An investigation into diet treatment for adults with previously untreated phenylketonuria and severe intellectual disability

J Intellect Disabil Res. 2000 Feb:44 ( Pt 1):53-9. doi: 10.1046/j.1365-2788.2000.00260.x.

Abstract

There is evidence in the literature which suggests that adults with previously untreated phenylketonuria (PKU) benefit from a low phenylalanine diet. A prospective study providing a phenylalanine-restricted diet to five subjects with severe intellectual disability arising from untreated PKU is reported. Physical, social and behavioural measures were used to monitor the effects of the diet Four out of the five subjects derived considerable benefit. It is concluded that the restricted diet is worth trying in most individuals with previously untreated PKU, and that possible benefits are in the areas of concentration, alertness, mood, irritability and adaptive behaviour.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Age Factors
  • Diet, Protein-Restricted
  • Female
  • Humans
  • Intellectual Disability / diet therapy*
  • Intellectual Disability / etiology
  • Intellectual Disability / psychology*
  • Male
  • Middle Aged
  • Monitoring, Physiologic
  • Patient Compliance
  • Phenylalanine / metabolism
  • Phenylketonurias / complications
  • Phenylketonurias / diet therapy*
  • Phenylketonurias / psychology*
  • Prospective Studies
  • Treatment Outcome

Substances

  • Phenylalanine