Clinical, demographic, and genetic aspects of the Werner syndrome in Japan.

A total of 196 typical patients with the Werner syndrome, a caricature of aging, was studied to define the details of the clinical manifestations, demography and genetic aspects in Japan. Most of the previously reported clinical characteristics and the autosomal recessive inheritance of this syndrome were confirmed, but no significant linkage was revealed between specific HLA type and the Werner syndrome. The frequency of the Werner syndrome in Japan was estimated using two methods which indicated approximately 300 cases among 100 million people.