title: Long-chain polyunsaturated fatty acid status in phenylketonuric patients treated with tetrahydrobiopterin.
abstract: OBJECTIVE
  To evaluate LCPUFA composition in PKU patients treated with BH(4).
  
  
  METHODS
  Cross-sectional study of plasma and erythrocyte LCPUFA composition of 13 PKU patients treated with BH(4) compared with data from 48 PKU patients on protein-restricted diet, and 17 mild HPA patients on free diet. PUFA were analysed by gas chromatography.
  
  
  RESULTS
  Plasma and erythrocyte docosahexaenoic acid (DHA), and LCPUFA deficiency markers did not show significant differences in PKU patients on BH(4) compared with those with mild HPA and our reference values, but they did in comparison with PKU on protein-restricted diet (p<0.0001). Essential fatty acids and arachidonic acid composition were not significantly different in any of the studied groups. DHA values correlate with the index of dietary control only in PKU patients on protein-restricted diet (p=0.002).
  
  
  CONCLUSIONS
  LCPUFA status is within the reference values in PKU patients treated with BH(4). This translates to a further advantage of BH(4) therapy.
authors: Department of Biochemistry, Barcelona, Spain. vilaseca@hsjdbcn.org
authors: Maria A
authors: MA
authors: Vilaseca
authors: Nilo
authors: N
authors: Lambruschini
authors: Lilianne
authors: L
authors: Gómez-López
authors: Alejandra
authors: A
authors: Gutiérrez
authors: Juan
authors: J
authors: Moreno
authors: Mireia
authors: M
authors: Tondo
authors: Rafael
authors: R
authors: Artuch
authors: Jaume
authors: J
authors: Campistol
mesh_terms: D000293:Adolescent; D001708:Biopterins / Q000031:analogs & derivatives* / Q000627:therapeutic use; D002648:Child; D002675:Child, Preschool; D004032:Diet; D004281:Docosahexaenoic Acids / Q000097:blood; D004912:Erythrocytes / Q000378:metabolism; D005231:Fatty Acids, Unsaturated / Q000097:blood*; D006801:Humans; D007223:Infant; D010661:Phenylketonurias / Q000097:blood* / Q000188:drug therapy*; D010743:Phospholipids / Q000097:blood; D012016:Reference Values
chemical_list: D005231:Fatty Acids, Unsaturated; D010743:Phospholipids; D001708:Biopterins; D004281:Docosahexaenoic Acids; C003402:sapropterin