ABSTRACT
<p><b>OBJECTIVE</b>To investigate the factors influencing the quality of life (QOL) of children with phenylketonuria (PKU) in Anhui Province, China.</p><p><b>METHODS</b>A total of 104 PKU children who were diagnosed and treated in three major maternal and child health hospitals in Anhui Province were enrolled as study subjects. The PedsQL™ 4.0 Generic Core Scales were used to evaluate the quality of life of these children. The multivariate logistic regression analysis was used to evaluate the factors influencing the QOL.</p><p><b>RESULTS</b>The 104 PKU children had significantly lower overall QOL score and scores on the subscales of physiological functioning, emotional functioning, and social functioning than the general school-age children (P<0.01). They also had a significantly lower score on the physiological domain consisting of emotional functioning, social functioning, and role functioning than the general school-age children (P<0.01). The multivariate logistic regression analysis showed that an older age (≥4 years) of PKU children was the risk factor for poor QOL (OR=8.569, P<0.01), and guardians' engagement at enterprises or institutions was the protective factor for QOL (OR=0.206, P<0.05).</p><p><b>CONCLUSIONS</b>PKU children have a low level of QOL, and age and guardians' occupation are factors influencing the QOL.</p>