{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,17]],"date-time":"2026-08-17T14:27:50Z","timestamp":1786976870325,"version":"build-2736575974"},"reference-count":38,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2019,4,3]],"date-time":"2019-04-03T00:00:00Z","timestamp":1554249600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/100009633","name":"Eunice Kennedy Shriver National Institute of Child Health and Human Development","doi-asserted-by":"publisher","award":["U54 HD061222"],"award-info":[{"award-number":["U54 HD061222"]}],"id":[{"id":"10.13039\/100009633","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000097","name":"National Center for Research Resources","doi-asserted-by":"publisher","award":["U54 RR019478"],"award-info":[{"award-number":["U54 RR019478"]}],"id":[{"id":"10.13039\/100000097","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000071","name":"National Institute of Child Health and Human Development","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100000071","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000097","name":"National Center for Research Resources","doi-asserted-by":"publisher","award":["NIH U54 HD061222"],"award-info":[{"award-number":["NIH U54 HD061222"]}],"id":[{"id":"10.13039\/100000097","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","award":["RR019478"],"award-info":[{"award-number":["RR019478"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","award":["U54"],"award-info":[{"award-number":["U54"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["American J of Med Genetics Pt A"],"published-print":{"date-parts":[[2019,6]]},"abstract":"<jats:p>\n                    Maladaptive behaviors are challenging and a source of stress for caregivers of individuals with Angelman Syndrome (AS). There is limited information on how these maladaptive behaviors vary over time among individuals with AS due to different genetic etiologies. In this study, caregivers of 301 individuals with AS were asked questions about their child's behavior and completed the Aberrant Behavior Checklist\u2010Community version (ABC\u2010C). Developmental functioning was evaluated with either the Bayley Scales of Infant Development, Third Edition (Bayley\u2010III) or the Mullen Scales of Early Learning (MSEL). Family functioning was assessed using the parent\u2010completed Parenting Stress Index (PSI) and the Family Quality of Life questionnaire (FQoL). Approximately 70% of participants had AS due to a deletion on the maternally\u2010inherited copy of chromosome 15q11q13. Results revealed that at baseline, individuals with AS had low scores in the domains of lethargy (mean: 2.6\u20134.2 depending on genotype) and stereotypy (mean: 2.3\u20134.2 depending on genotype). Higher cognitive functioning was associated with increased irritability (\n                    <jats:italic>r<\/jats:italic>\n                    =\u20090.32,\n                    <jats:italic>p<\/jats:italic>\n                    \u2009&lt;\u2009.01). Hyperactivity (\n                    <jats:italic>p<\/jats:italic>\n                    \u2009&lt;\u2009.05) and irritability (\n                    <jats:italic>p<\/jats:italic>\n                    &lt;\u2009.05) increased with age across all genotypes and should be ongoing targets for both behavioral and pharmacological treatment. Concerns for short attention span were endorsed by more than 70% of caregivers at baseline. Maladaptive behaviors, particularly hyperactivity, irritability and aggression, adversely affected parental stress, and family quality of life.\n                  <\/jats:p>","DOI":"10.1002\/ajmg.a.61140","type":"journal-article","created":{"date-parts":[[2019,4,3]],"date-time":"2019-04-03T10:22:51Z","timestamp":1554286971000},"page":"983-992","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":47,"title":["Maladaptive behaviors in individuals with Angelman syndrome"],"prefix":"10.1002","volume":"179","author":[{"given":"Anjali","family":"Sadhwani","sequence":"first","affiliation":[{"name":"Department of Psychiatry Boston Children's Hospital, Harvard Medical School  Boston Massachusetts"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jennifer M.","family":"Willen","sequence":"additional","affiliation":[{"name":"Division of Genetics and Genomics Boston Children's Hospital, Harvard Medical School  Boston Massachusetts"},{"name":"Department of Psychiatry Kennedy Krieger Institute  Baltimore Maryland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nicole","family":"LaVallee","sequence":"additional","affiliation":[{"name":"PROMETRIKA, LLC  Cambridge Massachusetts"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Miganush","family":"Stepanians","sequence":"additional","affiliation":[{"name":"PROMETRIKA, LLC  Cambridge Massachusetts"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hillary","family":"Miller","sequence":"additional","affiliation":[{"name":"Division of Genetics and Genomics, Department of Biostatistics Boston Children's Hospital, Harvard T.H. Chan School of Public Health  Boston Massachusetts"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sarika U.","family":"Peters","sequence":"additional","affiliation":[{"name":"Division of Developmental Medicine Vanderbilt University School of Medicine  Nashville Tennessee"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rene L.","family":"Barbieri\u2010Welge","sequence":"additional","affiliation":[{"name":"Developmental Evaluation Clinic Rady Children's Hospital San Diego  San Diego California"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lucia T.","family":"Horowitz","sequence":"additional","affiliation":[{"name":"Greenwood Genetic Center  Greenwood South Carolina"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lisa M.","family":"Noll","sequence":"additional","affiliation":[{"name":"Psychology Service Texas Children's Hospital, Baylor College of Medicine  Houston Texas"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rachel J.","family":"Hundley","sequence":"additional","affiliation":[{"name":"Division of Developmental Medicine Vanderbilt University School of Medicine  Nashville Tennessee"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lynne M.","family":"Bird","sequence":"additional","affiliation":[{"name":"Department of Pediatrics University of California  San Diego"},{"name":"Genetics\/Dysmorphology Rady Children's Hospital  San Diego California"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1593-6149","authenticated-orcid":false,"given":"Wen\u2010Hann","family":"Tan","sequence":"additional","affiliation":[{"name":"Division of Genetics and Genomics Boston Children's Hospital, Harvard Medical School  Boston Massachusetts"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2019,4,3]]},"reference":[{"key":"e_1_2_6_2_1","volume-title":"Parenting stress index manual","author":"Abidin R. 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