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A newly discovered large concentration of familial amyloid polyneuropathy (FAP) in Japan is clinically outlined. The importance of the urinary secretion of IgA in these cases is stressed. Dimethyl sulfoxide administration to these patients induced clinical improvements accompanied by increased excretion of low molecular weight proteins. Amino acid composition of amyloid protein of the Ogawa village-type FAP cases was similar to that of primary amyloidosis.

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