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Enzyme-substitution therapy with the yeast Saccharomyces cerevisiae in congenital sucrase-isomaltase deficiency.
N Engl J Med. 1987 May 21; 316(21):1306-9.NEJM

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.

Authors

No affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article
Research Support, Non-U.S. Gov't

Language

eng

PubMed ID

3553946

Citation

Harms, H K., et al. "Enzyme-substitution Therapy With the Yeast Saccharomyces Cerevisiae in Congenital Sucrase-isomaltase Deficiency." The New England Journal of Medicine, vol. 316, no. 21, 1987, pp. 1306-9.
Harms HK, Bertele-Harms RM, Bruer-Kleis D. Enzyme-substitution therapy with the yeast Saccharomyces cerevisiae in congenital sucrase-isomaltase deficiency. N Engl J Med. 1987;316(21):1306-9.
Harms, H. K., Bertele-Harms, R. M., & Bruer-Kleis, D. (1987). Enzyme-substitution therapy with the yeast Saccharomyces cerevisiae in congenital sucrase-isomaltase deficiency. The New England Journal of Medicine, 316(21), 1306-9.
Harms HK, Bertele-Harms RM, Bruer-Kleis D. Enzyme-substitution Therapy With the Yeast Saccharomyces Cerevisiae in Congenital Sucrase-isomaltase Deficiency. N Engl J Med. 1987 May 21;316(21):1306-9. PubMed PMID: 3553946.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Enzyme-substitution therapy with the yeast Saccharomyces cerevisiae in congenital sucrase-isomaltase deficiency. AU - Harms,H K, AU - Bertele-Harms,R M, AU - Bruer-Kleis,D, PY - 1987/5/21/pubmed PY - 1987/5/21/medline PY - 1987/5/21/entrez SP - 1306 EP - 9 JF - The New England journal of medicine JO - N. Engl. J. Med. VL - 316 IS - 21 N2 - 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. SN - 0028-4793 UR - https://www.unboundmedicine.com/medline/citation/3553946/Enzyme_substitution_therapy_with_the_yeast_Saccharomyces_cerevisiae_in_congenital_sucrase_isomaltase_deficiency_ L2 - http://www.nejm.org/doi/full/10.1056/NEJM198705213162104?url_ver=Z39.88-2003&rfr_id=ori:rid:crossref.org&rfr_dat=cr_pub=pubmed DB - PRIME DP - Unbound Medicine ER -