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Hereditary Fructose Intolerance in Childhood: Diagnosis, Management, and Course in 55 Patients

Hereditary Fructose Intolerance in Childhood: Diagnosis, Management, and Course in 55 Patients Abstract • The early manifestations of hereditary fructose intolerance are described in a series of 55 patients. Management of this metabolic disorder depends on the severity of liver impairment. When the patients are given a fructose-free diet, the improvement is dramatic but liver enlargement and fatty vacuolization of liver cells often persist. These hepatic findings were also observed in the five homozygous infants who were given a fructosefree diet from birth; this outcome may support the hypothesis that minimal amounts of fructose are essential for human beings. (Am J Dis Child 132:605-608, 1978) References 1. Cornblath M, Schwartz R: Disorders of Carbohydrate Metabolism in Infancy . Philadelphia, WB Saunders Co, 1976, p 325. 2. Odièvre M, Poirier C, Levillain P, et al: Etude des réponses glucosémiques, lactacidémiques et insulinémiques après administration intraveineuse rapide de doses variables de fructose chez l'enfant normal . Arch Fr Pédiatr 27:1057-1068, 1970. 3. Sibley JA, Lehninger AO: Determination of aldolase in animal tissues . J Biol Chem 177:859-872, 1949. 4. Beutler E, Baluda M: A simple spot screening test for galactosemia . J Lab Clin Med 68:137-141, 1966. 5. Cornblath M, Rosenthal IM, Reisner SH, et al.: Hereditary fructose intolerance . N Engl J Med 269:1271-1278, 1963.Crossref 6. Froesch ER, Wolf HP, Baitsch H, et al: Hereditary fructose intolerance: An inborn defect of hepatic fructose-1-phosphate splitting aldolase . Am J Med 34:151-157, 1963.Crossref 7. Levin B, Snodgrass GJAI, Oberholzer VG, et al: Fructosaemia: Observations on seven cases . Am J Med 45:826-838, 1968.Crossref 8. Odièvre M: Les difficultés du diagnostic de l'intolérance héréditaire au fructose chez le nourrisson . Arch Fr Pédiatr 26:5-19, 1969. 9. Dubois R, Loeb H, Malaisse-Lagae F, et al: Etude clinique et anatomopathologique de deux cas d'intolérance congénitale au fructose . Pédiatrie 20:5-14, 1965. 10. Morris RCJ: An experimental renal acidification defect in patients with hereditary fructose intolerance . J Clin Invest 47:1648-1663, 1968.Crossref 11. Odièvre M, Gautier M, Rieu D: Intolérance héréditaire au fructose du nourrisson: Evolution des lésions histologiques hépatiques sous traitement diététique prolongé . Arch Fr Pédiatr 26:433-443, 1969. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png American Journal of Diseases of Children American Medical Association

Hereditary Fructose Intolerance in Childhood: Diagnosis, Management, and Course in 55 Patients

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Publisher
American Medical Association
Copyright
Copyright © 1978 American Medical Association. All Rights Reserved.
ISSN
0002-922X
DOI
10.1001/archpedi.1978.02120310069014
Publisher site
See Article on Publisher Site

Abstract

Abstract • The early manifestations of hereditary fructose intolerance are described in a series of 55 patients. Management of this metabolic disorder depends on the severity of liver impairment. When the patients are given a fructose-free diet, the improvement is dramatic but liver enlargement and fatty vacuolization of liver cells often persist. These hepatic findings were also observed in the five homozygous infants who were given a fructosefree diet from birth; this outcome may support the hypothesis that minimal amounts of fructose are essential for human beings. (Am J Dis Child 132:605-608, 1978) References 1. Cornblath M, Schwartz R: Disorders of Carbohydrate Metabolism in Infancy . Philadelphia, WB Saunders Co, 1976, p 325. 2. Odièvre M, Poirier C, Levillain P, et al: Etude des réponses glucosémiques, lactacidémiques et insulinémiques après administration intraveineuse rapide de doses variables de fructose chez l'enfant normal . Arch Fr Pédiatr 27:1057-1068, 1970. 3. Sibley JA, Lehninger AO: Determination of aldolase in animal tissues . J Biol Chem 177:859-872, 1949. 4. Beutler E, Baluda M: A simple spot screening test for galactosemia . J Lab Clin Med 68:137-141, 1966. 5. Cornblath M, Rosenthal IM, Reisner SH, et al.: Hereditary fructose intolerance . N Engl J Med 269:1271-1278, 1963.Crossref 6. Froesch ER, Wolf HP, Baitsch H, et al: Hereditary fructose intolerance: An inborn defect of hepatic fructose-1-phosphate splitting aldolase . Am J Med 34:151-157, 1963.Crossref 7. Levin B, Snodgrass GJAI, Oberholzer VG, et al: Fructosaemia: Observations on seven cases . Am J Med 45:826-838, 1968.Crossref 8. Odièvre M: Les difficultés du diagnostic de l'intolérance héréditaire au fructose chez le nourrisson . Arch Fr Pédiatr 26:5-19, 1969. 9. Dubois R, Loeb H, Malaisse-Lagae F, et al: Etude clinique et anatomopathologique de deux cas d'intolérance congénitale au fructose . Pédiatrie 20:5-14, 1965. 10. Morris RCJ: An experimental renal acidification defect in patients with hereditary fructose intolerance . J Clin Invest 47:1648-1663, 1968.Crossref 11. Odièvre M, Gautier M, Rieu D: Intolérance héréditaire au fructose du nourrisson: Evolution des lésions histologiques hépatiques sous traitement diététique prolongé . Arch Fr Pédiatr 26:433-443, 1969.

Journal

American Journal of Diseases of ChildrenAmerican Medical Association

Published: Jun 1, 1978

References