Document Detail

Coeliac disease in Williams syndrome.
MedLine Citation:
PMID:  11694549     Owner:  NLM     Status:  MEDLINE    
BACKGROUND: Coeliac disease (CD) has been reported in several patients affected by chromosomal disorders, including Down syndrome (DS) and Turner syndrome (TS). CD has also been found in sporadic Williams syndrome (WS) patients. In this study, CD was evaluated in a consecutive series of patients with WS, in order to estimate if the prevalence of CD in WS patients is higher than in the general population. METHODS AND RESULTS: A consecutive series of 63 Italian patients with WS was studied by analysing the dosage of antigliadin antibodies (AGA) IgA and antiendomisium antibodies (AEA). In patients with positive AGA and AEA, small bowel biopsy was performed. The prevalence of CD in our WS population was compared with that estimated in a published series of 17 201 Italian students. Seven WS patients were found to be positive for AGA IgA and AEA. Six of them underwent small bowel biopsy, which invariably disclosed villous atrophy consistent with CD. The prevalence of CD in the present series of WS patients was 9.5% (6/63), compared to 0.54% (1/184) in the Italian students (p<0.001). CONCLUSION: The present results suggest that the prevalence of CD in WS is higher than in the general population and is comparable to that reported in DS and TS. AGA and AEA screening is recommended in patients with WS.
A Giannotti; G Tiberio; M Castro; F Virgilii; F Colistro; F Ferretti; M C Digilio; M Gambarara; B Dallapiccola
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Journal of medical genetics     Volume:  38     ISSN:  1468-6244     ISO Abbreviation:  J. Med. Genet.     Publication Date:  2001 Nov 
Date Detail:
Created Date:  2001-11-05     Completed Date:  2002-02-04     Revised Date:  2008-11-20    
Medline Journal Info:
Nlm Unique ID:  2985087R     Medline TA:  J Med Genet     Country:  England    
Other Details:
Languages:  eng     Pagination:  767-8     Citation Subset:  IM    
Servizio di Genetica Medica, Ospedale Bambino Gesù, IRCCS, Piazza S Onofrio 4, 00165, Roma, Italy.
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MeSH Terms
Celiac Disease / blood,  complications,  pathology*
Child, Preschool
Gliadin / immunology
Immunoglobulin A / blood
Immunoglobulin E / blood
Williams Syndrome / blood,  complications,  pathology*
Reg. No./Substance:
0/Immunoglobulin A; 37341-29-0/Immunoglobulin E; 9007-90-3/Gliadin

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