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• W4308789264 abstract "To determine Sturge-Weber syndrome (SWS) incidence and secondary glaucoma risk.Nationwide retrospective cohort study.The Korean National Health Insurance (NHI) claims database from 2002 to 2019 along with the registration-program database for rare intractable diseases (ie, the rare disease registry) were accessed to identify ophthalmologist/neurologist-confirmed SWS patients. SWS incidence was estimated in a same-birth-year population (ie, a birth cohort) from 2002 to 2009. Among the SWS patients born between 2002 and 2019, the incidence of SWS-associated glaucoma was estimated.During the 18-year observational period, a total of 1049 patients were registered as SWS. The mean birth-cohort SWS incidence was 3.08 (95% CI 2.52-3.64) per 100 000 people per year, with an approximate female-to-male ratio of 0.97:1. Among the 217 SWS patients born between 2002 and 2019, secondary glaucoma arose in 18 (8.3%) cases, including 12 males (66.7%). Among these 18 SWS-associated glaucoma cases, 15 (83.3%) were diagnosed before 1 year of age, and the other 3 (16.7%) between age 1 and 2 years. Among the 660 SWS patients under age 40 years during the study period, SWS-associated glaucoma was identified in 79 (12.0%) cases. Neurologic manifestations such as epilepsy, hemiparesis, and mental retardation did not significantly differ between SWS patients with and those without secondary glaucoma.This study identified birth-cohort SWS incidence and determined secondary-glaucoma risk in a population of East Asian ethnicity. These data could help to promote better understanding of the epidemiologic features of SWS patients." @default.
• W4308789264 created "2022-11-15" @default.
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• W4308789264 date "2023-03-01" @default.
• W4308789264 modified "2023-10-18" @default.
• W4308789264 title "Incidence of Sturge-Weber Syndrome and Risk of Secondary Glaucoma: A Nationwide Population-based Study Using a Rare Disease Registry" @default.
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• W4308789264 doi "https://doi.org/10.1016/j.ajo.2022.11.009" @default.
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