Profiles and Predictors of Family Functioning in Families of Children with Mowat-Wilson Syndrome: A Cross-Sectional Survey

莫瓦特-威尔逊综合征患儿家庭功能特征及预测因素:一项横断面调查

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Abstract

Introduction: Family functioning is a critical factor linked to child outcomes in neurodevelopmental disorders but remains unstudied in Mowat-Wilson syndrome (MWS), a rare condition with substantial caregiver burden. This study characterized family functioning in MWS and identified factors associated with family functioning. Materials and Methods: We employed a cross-sectional survey in a cohort of 42 patients with MWS. Family functioning was assessed using the Family Assessment Device (FAD). Clinical and behavioral characteristics included intellectual disability, adaptive functioning, autism spectrum disorder (ASD)-related behaviors, and CBCL-derived domains of social, emotional, and sleep-related concerns. Results: Among 42 children (mean age 4.9 years), severe/profound intellectual disability and adaptive deficits were common. Difficulties in family functioning were frequently reported. The highest proportions of scores above the published cut-off were observed in behavior control (90.5%) and affective involvement (76.2%). Higher monthly household income was independently associated with better general family functioning (β = -0.443, p = 0.008), whereas child age, intellectual disability severity, and adaptive impairment were not significantly associated with general functioning scores. Poorer general functioning was associated with higher scores on the CBCL-derived depressive symptom domain. Conclusions: Caregivers of children with MWS commonly reported difficulties in family functioning; behavior control was most affected, followed by affective involvement. Higher household income was independently associated with better general family functioning, suggesting that household income is an important socioeconomic correlate of family functioning.

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