Advertisement

Dünndarm A pp 627-646 | Cite as

Kohlenhydratintoleranz

  • W. F. Caspary
Part of the Handbuch der inneren Medizin book series (INNEREN, volume 3 / 3 / A)

Zusammenfassung

Unter Kohlenhydratintoleranz versteht man einen Krankheitszustand, bei dem ungeachtet der Ätiologie Zucker nicht resorbiert werden können. Als Ursachen kommen prinzipiell eine fehlerhafte Digestion (sehr selten = Fehlen von α-Amylase, häufiger: Fehlen oder Verminderung der Oligosaccharidasen) oder eine Störung der Resorption (Fehlen des Glukose-Galaktosetransportsystems, reduzierte Resorptionsfläche) in Frage. Bei der primären Kohlenhydrat-Intoleranz besteht ein angeborenes oder erworbenes Fehlen oder ein Mangel an einzelnen funktionellen Digestions- und Resorptionselementen, bei der sekundären Kohlenhydrat-Intoleranz sind durch morphologische und funktionelle Veränderungen sekundär Verdauung und Resorption gestört. Beide Mechanismen führen dazu, daß nicht resorbierte Kohlenhydrate im Darmlumen verbleiben und dort unspezifische Symptome entfalten. Wird ein aufgenommenes Kohlenhydrat im Darmlumen nicht gespalten oder resorbiert, so gelangt es in distalere Abschnitte des Dünndarms oder in den Dickdarm, die normalerweise mit Bakterien besiedelt sind (Caspary et al. 1981). Bakterien des Kolons spalten (Hydrolyse) und fermentieren Zucker zu Butyrat, Propionat, Laktat, Azetat (=kurzkettige Fettsäuren) sowie zu Wasserstoff (H2) und Kohlendioxyd (CO2) (Caspary et al. 1981) (Abb. 1). Die Fermentationsprodukte senken den normalen pH des Stuhls von pH 7–8 auf einen pH-Wert von 5–6. Die bakterielle Fermentation von Kohlenhydraten zu H2 hat man sich in der Diagnostik zur Erfassung einer Kohlenhydratintoleranz als H2-Atemtest nutzbar gemacht (Caspary 1978b) (s.S. 778 ff.).

Preview

Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.

