Skip to main content

Abstract

Carbohydrates are important constituents of the diet of man throughout the world and, following intestinal hydrolysis and absorption, provide a critical source of metabolic energy. Defects in hydrolysis and/or absorption result in retention of residues within the intestinal tract, and gastrointestinal symptoms, i.e. carbohydrate or sugar ‘intolerance’. Sugar intolerance is a common clinical problem and may have serious consequences, particularly when it occurs in early life.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 39.99
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 54.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

Preview

Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.

References

  1. A. M. Ugolev, (1965). Membrane (contact) digestion. Physiol. Rev., 45, 555

    PubMed  CAS  Google Scholar 

  2. G. M. Gray, (1975). Carbohydrate digestion and absorption. Role of the small intestine. N. Engl. J. Med., 292, 1225

    Article  PubMed  CAS  Google Scholar 

  3. A. D. Newcomer and D. B. McGill, (1966). Distribution of disaccharidase activity in the small bowel of normal and lactase deficient subjects. Gastroenterology, 51, 481

    PubMed  CAS  Google Scholar 

  4. M. Saito, E. Murakami and M. Suda, (1976). Circadian rhythms in disaccharidases of rat small intestine and its relation to food intake. Biochim. Biophys. Acta, 421, 177

    PubMed  CAS  Google Scholar 

  5. G. M. Gray, and N. A. Santiago, (1966). Disaccharide absorption in normal and diseased human intestine. Gastroenterology, 51, 489

    PubMed  CAS  Google Scholar 

  6. D. H. Alpers, and M. N. Cote, (1971). Inhibition of lactose hydrolysis by dietary sugars. Am. J. Physiol, 221, 865

    PubMed  CAS  Google Scholar 

  7. N-G. Asp, A. Dahlqvist and O. Koldovsky, (1969). Human small intestinal β-galactosidases. Separation and characterization of one lactase and one hetero β-galactosidase. Biochem. J., 114, 351

    PubMed  CAS  Google Scholar 

  8. G. M. Gray, and N. A. Santiago, (1969). Intestinal β-galactosidases. I. Separation and characterization of three enzymes in normal human intestine. J. Clin. Invest., 48, 716

    Article  PubMed  CAS  Google Scholar 

  9. N.-G. Asp, (1971). Human small intestinal β-galactosidases. Separation and characterization of three forms of an acid β-galactosidase. Biochem. J., 121, 299

    PubMed  CAS  Google Scholar 

  10. Z. Lojda, P. Fric and J. Jode, (1972). Histochemische Befunde in der Dunn-darmschleimhaut bei Storungen der Kohlenhydratabsorption. Dtsch. Z. Ver-dau. Stoffwechselkr., 32, 163

    CAS  Google Scholar 

  11. N.-G. Asp, and A. Dahlqvist, (1974). Intestinal β-galactosidases in adult low lactase activity and in congenital lactase deficiency. Enzyme, 18, 84

    PubMed  CAS  Google Scholar 

  12. K. A. Conklin, K. M. Yamashiro and G. M. Gray, (1975). Human intestinal sucrase-isomaltase. Identification of free sucrase and isomaltase and cleavage of the hybrid into active distinct subunits. J. Biol. Chem., 250, 5735

    PubMed  CAS  Google Scholar 

  13. J. Brunner, H. Hauser, G. Semenza and H. Wacker, (1977). Incorporation of pure hydrolases isolated from brush border membranes in single-bilayer lecithin vesicles. In Biochemistry of Membrane Transport, FEBS Symposium No. 42, p. 105. (Springer-Verlag Berlin)

    Google Scholar 

  14. D. Maestracci, (1976). Enzymic solubilisation of the human intestinal brush border membrane enzymes. Biochim. Biophys. Acta, 433, 469

    Article  PubMed  CAS  Google Scholar 

  15. D. H. Alpers, and B. Seetharam, (1977). Pathophysiology of diseases involving intestinal brush-border proteins. N. Engl. J. Med., 296, 1047

    Article  PubMed  CAS  Google Scholar 

  16. R. K. Crane, (1965) Na+-dependent transport in the intestine and other animal tissues. Fed. Proc, 24, 1000

    PubMed  CAS  Google Scholar 

  17. G. A. Kimmich, (1973). Coupling between Na+ and sugar transport in small intestine. Biochim. Biophys. Acta, 300, 31

    PubMed  CAS  Google Scholar 

  18. P. Honegger, and G. Semenza, (1973). Multiplicity of carriers for free glucalogues in hamster small intestine. Biochim. Biophys. Acta, 318, 390

