Prevalence of fragile-X syndrome and FRAXE among children with intellectual disability in a Caribbean island, Guadeloupe, French West Indies.
Expect fragile-X in about 1 in 15 Afro-Caribbean boys with moderate-severe ID, so test early.
01Research in Context
What this study did
Doctors in Guadeloupe tested children who had moderate or severe intellectual disability. They wanted to know how many had fragile-X syndrome.
They checked both boys and girls, but only boys tested positive.
What they found
About 1 in every 15 boys with ID had fragile-X. No girls with ID had it.
No one in the group had the related FRAXE condition.
How this fits with other research
Cryan et al. (1996) tracked how boys with fragile-X learn daily living skills. Their work helps you picture what the Guadeloupe boys may need next.
Plant et al. (2007) and Dougherty et al. (1996) counted ID in the Netherlands and Sydney. They looked at all causes, while A et al. zoomed in on fragile-X inside ID.
Belmonte et al. (2008) later bundled many studies like this one into a big review. The 1998 numbers still sit inside that wider story.
Why it matters
If you assess Afro-Caribbean boys with unexplained moderate-severe ID, think fragile-X. Order the DNA test early. Knowing the cause lets you write clearer goals, teach families what to expect, and connect them to fragile-X clinics and support groups.
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02At a glance
03Original abstract
Fragile-X syndrome (FXS) is the most common cause of inherited intellectual disability. Although FXS has been identified in all the main ethnic groups, little is known about its prevalence with respect to ethnicity. Since the identification of the FXS primary defect, diagnosis involving DNA analysis has been made possible, allowing efficient screening strategies to be considered. The present authors have carried out FXS screening among children belonging mainly to the Afro-Caribbean ethnic group (163 boys and 85 girls) affected with moderate to severe intellectual disability of previously unknown origin. We have found a 6.7% and 0% prevalence among boys and girls, respectively, yielding a minimum FXS incidence of 0.42 per 1000 male births per year. Family studies have resulted in genetic counselling for several individuals. FRAXE screening was also achieved and no FRAXE case was detected in this study.
Journal of intellectual disability research : JIDR, 1998 · doi:10.1046/j.1365-2788.1998.00064.x