Document Detail


Haddad syndrome: a case of an infant with central congenital hypoventilation syndrome and Hirschsprung disease.
MedLine Citation:
PMID:  18230845     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
This report presents an otherwise healthy infant who developed unexplained apnea and long-segment Hirschsprung disease. After extensive evaluation that included a paired-like homeobox 2b gene (PHOX2B) analysis, he was found to have Haddad syndrome, a congenital disorder that features central congenital hypoventilation syndrome in conjunction with Haddad syndrome. Recent work has associated polyalanine repeats within the PHOX2B gene on chromosome 4p12 with central congenital hypoventilation syndrome, whereas PHOX2B knockout mice develop aganglionic bowels.
Authors:
Daniel Lai; Brian Schroer
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Publication Detail:
Type:  Case Reports; Journal Article     Date:  2008-01-29
Journal Detail:
Title:  Journal of child neurology     Volume:  23     ISSN:  0883-0738     ISO Abbreviation:  J. Child Neurol.     Publication Date:  2008 Mar 
Date Detail:
Created Date:  2008-02-28     Completed Date:  2008-04-01     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  8606714     Medline TA:  J Child Neurol     Country:  Canada    
Other Details:
Languages:  eng     Pagination:  341-3     Citation Subset:  IM    
Affiliation:
College of Medicine, Ohio State University, Columbus, OH, USA. Lai.132@osu.edu
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MeSH Terms
Descriptor/Qualifier:
Abnormalities, Multiple / genetics*
Apnea / complications,  genetics*,  therapy
DNA Repeat Expansion
Hirschsprung Disease / complications,  genetics*
Homeodomain Proteins / genetics*
Humans
Hypoventilation / complications,  congenital,  genetics*
Infant
Infant, Newborn
Intestine, Large / abnormalities,  pathology
Male
Syndrome
Transcription Factors / genetics*
Chemical
Reg. No./Substance:
0/Homeodomain Proteins; 0/NBPhox protein; 0/Transcription Factors

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


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