An epidemiological study of Wilson's disease in the Republic of Ireland. Issue 3 (March 1993)

• Record Type: Journal Article
• Authors: Reilly, M; Daly, L; Hutchinson, M
• Is Part Of: Journal of neurology, neurosurgery and psychiatry. Volume 56:Issue 3(1993)
• Page Start: 298
• Page End: 300
• Publication Date: 1993-03
• Subjects: Neurology -- Periodicals; Nervous system -- Surgery -- Periodicals; Psychiatry -- Periodicals; 616.8
• DOI: 10.1136/jnnp.56.3.298
• Languages: English
• ISSNs: 0022-3050

Abstract

Abstract : In a population based study of the prevalence of Wilson's disease in the Republic of Ireland from 1970-89, 26 definite and probable cases were ascertained. The adjusted birth incidence rate was 17.0 per million live births (95% (confidence interval 9.9 to 27.2)) for the 20 year period 1950-69. The gene frequency was 0.41% (95% (confidence interval 0.31% to 0.52%) indicating that one in 122 of the population was a gene carrier. Allowing for a maximal degree of consanguinity, the gene frequency is reduced to 0.36% and the proportion of heterozygotes to one in 139 of the population. The consistency of the adjusted birth incidence rates over the decades 1950-59 and 1960-69 suggests a high degree of ascertainment of diagnosed cases of Wilson's disease.