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Title: 13C-labeled-starch breath test in congenital sucrase-isomaltase deficiency

Author
item ROBAYO-TORRES, CLAUDIA - Children'S Nutrition Research Center (CNRC)
item DIAZ-SOTOMAYOR, MARISELA - Children'S Nutrition Research Center (CNRC)
item HAMAKER, BRUCE - Purdue University
item BAKER, SUSAN - University Of Buffalo
item CHUMPITAZI, BRUNO - Baylor College Of Medicine
item OPEKUN, ANTONE - Baylor College Of Medicine
item NICHOLS, BUFORD - Children'S Nutrition Research Center (CNRC)

Submitted to: Journal of Pediatric Gastroenterology and Nutrition
Publication Type: Peer Reviewed Journal
Publication Acceptance Date: 11/2/2017
Publication Date: 6/1/2018
Citation: Robayo-Torres, C.C., Diaz-Sotomayor, M.M., Hamaker, B.R., Baker, S.S., Chumpitazi, B.P., Opekun, A.R., Nichols, B.L. 2018. 13C-labeled-starch breath test in congenital sucrase-isomaltase deficiency. Journal of Pediatric Gastroenterology and Nutrition. 66:S61-S64. https://doi.org/10.1097/MPG.0000000000001858.
DOI: https://doi.org/10.1097/MPG.0000000000001858

Interpretive Summary: Human starch digestion is a multienzyme process involving 6 different enzymes: salivary and pancreatic a-amylase; sucrase and isomaltase (from sucrose-isomaltase [SI]), and maltase and glucoamylase (from maltase-glucoamylase [MGAM]). Together these enzymes cleave starch to smaller absorbable glucose. Approximately 80% of all mucosal maltase activity is accounted for by SI and the reminder by MGAM. Studies suggest that starch may be poorly digested in those with congenital sucrase-isomaltase deficiency (CSID). Poor starch digestion in CSID was documented using a noninvasive 13C-breath test. We have demonstrated in CSID patients that all had low starch digestion. Our hypothesis that SI deficiency results in decreased digestion starches was confirmed. These results will aid other researchers focusing on starch digestion.

Technical Abstract: Human starch digestion is a multienzyme process involving 6 different enzymes: salivary and pancreatic a-amylase; sucrase and isomaltase (from sucrose-isomaltase [SI]), and maltase and glucoamylase (from maltase-glucoamylase [MGAM]). Together these enzymes cleave starch to smaller molecules ultimately resulting in the absorbable monosaccharide glucose. Approximately 80% of all mucosal maltase activity is accounted for by SI and the reminder by MGAM. Clinical studies suggest that starch may be poorly digested in those with congenital sucrase-isomaltase deficiency (CSID). Poor starch digestion occurs in individuals with CSID and can be documented using a noninvasive 13C-breath test (BT). 13C-Labled starch was used as a test BT substrate in children with CSID. Sucrase deficiency was previously documented in study subjects by both duodenal biopsy enzyme assays and 13C-sucrose BT. Breath 13CO2 was quantitated at intervals before and after serial C-substrate loads (glucose followed 75 minutes later by starch). Variations in metabolism were normalized against 13C-glucose BT (coefficient of glucose absorption). Control subjects consisted of healthy family members and a group of children with functional abdominal pain with biopsy-proven sucrase sufficiency. Children with CSID had a significant reduction of 13C-starch digestion mirroring that of their duodenal sucrase and maltase activity and 13C-sucrase BT. In children with CSID, starch digestion may be impaired. In children with CSID, starch digestion correlates well with measures of sucrase activity.