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- W2116721670 abstract "ABSTRACT – From 1902 to 1983, 68 cases of hepatolenticular degeneration (HLD) were discovered in Sardinia, with a mean frequency, in reference to number of live births, of 27.7 and a sex ratio of 1.83. The prevalence of the disease was seen to be higher over the last few decades. With regard to the geographic distribution of the disease, 3 high-frequency areas were evident, in Barbagia, in Campidano, and in the area surrounding the city of Sassari. In 38.23% of cases, the clinical picture was of hepatoneurologic type; hepatic forms have become more frequent over the last decades. The first symptoms were observed at mean age of 15 years 8 months. The number of asymptomatic cases was fairly consistent (22.05%). The median survival rate in subjects who received inadequate therapy was 6 years 4 months. Only 3 patients of the 45 treated with adequate therapy died. The gene frequency, calculated by the application of Dahlberg's formula, was extremely high." @default.
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- W2116721670 date "2009-01-29" @default.
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- W2116721670 title "Epidemiologic study of hepatolenticular degeneration (Wilson's disease) in Sardinia (1902-1983)" @default.
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- W2116721670 doi "https://doi.org/10.1111/j.1600-0404.1985.tb01546.x" @default.
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