Document Detail

Phenylalanine-tyrosine deficiency syndrome as a complication of the management of hereditary tyrosinemia.
MedLine Citation:
PMID:  835507     Owner:  NLM     Status:  MEDLINE    
A male infant with type I hereditary tyrosinemia developed a phenylalanine-tyrosine deficiency syndrome after receiving a synthetic diet which was low in these amino acids. The syndrome was characterized by growth failure, anorexia, lethargy, and hypotonia. Hypophenylalaninemia and hypotyrosinemia were discovered. The blood concentration of most other amino acids were increased. Supplementation of the patient's diet with phenylalanine and tyrosine resulted in a prompt and dramatic reversal of both clinical and biochemical abnormalities. Dietary therapy had no effect on the child's hepatic cirrhosis.
R M Cohn; M Yudkoff; B Yost; S Segal
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Publication Detail:
Type:  Case Reports; Journal Article; Research Support, U.S. Gov't, P.H.S.    
Journal Detail:
Title:  The American journal of clinical nutrition     Volume:  30     ISSN:  0002-9165     ISO Abbreviation:  Am. J. Clin. Nutr.     Publication Date:  1977 Feb 
Date Detail:
Created Date:  1977-03-31     Completed Date:  1977-03-31     Revised Date:  2006-11-15    
Medline Journal Info:
Nlm Unique ID:  0376027     Medline TA:  Am J Clin Nutr     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  209-14     Citation Subset:  AIM; IM    
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MeSH Terms
Amino Acid Metabolism, Inborn Errors / diet therapy
Amino Acids / blood
Liver Cirrhosis / pathology
Phenylalanine / deficiency*
Tyrosine / blood*,  deficiency
Reg. No./Substance:
0/Amino Acids; 55520-40-6/Tyrosine; 63-91-2/Phenylalanine

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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