The Behavioral Phenolype of Children with Velocardiofacial Syndrome

Velocardiofacial syndrome (VCFS) is one of the most common genetic, congenital diseases to date. The clinical symptoms of patients with VCFS have included up to 180 medical and psychological features, such as velopharyngeal insufficiency, cleft palate, schizophrenia or bipolar disorder, cognitive limitations, and behavioral or speech or language difficulties. The purpose of this study was to examine early personality patterns associated with VCFS, and to identify whether variables, including maternal personality, parent-child interaction, and family environment, moderated the relationship between children\u27s personality and behavior. The Hierarchical Personality Inventory for Children (HiPlC), NEO Five-Factor Inventory (NEO-FFl), Parenting Stress Index (PSl), Family Environment Scale (FES), and Behavioral Assessment System for Children, were completed by 34 maternal caregivers of children with VCFS, children with cleft palate and/or cleft lip and palate (CP/CLP), and children with no known medical conditions (NMC). Multiple regression and ANOVA\u27s were used to analyze the data. Findings from this study suggested that children with VCFS exhibit similar personality patterns when compared to children in other groups. Specifically, VCFS is associated with average levels of Emotional Stability, Extraversion, Benevolence, and Conscientiousness. However, children with VCFS demonstrated lower levels of Imagination when compared to other groups of children. Further, a larger percentage of children with VCFS obtained high scores on the Emotional Stability domain and low scores on the Extraversion, Imagination, Agreeableness, and Conscientiousness domains in comparison to other children. Maternal caregivers in the VCFS group exhibited average levels of personality traits (Neuroticism, Extraversion, Openness, Agreeableness, Conscientiousness) in comparison to caregivers in other groups. However, a larger percentage of caregivers in the VCFS group obtained high scores on the Neuroticism and Extraversion domains. VCFS dyads obtained significantly different and clinically elevated scores on the Parent- Child Dysfimctional Interaction domain in comparison to dyads in other groups. No statistically significant differences between groups in terms of Family Conflict and Family Cohesion emerged. However, a larger percentage of caregivers in the VCFS group reported high levels of Family Conflict in comparison to caregivers in other groups. Maternal Neuroticism and Parent-Child Dysfunctional Interaction influenced the relationship between children\u27s Emotional Stability and Behavioral Symptoms