Document Detail


Brachmann-de Lange syndrome: autosomal dominant inheritance and male-to-male transmission.
MedLine Citation:
PMID:  8989466     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
We report on familial occurrence of the Brachmann-de Lange syndrome (BDLS): a mildly affected father and his severely affected son and daughter who have different mothers. Both children are severely affected while the father has a much milder but definite BDLS phenotype. Our report documents the third example of male-to-male transmission and adds to the argument against exclusively maternal transmission in familial cases. In addition, our findings illustrate the occurrence of severe manifestations in cases of familial BDLS.
Authors:
R R McKenney; F F Elder; J Garcia; H Northrup
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  American journal of medical genetics     Volume:  66     ISSN:  0148-7299     ISO Abbreviation:  Am. J. Med. Genet.     Publication Date:  1996 Dec 
Date Detail:
Created Date:  1997-03-11     Completed Date:  1997-03-11     Revised Date:  2004-11-17    
Medline Journal Info:
Nlm Unique ID:  7708900     Medline TA:  Am J Med Genet     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  449-52     Citation Subset:  IM    
Affiliation:
Graduate School of Biomedical Sciences, University of Texas, Health Science Center, Houston 77030, USA.
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MeSH Terms
Descriptor/Qualifier:
Adult
Child, Preschool
De Lange Syndrome / genetics*,  pathology
Failure to Thrive
Fatal Outcome
Female
Genes, Dominant*
Humans
Infant, Newborn
Karyotyping
Male
Phenotype

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


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