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Department of Human Genetics, Hadassah University Hospital, Jerusalem, Israel.
Over a period of 15 years, 18 infants affected with Krabbe disease were diagnosed in Israel. None of the patients were Jews. Six were Druze from a large kindred in which a very high incidence of the disease was previously reported. The 12 other patients were Moslem Arabs; 7 were from two adjacent villages and most of them were found to be related, originating from a large kindred in which the incidence of the disease is 1/130 live births. Three other patients were from a third large kindred that also had a high incidence of Krabbe disease. Israel's population includes communities in which a high incidence of lysosomal diseases has been found. The incidence of these diseases in Israel may be reduced by delineating the populations at risk. With respect to Krabbe disease, as reported here, the tests for heterozygote detection are not sufficiently accurate for a screening of the populations at risk. Prevention is possible only by prenatal diagnosis in the families at risk.
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