Abstract
Intestinal disaccharidase activities were determined in 294 jejunal biopsies obtained from 254 children with various disorders of the small bowel, and alkaline phosphatase activity was measured in 251 biopsies. In normal mucosa a broad range of enzyme activity was found corresponding with the data in the literature.
A primary disaccharidase deficiency was observed in 5 children with congenital sucraseisomaltase deficiency and in a 12-year-old Egyptian boy with acquired lactase deficiency.
A secondary generalized depression of disaccharidase activity and a diminution of alkaline phosphatase activity existed chiefly in patients who had severe or moderate mucosal damage, also in active coeliac disease and during gluten loading, in protracted diarrhoea of infancy, chronic malabsorption of unknown origin and agammaglobulinemia. During remissions enzyme activities recovered together with mucosal improvement. Low levels of enzyme activities were also seen in some cases of protracted diarrhoea of infancy and chronic malabsorption of unknown origin although only mild mucosal lesions were demonstrated.
Zusammenfassung
In 294 Jejunumbiopsien von 254 Kindern mit verschiedenen Dünndarmerkrankungen wurde die Aktivität der intestinalen Disaccharidasen und in 251 Biopsien die Aktivität der alkalischen Phosphatase bestimmt. Bei Vorliegen einer normalen Mucosa fand sich eine breite Streuung der Enzymaktivitäten, die mit den Angaben im Schrifttum übereinstimmte.
Ein primärer Disaccharidasemangel wurde bei 5 Kindern mit kongenitalem Saccharase-Isomaltase-Mangel sowie bei einem 12jährigen Jungen ägyptischer Herkunft mit erworbenem Lactasemangel beobachtet.
Eine sekundäre, generelle Erniedrigung der Disaccharidaseaktivitäten und eine Verminderung der Aktivität der alkalischen Phosphatase bestand besonders dann, wenn schwere oder mittelschwere Mucosaveränderungen nachweisbar waren, so in der aktiven Phase der Cöliakie und unter Glutenbelastung, bei protrahierter infantiler Diarrhoe, chronischer Malabsorption unklarer Genese und Agammaglobulinämie. In der Remissionsphase erholten sich gleichzeitig mit der Restitution der Mucosa auch die Enzymaktivitäten. Bei protrahierter infantiler Diarrhoe und bei chronischer Malabsorption unklarer Genese zeigte sich in mehreren Fällen auch dann eine deutliche Verminderung der Enzymaktivitäten, wenn nur leichte Schleimhautveränderungen vorhanden waren.
Similar content being viewed by others
Literatur
Antonowicz, I., Reddy, V., Khaw, K.-T., Shwachman, H.: Lactase deficiency in patients with cystic fibrosis. Pediatrics 42, 492 (1968)
Arthur, A. B.: Intestinal disaccharidase deficiency in children with coeliac disease. Arch. Dis. Childh. 41, 519 (1966)
Auricchio, S., Rubino, A., Prader, A., Rey, J., Jos, J., Frézal, J., Davidson, M.: Intestinal glycosidase activities in congenital malabsorption of disaccharides. J. Pediat. 66, 555 (1965)
Berg, N. O., Dahlquist, A., Lindberg, T., Nordén, A.: Intestinal dipeptidases and disaccharidases in celiac disease in adults. Gastroenterology 59, 575 (1970)
Bessey, O. A., Lowry, O. H., Brock, M. J.: A method for the rapid determination of alkaline phosphatase with five cubic millimeters of serum. J. biol. Chem. 164, 321 (1946)
Bolin, T. D., Davis, A. E.: Primary lactase deficiency: genetic or acquired? Gastroenterology 62, 355 (1972)
Bolin, T. D., Davis, A. E., Seah, C. S., Chua, K. L., Yong, V., Kho, K. M., Siak, C. L., Jacob, E.: Lactose intolerance in Singapore. Gastroenterology 59, 76 (1970)
Bowie, M. D., Brinckman, G. L., Hansen, J. D. L.: Acquired disaccharide intolerance in malnutrition. J. Pediat. 66, 1083 (1965)
Burke, V., Danks, D. M.: Monosaccharide malabsorption in young infants Lancet 1966 I, 1177
Burke, V., Kerry, K. R., Anderson, C. M.: The relationship of dietary lactose to refractory diarrhoea in infancy. Aust. paediat. 1, 147 (1965)
Careddu, P., Giovannini, M., Cevini, G.: Vorübergehende Disaccharidintoleranz bei den Ernährungsstörungen des Säuglingsalters. Helv. paediat. Acta 18, 97 (1963)
