[Neuropsychological follow-up of children with phenylketonuria treated early]. / Follow-up neuro-psichico di bambini fenilchetonurici trattati precocemente.

We evaluated, yearly, the neuro-psychological development in 16 out of the 27 children with phenylketonuria detected via neonatal screening in the years 1980-90. 14 children had the classical form, while 2 had "variant" type of phenylketonuria. Development quotients always were slightly lower in patients than in controls, but the greatest difference was seen at 1 year of age (92.5 +/- 6.7 vs 101.6 +/- 2.2). At age 6 years, 4 children out of 11 had a IQ equal or below 90.2 of them had neonatal asphyxia, while the others showed, after age 2-4, constantly elevated phenylalanine levels. Four children had a bad metabolic control, as from the age of 2-4 year, and only 1 of them showed a normal IQ at 6 years of age. Regression analyses were used to determine the best predictors of IQ. Parents' social level stood out as the best predictor of IQ at 6 years of age. If this parameter was excluded, phenylalanine level, especially at age 4, best predicted IQ. The following variables had no influence: age at start of diet (they were all begun on diet within the first month of life), pre-diet phenylalanine levels and phenylalanine levels at 1 year of age (all children were, at this age, on good control), DQ (Development Quotient) at age 1. A high percentage (10 out 14) of hyperactive and psychologically instable children was also found, but with no correlations with IQ levels.

School-related behavior in children with cancer.

Ninety-one children with cancer (thirty-seven with leukemia and fifty-four with solid tumors)--school aged (elementary and middle school), residing in Emilia-Romagna Region (Italy), and receiving treatment at the Department of Pediatrics (III), University of Bologna, from August 1973 to April 1987--took part in a study of school behavior as perceived by the teacher. The research was carried out through a 29-item forced-choice questionnaire based on the questionnaire proposed by Deasy-Spinetta for a similar study in the U.S.A. Results revealed that children with cancer have a school behavior scoring at levels significantly lower than controls. There are more difficulties for children attending middle than elementary school, for children on therapy than off therapy, and for children with leukemia than with solid tumors. In conclusion, the main obstacle to school performance seems to be lower attendance, which is mainly due to treatment and clinic visits and probably in part to an overprotection and an excessive concern on the part of physicians and parents.

Intellectual function in long-term survivors of childhood acute lymphoblastic leukemia.

21 off-therapy ALL patients treated with chemotherapy plus cranial irradiation were assessed using the Wechsler Intelligence Scale for Children and compared with a group of 15 off-therapy solid tumor children treated with chemotherapy and extracranial irradiation. Children with leukemia achieved lower IQ scores than children with solid tumors. The ALL group was divided in two subgroups, by onset of disease and beginning of treatment: before (13 subjects) and after (8 subjects) 5 years of age. The results indicate that the disturbance of intellectual functioning is produced in ALL children irradiated after 5 years of age. In order to check on a possible delayed intellectual deterioration, all ALL children were also tested twice, with one year interval between the two tests. On the second assessment the scores for each subject were essentially the same as before. Further studies on a large number of long term survivors are being carried out in order to find out whether the deficit is transient or permanent.