Abstract

The activity of two galactosidases (lactase and hetero-β-galactosidase) and β-glucuronidase were studied in per oral duodenal biopsies in 50 infants and children. Ten patients served as controls and 40 had nutritional disorders including celiac disease (acute, and in remission), cystic fibrosis (CF), protein losing enteropathy, and some miscellaneous conditions. The values for the 10 control patients expressed in units/gm protein/minute ± S.D. follows: lactase 38.0 ± 13.4, H-β-gal-ase 1.42 ± 0.35., and β-glucuronidase 1.90 ± 0.45. In the acute stage of celiac disease the lactase values were markedly reduced, the H-β-gal-ase normal or slightly reduced, with normal activity for β-glucuronidase. In clinical remission and while still on a gluten-free diet the activity of lactase remained significantly reduced in seven of nine patients even after 2 to 10 years. The lysosomal enzymes H-β-gal-ase and β-glucuronidase were not strikingly affected in patients with CF although four of six patients showed low values for H-β-galactosidase. β-glucuronidase was not affected in a variety of intestinal disorders including those that severely affect the integrity of the intestinal mucosa. In the conditions studied there was no correlation between the activity of the two galactosidases, nor between the two lysosomal enzymes.

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