Cognition and tyrosine supplementation among school-aged children with phenylketonuria.
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Biomedical subjects
Publications and source records attributed to W van Doorninck.
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We determined the effect on intellectual performance and behavior of the age at which dietary control was lost in 119 10-year-old children with phenylketonuria (PKU) who had started on a diet low in phenylalanine before the age of 65 days. The children's diets were considered to be out of control when their blood phenylalanine concentration persistently exceeded 15 mg per deciliter. The age at which control was lost was the best, and frequently the only, predictor of the child's IQ at the age of 8 or 10 years and of the deficit in the child's IQ as compared with those of his or her unaffected siblings or parents. The age at which control was lost was also the best predictor of the deficit in scores on the Wide Range Achievement Test of children with PKU at the age of eight, as compared with their unaffected siblings, and of behavior problems (Louisville Behavior Checklist). Variables that were not significant included the maximal phenylalanine concentration before dietary therapy, the age at which treatment was started, and the family's coping abilities. The greatest deficiencies in all of these outcomes were observed among children who were out of dietary control before the age of six years. The highest correlation between the IQs of the children with PKU and their respective parents was observed among the children still on the diet at the age of eight years; the lowest, among those who had poor dietary control before the age of six. These findings suggest that phenylalanine restriction should continue after the age of eight years in children with PKU.
A controlled prospective study was conducted to evaluate possible effects of the use of three intrapartum fetal monitoring techniques on the offspring of high-risk mothers in labor after at least 34 weeks' gestation. Six hundred and ninety women were randomly assigned to one of the three monitoring groups: auscultation alone, electronic monitoring alone, and electronic monitoring with option to obtain fetal scalp pH. There were no significant differences among the three groups of offspring with respect to neonatal mortality or morbidity, Apgar scores, cord blood gas values, or Brazelton examinations at ages 2 to 3 days. Assessment of the infants at 9 months revealed no significant differences in their growth and development as assessed by physical examination. Bayley Scales of Infant Development, and Milani-Comparetti tests. The frequency of delivery by cesarean section was significantly higher in the electronically monitored group than in the auscultated group. This study failed to show beneficial effects of electronic fetal monitoring over auscultated monitoring for high-risk but relatively mature fetuses.
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