    Article  CAS  Google Scholar 

  19. E. S. Debnam, and R. J. Levin, (1976). Influence of specific dietary sugars on the jejunal mechanisms for glucose, galactose, and α-methyl glucoside absorption: evidence for multiple sugar carriers. Gut, 17, 92

    Article  PubMed  CAS  Google Scholar 

  20. P. Malathi, K. Ramaswamy, W. F. Caspary and R. K. Crane, (1973). Studies on the transport of glucose from disaccharides by hamster small intestine in vitro. I. Evidence for a disaccharidase-related transport system. Biochim. Biophys. Acta, 307, 613

    Article  PubMed  CAS  Google Scholar 

  21. K. Ramaswamy, P. Malathi, W. F. Caspary and R. K. Crane, (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, 39

    Article  PubMed  CAS  Google Scholar 

  22. K. Ramaswamy, P. Malathi and R. K. Crane, (1976). Demonstration of hydrolase-related glucose transport in brush border membrane vesicles prepared from guinea pig small intestine. Biochem. Biophys. Res. Commun., 68, 162

    Article  PubMed  CAS  Google Scholar 

  23. J. H. Annegers, (1964). Intestinal absorption of hexoses in the dog. Am. J. Physiol, 206, 1095

    PubMed  CAS  Google Scholar 

  24. C. D. Holdsworth and A. M. Dawson, (1965). Absorption of fructose in man. Proc. Soc. Exp. Biol. Med., 118, 142

    PubMed  CAS  Google Scholar 

  25. S. G. Schultz and C. K. Strecker, (1970). Fructose influx across the brush border of rabbit ileum. Biochim. Biophys. Acta, 211, 586

    Article  PubMed  CAS  Google Scholar 

  26. A. Holzel, V. Schwarz and K. W. Sutcliffe, (1959). Defective lactose absorption causing malnutrition in infancy. Lancet, 1, 1126

    Article  PubMed  CAS  Google Scholar 

  27. B. Levin, J. M. Abraham, E. A. Burgess and P. G. Wallis, (1970). Congenital lactose malabsorption. Arch. Dis. Child., 45, 173

    Article  PubMed  CAS  Google Scholar 

  28. A. U. Freiburghaus, J. Schmitz, M. Schindler, H. W. Rotthauwe, P. Kuitunen, K. Launiala and B. Hadorn, (1976). Protein patterns of brush border fragments in congenital lactose malabsorption and in specific hypolactasia of the adult. N. Engl. J. Med., 294, 1030

    Article  PubMed  CAS  Google Scholar 

  29. Sunshine and N. Kretchmer, (1964). Intestinal disaccharidases. Absence in two species of sea lions. Science, 144, 850

    Article  PubMed  CAS  Google Scholar 

  30. G. M. Gray, (1978). Intestinal disaccharidase deficiencies and glucose galactose malabsorption. In J. B. Stanberg, J. B. Wyngaarden and D. S. Fredrickson (eds). The Metabolic Basis of Inherited Disease, 4th Ed. pp. 1526–1536. (pmMcGraw Hill Book Company, New York)

    Google Scholar 

  31. N.-G. Asp, N.O. Berg, A. Dahlqvist, J. Jassila and H. Salmi, (1971). The activity of three different small intestinal β-galactosidases in adults with and without lactase deficiency. Scand. J. Gastroenterol, 6, 755

    Article  PubMed  CAS  Google Scholar 

  32. C. Nordstrom and A. Dahlqvist, (1973). Quantitative distribution of some enzymes along the villi and crypts of human small intestine, Scand. J. Gastroenterol, 8, 407

    Google Scholar 

  33. R. K. Crane, D. Menard, H. Preiser and J. J. Cerda, (1976). The molecular basis of brush border membrane disease. In L. Botz, J. F. Hoffman and A. Leaf (eds), Membranes and Diseases: International Conference on Biological Membranes, pp. 229–241. (Raven Press, New York)

