An investigation into diet treatment for adults with previously untreated phenylketonuria and severe intellectual disability.
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.
Fitzgerald
B
B
Learning Difficulties Service, Chase Farm Hospital, Enfield, UK. brian.fitzgerald@virgin.net
Morgan
J
J
Keene
N
N
Rollinson
R
R
Hodgson
A
A
Dalrymple-Smith
J
J
D000328:Adult; D000367:Age Factors; D018753:Diet, Protein-Restricted; D005260:Female; D006801:Humans; D008607:Intellectual Disability / Q000178:diet therapy* / Q000209:etiology / Q000523:psychology*; D008297:Male; D008875:Middle Aged; D008991:Monitoring, Physiologic; D010349:Patient Compliance; D010649:Phenylalanine / Q000378:metabolism; D010661:Phenylketonurias / Q000150:complications / Q000178:diet therapy* / Q000523:psychology*; D011446:Prospective Studies; D016896:Treatment Outcome
D010649:Phenylalanine