Use of a new amino acid mixture in the diet therapy of patients with classical phenylketonuria
Riva, E.; Paccanelli, S.; Longhi, R.; Giovannini, M.
La Pediatria Medica e Chirurgica Medical and Surgical Pediatrics 5(6): 521-530
1983
ISSN/ISBN: 0391-5387 PMID: 6681058 Document Number: 210466
The treatment of classic PKU is based on the dietary restriction of phenylalanine. The present study was planned to evaluate the effectiveness of a new amino acid mixture (ASP-Nestlé) in the dietary treatment of ten PKU patients (age 2 to 9 years, mean 5 years). The children were given this product for a minimum of 3 and a maximum of 12 months (mean 8 months). The aminoacid mixture, diluted in water or various flavored drinks, was favorably accepted by the patients. No clinical signs of hypophenylalaninemia were noticed and the children grew regularly in height and weight. The biochemical parameters, evaluated before during and after the treatment, showed a good nutritional balance. The plasma phenylalanine control was excellent, when compared to the plasma phenylalanine range obtained with other dietary regimens.