Abstract
Fructose is one of the main sweetening agents in the human diet. It is found in the free form in honey, fruit, and many vegetables and associated with glucose in the form of the disaccharide sucrose in even more numerous foods and beverages. Sorbitol, also widely distributed in fruit and vegetables, is converted into fructose in the liver by sorbitol dehydrogenase (Fig. 1). Two inborn errors of fructose metabolism are known. Essential fructosuria is a completely harmless anomaly characterized by the appearance of fructose in the urine after the intake of fructose-containing foods. In hereditary fructose intolerance (HFI), fructose provokes prompt gastrointestinal discomfort and hypoglycemia upon ingestion, although sensitivity varies from patient to patient; it may cause liver and kidney failure when taken persistently and becomes life-threatening when given intravenously. Fructose-1,6-bisphosphatase deficiency, sometimes also considered an inborn error of fructose metabolism, will be discussed in Chap. 8. It is manifested by the appearance of hypoglycemia and lactic acidosis during fasting and may also be life-threatening.
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References
Steinitz H, Mizrahy 0 (1969) Essential fructosuria and hereditary fructose intolerance. N Engl J Med 280: 222
Shapira F, Shapira G, Dreyfus JC (1961/1962) La lésion enzymatique de la fructosurie bénigne. Enzymol Biol Clin 1: 170–175
Kraffczyk F, Helger R, Bremer HJ (1972) Thin-layer chromatographic screening tests for carbohydrate anomalies in plasma, urine and faeces. Clin Chim Acta 42: 303–308
Lasker M (1941) Essential fructosuria. Hum Biol 13: 51–63
Froesch ER, Prader A, Labhart A, Stuber HW, Wolf HP (1957) Die hereditäre Fructoseintoleranz, eine bisher nicht bekannte kongenitale Stoffwechselstörung. Schweiz Med Wochenschr 87: 1168–1171
Baerlocher K, Gitzelmann R, Steinmann B, Gitzelmann-Cumarasamy N (1978) Hereditary fructose intolerance in early childhood: a major diagnostic challenge. Survey of 20 symptomatic cases. Helv Paediatr Acta 33: 465–487
Mock DM, Perman JA, Thaler MM, Morris RC Jr (1983) Chronic fructose intoxication after infancy in children with hereditary fructose intolerance. A cause of growth retardation. N Engl J Med 309: 764–770
Lameire N, Mussche M, Baele G, Kint J, Ringoir S (1978) Hereditary fructose intolerance: a difficult diagnosis in the adult. Am J Med 65: 416–423
Hers HG, Joassin G (1961) Anomalie de l’aldolase hépatique dans l’intolérance au fructose. Enzymol Biol Clin 1: 4–14
Van den Berghe G (1978) Metabolic effects of fructose in the liver. Curr Top Cell Regul 13: 97–135
Oberhaensli RD, Rajagopalan B, Taylor DJ, Radda GK, Collins JE, Leonard JV, Schwarz HP, Herschkowitz N (1987) Study of hereditary fructose intolerance by use of 31P magnetic resonance spectroscopy. Lancet ii: 931–934
Woods HF, Alberti KGMM (1972) Dangers of intravenous fructose. Lancet ii: 1354–1357
Steinmann B, Gitzelmann R (1981) The diagnosis of hereditary fructose intolerance. Helv Paediatr Acta 36: 297–316
Cornblath M, Schwartz R (1991) Hereditary fructose intolerance. In: Cornblath M, Schwartz R (eds) Disorders of carbohydrate metabolism in infancy. 3rd edn. Blackwell, London, pp 391–403
Odièvre M, Gentil C, Gautier M, Alagille D (1978) Hereditary fructose intolerance in childhood. Diagnosis, management and course in 55 patients. Am J Dis Child 132: 605–608
Cross NCP, DeFranchis R, Sebastio G, Dazzo C, Tolan DR, Gregori C, Odièvre M, Vidail-het M, Romano V, Mascali G, Romano C, Musumeci S, Steinmann B, Gitzelmann R, Cox TM (1990) Molecular analysis of aldolase B genes in hereditary fructose intolerance. Lancet is 306–309
Cross NCP, Cox TM (1990) Hereditary fructose intolerance. Int J Biochem 22: 685–689
Tolan DR, Brooks CC (1992) Molecular analysis of common aldolase B alleles for hereditary fructose intolerance in North Americans. Biochem Med Metab Biol 48: 19–25
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© 1995 Springer-Verlag Berlin Heidelberg
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Van den Berghe, G. (1995). Disorders of Fructose Metabolism. In: Fernandes, J., Saudubray, JM., Van den Berghe, G., Tada, K., Buist, N.R.M. (eds) Inborn Metabolic Diseases. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-662-03147-6_7
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DOI: https://doi.org/10.1007/978-3-662-03147-6_7
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