Abstract
Extract: Ten patients affected by congenital deficiency of intestinal sucrase and isomaltase activities were studied. The intraluminal α-amylolysis of amylopectin was found to be normal in these patients. It is incomplete during the 1st year of life, both in patients and in controls, as a consequence of the lower α-amylase activity in this age group.
Maltotriose was well tolerated in two normal controls ages 32 and 3 months and in one 31-month-old patient: in these subjects an oral tolerance test with the trisaccharide was followed by a rapid increase of blood glucose (46–58 mg/100 ml) and did not cause diarrhea. However, in a 6-month-old patient fermentative diarrhea and low increase of blood glucose (24 mg/100 ml) followed oral load with maltotriose. In all patients, enzyme assays on intestinal biopsy specimens showed that the glucamylase activity of the heat-resistant maltase(s) was normal, while that of the sucrase-iso-maltase complex was decreased.
These results suggest that starch malabsorption in congenital sucrose and isomaltose intolerance results from deficiency of the enzymatic activities of the sucrase-isomaltase complex involved in starch digestion.
Speculation: Malabsorption of starch can be expected to result from: (1) deficiency of lpancreatic amylase; (3) glucose malabsorption; (4) higher intestinal flow rate. The results in this study show that one particular form of starch malabsorption is a consequence of congential deficiency of the activities of the sucrase-isomaltase complex involved in starch digestion. The methods used here should prove to be useful also in the investigation of other forms of starch malabsorption.
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Aurigchio, S., Ciccimarra, F., Moauro, L. et al. Intraluminal and Mucosal Starch Digestion in Congenital Deficiency of Intestinal Sucrase and Isomaltase Activities. Pediatr Res 6, 832–839 (1972). https://doi.org/10.1203/00006450-197211000-00004
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DOI: https://doi.org/10.1203/00006450-197211000-00004