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Phenylketonuria: Dietary and therapeutic challenges
Author(s) -
Giovannini M.,
Verduci E.,
Salvatici E.,
Fiori L.,
Riva E.
Publication year - 2007
Publication title -
journal of inherited metabolic disease
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.462
H-Index - 102
eISSN - 1573-2665
pISSN - 0141-8955
DOI - 10.1007/s10545-007-0552-8
Subject(s) - polyunsaturated fatty acid , docosahexaenoic acid , tetrahydrobiopterin , medicine , long chain , endocrinology , biology , physiology , fatty acid , biochemistry , chemistry , nitric oxide synthase , nitric oxide , polymer science
Summary PKU subjects need special attention in the definition of optimal supplementation of nutrients, which may be insufficient in relation to the type of diet and may otherwise manifest symptoms of deficit. In particular, it is necessary to pay great attention to the long‐chain polyunsaturated fatty acid (LC‐PUFA) levels in relation to correct development of the central nervous system. On the basis of numerous beneficial effects currently known, a permanent supplementation with LC‐PUFAs, in particular with docosahexaenoic acid, should be considered. Moreover, new formulas, Phe‐free peptides, and ‘modulated’ amino acid preparations might help in preventing nutritional deficiencies and imbalances, with the ultimate aim of improving growth. New strategies—such as supply of tetrahydrobiopterin—need to be optimized in terms of targets, patients and expected outcomes.