    Google Scholar 

  34. N. S. Rosensweig, (1971). Adult lactase deficiency — genetic control or adaptive response? Gastroenterology, 60, 464

    PubMed  CAS  Google Scholar 

  35. T. D. Bolin, R. C. Pirola and A. E. Davis, (1969). Adaptation of intestinal lactase in the rat. Gastroenterology, 57, 406

    PubMed  CAS  Google Scholar 

  36. M. D. Kogut, G. N. Donnell and K. N. F. Shaw, (1967). Studies of lactose absorption in patients with galactosemia. J. Pediatr., 71, 75

    Article  PubMed  CAS  Google Scholar 

  37. O. Ransome-Kuti, N. Kretchmer, J. D. Johnson and J. T. Gribble, (1975). A genetic study of lactose digestion in Nigerian families. Gastroenterology, 68, 431

    PubMed  CAS  Google Scholar 

  38. A. D. Newcomer, H. Gordon, P. J. Thomas and D. B. McGill, (1977). Family studies of lactase deficiency in the American Indian. Gastroenterology, 73,985

    PubMed  CAS  Google Scholar 

  39. J. D. Johnson, F. J. Simoons, R. Hurwitz, A. Grange, C. H. Mitchell, F. Sinatra, P. Surchin, W. V. Robertson, P. H. Bennett and N. Kretchmer, (1977). Lactose malabsorption among the Pima Indians of Arizona. Gastroenterology, 73, 1299

    PubMed  CAS  Google Scholar 

  40. H. A. Weijers, J. H. van de Kramer, D. A. A. Mossel and W. K. Dicke, (1960). Diarrhoea caused by a deficiency of sugar-splitting enzymes. Lancet, 2, 296

    Article  PubMed  CAS  Google Scholar 

  41. M. L. Peterson and R. Herber, (1967). Intestinal sucrase deficiency. Trans. Assoc. Am. Physicians, 80, 275

    PubMed  CAS  Google Scholar 

  42. A. McNair, E. Gudmand-Hjoyer, S. Jarnum and L. Orrild, (1972). Sucrose malabsorption in Greenland. Br. Med. J., 2, 19

    Article  PubMed  CAS  Google Scholar 

  43. I. Antonowicz, J. D. Lloyd Still, K. T. Khaw and H. Shwachman, (1972). Congenital sucrase-isomaltase deficiency. Observations over a period of six years. Pediatrics, 49, 847

    PubMed  CAS  Google Scholar 

  44. M. E. Ament, D. R. Perera and L. J. Esther, (1973). Sucrase-isomaltase deficiency — a frequently misdiagnosed disease. J. Pediatr., 83, 721

    Article  PubMed  CAS  Google Scholar 

  45. R. Dubs, B. Steinmann and R. Gitzelmann, (1973). [atDemonstration of an inactive enzyme antigen in sucrase-isomaltase deficiency. Helv. Paediatr. Acta, 28, 187

    PubMed  CAS  Google Scholar 

  46. A. U. Freiburghaus, R. Dubs, B. Hadorn, H. Gaze, H. P. Hauri and R. Gizelmann, (1977). The brush border membrane in hereditary sucrase-isomaltase deficiency — abnormal protein pattern and presence of immuno-reactive enzyme. Eur. J. Clin. Invest., 7, 455

    Article  PubMed  CAS  Google Scholar 

  47. H. Preiser, D. Menard, R. K. Crane and J. J. Cerda, (1974). Deletion of enzyme protein from the brush border membrane in sucrase-isomaltase deficiency. Biochim. Biophys. Acta, 363,279

    Article  PubMed  CAS  Google Scholar 

  48. J. Schmitz, C. Conmegrain, D. Maestracci and J. Rey, (1974). Absence of brush border sucrase-isomaltase complex in congenital sucrose intolerance. Biomedicine, 21, 440

    PubMed  CAS  Google Scholar 

  49. G. M. Gray, K. A. Conklin and R. R. W. Townley, (1976). Sucrase-isomaltase deficiency. Absence of an inactive enzyme variant. N. Engl. J. Med., 294, 750

    Article  PubMed  CAS  Google Scholar 

  50. J. Madzarovova-Nohejlova, (1973). Trehalase deficiency in a family. Gastroenterology, 65, 130

    PubMed  CAS  Google Scholar 

  51. B. Lindquist and G. W. Meeuwisse, (1962). Chronic diarrhoea caused by monosaccharide malabsorption. Acta Paediatr., 51, 674

