Abstract
Previous studies of intellectual development in children who had experienced transient neonatal tyrosinemia (TNT) generally have shown no adverse effects from this disorder. However, the patients were usually prematures with only modest tyrosine elevations persisting <1 mo. In contrast, this report presents the results of extensive psychological testing of 15 children (mean age 4.7±0.8 yrs) who had had moderately severe TNT (mean 25.0±9.4 mg%) for a mean duration of 50±14.4 days after uneventful term births. Their TNT was discovered through phenylketonuria screening and all had been fed a high protein evaporated milk formula. In comparison to carefully matched controls (for age, sex, socioeconomic status, locale and family background), the TNT group had significantly lower intellectual development by both the McCarthy Scales (77.1±16.5 vs 92.6±13.2, p=0.008) and the Illinois Test of Psycholinguistic Ability (92.3±13.3 vs 109.9±15, p=0.002). Additionally, the TNT group scored significantly lower in subtests of verbal abilities, fine motor skills and higher cognitive skills. However, visual perception, visual memory and visual reasoning were not significantly affected. These results indicate that severe, prolonged TNT is injurious to the developing central nervous system. Extensive public health measures are needed to determine the extent of this disorder and to reduce its occurrence.
Article PDF
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Mamunes, P., Prince, P., Thornton, N. et al. INTELLECTUAL DEFICITS AFTER TRANSIENT TYROSINEMIA IN TERM NEONATES. Pediatr Res 8, 344 (1974). https://doi.org/10.1203/00006450-197404000-00026
Issue Date:
DOI: https://doi.org/10.1203/00006450-197404000-00026