ARS | Publication request: Carbohydrate digestion in congenital sucrase isomaltase deficient and recurrent abdominal pain children assesed by 13C- starch breath test

Submitted to: Gastroenterology
Publication Type: Abstract Only
Publication Acceptance Date: May 1, 2010
Publication Date: May 1, 2010
Citation: Robayo-Torres, C.C., Opekun, A.R., Quezada-Calvillo, R., Diaz-Sotomayor, M., Baker, S.S., Nichols, B.L. 2010. Carbohydrate digestion in congenital sucrase isomaltase deficient and recurrent abdominal pain children assesed by 13C- starch breath test [abstract]. Gastroenterology. 138(Suppl.1)S-139.

Technical Abstract: Starches contribute about half of the food energy needs to the weaned child's diet. Malabsorption of sucrose is associated with abdominal pain, bloating and diarrhea. A genetic disorder called Congenital Sucrase-Isomaltase Deficiency (CSID) is suspected when these symptoms follow sugar ingestion and are relieved by a sugar elimination diet. Diagnosis is made upon demonstration of very low or absent sucrase activities in duodenal biopsy enzyme assays with a normal morphology. Treatment is by sucrose elimination diet and oral enzyme supplementation with sacrosidase. Clinical reviews of CSID have suggested that some of these patients also have starch malabsorption. The presence of coexisting starch and sucrose intolerance makes childhood dietary management very difficult and reduces the value of oral sacrosidase supplements. Confirmation of starch intolerance is made difficult because of lack of a specific substrate in mucosal biopsy enzyme assays and the shared SI and MGAM contributions to both maltase and glucoamylase assays results. The aim of this study was to examine starch digestion in a series of children with CSID and a population with similar symptoms of recurrent abdominal pain. The study included eleven adult control subjects; eleven patients with CSID; and 10 RAP age matched patients in oral breath test using 20mg of UL-13C-substrates and periodic 13CO2 breath enrichment analyses normalized for % of glucose oxidation (CGO%) as previously reported. UL-13C-substrates (G & ST; 20 mg. each, Isotec, Miamisberg, OH) were given in 10 gm unlabeled glucose oligomers (10%) vehicle after an overnight fast and reference sampling on 2 separate days. Serial 13CO2 breath enrichments (every 15 min x 9) were assayed using a 13CO2 infrared spectrophotometer. A Coefficient of Glucose Oxidation (CGO) was calculated for breath enrichments to adjust for individual differences. Normal adult control subjects were used to define CGO lower reference levels (LL) defined as mean-1 SD (75 +/- 19 CGO%, LL 56. Because CGO were found to be relatively constant in the period of 60-90' after load these values were averaged for each individual. The normal controls had a LL value for starch of 56% CGO. Eight of ten RAP patients fell below control LL. Within the RAP group (46 +/- 24%CGO), the LL was of 20% and 1 had < 20%. CSID patients (19 +/- 11%CGO with 6 < 20% and 2 = 20%. We concluded that starch digestion by CSID patients was significantly below the 20% %CGO ST/G LL found in RAP patients (p=0.000) and both CSID and RAP populations failed significantly below the normal LL of 56 %CGO.