Abstract
Intractable diarrhea of infancy is a heterogeneous group of disorders resulting in protracted diarrhea. Secondary carbohydrate intolerance plays an important role in this regard. These are usually related to lactase and sucrase deficiency. More recently, glucoamylase deficiency has been described. In all of the above instances, a marked enteritis was present. We report 2 patients who continued to have diarrhea, with positive reducing substances in the stool, while on diets with glucose polymers as the sole CHO source. These patients aged 4 & 9 months, presented with diarrhea of 1 and 4 months duration. Jejunal biopsies were normal under light microscopy and on EM. Despite minimal morphological changes, both patients had a marked decrease in lactase, sucrase, maltase, and glucoamylase activity in jejunal biopsy specimens. Duodenal fluid, amylase, and trypsin were normal. Formulae (RCF & 3232A), with glucose monosaccharide as the CHO source, alleviated the diarrhea. Conclusions: 1. Glucoamylase deficiency is a significant clinical entity and is important in perpetuating diarrhea in these patients. 2. Light microscopy of small intestinal biopsy may be normal. 3. Formulae containing glucose and fructose monosaccharides as the CHO source alleviate the diarrhea.
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Rome, J., Katz, A. & Flores, A. PROTRACTED DIARRHEA OF INFANCY: THE IMPORTANCE OF GLUCOAMYLASE DEFICIENCY. Pediatr Res 18 (Suppl 4), 210 (1984). https://doi.org/10.1203/00006450-198404001-00702
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DOI: https://doi.org/10.1203/00006450-198404001-00702