Open Access13 C‐Labeled‐Starch Breath Test in Congenital Sucrase‐isomaltase Deficiency
Author(s) -
RobayoTorres Claudia C.,
DiazSotomayor Marisela,
Hamaker Bruce R.,
Baker Susan S.,
Chumpitazi Bruno P.,
Opekun Antone R.,
Nichols Buford L.
Publication year - 2018
Publication title -
journal of pediatric gastroenterology and nutrition
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.206
H-Index - 131
eISSN - 1536-4801
pISSN - 0277-2116
DOI - 10.1097/mpg.0000000000001858
Subject(s) - sucrase , maltase , starch , digestion (alchemy) , endocrinology , medicine , amylase , carbohydrate , biochemistry , carbohydrate metabolism , enzyme , chemistry , chromatography
Background and Hypotheses: Human starch digestion is a multienzyme process involving 6 different enzymes: salivary and pancreatic α‐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 13 C‐breath test (BT). Methods: 13 C‐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 13 C‐sucrose BT. Breath 13 CO 2 was quantitated at intervals before and after serial 13 C‐substrate loads (glucose followed 75 minutes later by starch). Variations in metabolism were normalized against 13 C‐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. Results: Children with CSID had a significant reduction of 13 C‐starch digestion mirroring that of their duodenal sucrase and maltase activity and 13 C‐sucrase BT. Conclusions: In children with CSID, starch digestion may be impaired. In children with CSID, starch digestion correlates well with measures of sucrase activity.