Lipid status and fatty acid metabolism in phenylketonuria
| Autor: | Giacomo Biasucci, Carlo Agostoni, Elisabetta Riva, Marcello Giovannini, D. Luotti |
|---|---|
| Rok vydání: | 1995 |
| Předmět: |
congenital
hereditary and neonatal diseases and abnormalities medicine.medical_specialty Adolescent Diet therapy Biology chemistry.chemical_compound Internal medicine Phenylketonurias Genetics medicine Humans Child Genetics (clinical) chemistry.chemical_classification Fatty acid metabolism Cholesterol Fatty Acids Infant Newborn nutritional and metabolic diseases Infant Lipid metabolism Lipid Metabolism Lipids Endocrinology chemistry Eicosanoid Docosahexaenoic acid Child Preschool lipids (amino acids peptides and proteins) Arachidonic acid Polyunsaturated fatty acid |
| Zdroj: | Journal of inherited metabolic disease. 18(3) |
| ISSN: | 0141-8955 |
| Popis: | Owing to dietary treatment, patients with phenylketonuria (PKU) are supplied with only small amounts of animal fats and show low blood cholesterol levels and a poor long-chain polyunsaturated fatty acid (LC-PUFA) status. Endogenous synthesis should compensate for the physiological need for cholesterol for growth, but supplementary exogenous LC-PUFA seems advisable. PKU infants could develop depletion of docosahexaenoic acid, fundamental for the functional development of the central nervous system. The availability of arachidonic acid from plasma in PKU has been found to be related to dietary compliance, and seems to influence the synthesis of arachidonate-derived eicosanoids. Trials of dietary supplementation of PKU patients with oils containing LC-PUFA are in progress, but the functional consequences of these supplementations need further investigation. |
| Databáze: | OpenAIRE |
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