Motor Development Skills of 1- to 4-Year-Old Iranian Children with Early Treated Phenylketonuria

Objective: To gauge the gross and fine motor development of early treated phenylketonuria (ETPKU) in children in the age range of 1-4 years. Materials & Methods: The cross-sectional analytic study was conducted in PKU clinics (reference clinics for PKU follow-up), Tehran, Iran. Seventy children with ETPKU were selected as the case group. ETPKU children were those with early and continuous treatment with a phenylalanine restricted regimen. Also, 100 healthy children matched with the ETPKU group for age were randomly selected from 4 kindergartens in four districts of Tehran as the control group. The measurements consisted of a demographic questionnaire, Peabody Developmental Motor Scale-2 (PDMS-2) and pediatrician assessment. Motor quotients were determined by PDMS-2 and then compared in both groups through two independent samples t-test. Results: The mean ages in case and control group were 28.5 (+/- 11.6) and 29.7 (+/- 11.3) months, respectively. The comparisons of the mean fine (p<0.001), gross (P=0.010), and total (p<0.001) developmental motor quotients (DMQs) showed statistically significant differences between the two groups. Conclusion: The result showed that screening and following a current phenylalanine-restricted diet in ETPKU children have not completely reduced the motor delay and they have had lower motor development than normal children. As a result, were more emphasized early detection and early rehabilitation for all of ETPKU children.