Disaccharidase activities in small intestinal mucosa in patients with cystic fibrosis.

The disaccharidase activities in small intestinal biopsies were related to the morphology of the mucosa and the ages of 63 patients with cystic fibrosis and 177 healthy control subjects of Caucasian origin. In patients with CF and in the healthy control subjects under 5 years of age with normal intestinal mucosa, no low lactase activity was found. In those patients with CF who were over 5 years of age, one group had high and one group had low lactase activity, as occurs in healthy Caucasian control subjects of the same age. This finding supports the view that in patients with CF, lactase deficiency is not related to the disease entity. In patients with or without CF who had the same degree of mucosal atrophy, the decrease of disaccharidase activities followed the same pattern, indicating that enzyme activities are affected to the same extent by the damage of the mucosa. In patients with CF with pancreatic insufficiency, the disaccharidase activities were significantly (P less than 0.001) higher when compared to those in control subjects of the same age and ethnic group, although the increase was not uniform in all patients with cystic fibrosis.     

Functional intestinal obstruction in premature infants

Functional intestinal obstruction in two sets of twins is described. In each case the smaller twin was affected. The diagnosis was a clinical one which was supported by radiological findings. The stools from these two infants had a lower than normal water content.     

Disaccharidase activities in jejunal fluid.

Jejunal fluid and mucosal tissue were obtained simultaneously from the same jejunal site in a group of 29 children by a modified biopsy procedure. Lactase, maltase, and sucrase activities were measured in both fluid and mucosal specimens using the same analytical method. The fluid enzyme activities showed highly significant positive correlations with the same enzyme activity in the relevant tissue samples. Relative concentrations of disaccharidase enzymes represented by sucrase: lactase activity ratios also showed a highly significant positive correlation between fluid and tissue. This close relation suggests that the mucosa is the sole or predominant source of disaccharidase activity in the intestinal fluid. The results of kinetic studies comparing tissue and fluid enzyme characteristics also indicate a mucosal origin for the fluid enzyme activities. We conclude that disaccharidase activities in jejunal fluid reflect closely local tissue values and that these measurements may be useful in assessing mucosal enzyme activity in infants in whom jejunal biopsy is not possible.     

Lower urinary tract obstruction secondary to congenital bladder diverticula in infants

Purpose  

Congenital primary bladder diverticulum is a rare condition and may present with urinary infection; other forms of presentation are rare. We present a series of infants who presented with urinary retention secondary to large primary bladder diverticulum.     

Disaccharidase activities, jejunal morphology, and carbohydrate tolerance in children with chronic diarrhea

Children with chronic diarrhea were examined for their carbohydrate tolerance, small bowel morphology, and specific disaccharidase activities to determine whether disaccharidase enzymatic activity is related to villus height and thus may be viewed as an independent variable that controls digestive capacity. Results indicated that 65-78% of all abnormal biopsy samples had specific disaccharidase activities that fell within the normal range. Clinical disaccharide tolerance studies indicated that specific enzyme activity did not correlate with disaccharide tolerance.     

Disaccharidase and lysosomal enzyme activities in amniotic fluid, intestinal mucosa and meconium. Correlation between morphology and disaccharidase activities in human fetal small intestine.

Brush border membrane bound disaccharidases (sucrase and maltase) and lysosomal enzyme (alpha-glucosidase, beta-D-fucosidase and N-acetyl-beta-glucosaminidase) activities awere studied in amniotic fluid (AF). The above enzymes except N-acetyl-beta-glucosaminidase showed a decrease in activity with gestational age beginning at about the 19th week. The activities of sucrase and maltase correlate with the morphological maturation of fetal intestinal mucosa. The distribution of disaccharidases and lysosomal alpha-glucosidase in AF and intestinal mucosa showed different patterns suggesting that these enzymes originate in diverse fetal tissues.     

Disaccharidase activities in Belgian children: reference intervals and comparison with non-Belgian Caucasian children

Aim: To establish reference values for disaccharidase activities in Belgian children and to compare enzyme activities with those of non-Belgian Caucasian children. Methods: Data from Belgian children who had undergone endoscopic jejunal biopsies (1994-2000) for suspected malabsorption were reviewed. The patients were divided into three groups based on histology: (A) normal (n = 201), (B) moderate changes (n = 58) and (C) (sub)total atrophy (n = 14). The 95% reference limits for disaccharidase activities (U/g protein) were calculated for group A after exclusion of patients with a positive hydrogen breath test, a history of lactose intolerance or coeliac disease (final population: n = 151, 0.1-12 y). Values were compared with those of 34 non-Belgian Caucasian children with normal histology (28 of Mediterranean origin). Results: The reference limits (90% confidence interval) were 86 (65-111)-423 (366-494) for maltase, 9 (6-12)-91 (78-122) for lactase and 24 (18-30)-155 (120-184) for sucrase. No gender-related differences in enzyme activities were found. Lactase levels showed a slight decrease with increasing age. Disaccharidase activities of children with histologically confirmed mucosal injury were significantly lower than those of children with normal histology: median values for groups A, B and C were 208, 181 and 96, respectively, for maltase, 40, 28 and 7, respectively, for lactase and 69, 54 and 25, respectively, for sucrase. Median disaccharidase activities in biopsies with normal histology were lower in non-Belgian children, the difference being only statistically significant for lactase, 33 versus 40.

Conclusion: The reference values for Belgian children are well in line with other reported values from Caucasian children. Although enzyme activities are lower in children with histologically confirmed mucosal damage, they do not allow differentiation between histology groups. Lower lactase values were found in non-Belgian children.