Autosomal recessive muscular dystrophy in Manitoba Hutterites

MHK Shokeir, NL Kobrinsky - Clinical Genetics, 1976 - Wiley Online Library
MHK Shokeir, NL Kobrinsky
Clinical Genetics, 1976Wiley Online Library
A slowly progressive type of muscular dystrophy affecting 11 known members of several
Southern Manitoba Hutterite colonies is described. Though encompassing the facial
characteristics of the facio‐scapulo‐humeral type and the proximal distribution of the limb‐
girdle type, it was felt that this disease represents a distinct type of muscular dystrophy with
autosomal recessive inheritance. Since all “affected” colonies can be traced to one founding
colony in South Dakota, the disease may have been introduced from Europe between 1874 …
A slowly progressive type of muscular dystrophy affecting 11 known members of several Southern Manitoba Hutterite colonies is described. Though encompassing the facial characteristics of the facio‐scapulo‐humeral type and the proximal distribution of the limb‐girdle type, it was felt that this disease represents a distinct type of muscular dystrophy with autosomal recessive inheritance.
Since all “affected” colonies can be traced to one founding colony in South Dakota, the disease may have been introduced from Europe between 1874 and 1879. Furthermore, normal fertility and a high degree of inbreeding in a genetically isolated population have contributed to the maintenance of the disease in the population.
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