{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,10]],"date-time":"2026-09-10T05:47:39Z","timestamp":1789019259414,"version":"build-2803163510"},"reference-count":48,"publisher":"Wiley","issue":"10","license":[{"start":{"date-parts":[[2023,7,20]],"date-time":"2023-07-20T00:00:00Z","timestamp":1689811200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/100007857","name":"Intellectual and Developmental Disabilities Research Center","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100007857","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100006792","name":"Hartwell Foundation","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100006792","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","award":["P50HD103538"],"award-info":[{"award-number":["P50HD103538"]}],"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":["K08HD086250"],"award-info":[{"award-number":["K08HD086250"]}],"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":[[2023,10]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>The aim of this study was to provide a descriptive overview of the social characteristics associated with Wiedemann\u2010Steiner syndrome (WSS). A total of 24 parents of children\/adults with WSS (11F, mean age\u2009=\u200912.94\u2009years, SD\u2009=\u20098.00) completed the Social Responsiveness Scale 2nd Edition (SRS\u20102); Colorado Learning Difficulties Questionnaire (CLDQ) and Strengths and Difficulties Questionnaire (SDQ). Almost half our sample reported a diagnosis of autism spectrum disorder (ASD) and 70% had intellectual disability. On the SDQ, over 90% of participants were rated in borderline\/clinical ranges in Peer Problems, yet the majority fell within normal limits in Prosocial Behaviors. Most fell in the moderate\/severe difficulties ranges across SRS\u20102 Social Cognition, Communication, and Restricted\/Repetitive Behaviors scales (all &gt;70%); whereas substantially less participants met these ranges for deficits in Social Awareness (50%) and Social Motivation (33.33%). A pattern of relatively strong prosocial skills and social drive in the context of difficulties with inflexible behaviors, social cognition, and communication was observed, regardless of gender, ASD or intellectual disability diagnosis. The social phenotype associated with WSS is characterized by some autistic features paired with unusually high social motivation and prosocial tendencies.<\/jats:p>","DOI":"10.1002\/ajmg.a.63351","type":"journal-article","created":{"date-parts":[[2023,7,20]],"date-time":"2023-07-20T09:24:06Z","timestamp":1689845046000},"page":"2591-2601","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":7,"title":["The social phenotype associated with <scp>Wiedemann\u2010Steiner<\/scp> syndrome: Autistic traits juxtaposed with high social drive and prosociality"],"prefix":"10.1002","volume":"191","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-7193-4300","authenticated-orcid":false,"given":"Rowena","family":"Ng","sequence":"first","affiliation":[{"name":"Kennedy Krieger Institute  Baltimore Maryland USA"},{"name":"Department of Psychiatry and Behavioral Sciences Johns Hopkins University School of Medicine  Baltimore Maryland USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Allison","family":"Kalinousky","sequence":"additional","affiliation":[{"name":"Department of Genetic Medicine Johns Hopkins University School of Medicine  Baltimore Maryland USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jill A.","family":"Fahrner","sequence":"additional","affiliation":[{"name":"Department of Genetic Medicine Johns Hopkins University School of Medicine  Baltimore Maryland USA"},{"name":"Department of Pediatrics Johns Hopkins University School of Medicine  Baltimore Maryland USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hans Tomas","family":"Bjornsson","sequence":"additional","affiliation":[{"name":"Department of Genetic Medicine Johns Hopkins University School of Medicine  Baltimore Maryland USA"},{"name":"Department of Pediatrics Johns Hopkins University School of Medicine  Baltimore Maryland USA"},{"name":"Faculty of Medicine University of Iceland  Reykjavik Iceland"},{"name":"Landspitali University Hospital  Reykjavik Iceland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jacqueline","family":"Harris","sequence":"additional","affiliation":[{"name":"Kennedy Krieger Institute  Baltimore Maryland USA"},{"name":"Department of Genetic Medicine Johns Hopkins University School of Medicine  Baltimore Maryland USA"},{"name":"Department of Pediatrics Johns Hopkins University School of Medicine  Baltimore Maryland USA"},{"name":"Department of Neurology Johns Hopkins University School of Medicine  Baltimore Maryland USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2023,7,20]]},"reference":[{"key":"e_1_2_10_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ejmg.2017.03.006"},{"key":"e_1_2_10_3_1","doi-asserted-by":"publisher","DOI":"10.3389\/fpsyg.2012.00247"},{"key":"e_1_2_10_4_1","doi-asserted-by":"publisher","DOI":"10.1111\/cge.13254"},{"key":"e_1_2_10_5_1","doi-asserted-by":"publisher","DOI":"10.1038\/nn.4276"},{"key":"e_1_2_10_6_1","doi-asserted-by":"publisher","DOI":"10.1162\/089892900561959"},{"issue":"1","key":"e_1_2_10_7_1","first-page":"1","article-title":"Investigating the validation of the Chinese Mandarin version of the Social Responsiveness Scale in a Mainland China child population","volume":"17","author":"Cen C. 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