Children with phenylalanine-hydroxylase deficiency (type-I hyperphenylalaninemia, HPA) follow a low-phenylalanine diet, severely restricted in animal foods and long-chain polyunsaturated fatty acids (LCPUFA). Consequently, they have a poor LCPUFA status, particularly for docosahexaenoic acid (DHA). DHA is relevant to visual and neural development.
Effects of long-chain polyunsaturated fatty acid supplementation on fatty acid status and visual function in treated children with hyperphenylalaninemia / C. Agostoni, N. Massetto, G. Biasucci, A. Rottoli, M. Bonvissuto, M. G. Bruzzese, M. Giovannini, E. Riva. - In: THE JOURNAL OF PEDIATRICS. - ISSN 0022-3476. - 137:4(2000 Oct), pp. 504-509.
Effects of long-chain polyunsaturated fatty acid supplementation on fatty acid status and visual function in treated children with hyperphenylalaninemia
C. AgostoniPrimo
;M. GiovanniniPenultimo
;E. RivaUltimo
2000
Abstract
Children with phenylalanine-hydroxylase deficiency (type-I hyperphenylalaninemia, HPA) follow a low-phenylalanine diet, severely restricted in animal foods and long-chain polyunsaturated fatty acids (LCPUFA). Consequently, they have a poor LCPUFA status, particularly for docosahexaenoic acid (DHA). DHA is relevant to visual and neural development.
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