How practical are recommendations for dietary control in phenylketonuria?

被引：242

作者：

Walter, JH ^{[1
]}

White, FJ

Hall, SK

MacDonald, A

Rylance, G

Boneh, A

Francis, DE

Shortland, GJ

Schmidt, M

Vail, A

机构：

[1] Royal Manchester Childrens Hosp, Willink Biochem Genet Unit, Manchester M27 4HA, Lancs, England

[2] Birmingham Childrens Hosp, Birmingham, W Midlands, England

[3] Royal Melbourne Childrens Hosp, Murdoch Childrens Res Inst, Melbourne, Vic, Australia

[4] Univ Wales Hosp, Dept Child Hlth, Cardiff CF4 4XW, S Glam, Wales

[5] Univ Manchester, Biostat Grp, Manchester, Lancs, England

来源：

LANCET | 2002年 / 360卷 / 9326期

关键词：

D O I：

10.1016/S0140-6736(02)09334-0

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

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 alms 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.

引用

页码：55 / 57

页数：3