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An investigation of the hyperaminoaciduria in phenylketonuria associated with the feeding of certain commercial low-phenylalanine preparations

Published online by Cambridge University Press:  09 March 2007

Barbara E. Clayton ,
A. F. Heeley  and
Mary Heeley
Barbara E. Clayton
Affiliation:
Department of Chemical Pathology, The Hospital for Sick Children and Institure of Child Health, London, WC 1
A. F. Heeley
Affiliation:
Department of Chemical Pathology, The Hospital for Sick Children and Institure of Child Health, London, WC 1
Mary Heeley
Affiliation:
Department of Chemical Pathology, The Hospital for Sick Children and Institure of Child Health, London, WC 1
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Abstract

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1. Hypertryptophanaemia, hypertryptophanuria and to a lesser degree a generalized hyperaminoaciduria were observed in phenylketonuric patients receiving certain commercial low-phenylalanine diets containing DL-tryptophan.

2. The generalized hyperaminoaciduria was associated with the ingestion of acid hydro-lysates of protein, but not with the ingestion of enzymic hydrolysates or D-tryptophan.

3. Alanine recovered from the urine of these treated patients had a D-isomer content of approx. 50%. This amount of urinary D-alanine could be derived from the ingestion of an acid hydrolysate of protein in which the amino acids had racemized to the extent of 2–3%.

Type
Research Article
Information
British Journal of Nutrition , Volume 24 , Issue 2 , June 1970 , pp. 573 - 580
Copyright
Copyright © The Nutrition Society 1970

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