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Tay–Sachs disease, which can present as a fatal illness of children that causes mental deterioration prior to death, was historically extremely common among Ashkenazi Jews, [19] with lower levels of the disease in some Pennsylvania Dutch, Italian, Irish Catholic, and French Canadian descent, especially those living in the Cajun community of ...
Ashkenazi Jews have a high incidence of Tay–Sachs and other lipid storage diseases. In the United States, about 1 in 27 to 1 in 30 Ashkenazi Jews is a recessive carrier. The disease incidence is about 1 in every 3,500 newborn among Ashkenazi Jews. [39]
Ashkenazi Jews have been screened as Tay–Sachs carriers since carrier testing began in 1971. Since the 1970s, many Jewish communities have embraced genetic screening, and in 1971, Israel became the first country to offer free genetic screening [1] and counseling for Tay–Sachs disease and other diseases, leading to international discussion about the proper scope of genetic testing.
Taken together, sphingolipidoses have an incidence of approximately 1 in 10,000, but substantially more in certain populations such as Ashkenazi Jews. Enzyme replacement therapy is available to treat mainly Fabry disease and Gaucher disease, and people with these types of sphingolipidoses may live well into adulthood. The other types are ...
Researchers suggest their study challenges the previous view that disease-related variants associated with Ashkenazi Jewish populations only became more common in the past 600 years.
In both the Ashkenazi and Sephardi Jewish communities, there is an increased rate of a number of genetic disorders such as Tay–Sachs disease, an autosomal recessive disorder that goes unnoticed in carriers, but is fatal within the first few years of life in almost all homozygotes.
Tay–Sachs disease, an autosomal recessive disorder most common among Ashkenazi Jews, French Canadians of Saguenay–Lac-Saint-Jean, Cajuns of Louisiana and Old Order Amish of Pennsylvania [48] [49] Hereditary hemochromatosis, most common among persons having Northern European ancestry, in particular those people of Celtic descent
Hackers have compiled a giant apparent list of people with Ashkenazi Jewish ancestry after taking that information from the genetic testing service 23andMe, which is now being shared on the internet.