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September 1957

The Dietary Phenylalanine Requirements and Tolerances of Phenylketonuric Patients

Author Affiliations

From the Department of Pediatrics, Harvard Medical School, and the Department of Medicine, The Children's Medical Center. Present address: Children's Memorial Hospital, 707 Fullerton Ave., Chicago 14 (Dr. Hsia).

AMA Am J Dis Child. 1957;94(3):224-230. doi:10.1001/archpedi.1957.04030040006002

Introduction  Recently Rose and his co-workers 1 have shown that normal adults require a minimum of 0.8 to 1.1 gm. per day of L-phenylalanine, but that this figure may be reduced to about 0.3 gm. per day in the presence of excess tyrosine.2 Snyderman and her associates3 have estimated the minimal L-phenylalanine requirement of normal infants at 90 mg. per kilogram per day, but 60 mg. per kilogram per day sufficed in three of the six infants tested. Information has not been previously available as to the minimal or optimal phenylalanine intake of patients with phenylketonuria, and this becomes a point of some importance because of the low-phenylalanine diets currently being administered as a form of experimental treatment.4-10 The body tissues of phenylketonuric patients contain phenylalanine in the same proportion as the tissues of normal persons,11 and one must thus aim to provide sufficient dietary phenylalanine

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