Impaired prenatal and postnatal growth in Dutch patients with phenylketonuria. The National PKU Steering Committee.
AUTOR(ES)
Verkerk, P H
RESUMO
OBJECTIVE--To assess whether physical growth is affected in early treated Dutch patients with phenylketonuria (PKU). METHODS--The birth weights of all 137 early detected patients with PKU born in the period from 1974 to 1988 in the Netherlands were compared with reference values. Height, head circumference, and weight were measured at the age at which treatment started (commonly about 2-3 weeks), at 6 months of age, and yearly from the child's first birthday up to the age of 10 years. These measurements were compared with reference values. RESULTS--The adjusted birth weight in patients with PKU was 141 g (95% confidence interval (CI) 66 to 216 g) less than Dutch reference values by Kloosterman and 103 g (95% CI 9 to 196 g) less compared with the birth weight of another reference group. At the age at which treatment started, z scores of patients for height by age were -0.23 (95% CI -0.44 to -0.02) and z scores for head circumference by the age were -0.25 (95% CI -0.44 to -0.06). From the age at which treatment started up to the age of 3 years z scores for height by age further decreased to -0.74 (95% confidence interval -0.93 to -0.56), after which no additional decrease occurred. In contrast, z scores for head circumference increased from -0.25 at the first visit to 0.08 (95% CI -0.14 to 0.30) at the age of 1 year, after which they remained close to zero. Weight by height was close to the expected centiles for all ages. CONCLUSION--Patients with PKU are growth retarded at birth and have smaller head circumferences than the normal population. In Dutch patients further growth retardation occurs in the first three years of life.
ACESSO AO ARTIGO
http://www.pubmedcentral.nih.gov/articlerender.fcgi?artid=1029938Documentos Relacionados
- Significant phenylalanine hydroxylation in vivo in patients with classical phenylketonuria.
- Time course from first symptom to treatment in patients with non-small cell lung cancer referred for radiotherapy: a report by the CHART Steering Committee.
- Royal College of Pathologists' accreditation pilot study: a year later. The College Audit Steering Committee.
- Arginase deficiency and phenylketonuria.
- Royal College of Pathologists' United Kingdom pilot study of laboratory accreditation. The College Accreditation Steering Committee.