Literatur

  1. Alpers DH, Cote MN (1971) Inhibition of lactose hydrolysis by dietary sugars. Am J Physiol 221:865PubMedGoogle Scholar
  2. Alpers DH, Seetharam B (1977) Pathophysiology of diseases involving intestinal brush border proteins. N Engl J Med 296:1047PubMedCrossRefGoogle Scholar
  3. Ament ME, Perera DR, Esther LJ (1973) Sucrase-isomaltase deficiency — a frequently misdiagnosed disease. J Pediatr 83:721PubMedCrossRefGoogle Scholar
  4. Antonowicz I, Lloyd Still JD, Khaw KT et al. (1972) Congenital sucrase-isomaltase deficiency. Observations over a period of six years. Pediatrics 49:847PubMedGoogle Scholar
  5. Arashima S, Matsuda I, Sato N et al. (1971) Intestinal disaccharidase activity in an infant with lactose intolerance. Helv Paediatr Acta 26:215PubMedGoogle Scholar
  6. Asp N-G (1971) Human small intestinal β-galactosidases. Separation and characterization of three forms of an acid β-galactosidase. Biochem J 121:299PubMedGoogle Scholar
  7. Asp N-G, Dahlqvist A (1974) Intestinal β-galactosidases in adult low lactase activity and in congenital lactase deficiency. Enzymes 18:84Google Scholar
  8. Asp N-G, Berg NO, Dahlqvist A et al. (1971) The activity of three different small intestinal β-galactosidases in adults with and without lactase deficiency. Scand J Gastroenterol 6:755PubMedCrossRefGoogle Scholar
  9. Auricchio S, Rubino A, Landolt M et al. (1963) Isolated intestinal lactase deficiency in the adult. Lancet 2:324PubMedCrossRefGoogle Scholar
  10. Berg NO, Dahlqvist A, Lindberg T et al. (1969) Severe familial lactose intolerance — a gastrogen disorder? Acta Paediatr Scand 58:525PubMedCrossRefGoogle Scholar
  11. Berg NO, Dahlqvist A, Lindberg T (1979) A boy with severe infantile gastrogen lactose intolerance and acquired lactase deficiency. Acta Paediatr Scand 68:751PubMedGoogle Scholar
  12. Bergoz R (1971) Trehalose malabsorption causing intolerance to mushrooms. Gastroenterology 60:909PubMedGoogle Scholar
  13. Birge SJ, Kentmann HT, Cuatrecasas P et al. (1967) Osteoporosis, intestinal lactase deficiency and low dietary calcium intake. N Engl J Med 276:445PubMedCrossRefGoogle Scholar
  14. Bolin TD, Pirola RC, Davis AE (1969) Adaptation of intestinal lactase in the rat. Gastroenterology 57:406PubMedGoogle Scholar
  15. Burgess EA, Levin B, Mahalanabis D et al. (1964) Hereditary sucrose intolerance: levels of sucrase activity in jejunal mucosa. Arch Dis Child 39:431PubMedCrossRefGoogle Scholar
  16. Cady AB, Rhodes JB, Littman A, Crane RK (1967) Significance of lactase deficit in ulcerative colitis. J Lab Clin Med 70:279PubMedGoogle Scholar
  17. Caspary WF (1975 a) Resorption von Kohlenhydraten und Proteinen im Dünndarm unter normalen und krankhaften Bedingungen. In: Bartelheimer H, Kühn HA, Becker W, Stelzner F (Hrsg) Gastroenterologie und Stoffwechsel. Thieme, StuttgartGoogle Scholar
  18. Caspary WF (1975 b) Atemanalytische Tests in der gastroenterologischen Diagnostik. Z Gastroenterol 13:704PubMedGoogle Scholar
  19. Caspary WF (1977) Kohlenhydratresorption und Malabsorption. Leber Magen Darm 7:150PubMedGoogle Scholar
  20. Caspary WF (1978a) Enzymopathien. In: Bartelheimer H, Classen M, Ossenberg FW (Hrsg) Der kranke Dünndarm. Witzstock, Baden-Baden, S 175Google Scholar
  21. Caspary WF (1978 b) Breath tests. Clin Gastroenterol 7/2:351Google Scholar
  22. Caspary WF, Winckler K, Lankisch PG et al. (1975) Influence of exocrine and endocrine pancreatic function on intestinal brush border enzymatic activities. Gut 16:89PubMedCrossRefGoogle Scholar
  23. Caspary WF, Lembcke B, Elsenhans B (1981) Bacterial fermentation of carbohydrate within the gastrointestinal tract. In: Read NW (ed) Diarrhoea: New insights. Clinical Research Reviews. Janssen Pharmaceutical, Marlow, England, p 107Google Scholar
  24. Conklin KA, Yamashiro LM, Gray GM (1975) Human intestinal sucrase-isomaltase. Identification of free sucrase and isomaltase and cleavage of the hybrid into active distinct subunits. J Biol Chem 250:5735PubMedGoogle Scholar
