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Adult onset sucrase-isomaltase deficiency with secondary disaccharidase deficiency resulting from severe dietary carbohydrate restriction

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Summary

A 40-year-old man with sucrase-isomaltase deficiency, diagnosed 14 years previously, presented with troublesome diarrhea. Brush-border alkaline phosphatase and all disaccharidases, except lactase, were significantly depressed. Dietary assessment revealed severe self-imposed carbohydrate restriction to about 25 g/day. A 150-g carbohydrate diet with restricted starch and no sucrose over nine months resulted in normal trehalase and alkaline phosphatase activities, and maltase activity improved to half normal. Sucrase and isomaltase remained depressed and did not change after three weeks of sucrose 50 g/day. It is concluded that a self-imposed therapeutic diet of severe carbohydrate restriction resulted in secondary brush-border enzyme deficiencies.

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Cooper, B.T., Scott, J., Hopkins, J. et al. Adult onset sucrase-isomaltase deficiency with secondary disaccharidase deficiency resulting from severe dietary carbohydrate restriction. Digest Dis Sci 28, 473–477 (1983). https://doi.org/10.1007/BF02430538

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  • DOI: https://doi.org/10.1007/BF02430538

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