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Effect of Phenylalanine-Restricted Diet in Phenylketonuria II

Effect of Phenylalanine-Restricted Diet in Phenylketonuria II Abstract In an earlier paper two of us (F. A. H. and C. W. S.) presented the short-term psychological and behavioral changes observed in two preschool phenylketonuric children treated with a phenylalanine-restricted diet.1 In addition, a newborn sibling of one of the cases, who had received dietary treatment since 8 weeks of age, was reported as developing normally through the first 9 months of life. The present paper offers an additional year of follow-up on these three patients. It also reports on the changes observed in a second newborn and two 18-month-old children under long-term therapy. Dietary Program The phenylalanine-restricted diet used in this series is that outlined by Armstrong and Tyler.2,3 Patients are given a phenylalanine-free diet consisting of a protein hydrolysate basal mix* (4.2 gm/kg/day), fat in the form of a vegetable oil (3.0 gm/kg/day) and CHO (10 gm/kg/day) for three weeks. On such a diet References 1. Horner, F. A., and Streamer, C. W.: Effect of a Phenylalanine-Restricted Diet on Patients with Phenylketonuria , J. A. M. A. 161:1628 ( (Aug. 25) ) 1956.Crossref 2. Tyler, F. H., and Armstrong, M. D.: Diseases of Nervous System: Metabolic Aspects of Some Neurological and Muscular Disorders , Ann. Rev. Med. 5:207, 1954.Crossref 3. Armstrong, M. D., and Tyler, F. H.: Studies on Phenylketonuria: I. Restricted Phenylalanine Intake in Phenylketonuria , J. Clin. Invest. 34:565 ( (April) ) 1955.Crossref 4. Armstrong, M. D., and Binkley, E. L.: Studies on Phenylketonuria: V. , Proc. Soc. Exper. Biol. & Med. 93:418 ( (Dec.) ) 1956. 5. Jervis, G. A.: Phenylpyruvic Oligophrenia: Introductory Study of 50 Cases of Mental Deficiency Associated with Excretion of Phenylpyruvic Acid , Arch. Neur. & Psychiat. 38:944 ( (Nov.) ) 1937. 6. Bickel, H.; Gerrard, J., and Hickmans, E. M.: Influence of Phenylalanine Intake on Chemistry and Behaviour of a Phenylketonuric Child , Acta Paediat. 43:64 ( (Jan.) ) 1954. 7. Woolf, L. I.; Griffiths, R., and Moncrieff, A.: Treatment of Phenylketonuria with a Diet Low in Phenylalanine , Brit. M. J. 1:57 ( (Jan. 8) ) 1955. 8. Berendes, H.: Paper presented at Western Society for Pediatric Research, Fourth Annual Meeting, Salt Lake City, October, 1956. 9. Low, N. L.; Armstrong, M. D., and Carlisle, J. W.: Phenylketonuria: Two Unusual Cases , Lancet 2:917 ( (Nov. 3) ) 1956. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png A.M.A. Journal of Diseases of Children American Medical Association

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References (11)

Publisher
American Medical Association
Copyright
Copyright © 1957 American Medical Association. All Rights Reserved.
ISSN
0096-6916
DOI
10.1001/archpedi.1957.02060040617003
Publisher site
See Article on Publisher Site

Abstract

Abstract In an earlier paper two of us (F. A. H. and C. W. S.) presented the short-term psychological and behavioral changes observed in two preschool phenylketonuric children treated with a phenylalanine-restricted diet.1 In addition, a newborn sibling of one of the cases, who had received dietary treatment since 8 weeks of age, was reported as developing normally through the first 9 months of life. The present paper offers an additional year of follow-up on these three patients. It also reports on the changes observed in a second newborn and two 18-month-old children under long-term therapy. Dietary Program The phenylalanine-restricted diet used in this series is that outlined by Armstrong and Tyler.2,3 Patients are given a phenylalanine-free diet consisting of a protein hydrolysate basal mix* (4.2 gm/kg/day), fat in the form of a vegetable oil (3.0 gm/kg/day) and CHO (10 gm/kg/day) for three weeks. On such a diet References 1. Horner, F. A., and Streamer, C. W.: Effect of a Phenylalanine-Restricted Diet on Patients with Phenylketonuria , J. A. M. A. 161:1628 ( (Aug. 25) ) 1956.Crossref 2. Tyler, F. H., and Armstrong, M. D.: Diseases of Nervous System: Metabolic Aspects of Some Neurological and Muscular Disorders , Ann. Rev. Med. 5:207, 1954.Crossref 3. Armstrong, M. D., and Tyler, F. H.: Studies on Phenylketonuria: I. Restricted Phenylalanine Intake in Phenylketonuria , J. Clin. Invest. 34:565 ( (April) ) 1955.Crossref 4. Armstrong, M. D., and Binkley, E. L.: Studies on Phenylketonuria: V. , Proc. Soc. Exper. Biol. & Med. 93:418 ( (Dec.) ) 1956. 5. Jervis, G. A.: Phenylpyruvic Oligophrenia: Introductory Study of 50 Cases of Mental Deficiency Associated with Excretion of Phenylpyruvic Acid , Arch. Neur. & Psychiat. 38:944 ( (Nov.) ) 1937. 6. Bickel, H.; Gerrard, J., and Hickmans, E. M.: Influence of Phenylalanine Intake on Chemistry and Behaviour of a Phenylketonuric Child , Acta Paediat. 43:64 ( (Jan.) ) 1954. 7. Woolf, L. I.; Griffiths, R., and Moncrieff, A.: Treatment of Phenylketonuria with a Diet Low in Phenylalanine , Brit. M. J. 1:57 ( (Jan. 8) ) 1955. 8. Berendes, H.: Paper presented at Western Society for Pediatric Research, Fourth Annual Meeting, Salt Lake City, October, 1956. 9. Low, N. L.; Armstrong, M. D., and Carlisle, J. W.: Phenylketonuria: Two Unusual Cases , Lancet 2:917 ( (Nov. 3) ) 1956.

Journal

A.M.A. Journal of Diseases of ChildrenAmerican Medical Association

Published: Jun 1, 1957

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