Document Detail


Proximal interstitial 1p36 deletion syndrome: the most proximal 3.5-Mb microdeletion identified on a dysmorphic and mentally retarded patient with inv(3)(p14.1q26.2).
MedLine Citation:
PMID:  18835671     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
From the investigation by microarray-based comparative genomic hybridization (aCGH), a new syndrome with "atypical" proximal interstitial deletion of 1p36.23-36.11 has been suggested. Here, we report on an 8.5-year-old girl with psychomotor developmental delay and a dysmorphic appearance. Although her G-banded chromosomal analysis showed inv(3)(p14.1q26.2), detailed FISH analyses denied pathogenic deletions around the breakpoints of chromosome 3. Accordingly, aCGH analysis was performed to identify a genomic aberration related to her phenotype, and a 3.5-Mb interstitial deletion of 1p36.13-36.12 was revealed. This deletion was the most proximal interstitial deletion of 1p36. Compared to the previously reported patients, abnormally shaped teeth, delayed tooth eruption, and leg malformation are unique phenotypes only to this patient, which might be due to the centromeric unique deletion region with 0.8-Mb.
Authors:
Keiko Shimojima; Marco T Páez; Kenji Kurosawa; Toshiyuki Yamamoto
Publication Detail:
Type:  Case Reports; Journal Article; Research Support, Non-U.S. Gov't     Date:  2008-10-05
Journal Detail:
Title:  Brain & development     Volume:  31     ISSN:  1872-7131     ISO Abbreviation:  Brain Dev.     Publication Date:  2009 Sep 
Date Detail:
Created Date:  2009-08-03     Completed Date:  2009-09-25     Revised Date:  2009-11-19    
Medline Journal Info:
Nlm Unique ID:  7909235     Medline TA:  Brain Dev     Country:  Netherlands    
Other Details:
Languages:  eng     Pagination:  629-33     Citation Subset:  IM    
Affiliation:
International Research and Educational Institute for Integrated Medical Sciences (IREIIMS), Tokyo Women's Medical University, Tokyo, Japan.
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MeSH Terms
Descriptor/Qualifier:
Abnormalities, Multiple / genetics*
Child
Chromosome Deletion*
Chromosome Inversion
Chromosome Mapping
Chromosomes, Human, Pair 1 / genetics*
Chromosomes, Human, Pair 3*
Craniofacial Abnormalities / diagnosis,  genetics
Female
Humans
In Situ Hybridization, Fluorescence
Mental Retardation / diagnosis,  genetics
Muscle Hypotonia / diagnosis,  genetics
Oligonucleotide Array Sequence Analysis
Phenotype
Syndrome

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