  25. Cook GC, Lee FD (1966) The jejunum after kwashiorkor. Lancet 2:1263PubMedCrossRefGoogle Scholar
  26. Cooper BT, Candy DCA, Harries JT et al. (1979) Subcellular fractionation studies of the intestinal mucosa in congenital sucrase-isomaltase deficiency. Clin Sci 57:181PubMedGoogle Scholar
  27. Crane RK, (1965) Na+ dependent transport in the intestine and other animal tissues. Fed Proc 24:1000PubMedGoogle Scholar
  28. Crane RK, Menard D, Preiser H et al. (1976) The molecular basis of brush border membrane disease. In: Botz L, Hoffmann JF, Leaf A (eds) Membranes and diseases: International Conference on Biological Membranes. Raven Press, New York, p 229Google Scholar
  29. Dubs R, Steinmann B, Gitzelmann R (1973) Demonstration of an inactive enzyme antigen in sucrase-isomaltase deficiency. Helv Paediatr Acta 28:187PubMedGoogle Scholar
  30. Durand P (1958) Lattosuria idiopatica in una paziente con diarrea cronica ed acidosis. Minerva Pediátr 10:706PubMedGoogle Scholar
  31. Eggermont E, Hers HG (1969) The sedimentation properties of the intestinal α-glucosidases of normal human subjects and of patients with sucrose intolerance. Eur Biochem 9:488CrossRefGoogle Scholar
  32. Eggermont E, Loeb H (1966) Glucose-galactose intolerance. Lancet 2:343CrossRefGoogle Scholar
  33. Elsas LJ, Lambe DW (1973) Familial glucose-galactose malabsorption: Remission of glucose intolerance. J Pediatr 83:226PubMedCrossRefGoogle Scholar
  34. Elsas LJ, Hillmann RE, Patterson JH et al. (1970) Renal and intestinal hexose transport in familial glucose-galactose malabsorption. J Clin Invest 49:576PubMedCrossRefGoogle Scholar
  35. Fairclough PD, Clark ML, Dawson AM et al. (1978) Absorption of glucose and maltose in congenital glucose-galactose malabsorption. Pediatr Res 12:1112PubMedCrossRefGoogle Scholar
  36. Frank G, Brunner J, Hauser H et al. (1978) The hydrophobic anchor of small intestinal sucrase-isomaltase. FEBS Lett 96:183PubMedCrossRefGoogle Scholar
  37. Freiburghaus AU, Schmitz J, Schindler M et al. (1976) Protein patterns of brush-border fragments in congenital lactose malabsorption and in specific hypolactasia of the adult. N Engl J Med 294:1030PubMedCrossRefGoogle Scholar
  38. Freiburghaus AU, Dubs R, Hadorn B et al. (1977) The brush border membrane in hereditary sucrase isomaltase deficiency. Abnormal protein pattern and presence of immunoreactive enzyme. Eur J Clin Invest 7:455PubMedCrossRefGoogle Scholar
  39. Goldstein F, Karacadag S, Wirths CW et al. (1970) Intraluminal small intestinal utilization of D-xylose by bacteria. A limitation of D-xylose absorption test. Gastroenterology 59:380PubMedGoogle Scholar
  40. Gray GM (1975) Carbohydrate digestion and absorption. Role of the small intestine. N Engl J Med 292:1225PubMedCrossRefGoogle Scholar
  41. Gray GM (1978) Intestinal disaccharidase deficiencies and glucose-galactose malabsorption. In: Stanbury JB, Wyngaarden JB, Frederickson DS (eds) The metabolic basis of inherited disease, 4th edn. McGraw-Hill, New York, p 1526Google Scholar
  42. Gray GM, Santiago NA (1966) Disaccharide absorption in normal and diseased human intestine. Gastroenterology 51:489PubMedGoogle Scholar
  43. Gray GM, Santiago NA (1969) Intestinal β-galactosidases. I. Separation and characterization of three enzymes in normal human intestine. J Clin Invest 48:716PubMedCrossRefGoogle Scholar
  44. Gray GM, Walter MW, Colver EH (1968) Persistent deficiency of intestinal lactase in apparently cured tropical sprue. Gastroenterology 54:552PubMedGoogle Scholar
  45. Gray GM, Conklin KA, Townley RR (1976) Sucrase-isomaltase deficiency. Absence of an inactive enzyme variant. N Engl J Med 294:750PubMedCrossRefGoogle Scholar
  46. Harris JT (1982) Disorders of carbohydrate absorption. Clin Gastroenterol 11/1:17Google Scholar
  47. Hauri HP, Quaroni A, Isselbacher KJ (1979) Biogenesis of intestinal plasma membrane: post-translational route and cleavage of sucrase-isomaltase. Proc Natl Acad Sci USA 76:5183PubMedCrossRefGoogle Scholar
