Objective: To describe four infants with protracted diarrhea caused by glucose polymer intolerance resulting from congenital sucrase-isomaltase deficiency.
Methods: The diagnosis of congenital sucrase-isomaltase deficiency was established by mucosal disaccharidase assay. In each case the clinical response to discontinuation of glucose polymer was documented.
Results: The median age at the onset of symptoms was 3 weeks (range, 2 to 16 weeks). In three infants the formula had been prescribed for presumed postgastroenteritis diarrhea, and in a fourth it was begun after resection of a short-segment congenital ileal atresia. In each infant watery diarrhea occurred and persisted for many months, and it was assumed that the original gastrointestinal disorder was responsible. In two cases, parenteral nutrition was administered for persistent failure to thrive. Ultimately, investigation revealed the underlying congenital sucrase-isomaltase deficiency, and elimination of glucose polymer from the diet led to immediate recovery in each case.
Conclusion: Congenital sucrase-isomaltase deficiency should be considered a possible cause of protracted diarrhea in infants receiving glucose polymer-based feedings.