Chandra, R. K., Pawa, R. R., Ghai, O. P.: Sugar intolerance in malnourished infants and children. Brit. med. J. 1968 IV, 611
Clarke, J. T., Quillian, W., Shwachman, H.: Chronic diarrhea and failure to thrive due to intestinal disaccharides insufficiency. Pediatrics 34, 807 (1964)
Coello-Ramirez, P., Gutierrez-Topete, G., Lifshitz, F.: Pneumatosis intestinalis. Amer. J. Dis. Child. 120, 3 (1970)
Crosby, W. H., Kugler, H. W.: Intraluminal biopsy of the small intestine. The intestinal biopsy capsule. Amer. J. dig. Dis. 2, 236 (1957)
Cuatrecasas, P., Lockwood, D. H., Caldwell, J. R.: Lactase deficiency in the adult: a common occurence. Lancet 1965 I, 14
Dahlquist, A.: Assay of intestinal disaccharidases. Anal. Biochem.. 22, 99 (1968)
Dahlquist, A., Lindberg, T., Meeuwisse, G., Akerman, M.: Intestinal, dipeptidases and disaccharidases in children with malabsorption. Acta paediat. scand. 59, 621 (1970)
Eggstein, M., Kreutz, F. M.: Vergleichende Untersuchungen zur quantitativen Eiweißbestimmung im Liquor und eiweißarmen Lösungen. Klin. Wschr. 33, 879 (1955)
Ferguson, A., Watson, W. C., Maxwell, J. D., Fell, G. S.: Alkaline phosphatase levels in normal and diseased small bowel. Gut 9, 96 (1968)
Gracey, M., Burke, V.: Sugar-induced diarrhoea, in children. Arch. Dis. Childh. 48, 331 (1973)
Gray, G. M.: Problems with lactose tolerance tests. Gastroenterology 53, 496 (1967)
Gray, G. M., Santiago, N. A.: Disaccharide absorption in normal and diseased human intestine. Gastroenterology 51, 489 (1966)
Habte, D., sterky, G., Hjalmarsson, B.: Lactose malabsorption in Ethiopian children. Acta paediat. scand. 62, 649 (1973)
Haemmerli, U. P., Kistler, H. J., Ammann, R., Auricchio, S., Prader, A.: Lactasemangel der Dünndarmmucosa als Ursache gewisser Formen erworbener Milchintoleranz bei Erwachsenen. Helv. med. Acta 30, 693 (1963)
Hamilton, J. R., Lynch, M. J., Reilly, B. J.: Active coeliac disease in childhood. Quart. J. Med. 38, 135 (1969)
Harms, K.: Die Bedeutung des oralen d-Xylose-Testes in der Diagnostik und Verlaufskontrolle der Cöliakie. Z. Kinderheilk. 114, 221 (1973)
Holzel, A.: Sugar malabsorption due to deficiencies of disaccharidase activities and of monosaccharide transport. Arch. Dis. Childh. 42, 341 (1967)
Holzel, A., Schwarz, V., Sutcliffe, K. W.: Defective lactose absorption causing malnutrition in infancy. Lancet 1959 I, 1126
Huang, S.-S., Bayless, Th. M.: Lactose intolerance in healthy children. New Engl. J. Med. 276, 1283 (1967)
Keusch, G. T., Troncale, F. J., Miller, L. H., Promadhat, V., Anderson, P. R.: Acquired lactose malabsorption in Thai children. Pediatrics 43, 540 (1969)
Levin, B., Abraham, J. M., Burgess, E. A., Wallis, P. G.: Congenital lactose malabsorption. Arch. Dis. Childh. 45, 173 (1970)
Lifshitz, F., Holman, G. H.: Dissaccharidase deficiency with steatorrhea. J. Pediat. 64, 34 (1964)
Lifshitz, F., Coello-Ramirez, P., Contreras-Gutierrez M. L.: The response of infants to carbohydrate oral loads after recovery from diarrhea. J. Pediat. 79, 612 (1971)
Lifshitz, F., Coello-Ramirez, P., Gutierrez-Topete, G.: Monosaccharide intolerance and hypoglycemia in infants with diarrhea. I. Clinical course of 23 infants. J. Pediat. 77, 595 (1970)
Lifshitz, F., Coello-Ramirez, P., Gutierrez-Topete, G.: Monosaccharide intolerance and hypoglycemia in infants with diarrhea. II. Metabolic studies in 23 infants. J. Pediat. 77, 604 (1970)
Lifshitz, F., Coello-Ramirez, P., Gutierrez-Topete, G., Cornado-Cornet, M. C.: Carbohydrate intolerance in infants with diarrhea. J. Pediat. 79, 760 (1971)
Lifshitz, F., Klotz, A. P., Holman, G. H.: Intestinal, disaccharidase deficiencies in glutensensitive enteropathy. Amer. J. dig. Dis. 10, 47 (1965)
Lloyd-Still, J.: Gastroenteritis with secondary disaccharide intolerance. Acta paediat. scand. 58, 147 (1969)
Lowry, O. H., Rosebrough, N. J., Farr, A. L., Randall, R. J.: Protein measurement with the folin phenol reagent. J. biol. Chem. 193, 265 (1951)
