{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,28]],"date-time":"2026-09-28T21:13:23Z","timestamp":1790630003566,"version":"4.1.0"},"reference-count":31,"publisher":"Bioscientifica","issue":"5","license":[{"start":{"date-parts":[[2023,3,7]],"date-time":"2023-03-07T00:00:00Z","timestamp":1678147200000},"content-version":"unspecified","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"content-domain":{"domain":["ec.bioscientifica.com"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2023,3,7]]},"abstract":"<jats:p>Investigating sex chromosome trisomies (SCTs) may help in understanding neurodevelopmental pathways underlying the risk for neurobehavioral problems and psychopathology. Knowledge about the neurobehavioral phenotype is needed to improve clinical care and early intervention for children with SCT. This is especially relevant considering the increasing number of early diagnosed children with the recent introduction of noninvasive prenatal screening. The TRIXY Early Childhood Study is a longitudinal study designed to identify early neurodevelopmental risks in children with SCT, aged 1\u20137 years. This review summarizes the results from the TRIXY Early Childhood Study, focusing on early behavioral symptoms in areas of autism spectrum disorder, attention-deficit hyperactivity disorder, and communication disorders, and underlying neurocognitive mechanisms in domains of language, emotion regulation, executive functioning, and social cognition. Behavioral symptoms were assessed through structured behavior observation and parental questionnaires. Neurocognition was measured using performance tests, eyetracking, and psychophysiological measures of arousal. In total, 209 children aged 1\u20137 years were included: 107 children with SCT (33 XXX, 50 XXY, and 24 XYY) and 102 age-matched population controls. Study outcomes showed early behavioral symptoms in young children with SCT, and neurocognitive vulnerabilities, already from an early age onward. Neurobehavioral and neurocognitive difficulties tended to become more pronounced with increasing age and were rather robust, independent of specific karyotype, pre\/postnatal diagnosis, or ascertainment strategy. A more longitudinal perspective on neurodevelopmental \u2018at-risk\u2019 pathways is warranted, also including studies assessing the effectiveness of targeted early interventions. Neurocognitive markers that signal differences in neurodevelopment may prove to be helpful in this. Focusing on early development of language, social cognition, emotion regulation, and executive functioning may help in uncovering early essential mechanisms of (later) neurobehavioral outcome, allowing for more targeted support and early intervention.<\/jats:p>","DOI":"10.1530\/ec-22-0494","type":"journal-article","created":{"date-parts":[[2023,3,7]],"date-time":"2023-03-07T05:07:07Z","timestamp":1678165627000},"update-policy":"https:\/\/doi.org\/10.1530\/crossmarkpolicy-10","source":"Crossref","is-referenced-by-count":7,"title":["Neurocognitive and behavioral development in young children (1\u20137 years) with sex chromosome trisomy"],"prefix":"10.1530","volume":"12","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-9179-7515","authenticated-orcid":true,"given":"Sophie","family":"van Rijn","sequence":"first","affiliation":[{"name":"Clinical Neurodevelopmental Sciences, Leiden University, Wassenaarseweg, Leiden, The Netherlands"},{"name":"TRIXY Center of Expertise, Leiden University Treatment and Expertise Centre (LUBEC), Sandifortdreef, Leiden, The Netherlands"},{"name":"Leiden Institute for Brain and Cognition, Leiden University, Wassenaarseweg, Leiden, The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6429-5835","authenticated-orcid":true,"given":"Kimberly","family":"Kuiper","sequence":"additional","affiliation":[{"name":"Clinical Neurodevelopmental Sciences, Leiden University, Wassenaarseweg, Leiden, The Netherlands"},{"name":"TRIXY Center of Expertise, Leiden University Treatment and Expertise Centre (LUBEC), Sandifortdreef, Leiden, The Netherlands"},{"name":"Leiden Institute for Brain and Cognition, Leiden University, Wassenaarseweg, Leiden, The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nienke","family":"Bouw","sequence":"additional","affiliation":[{"name":"Clinical Neurodevelopmental Sciences, Leiden University, Wassenaarseweg, Leiden, The Netherlands"},{"name":"TRIXY Center of Expertise, Leiden University Treatment and Expertise Centre (LUBEC), Sandifortdreef, Leiden, The Netherlands"},{"name":"Department of Child and Adolescent Psychiatry\/Psychology, Erasmus MC, Sophia Children\u2019s Hospital, Dr. Molewaterplein, Rotterdam, The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Evelien","family":"Urbanus","sequence":"additional","affiliation":[{"name":"Clinical Neurodevelopmental Sciences, Leiden University, Wassenaarseweg, Leiden, The Netherlands"},{"name":"TRIXY Center of Expertise, Leiden University Treatment and Expertise Centre (LUBEC), Sandifortdreef, Leiden, The Netherlands"},{"name":"Department of Clinical, Neuro, and Developmental Psychology, Vrije Universiteit Amsterdam, Van der Boechorststraat, Amsterdam, The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hanna","family":"Swaab","sequence":"additional","affiliation":[{"name":"Clinical Neurodevelopmental Sciences, Leiden University, Wassenaarseweg, Leiden, The Netherlands"},{"name":"TRIXY Center of Expertise, Leiden University Treatment and Expertise Centre (LUBEC), Sandifortdreef, Leiden, The Netherlands"},{"name":"Leiden Institute for Brain and Cognition, Leiden University, Wassenaarseweg, Leiden, The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"416","reference":[{"key":"bib1","series-title":"Journal of Clinical Endocrinology and Metabolism","first-page":"622","article-title":"Prenatal and postnatal prevalence of Klinefelter syndrome: a national registry study","volume":"88","author":"Bojesen","year":"2003","unstructured":"Bojesen AJuul S & Gravholt CH. 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