title: Enzyme-substitution therapy with the yeast Saccharomyces cerevisiae in congenital sucrase-isomaltase deficiency.
abstract: Sucrase-isomaltase deficiency is an inherited disaccharidase deficiency that leads to malabsorption of sucrose, with resulting diarrhea and abdominal distention and cramps. We investigated the sucrose-splitting effect of viable yeast cells in eight children with congenital sucrase-isomaltase deficiency, by means of the sucrose hydrogen breath test. This test is based on the fact that hydrogen is released from the malabsorbed sucrose by the colonic microflora. We found that 0.3 g of lyophilized Saccharomyces cerevisiae, given after loading with 2 g of sucrose per kilogram of body weight, reduced hydrogen excretion in all patients, on average by 70 percent, in parallel with a complete loss or evident reduction of clinical symptoms. In vitro, lyophilized and fresh S. cerevisiae (fresh baker's yeast) had appreciable sucrase activity, a low isomaltase and maltase activity, and virtually no lactase activity. The sucrase activity was more inhibited by undiluted than by diluted gastric juice. We conclude that patients with congenital sucrase-isomaltase deficiency who intentionally or unintentionally consume sucrose can ameliorate the malabsorption by subsequently ingesting a small amount of viable yeast cells, preferably on a full stomach.
mesh_terms: D000293:Adolescent; D000328:Adult; D001944:Breath Tests; D002239:Carbohydrate Metabolism, Inborn Errors / Q000628:therapy*; D002648:Child; D002675:Child, Preschool; D005260:Female; D005612:Freeze Drying; D006801:Humans; D006859:Hydrogen / Q000032:analysis; D007223:Infant; D007787:Lactose Intolerance / Q000378:metabolism; D008297:Male; D009097:Multienzyme Complexes / Q000172:deficiency*; D012441:Saccharomyces cerevisiae / Q000201:enzymology*; D013394:Sucrase-Isomaltase Complex / Q000172:deficiency*
chemical_list: D009097:Multienzyme Complexes; D006859:Hydrogen; D013394:Sucrase-Isomaltase Complex