To determine the prevalence of short
polymers of
glucose and
starch malabsorption caused by small intestinal
glucoamylase deficiency in children with chronic
diarrhea, we studied small bowel biopsy specimens from 511 children (aged 1 month to 9 years) with chronic
diarrhea evaluated at 54 medical centers.
Glucoamylase and
disaccharidase (
lactase,
sucrase,
maltase, and
palatinase)
enzyme assays were performed. Of the 511 children, 15 had
glucoamylase deficiency. Six who had significant small intestinal mucosal injury and
disaccharidase deficiencies were defined as having secondary
glucoamylase deficiency; the other nine patients with normal mucosal morphologic features were defined as having primary
glucoamylase deficiency.
Secretin tests showed normal pancreatic
amylase values for age in all seven children tested. Four of them had abnormal findings on tolerance tests for
starch and short
polymers of
glucose (rise in
blood glucose concentration: < 20 mg/dl) and reducing substances in stools, and three of these four had symptoms of intolerance (abdominal distention,
flatulence, and
diarrhea). All seven patients responded to a
starch elimination diet. After reintroduction of a
starch diet,
diarrhea recurred in four patients; this was alleviated 48 hours after reelimination of
starch. We conclude that intestinal
glucoamylase deficiency is present in some patients with chronic
diarrhea.