Document Detail


Bardet-biedl syndrome and brain abnormalities.
MedLine Citation:
PMID:  17607597     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Bardet-Biedl syndrome (BBS) is a rare autosomal recessive disorder with clinical and genetic heterogeneity. The main features are obesity, polydactyly, pigmentary retinopathy, learning disabilities, hypogonadism, and renal abnormalities. To date, eleven genes have been cloned but there is still little knowledge about genotype/phenotype correlations. We describe three additional cases with BBS and cerebral abnormalities and focus on cerebellar abnormalities in BBS.
Authors:
C Rooryck; S Pelras; J-F Chateil; C Cances; B Arveiler; A Verloes; D Lacombe; C Goizet
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Neuropediatrics     Volume:  38     ISSN:  0174-304X     ISO Abbreviation:  Neuropediatrics     Publication Date:  2007 Feb 
Date Detail:
Created Date:  2007-07-03     Completed Date:  2007-10-25     Revised Date:  2008-01-16    
Medline Journal Info:
Nlm Unique ID:  8101187     Medline TA:  Neuropediatrics     Country:  Germany    
Other Details:
Languages:  eng     Pagination:  5-9     Citation Subset:  IM    
Affiliation:
Service de Génétique Médicale, CHU Pellegrin-Enfants, Bordeaux, France.
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MeSH Terms
Descriptor/Qualifier:
Bardet-Biedl Syndrome / genetics,  pathology*
Cerebellum / abnormalities*
Child
Child, Preschool
Female
Humans
Infant
Infant, Newborn
Male

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