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Irritability in children with RASopathies, insights into emotional dysregulation and social impairment.

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Title: Irritability in children with RASopathies, insights into emotional dysregulation and social impairment.
Authors: Serur, Yaffa (AUTHOR), Fuhrmann, Naomi (AUTHOR), Russo, Odeya (AUTHOR), Green, Tamar (AUTHOR)
Source: European Child & Adolescent Psychiatry. Nov2025, Vol. 34 Issue 11, p3497-3507. 11p.
Subjects: Emotion regulation, Noonan syndrome, Attention-deficit hyperactivity disorder, T-test (Statistics), Data analysis, Research funding, Questionnaires, Fisher exact test, Multiple regression analysis, Affective disorders, Anxiety, Descriptive statistics, Mann Whitney U Test, Chi-squared test, Neurofibromatosis, Behavior disorders in children, Child development deviations, Cognition disorders, Social skills, Child Behavior Checklist, Statistics, Comparative studies, Social participation, Comorbidity, Mental depression, Disease complications, Psychosocial factors
Abstract: RASopathies, including Noonan Syndrome (NS) and Neurofibromatosis type 1 (NF1), are developmental disorders caused by germline mutations in genes of the RAS-mitogen-activated protein kinase pathway (RAS-MAPK). This study investigates irritability, a highly prevalent transdiagnostic construct, in children with NS and NF1 and the impact of RASopathy status on the associations between irritability, emotional dysregulation-related disorders, and social impairment. The sample comprises 174 children aged 4–17 (age mean = 9.49; 98 females), including 113 children with RASopathies (NS n = 85, NF1 n = 28) and 61 age-sex-matched typically developed (TD) children. We used parent proxy questionnaires (CBCL, SRS) to assess irritability, symptoms of ADHD, defiance, anxiety/depression, and social impairment while controlling for cognitive measures (IQ). Children with RASopathies exhibited higher irritability than TD children (mean difference = 1.09; p <.001). Children with NS showed a weaker association between irritability and ADHD symptoms compared to TD children (p =.032, ηp2 = 0.03) and a stronger association between irritability and social impairment compared to both TD (p =.033, ηp2 = 0.03) and NF1 groups (p =.009, ηp2 = 0.06). We present novel and clinically significant findings showing high irritability in children with NS and NF1. Our study provides syndrome-specific results, suggesting differences in the mechanisms involved in irritability, ADHD, and social processes. In essence, children with RASopathies showed a highly irritable profile associated with ADHD symptoms and social impairment, with a significantly stronger association between irritability and social processes in NS. Our results suggest that developing prevention and treatments targeting irritability can distinctly affect the trajectories of neurodevelopmental disorders in children with NS and NF1. [ABSTRACT FROM AUTHOR]
Copyright of European Child & Adolescent Psychiatry is the property of Springer Nature and its content may not be copied or emailed to multiple sites without the copyright holder's express written permission. Additionally, content may not be used with any artificial intelligence tools or machine learning technologies. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)
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  Data: RASopathies, including Noonan Syndrome (NS) and Neurofibromatosis type 1 (NF1), are developmental disorders caused by germline mutations in genes of the RAS-mitogen-activated protein kinase pathway (RAS-MAPK). This study investigates irritability, a highly prevalent transdiagnostic construct, in children with NS and NF1 and the impact of RASopathy status on the associations between irritability, emotional dysregulation-related disorders, and social impairment. The sample comprises 174 children aged 4–17 (age mean = 9.49; 98 females), including 113 children with RASopathies (NS n = 85, NF1 n = 28) and 61 age-sex-matched typically developed (TD) children. We used parent proxy questionnaires (CBCL, SRS) to assess irritability, symptoms of ADHD, defiance, anxiety/depression, and social impairment while controlling for cognitive measures (IQ). Children with RASopathies exhibited higher irritability than TD children (mean difference = 1.09; p &lt;.001). Children with NS showed a weaker association between irritability and ADHD symptoms compared to TD children (p =.032, ηp2 = 0.03) and a stronger association between irritability and social impairment compared to both TD (p =.033, ηp2 = 0.03) and NF1 groups (p =.009, ηp2 = 0.06). We present novel and clinically significant findings showing high irritability in children with NS and NF1. Our study provides syndrome-specific results, suggesting differences in the mechanisms involved in irritability, ADHD, and social processes. In essence, children with RASopathies showed a highly irritable profile associated with ADHD symptoms and social impairment, with a significantly stronger association between irritability and social processes in NS. Our results suggest that developing prevention and treatments targeting irritability can distinctly affect the trajectories of neurodevelopmental disorders in children with NS and NF1. [ABSTRACT FROM AUTHOR]
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  Data: &lt;i&gt;Copyright of European Child &amp; Adolescent Psychiatry is the property of Springer Nature and its content may not be copied or emailed to multiple sites without the copyright holder&#39;s express written permission. Additionally, content may not be used with any artificial intelligence tools or machine learning technologies. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract.&lt;/i&gt; (Copyright applies to all Abstracts.)
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      – Type: doi
        Value: 10.1007/s00787-025-02767-w
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        Text: English
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        PageCount: 11
        StartPage: 3497
    Subjects:
      – SubjectFull: Emotion regulation
        Type: general
      – SubjectFull: Noonan syndrome
        Type: general
      – SubjectFull: Attention-deficit hyperactivity disorder
        Type: general
      – SubjectFull: T-test (Statistics)
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      – SubjectFull: Data analysis
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      – SubjectFull: Research funding
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      – SubjectFull: Questionnaires
        Type: general
      – SubjectFull: Fisher exact test
        Type: general
      – SubjectFull: Multiple regression analysis
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      – SubjectFull: Affective disorders
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      – SubjectFull: Anxiety
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      – SubjectFull: Descriptive statistics
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      – SubjectFull: Mann Whitney U Test
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      – SubjectFull: Neurofibromatosis
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      – SubjectFull: Statistics
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      – SubjectFull: Disease complications
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      – SubjectFull: Psychosocial factors
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      – TitleFull: Irritability in children with RASopathies, insights into emotional dysregulation and social impairment.
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              Text: Nov2025
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