Document Detail


c. 595-596 insC of FOXC2 underlies lymphedema, distichiasis, ptosis, ankyloglossia, and Robin sequence in a Thai patient.
MedLine Citation:
PMID:  20186799     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Lymphedema-distichiasis syndrome is a rare primary lymphedema inherited as an autosomal dominant disorder. The characteristic features consist of late onset-lymphedema and distichiasis together with other occasionally seen features including varicose vein, cleft palate, ptosis, and congenital heart diseases. FOXC2 is the gene found to be associated with this syndrome. We report here the first Thai patient who has characteristic features of this syndrome and the infrequently described features including ankyloglossia, and Robin sequence which consists of glossoptosis, cleft palate, and micrognathia. Mutation analysis of FOXC2 revealed c. 595-596 insC.
Authors:
Pranoot Tanpaiboon; Piranit Kantaputra; Karn Wejathikul; Wirawit Piyamongkol
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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 Mar 
Date Detail:
Created Date:  2010-03-01     Completed Date:  2010-04-12     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  101235741     Medline TA:  Am J Med Genet A     Country:  United States    
Other Details:
Languages:  eng     Pagination:  737-40     Citation Subset:  IM    
Copyright Information:
(c) 2010 Wiley-Liss, Inc.
Affiliation:
Faculty of Medicine, Department of Pediatrics, Chiang Mai University, Chiang Mai, Thailand. tanpaiboon1@yahoo.com
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MeSH Terms
Descriptor/Qualifier:
Abnormalities, Multiple / genetics*
Blepharoptosis / genetics
Child
DNA Mutational Analysis
Eyelashes / abnormalities
Forkhead Transcription Factors / genetics*
Frameshift Mutation
Genes, Dominant
Humans
Lingual Frenum / abnormalities
Lymphedema / genetics*,  radiography
Male
Mutagenesis, Insertional*
Pierre Robin Syndrome / genetics*
Syndrome
Thailand
Chemical
Reg. No./Substance:
0/Forkhead Transcription Factors; 0/mesenchyme fork head 1 protein

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


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