Lubos, M. C., Gerrard, J. W., Buchan, D. J.: Disaccharidase activities in milk-sensitive and celiac patients. J. Pediat. 70, 325 (1967)
Lücking, Th., Burdelski, M.: Kongenitaler Saccharase-Isomaltase-Mangel. Beobachtungen über einen Zeitraum von 6 Jahren. Dtsch. med. Wschr. (im Druck)
Lücking, Th., Grüttner, R.: Über die diagnostische Wertigkeit des D-Xylose-Resorptionstestes. Mschr. Kinderheilk. 119, 576 (1971)
Lücking, Th., Grüttner, R.: Chronische Diarrhoe und schweres Malabsorptionssyndrom nach Infektion mit Dyspepsie-Coli. Mschr Kinderheilk. 121, 376 (1973)
McCarthy, C. F., Gough, K. R., Rodriguez, M., Read, A. E.: Peroral intestinal mucosal biopsy with the small Crosby capsule. Brit. med. J. 1964 I, 1620
Nordio, S., Lamedica, G. M., Vignolo, L., Berio, A.: Six cases of lactose intolerance. Lactose intolerance and coeliac disease. Disaccharidases activity in the intestinal mucosa ascertained with the peroral biopsy. Ann. paediat. 204, 3 (1965)
Olatunbosun, D. A., Adadevoh, B. K.: Lactose intolerance in Nigerian children. Acta paediat. scand. 61, 715 (1972)
Padykula, H. A., Strauss, E. W., Ladman, A. J., Gardner, F. H.: A morphologic and histochemical analysis of the human jejunal epithelium in nontropical sprue. Gastroenterology 40, 735 (1961)
Plotkin, G. R., Issebacher, K. J.: Secondary disaccharidase deficiency in adult coeliac disease (non-tropical sprue) and other malabsorption states. New Engl. J. Med. 271, 1033 (1964)
Rey, J., Frézal, J.: Les anomalies des disaccharidases. Arch. franç. Pédiat. 24, 65 (1967)
Riecken, E. O., Stewart, J. S., Dowling, R. H.: Neuere Methoden in der Diagnostik intestinaler Störungen. Internist 7, 209 (1966)
Rodriguez-de-Curet, H., Lugo-de-Rivera, C., Torres-Pinedo, R.: Studies on infant diarrhea. IV. Sugar transit and absorption in small intestine after feeding. Gastroenterology 59, 396 (1970)
Salazar de Sousa, C.: Infant malnutrition caused by sugar intolerance. Ann. Paediat. Fenn. 11, 101 (1965)
Sheehy, T. W., Anderson, P. R.: Disaccharidase activity in normal and diseased small bowel. Lancet 1965 II, 1
Sheldon, W., Tempany, E.: Small intestinal peroral biopsy in coeliac children. Gut 7, 481 (1966)
Shiner, M.: Jejunal biopsy tube. Lancet 1956 I, 85
Shmerling, D. H., Auricchio, S., Rubino, A., Hadorn, B., Prader, A.: Der sekundäre Mangel an intestinaler Disaccharidaseaktivität bei der Coeliakie. Helv. paediat. Acta 19, 507 (1964)
Shwachman, H., Lloyd-Still, J. D., Khaw, K.-T., Antonowicz, T.: Protracted diarrhea of infancy treated by intravenous alimentation. II. Studies of small intestinal biopsy results. Amer. J. Dis. Child. 125, 365 (1973)
Sunshine, P., Kretchmer, N.: Studies of small intestine during development. III. Infantile diarrhea associated with intolerance to disaccharides. Pediatrics 34, 38 (1964)
Townley, R. R. W.: Disaccharidase deficiency in infancy and childhood. Pediatrics 38, 127 (1966)
Townley, R. R. W., Khaw, K.-T., Shwachman, H.: Quantitative assay of disaccharidase activities of small intestinal mucosal biopsy specimens in infancy and childhood. Pediatrics 36, 911 (1965)
Weijers, H. A., van de Kamer, J. H., Dicke, W. K., Ijsseling, J.: Diarrhoea caused by deficiency of sugar splitting enzymes. Acta paediat. (Uppsala) 50, 55 (1961)
Weijers H. A., Lindquist, B., Anderson, C. M., Rey, J., Shmerling, D. H., Visakorpi, J. K., Hadorn, B., Grüttner, R.: Diagnostic criteria in coeliac disease. Acta paediat. scand. 59, 461 (1970)
Welsh, J. D., Porter, M. G.: Reversible secondary disaccharidase deficiency. Amer. J. Dis. Child. 113, 716 (1967)
Welsh, J. D., Zschiesche, O. M., Anderson., J., Walker, A.: Intestinal disaccharidase activity in celiac sprue (glutensensitive enteropathy). Arch. intern. Med. 123 33 (1969)
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Lücking, T. Intestinale Aktivität von Disaccharidasen und alkalischer Phosphatase in Jejunumbiopsien bei kindlichen Dünndarmerkrankungen. Eur J Pediatr 121, 263–277 (1976). https://doi.org/10.1007/BF00443019
Received:
Issue Date:
DOI: https://doi.org/10.1007/BF00443019