ERIC Number: EJ821494
Record Type: Journal
Publication Date: 2009-Jan
Abstractor: As Provided
Reference Count: 38
Cancer Incidence among Persons with Fragile X Syndrome in Finland: A Population-Based Study
Sund, Reijo; Pukkala, E.; Patja, K.
Journal of Intellectual Disability Research, v53 n1 p85-90 Jan 2009
Background: Fragile X syndrome is a common inheritable cause of intellectual disability (ID) and is characterised by a large number of CGG repeats at the gene "FMR1" located on the X-chromosome. It has been reported that this genetic mechanism may protect against malignant transformations. Methods: We extracted from the Finnish registry on persons with ID a cohort of 302 persons with a fragile X diagnosis during 1982-1986. Follow-up for cancer incidence was performed in the Finnish Cancer Registry until the end of the year 2005. Results: There were 11 reported cancers during the mean follow-up of 21.4 years per person. The expected number of cancers based on the average Finnish population was 13.8 and no statistically significant protective effect was detected [standardised incidence ratios (SIR) 0.80, confidence interval (CI) 95% 0.40-1.4]. An increased risk for lip cancer was found (SIR 23, CI 95% 2.8-85). Conclusions: Confirmation of hypotheses about the mechanisms linking FXS and cancer needs further research.
Descriptors: Incidence, Mental Retardation, Cancer, Foreign Countries, Genetics, Clinical Diagnosis, Risk, Statistical Analysis
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Publication Type: Journal Articles; Reports - Research
Education Level: N/A
Authoring Institution: N/A
Identifiers - Location: Finland