    Article  PubMed  CAS  Google Scholar 

  52. A. J. Schneider, W. B. Kinter and C. E. Stirling, (1966). Glucose-galactose malabsorption. N. Engl. J. Med., 274, 305

    Article  PubMed  CAS  Google Scholar 

  53. S. F. Phillips and D. B. McGill, (1973). Glucose-galactose malabsorption in an adult: perfusion studies of sugar, electrolyte, and water transport. Am. J. Dig. Dis., 18, 1017

    Article  PubMed  CAS  Google Scholar 

  54. P. D. Wimberley, J. T. Harries and E. A. Burgess, (1974). Congenital glucose-galactose malabsorption. Proc. R. Soc. Med., 67, 755

    Google Scholar 

  55. W. S. Hughes and J. R. Senior, (1975). The glucose-galactose malabsorption syndrome in a 23 year old woman. Gastroenterology, 68, 142

    PubMed  CAS  Google Scholar 

  56. G. W. Meeuwisse and A. Dahlqvist, (1968). Glucose-galactose malabsorption. A study with biopsy of the small intestinal mucosa. Acta Paediatr. Scand., 57, 273

    Article  PubMed  CAS  Google Scholar 

  57. E. Eggermont and H. Loeb, (1966). Glucose-galactose intolerance. Lancet, 2, 343

    Article  Google Scholar 

  58. L. J. Elsas, R. E. Hillman, J. H. Patterson and L. Rosenberg, (1970). Renal and intestinal hexose transport in familial glucose-galactose malabsorption. J. Clin. Invest., 49, 576

    Article  PubMed  CAS  Google Scholar 

  59. P. D. Fairclough, M. L. Clark, A. M. Dawson, D. B. A. Silk, P. J. Milla and J. T. Harries, (1979). Absorption of glucose and maltose in congenital glucose-galactose malabsorption. Pediatr. Res. (In press)

    Google Scholar 

  60. R. G. Hendrickse, M. A. W. Wooldridge and A. Russell, (1977). Lactulose in baby milks causing diarrhoea simulating lactose intolerance. Br. Med. J., 1, 1194

    Article  PubMed  CAS  Google Scholar 

  61. G. Zoppi, G. Andreotti, F. Pajno-Ferrara, D. M. Njai and D. Gaburro, (1972). Exocrine pancreas function in premature and full term neonates. Pediatr. Res., 6, 880

    Article  PubMed  CAS  Google Scholar 

  62. P. J. P. Roberts and W. J. Whelan, (1960). The mechanism of carbohydrase action. 5. Action of human salivary α-amylase on amylopectin and glycogen. Biochem. J., 76, 246

    PubMed  CAS  Google Scholar 

  63. J. T. Harries and D. E. M. Francis, (1968). Temporary monosaccharide intolerance. Acta Paediatr. Scand., 57, 505

    Article  PubMed  CAS  Google Scholar 

  64. F. Lifshitz, P. Coello-Ramirez and G. Guttierrez-Toppette, (1970). Monosaccharide intolerance and hypoglycaemia in infants with diarrhoea. I. Clinical course in 23 infants. J. Pediatr., 77, 595

    Article  PubMed  CAS  Google Scholar 

  65. F. Lifshitz, P. Coello-Ramirez and G. Guttierrez-Toppette, (1970). Monosaccharide intolerance and hypoglycaemia in infants with diarrhoea. II. Metabolic studies in 23 infants. J. Pediatr., 11, 604

    Google Scholar 

Download references

Authors

Editor information

Editors and Affiliations

Rights and permissions

Reprints and permissions

Copyright information

© 1980 The Society for the Study of Inborn Errors of Metabolism

About this chapter

Cite this chapter

Harries, J.T., Muller, D.P.R. (1980). Basic causes of carbohydrate malabsorption. In: Burman, D., Holton, J.B., Pennock, C.A. (eds) Inherited Disorders of Carbohydrate Metabolism. Springer, Dordrecht. https://doi.org/10.1007/978-94-009-9215-3_2

Download citation

  • DOI: https://doi.org/10.1007/978-94-009-9215-3_2

  • Publisher Name: Springer, Dordrecht

  • Print ISBN: 978-94-009-9217-7

  • Online ISBN: 978-94-009-9215-3

  • eBook Packages: Springer Book Archive

Publish with us

Policies and ethics