  48. Hauri HP, Quaroni A, Isselbacher KJ (1980) Monoclonal antibodies to sucrase-isomaltase: probes for the study of postnatal development and biogenesis of the intestinal microvillus membrane. Proc Natl Acad Sci USA 77:6629PubMedCrossRefGoogle Scholar
  49. Hirschkorn N, Molla A (1969) Reversible jejunal disaccharidase deficiency in cholera and other diarrheal diseases. John Hopkins Med J 125:291Google Scholar
  50. Holzel A, Schwarz V, Sutcliffe KW (1959) Defective lactose absorption causing malnutrition in infancy. Lancet 1:1126PubMedCrossRefGoogle Scholar
  51. Hoskova A, Sabacky J, Mrskos A et al. (1980) Severe lactose intolerance with lactosuria and vomiting. Arch Dis Child 55:304PubMedCrossRefGoogle Scholar
  52. Hughes WS, Senior JR (1975) The glucose-galactose malabsorption syndrome in a 23-year-old woman. Gastroenterology 68:142PubMedGoogle Scholar
  53. Johnson JD, Simoons FJ, Hurwitz R et al. (1977) Lactose malabsorption among the Pirna Indians of Arizona. Gastroenterology 73:1299PubMedGoogle Scholar
  54. Kelly JJ, Alpers DH (1973) Properties of human intestinal glucoamylase. Biochim Biopys Acta 315:113Google Scholar
  55. Kern F, Struthers JE, Attwood WL (1963) Lactose intolerance as a cause of steatorrhea in an adult. Gastroenterology 45:477PubMedGoogle Scholar
  56. Kogut MD, Donnell GN, Shaw KNF (1967) Studies of lactose absorption in patients with galactosemia. J Pediatr 71:75PubMedCrossRefGoogle Scholar
  57. Kojecký Z, Matlocha Z (1965) Quantitative differences of intestinal disaccharidase activity following the resection of the stomach. Gastroenterologia 104:343PubMedCrossRefGoogle Scholar
  58. Levin B, Abraham JM, Burgess EA, Wallis PG (1970) Congenital lactose malabsorption. Arch Dis Child 45:173PubMedCrossRefGoogle Scholar
  59. Levitt MD, Donaldson RM jr (1970) Use of respiratory hydrogen (H2) excretion to detect carbohydrate malabsorption. J Lab Clin Med 75:9375Google Scholar
  60. Lifshitz F, Klotz AP, Holman GH (1968) Intestinal disaccharidase deficiencies in gluten-sensitive enteropathy. Am J Dig Dis 10:47CrossRefGoogle Scholar
  61. Lindquist B, Meeuwisse GW (1962) Chronic diarrhoe caused by monosaccharide malabsorption. Acta Paediatr 51:674PubMedCrossRefGoogle Scholar
  62. Lorenz-Meyer H, Blum AL, Haemmerli HP, Semenza G (1972) A second enzyme defect in acquired lactase deficiency: lack of small intestinal phlorizin-hydrolase. Eur J Clin Invest 2:326PubMedCrossRefGoogle Scholar
  63. Madzarovova-Nohejlova J (1973) Trehalase deficiency in a family. Gastroenterology 65:130PubMedGoogle Scholar
  64. Maestracci D (1976) Enzymic solubilisation of the human intestinal brush border membrane enzymes. Biochim Biophys Acta 433:469PubMedCrossRefGoogle Scholar
  65. Malathi P, Ramaswamy K, Caspary WF, Crane RK (1973) Studies on the transport of glucose from disaccharides by hamster small intestine in vitro. 1. Evidence for a disaccharidase-related transport system. Biochim Biophys Acta 307:613PubMedCrossRefGoogle Scholar
  66. McMichael HB, Webb J, Dawson HM (1965) Lactase deficiency in adults: A cause of “functional” diarrhoea. Lancet 1:717PubMedCrossRefGoogle Scholar
  67. McNair A, Gudmand-Hoyer E, Jarnum S, Orrild L (1972) Sucrose malabsorption in Greenland. Br Med J 2:19PubMedCrossRefGoogle Scholar
  68. Meeuwisse GW, Dahlqvist A (1968) Glucose-galactose malabsorption: a study with biopsy of the small intestinal mucosa. Acta Paediatr Scand 57:273PubMedCrossRefGoogle Scholar
  69. Moncrieff A, Wilkinson RH (1954) Sucrosuria with mental defect and hiatus hernia. Acta Paediatr Scand [Suppl] 100:495CrossRefGoogle Scholar
  70. Newcomer AD, McGill DB (1966) Distribution of disaccharidase activity in the small bowel of normal and lactase deficient subjects. Gastroenterology 51:481PubMedGoogle Scholar
  71. Newcomer AD, McGill DB (1967) Incidence of lactase-deficiency in ulcerative colitis. Gastroenterology 53:890PubMedGoogle Scholar
  72. Newcomer AD, Gordon H, Thomas PJ, McGill DB (1977) Family studies of lactase deficiency in the American Indian. Gastroenterology 73:985PubMedGoogle Scholar
