Biomedical subjects
F Plainguet
Publications and source records attributed to F Plainguet.
[Quality of life of children after organ transplantation].
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Long-term follow up of patients with classical phenylketonuria after diet relaxation at 5 years of age. The Paris Study.
The age for discontinuing dietary treatment of phenylketonuria (PKU) has been a worldwide source of controversy for many years. It is the reason we report here the results of a prospective, controlled study in which the diet was relaxed at 5 years of age in 31 so far well-treated children with classical PKU. The increase of phenylalanine (Phe) plasma levels to about 1500 mumol/l (25 mg/dl) after relaxing the diet was not associated with any significant decline of intellectual performance as measured by the Wechsler scores. Paired comparisons at 7-8 years and 11-13 years of age (n = 12) have shown WISC scores of 102.6 +/- 16.2 and 104.8 +/- 16, respectively, which were not significantly different. Similarly, paired comparisons at 9-10 years and 14-16 years (n = 6) did not demonstrate a significant loss of IQ points (107.7 +/- 13 vs 104.8 +/- 18). Of course, it is possible to argue that we should have observed an increase in IQ with increasing age in our patients and that the absence of deterioration cannot be considered by itself as a good result. Nevertheless, it cannot be excluded that the subtle but global intellectual impairments that have been documented in early-treated subjects are, to a very substantial degree, determined in the pre-school years, long before there is any question of stopping or relaxing the diet.
[Intellectual development after relaxing the diet at the age of 5 years in typical phenylketonuria].
BACKGROUND: No satisfactory controlled trial has yet been completed on typical phenylketonuria (PKU) patients whose treatment was relaxed at the age of 5 years. METHODS: 27 children having typical PKU were treated before the age of 3 months. The intake of phenylalanine and protein was carefully regulated during the first 5 years of life, after which the treatment was relaxed. All children were evaluated after at least 6 years on the relaxed diet. Their IQ scores and school performance were related to the degree of dietary control and plasma phenylalanine values. RESULTS: The IQ scores at 5 years of age were 100 +/- 10.8. Continued evaluation showed that IQ scores remained unchanged. Poor school performance was twice as frequent as in general population; the deficit in the IQ score of this group was 8 points below that of normal sibs. There was no correlation between plasma phenylalanine and the IQ score after the age of 5 years. The positive control decreased with aged. CONCLUSIONS: Children with typical PKU have an IQ deficit relative to their normal sibs just before relaxing treatment. Good dietary control until 5 years of age, maternal intelligence and continuing evaluation during relaxing diet are the best conditions for optimal intellectual progress. There is no evidence that continued treatment during adolescence is beneficial.