How practical are recommendations for dietary control in phenylketonuria?
In patients with phenylketonuria, blood phenylalanine concentration during childhood is the major determinant of cognitive outcome. Guidelines provide age-related recommendations for treatment. To ascertain the extent to which these aims are achievable, we audited results from four centres for the years 1994-2000. The median proportion of samples with phenylalanine concentrations above those recommended was less than 30% for those younger than age 10 years but almost 80% for those aged 15 years and older. Similarly, the median frequency of blood sampling, expressed as a proportion of that recommended, was more than 80% for patients younger than 10 years but less than 50% by age 15 years. Our results indicate the difficulty of maintaining control in phenylketonuria, especially in older rather than younger children.
Walter
J H
JH
Willink Biochemical Genetic Unit, Royal Manchester Children's Hospital, Manchester M27 4HA, UK. jhwalter@doctors.org.uk
White
F J
FJ
Hall
S K
SK
MacDonald
A
A
Rylance
G
G
Boneh
A
A
Francis
D E
DE
Shortland
G J
GJ
Schmidt
M
M
Vail
A
A
D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D006801:Humans; D007223:Infant; D015337:Multicenter Studies as Topic; D010349:Patient Compliance; D010649:Phenylalanine / Q000097:blood; D010661:Phenylketonurias / Q000178:diet therapy*; D016896:Treatment Outcome
D010649:Phenylalanine