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Adequacy of Low-Phenylalanine Diet

Adequacy of Low-Phenylalanine Diet Abstract AN INCREASING number of phenylketonuric infants are being fed a low-phenylalanine diet with the aim of preventing the development of mental retardation. Severe phenylalanine deprivation may result from such a diet unless the serum phenylalanine level is closely monitored and necessary adjustments of intake are promptly made. The observed lag in weight gain and linear growth1,2 in phenylketonuric infants on a low-phenylalanine diet, should not be considered a "necessary evil" or a "minor price to pay," but rather should prompt the search for a potentially correctable dietary imbalance. The consequences of amino acid imbalance on animal growth have been discussed recently by Harper et al.3 If the dietary intake of an indispensable amino acid is maintained at a suboptimal level, any further administration of an amino acid mixture with exclusion of the limiting amino acid is bound to produce more marked signs of protein deficiency, such as growth References 1. Centerwall, W.R., et al: Phenylketonuria: II. Results of Treatment in Infants and Young Children , J Pediat 59:102, 1961.Crossref 2. Fisch, R.O.; Gravem, H.J.; and Feinberg, S.B.: Growth and Bone Characteristics of Phenylketonurics , Amer J Dis Child 112:3, 1966.Crossref 3. Harper, A.E., et al: Some New Thoughts on Amino Acid Imbalance , Fed Proc 23:1087, 1964. 4. Sutherland, B.S.; Umbarger, B.; and Berry, H.K.: The Treatment of Phenylketonuria , Amer J Dis Child 111:505, 1966.Crossref 5. Sherman, J.D.; Greenfield, J.B.; and Ingall, D.: Reversible Bone Marrow Vacuolizations in Phenylketonunia , New Eng J Med 270:810, 1964.Crossref 6. Murdoch, M.D., and Holman, G.H.: Roentgenologic Bone Changes in Phenylketonuria: Relation to Dietary Phenylalanine and Serum Alkaline Phosphatase , Amer J Dis Child 107:523, 1964.Crossref 7. Dodge, P.R., et al: Hypoglycemia Complicating Treatment of Phenylketonuria , New Eng J Med 260:1104, 1959.Crossref 8. Farber, S., and Vawter, G.F.: Clinical Pathological Conference , J Pediat 63:667, 1963.Crossref 9. Hsia, D.Y.: Phenylketonuria: A Study of Human Biochemical Genetics , Pediatrics 38:173, 1966. 10. Benton, J.W., et al: Modification of the Schedule of Myelination in the Rat by Early Nutritional Deprivation , Pediatrics 38:801, 1966. 11. Davison, A.N., and Dobbing, J.: Myelination as a Vulnerable Period in Brain Development , Brit Med Bull 22:40, 1966. 12. Cravioto, J., and Robles, B.: Evolution of Adaptive and Motor Behavior During Rehabilitation From Kwashiorkor , Amer J Orthopsychiat 35:449, 1965.Crossref 13. Centerwall, W.R., et al: Phenylketonuria: I. Dietary Management of Infants and Young Children , J Pediat 59:93, 1961.Crossref http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png American Journal of Diseases of Children American Medical Association

Adequacy of Low-Phenylalanine Diet

American Journal of Diseases of Children , Volume 113 (5) – May 1, 1967

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Publisher
American Medical Association
Copyright
Copyright © 1967 American Medical Association. All Rights Reserved.
ISSN
0002-922X
DOI
10.1001/archpedi.1967.02090200054002
Publisher site
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Abstract

Abstract AN INCREASING number of phenylketonuric infants are being fed a low-phenylalanine diet with the aim of preventing the development of mental retardation. Severe phenylalanine deprivation may result from such a diet unless the serum phenylalanine level is closely monitored and necessary adjustments of intake are promptly made. The observed lag in weight gain and linear growth1,2 in phenylketonuric infants on a low-phenylalanine diet, should not be considered a "necessary evil" or a "minor price to pay," but rather should prompt the search for a potentially correctable dietary imbalance. The consequences of amino acid imbalance on animal growth have been discussed recently by Harper et al.3 If the dietary intake of an indispensable amino acid is maintained at a suboptimal level, any further administration of an amino acid mixture with exclusion of the limiting amino acid is bound to produce more marked signs of protein deficiency, such as growth References 1. Centerwall, W.R., et al: Phenylketonuria: II. Results of Treatment in Infants and Young Children , J Pediat 59:102, 1961.Crossref 2. Fisch, R.O.; Gravem, H.J.; and Feinberg, S.B.: Growth and Bone Characteristics of Phenylketonurics , Amer J Dis Child 112:3, 1966.Crossref 3. Harper, A.E., et al: Some New Thoughts on Amino Acid Imbalance , Fed Proc 23:1087, 1964. 4. Sutherland, B.S.; Umbarger, B.; and Berry, H.K.: The Treatment of Phenylketonuria , Amer J Dis Child 111:505, 1966.Crossref 5. Sherman, J.D.; Greenfield, J.B.; and Ingall, D.: Reversible Bone Marrow Vacuolizations in Phenylketonunia , New Eng J Med 270:810, 1964.Crossref 6. Murdoch, M.D., and Holman, G.H.: Roentgenologic Bone Changes in Phenylketonuria: Relation to Dietary Phenylalanine and Serum Alkaline Phosphatase , Amer J Dis Child 107:523, 1964.Crossref 7. Dodge, P.R., et al: Hypoglycemia Complicating Treatment of Phenylketonuria , New Eng J Med 260:1104, 1959.Crossref 8. Farber, S., and Vawter, G.F.: Clinical Pathological Conference , J Pediat 63:667, 1963.Crossref 9. Hsia, D.Y.: Phenylketonuria: A Study of Human Biochemical Genetics , Pediatrics 38:173, 1966. 10. Benton, J.W., et al: Modification of the Schedule of Myelination in the Rat by Early Nutritional Deprivation , Pediatrics 38:801, 1966. 11. Davison, A.N., and Dobbing, J.: Myelination as a Vulnerable Period in Brain Development , Brit Med Bull 22:40, 1966. 12. Cravioto, J., and Robles, B.: Evolution of Adaptive and Motor Behavior During Rehabilitation From Kwashiorkor , Amer J Orthopsychiat 35:449, 1965.Crossref 13. Centerwall, W.R., et al: Phenylketonuria: I. Dietary Management of Infants and Young Children , J Pediat 59:93, 1961.Crossref

Journal

American Journal of Diseases of ChildrenAmerican Medical Association

Published: May 1, 1967

References