Combined assessment of intestinal disaccharidases in congenital asucrasia by differential urinary disaccharide excretion

Investigation of intestinal disaccharide hydrolysis and permeability by means of a non-invasive differential sugar absorption test was performed in a family containing two siblings with primary sucrase-isomaltase deficiency. The procedure, which depends on measurement of urinary excretion ratios aft...

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Bibliographic Details
Published in:Journal of clinical pathology 1990-05, Vol.43 (5), p.406-409
Main Authors: Maxton, D G, Catt, S D, Menzies, I S
Format: Article
Language:English
Subjects:
asucrasia
Biological and medical sciences
Child
Chromatography, Thin Layer - methods
Disaccharidases - deficiency
Disaccharides - urine
Humans
Hydrolysis
Intestinal Absorption - physiology
Investigative techniques, diagnostic techniques (general aspects)
Lactose
Lactulose
Male
Medical sciences
Pathology. Cytology. Biochemistry. Spectrometry. Miscellaneous investigative techniques
Sucrose
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Summary:Investigation of intestinal disaccharide hydrolysis and permeability by means of a non-invasive differential sugar absorption test was performed in a family containing two siblings with primary sucrase-isomaltase deficiency. The procedure, which depends on measurement of urinary excretion ratios after the oral administration of lactose, sucrose, palatinose, lactulose and L-rhamnose, is capable of simultaneous determination of intestinal lactase, sucrase, and isomaltase activity and lactulose:rhamnose permeability. The results corresponded well with those of disaccharidase assay and histological findings in jejunal biopsy tissue obtained from the patients. Palatinose proved a satisfactory substrate for in vivo assessment of intestinal isomaltase activity. The method described provides a reliable and comprehensive assessment of intestinal disaccharide hydrolysis, and simultaneous estimation of permeability assists discrimination of primary from secondary disaccharidase deficiency. The ability to assess three different disaccharidase activities in addition to intestinal permeability by means of a single test, and the simplicity of preservation and transport of urine samples for sugar analysis, makes this a convenient, definitive method for the investigation of defective sugar absorption in both patients and population groups.
ISSN:0021-9746
1472-4146
DOI:10.1136/jcp.43.5.406