title: Dietary long-chain polyunsaturated fatty acid supplementation in infants with phenylketonuria: a randomized controlled trial.
abstract: BACKGROUND
  Pre- and postnatal tissue accretion of long-chain polyunsaturated fatty acids (LCPUFA) has been related to visual and cognitive development in healthy children in several studies. Children with phenylketonuria (PKU) consume diets with very low contents of preformed LCPUFA. We studied prospectively the LCPUFA status in infants with PKU without or with LCPUFA supplementation during the first year of life.
  
  
  METHODS
  Infants with PKU were enrolled at diagnosis (<4 weeks of age) and randomized double blind to phenylalanine-free amino acid supplements without LCPUFA (n = 11) or with both arachidonic (AA, 0.46 wt%) and docosahexaenoic acids (DHA, 0.27 wt%) (n = 10). At enrolment and again at 1, 2, 3, 4, 6, 9 and 12 months, plasma phospholipid fatty acids were measured and dietary intakes were calculated from dietary protocols.
  
  
  RESULTS
  Unsupplemented patients showed a marked LCPUFA depletion to levels clearly below those observed in healthy breast-fed infants. In contrast, supplemented infants had stable and higher LCPUFA levels than unsupplemented infants, reaching significant differences for AA values at 3, 4 and 6 months, and for DHA values at 1, 3, 4, 6, 9 and 12 months. Plasma phospholipid levels correlated closely with estimated dietary intakes of preformed LCPUFA.
  
  
  CONCLUSIONS
  Low LCPUFA intakes with PKU diets induce marked depletion of AA and particularly of DHA in the first year of life. Thus endogenous synthesis of LCPUFA from precursors supplied by diet seems unable to compensate for low LCPUFA intakes. LCPUFA supplementation of PKU diets during the first year of life effectively enhances LCPUFA status to levels comparable to those of healthy breast-fed infants.
authors: Department of Paediatrics, University of Munich, Munich, Germany. Berthold.Koletzko@med.uni-muenchen.de
authors: B
authors: B
authors: Koletzko
authors: T
authors: T
authors: Sauerwald
authors: H
authors: H
authors: Demmelmair
authors: M
authors: M
authors: Herzog
authors: U
authors: U
authors: von Schenck
authors: H
authors: H
authors: Böhles
authors: U
authors: U
authors: Wendel
authors: J
authors: J
authors: Seidel
mesh_terms: D001724:Birth Weight; D049628:Body Size; D001835:Body Weight / Q000187:drug effects; D019587:Dietary Supplements; D005231:Fatty Acids, Unsaturated / Q000627:therapeutic use*; D005504:Food Analysis; D005865:Gestational Age; D006801:Humans; D007223:Infant; D007225:Infant Food; D007231:Infant, Newborn; D010661:Phenylketonurias / Q000188:drug therapy*
chemical_list: D005231:Fatty Acids, Unsaturated