov 
Date Detail:
Created Date:  2009-11-05     Completed Date:  2010-01-06     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  101235741     Medline TA:  Am J Med Genet A     Country:  United States    
Other Details:
Languages:  eng     Pagination:  2513-21     Citation Subset:  IM    
Copyright Information:
Copyright 2009 Wiley-Liss, Inc.
Affiliation:
Secci??n de Citogen??tica, Centro de Bioqu??mica y Gen??tica Cl??nica, Hospital U. Virgen de la Arrixaca, El Palmar, Murcia, Spain. ascension.vera3@carm.es
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MeSH Terms
Descriptor/Qualifier:
Abnormalities, Multiple / genetics*
Adult
Chromosome Aberrations*
Chromosome Banding
Chromosomes, Artificial, Bacterial / genetics
Chromosomes, Human, Pair 5 / genetics*
Cri-du-Chat Syndrome / genetics*
Fatal Outcome
Female
Humans
In Situ Hybridization, Fluorescence
Infant, Newborn
Male
Meiosis
Phenotype
Pregnancy
Syndrome
Trisomy / genetics*

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


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