  73. Peterson ML, Herber R (1967) Intestinal sucrase deficiency. Trans Assoc Am Physicians 80: 275PubMedGoogle Scholar
  74. Phillips SF, McGill DB (1974) Gluclose-galactose malabsorption in an adult: perfusion studies of sugar, electrolyte and water transport. Am J Dig Dis 18:1017CrossRefGoogle Scholar
  75. Plotkin GR, Isselbacher KJ (1964) Secondary disaccharide deficiency in adult celiac disease (non-tropical sprue) and other malabsorption states. N Engl J Med 271:1033PubMedCrossRefGoogle Scholar
  76. Preiser H, Menard D, Crane RK, Cerda JJ (1974) Deletion of enzyme protein from the brush border membrane in sucrase-isomaltase deficiency. Biochim Biophys Acta 363:279PubMedCrossRefGoogle Scholar
  77. Prinsloo JG, Wittmann W, Kruger H, Freier E (1971) Lactose absorption and mucosal disaccharidases in convalescent pellagra and kwashiorkor children. Arch Dis Child 46:474PubMedCrossRefGoogle Scholar
  78. Ramaswamy K, Malathi P, Caspary WF, Crane RK (1974) Studies on the transport of glucose from disaccharides by hamster small intestine in vitro. II. Characteristics of the disaccharidase-related transport system. Biochim Biophys Acta 345:39PubMedCrossRefGoogle Scholar
  79. Ramaswamy K, Malathi P, Crane RK (1976) Demonstration of hydrolase-related glucose transport in brush border membrane vesicles prepared from guinea pig small intestine. Biochem Biophys Res Commun 68:162PubMedCrossRefGoogle Scholar
  80. Ransome-Kuti O, Kretschmer N, Johnson JD et al. (1975) A genetic study of lactose digestion in Nigerian families. Gastroenterology, 68, 431PubMedGoogle Scholar
  81. Rosensweig NS (1971) Adult lactase deficiency: genetic control or adaptive response? Gastroenterology 60:464PubMedGoogle Scholar
  82. Satio M, Murakami E, Suda M (1976) Circadian rhythms in disaccharidases of rat small intestine and its relation to food intake. Biochim Biophys Acta 421:177CrossRefGoogle Scholar
  83. Schmitz J, Commergrain C, Maestracci D, Rey J (1974) Absence of brush border sucrase-isomaltase complex in congenital sucrose intolerance. Biomedicine [Express] 21:440Google Scholar
  84. Schmitz J, Bresson JL, Triadou N et al. (1980) Analyse en électrophorèse sur gel de Polyacrylamide des protéines de la membrane microvillositaire et d’une fraction cytoplasmatique dans 8 cas d’intolérance congénitale au saccharose. Gastroenterol Clin Biol 4:251PubMedGoogle Scholar
  85. Schneider AJ, Kinter WB, Stirling CE (1966) Glucose-galactose malabsorption. N Engl J Med 274:305PubMedCrossRefGoogle Scholar
  86. Shmerling DH, Auricchio S, Rubino A et al. (1964) Der sekundäre Mangel an intestinaler Disaccharidasenaktivität bei der Cöliakie: Quantitative Bestimmung der Enzymaktivität und klinische Beurteilung. Helv Paediatr Acta 19:507PubMedGoogle Scholar
  87. Skovbjerg H, Gudmand-Hoyer E, Fenger H (1980) Immunoelectrophoretic studies on human small intestinal brush border proteins — amount of lactase protein in adult type hypolactasia. Gut 21:360PubMedCrossRefGoogle Scholar
  88. Weijers HA, Kamer JH van de (1962) Diarrhoea caused by deficiency of sugar splitting enzymes IL Acta Paediatr 51:371CrossRefGoogle Scholar
  89. Weijers HA, Kamer JH van de, Mossel DAA, Dicke WK (1960) Diarrhoea caused by deficiency of sugar splitting enzymes. Lancet 2:296PubMedCrossRefGoogle Scholar
  90. Welsh JD, Zschiesche OM, Anderson J et al. (1969) Intestinal disaccharidase activity in celiac sprue (gluten-sensitive enteropathy). Arch Intern Med 123:33PubMedCrossRefGoogle Scholar
  91. Weser E, Rubin W, Ross L et al. (1965) Lactase deficiency in patients with the “irritable-colon” syndrome. N Engl J Med 273:1070PubMedCrossRefGoogle Scholar
  92. Wimberley PD, Harries JT, Burgess EA (1974) Congenital glucose-galactose malabsorption. Proc R Soc Med 67:755Google Scholar
  93. Woodruff GG (1958) Sucrosuria in association with mental deficiency and hiatal hernia. J Pediatr 52:66PubMedCrossRefGoogle Scholar

Copyright information

© Springer-Verlag Berlin Heidelberg 1983

Authors and Affiliations

  • W. F. Caspary

There are no affiliations available

Personalised recommendations