Purchase this article with an account.
E. L. Hamblion, A. T. Moore, J. S. Rahi; Incidence of Childhood Onset Hereditary Retinal Disorders in the UK; Variation by Age, Ethnicity and Socioeconomic Status. Invest. Ophthalmol. Vis. Sci. 2009;50(13):4690.
Download citation file:
© ARVO (1962-2015); The Authors (2016-present)
To provide currently unavailable information on the burden of childhood onset hereditary retinal disorders within the UK, focussing on variations by age, ethnicity, and socioeconomic group. This is essential epidemiological information for evaluating and monitoring trends and planning future treatment programmes.
National active surveillance through the British Ophthalmological Surveillance Unit for 18 months (starting August 2006). Ophthalmologists reported any child newly diagnosed as having a stationary or progressive hereditary retinal disorder, irrespective of level of visual function and whether the condition was isolated or part of a systemic disorder.Clinical and sociodemographic data on each child were collected at notification and 9 months later using specifically developed questionnaires.
261 patients were reported with 27 distinct diagnoses, 21% diagnosed by 2 years of age. Cumulative incidence by age 16 was 2.5 (95% CI 2.38-2.63) /10,000. Asian compared to white ethnicity was associated with an increased risk of having a retinal disorder by age 16, Relative risk = 5.1 (95%CI 4.18-6.14). 27% of the children in the study were of Asian ethnicity compared with 6% of the UK under 16 population being Asian. Those in the lowest socio-economic quintile were over represented (30% in the study compared with 20% in the national population, p value < 0.05).
The incidence of childhood onset hereditary retinal disorders in the UK is higher than anticipated. Hereditary retinal disorders are more common in those of Asian ethnicity and those from more deprived backgrounds. These results have implications for the planning of service provision and of future treatment programmes as novel therapies become accessible.
This PDF is available to Subscribers Only