title: Small intestinal brush border enzymes in cystic fibrosis.
abstract: The study concerns the maltase, saccharase, lactase and alkaline phosphatase activity in small intestinal biopsy specimens from 61 consecutively admitted, untreated, Caucasian cystic fibrosis patients. A group of 319 age matched controls admitted during the same time period for undefined gastrointestinal or nutritional disorders acted as the controls. In order to eliminate morphological damage as a confounding factor, the enzyme activities were studied in small intestinal biopsy specimens having both normal stereomicroscopic and histological features. It was shown that neither maltase nor saccharase activity was different in the two groups, in contrast to lactase and alkaline phophatase activity, that was significantly lower in cystic fibrosis patients. The differences could not be explained by the nutritional status as judged by the body mass index. Lactase activity is known to be easily affected by numerous enteropathies. As the information on alkaline phosphatase activity is limited, the low activity is discussed in more detail. Taking into account the literature data, the low alkaline phosphatase activity is tentatively attributed either to enhanced release from the brush border or to the faulty handling of alkaline phophatase protein in the post-golgi compartments secondary to the accumulation of incorrectly glycosylated CFTR in the same cell structures.
authors.affiliation: Department of Paediatrics, University of Leuven, Belgium.
authors.forename: S
authors.initials: S
authors.lastname: Van Biervliet
authors.forename: E
authors.initials: E
authors.lastname: Eggermont
authors.forename: H
authors.initials: H
authors.lastname: Carchon
authors.forename: G
authors.initials: G
authors.lastname: Veereman
authors.forename: K
authors.initials: K
authors.lastname: Deboeck
mesh_terms: D000469:Alkaline Phosphatase / Q000378:metabolism; D001706:Biopsy; D016022:Case-Control Studies; D003550:Cystic Fibrosis / Q000201:enzymology*; D005260:Female; D006026:Glycoside Hydrolases / Q000378:metabolism; D006801:Humans; D007223:Infant; D007413:Intestinal Mucosa / Q000166:cytology / Q000201:enzymology; D007421:Intestine, Small / Q000201:enzymology*; D043322:Lactase; D008297:Male; D008871:Microvilli / Q000201:enzymology; D009752:Nutritional Status; D010179:Pancreas / Q000503:physiopathology; D000520:alpha-Glucosidases / Q000378:metabolism; D043324:beta-Fructofuranosidase; D001616:beta-Galactosidase / Q000378:metabolism
chemical_list: D000469:Alkaline Phosphatase; D006026:Glycoside Hydrolases; D043322:Lactase; D000520:alpha-Glucosidases; D001616:beta-Galactosidase; D043324:beta-Fructofuranosidase