XXXX in Israel.

@entity1 @entity74 @entity13785
Eighteen @entity1 with @entity13785 have been diagnosed in Israel during the last 15 years. All @entity1 were Palestinian Arabs, with the exception of two siblings from a Jewish Iraqi family. Clinically all @entity1 had the infantile type ( @entity13785 ), except one who had the juvenile type. Muscle glycogen content varied from 4 to 17% wet weight. Muscle alpha-glucosidase activity was zero in 10 of 17 @entity1 examined. Among the seven @entity1 in whom residual activity was present, the highest value was 18% of normal. Leukocyte alpha-glucosidase activity was highly variable, making this tissue unfit for enzymatic diagnosis of the disease. A marked heterogeneity was found in pH profiles of muscle and leukocyte alpha-glucosidase activity. A high prevalence of the disease in the Arab population was noted. In spite of a high rate of consanguinity, only a small number of autosomal recessively @entity74 have been shown to be unusually prevalent in the Arab population. In view of the serious prognosis of this disease, prenatal diagnosis should be offered to affected families.
