title: Parent stress across molecular subtypes of children with Angelman syndrome.
abstract: BACKGROUND
  Parenting stress has been consistently reported among parents of children with developmental disabilities. However, to date, no studies have investigated the impact of a molecular subtype of Angelman syndrome (AS) on parent stress, despite distinct phenotypic differences among subtypes.
  
  
  METHODS
  Data for 124 families of children with three subtypes of AS: class I and II deletions (n = 99), imprinting centre defects (IC defects; n = 11) and paternal uniparental disomy (UPD; n = 14) were drawn from the AS Rare Diseases Clinical Research Network (RDCRN) database and collected from five research sites across the Unites States. The AS study at the RDCRN gathered health information to understand how the syndrome develops and how to treat it. Parents completed questionnaires on their perceived psychological stress, the severity of children's aberrant behaviour and children's sleep patterns. Children's adaptive functioning and developmental levels were clinically evaluated.
  
  
  RESULTS
  Child-related stress reached clinical levels for 40% of parents of children with deletions, 100% for IC defects and 64.3% for UPD. Sleep difficulties were similar and elevated across subtypes. There were no differences between molecular subtypes for overall child and parent-related stress. However, results showed greater isolation and lack of perceived parenting skills for parents of children with UPD compared with deletions. Better overall cognition for children with deletions was significantly related to more child-related stress while their poorer adaptive functioning was associated with more child-related stress. For all three groups, the severity of children's inappropriate behaviour was positively related to different aspects of stress.
  
  
  CONCLUSIONS
  How parents react to stress depends, in part, on children's AS molecular subtype. Despite falling under the larger umbrella term of AS, it is important to acknowledge the unique aspects associated with children's molecular subtype. Identifying these factors can lead to tailored interventions that fit the particular needs of families of children with different AS subtypes.
authors.affiliation: Department of Child and Adolescent Development, California State University, Northridge, California, USA.
authors.forename: N
authors.initials: N
authors.lastname: Miodrag
authors.affiliation: Department of Pediatrics, Vanderbilt University, Nashville, Tennessee, USA.
authors.forename: S
authors.initials: S
authors.lastname: Peters
mesh_terms: D000293:Adolescent; D000328:Adult; D017204:Angelman Syndrome / Q000145:classification* / Q000235:genetics / Q000451:nursing*; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D008875:Middle Aged; D010290:Parents / Q000523:psychology*; D000066553:Problem Behavior / Q000523:psychology*; D013315:Stress, Psychological / Q000523:psychology*; D055815:Young Adult
keywords: Angelman syndrome; behaviour problems; molecular subtypes; parent stress