title: Inborn errors of metabolism with a protein-restricted diet: effect on polyunsaturated fatty acids.
abstract: Previous studies have shown that phenylketonuric patients display a deficiency in long-chain polyunsaturated fatty acids. A study has now been performed on 13 cases (5 with methylmalonic acidaemia and 8 with urea cycle disorders) whose dietary treatment also implies a limitation in protein-rich food. Plasma and red-cell phospholipid fatty acid profiles were studied. The most relevant results were a lower percentage of docosahexaenoic acid in plasma and red-cell phospholipids (0.91% +/- 0.53% vs 2.88% +/- 1.17% and 2.07% +/- 0.92% vs 3.62% +/- 1.01% (p < 0.001)) and a lower percentage of arachidonic acid in plasma (5.22% +/- 2.02% vs 8.3% +/- 2.11% (p < 0.001)). A long-chain polyunsaturated acid deficiency has also been confirmed in this group of metabolic patients and a dietary supplement is recommended since this population is subject to a special risk factor with regard to adequate psychomotor development. By extrapolating these data to the general population, the possibility can be inferred that long-chain polyunsaturated fatty acids are semi-essential in infant nutrition far beyond the breast-feeding period.
authors.affiliation: Department of Pediatrics, Cruces Hospital, University of the Basque Country, Spain.
authors.forename: P
authors.initials: P
authors.lastname: Sanjurjo
authors.forename: J I
authors.initials: JI
authors.lastname: Ruiz
authors.forename: M
authors.initials: M
authors.lastname: Montejo
mesh_terms: D000592:Amino Acid Metabolism, Inborn Errors / Q000097:blood / Q000178:diet therapy; D016718:Arachidonic Acid / Q000097:blood; D002648:Child; D002675:Child, Preschool; D002956:Citrulline / Q000097:blood; D018753:Diet, Protein-Restricted / Q000009:adverse effects*; D004281:Docosahexaenoic Acids / Q000097:blood; D005229:Fatty Acids, Monounsaturated / Q000097:blood; D005231:Fatty Acids, Unsaturated / Q000097:blood*; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D019787:Linoleic Acid / Q000097:blood; D008661:Metabolism, Inborn Errors / Q000097:blood / Q000178:diet therapy*; D008764:Methylmalonic Acid / Q000097:blood; D019814:Myristic Acid / Q000097:blood; D020163:Ornithine Carbamoyltransferase Deficiency Disease; D010743:Phospholipids / Q000097:blood; D017962:alpha-Linolenic Acid / Q000097:blood
chemical_list: D005229:Fatty Acids, Monounsaturated; D005231:Fatty Acids, Unsaturated; D010743:Phospholipids; D019814:Myristic Acid; D017962:alpha-Linolenic Acid; C008757:palmitoleic acid; D004281:Docosahexaenoic Acids; D016718:Arachidonic Acid; D002956:Citrulline; D008764:Methylmalonic Acid; D019787:Linoleic Acid