Brain and Behavior during Puberty in Klinefelter Syndrome.
克兰费尔特综合征青春期的大脑和行为。
基本信息
- 批准号:10658503
- 负责人:
- 金额:$ 33.11万
- 依托单位:
- 依托单位国家:美国
- 项目类别:
- 财政年份:2018
- 资助国家:美国
- 起止时间:2018-08-17 至 2024-05-31
- 项目状态:已结题
- 来源:
- 关键词:AccelerationAddressAdministrative SupplementAdolescentAffectAffectiveAgeAmygdaloid structureAnteriorAnxietyAttentionAttention deficit hyperactivity disorderAwarenessBehaviorBehavioralBirthBrainCOVID-19 pandemicChildChildhoodClinicalClinical ManagementCognitionCognitiveDataData CollectionDevelopmentDiagnosisDiagnosticDiffusion Magnetic Resonance ImagingEmotionalEndocrinologistExecutive DysfunctionExhibitsFailureFunctional Magnetic Resonance ImagingGenetic DiseasesGoalsHomeHormonesImpairmentInferior frontal gyrusInsula of ReilInterdisciplinary StudyKlinefelter&aposs SyndromeKnowledgeLanguageLearningLearning DisabilitiesMental DepressionModificationMoodsMultimodal ImagingNeurophysiology - biologic functionOutcomeParentsParticipantPhenotypePsyche structurePubertyReportingResearchSample SizeSamplingSchoolsSeveritiesSocial FunctioningStructureSymptomsTestingTestosteroneTimeVariantVocationX Chromosomeanxiety symptomsbehavior measurementboysbrain behaviorcingulate cortexcohortcomparison groupdepressive symptomsemotional functioningemotional symptomexecutive functionexperienceflexibilityimprovedlongitudinal analysislongitudinal designmaleneuralneuroimagingperipubertal periodprospectiverecruitresponsesecondary analysissex chromosome aneuploidysocialsocial skillstestosterone replacement therapytimelinetreatment effect
项目摘要
Klinefelter syndrome (KS) is a highly prevalent sex chromosome aneuploidy that is characterized by an
increased number of X chromosomes (47,XXY) in phenotypic males. Despite its frequent occurrence (up to 1 in
500 male births), clinicians often have little awareness about the diagnosis or management of cognitive-behavioral
and social-emotional symptoms of KS, which can include impairments in language-based learning, deficits in
executive functioning and social skills, and increased symptoms of depression and anxiety. Parents of children
with KS often report greater concerns about these behavioral disturbances relative to commonly observed
physical features in KS (e.g., tall stature and testicular failure), since they have the potential to affect everyday
function at home and at school, and can negatively influence long term social and vocational outcomes.
Most males with KS first experience testicular failure (i.e., deficiency in testosterone and/or hypogonadal
symptoms) starting in mid-puberty. For this reason, pediatric endocrinologists typically prescribe testosterone
replacement therapy (TRT) starting around this time. However, there remain large gaps in knowledge and
fundamental questions about the effects of this treatment on neural and behavioral development in males with KS,
despite well-documented effects of testosterone on the brain and cognition in typically developing males.
The prospective, longitudinal, multi-time point research proposed here would be the first to assess the
effects of TRT, or lack thereof (i.e., testicular failure) on the development of the brain and behavior in adolescents
with KS using multiple levels of analysis (cognitive-behavioral and social-emotional functioning, multi-modal
imaging, pubertal status, hormone levels). Seventy boys with KS and a comparison group of 58 age- and IQ-
matched neurotypical boys between the ages of 8 and 17 years will be recruited and followed annually for up to
three years. Three specific aims will be addressed: (1) to examine the effects of TRT on executive function and
social-emotional symptoms in KS (2) to evaluate the effects of TRT on brain structure and function in KS and, (3)
to elucidate brain-behavior associations in KS (exploratory). We will also examine associations between the
severity testicular failure and brain and behavioral measures.
The multidisciplinary research we propose offers a first-of-its-kind opportunity to provide an increased
understanding of how TRT exerts its beneficial effect on cognition, behavior and mood in boys with KS during
the peripubertal period. The results of this study are intended to lead to the development of improved clinical
management of cognitive-behavioral and social-emotional symptoms in boys with KS while advancing our
general knowledge of neural changes underlying cognition, behavior and mood during male puberty.
Klinefelter综合征(KS)是一种高度流行的性染色体非整倍体,其特征是
表型男性X染色体(47,XXY)数目增加。尽管它经常发生(高达1年
500名男性出生),临床医生通常对认知行为障碍的诊断或处理知之甚少
以及KS的社交情绪症状,其中可能包括基于语言的学习障碍,
执行功能和社交技能,以及抑郁和焦虑症状的增加。子女的父母
与通常观察到的相比,KS经常报告对这些行为障碍的更多担忧
KS的身体特征(例如,高个子和睾丸衰竭),因为它们有可能影响日常生活
在家庭和学校发挥作用,并可能对长期的社会和职业成果产生负面影响。
大多数患有KS的男性首先出现睾丸功能衰竭(即睾丸激素缺乏和/或性腺功能减退
症状)从青春期中期开始。出于这个原因,儿科内分泌科医生通常会开出睾丸素。
大约在这个时候开始替代疗法(TRT)。然而,在知识和知识方面仍然存在很大差距
关于这种治疗对男性KS患者神经和行为发育影响的基本问题,
尽管睾丸激素对大脑和认知的影响已经得到了很好的证明,但通常是发育中的男性。
这里提出的前瞻性、纵向、多时间点研究将是第一次评估
TRT是否存在(即睾丸衰竭)对青少年大脑发育和行为的影响
KS使用多层次的分析(认知-行为和社会-情感功能,多模式
成像、青春期状态、荷尔蒙水平)。70名患有KS的男孩和58岁智商的对照组--
匹配的8到17岁的神经典型男孩将被招募,并每年跟踪调查最多
三年了。三个具体目标将被解决:(1)检查TRT对执行功能的影响和
KS患者的社会情绪症状(2)评估TRT对KS患者脑结构和功能的影响
目的:阐明KS(探索性)中的脑行为关联。我们还将研究
严重程度、睾丸衰竭、脑和行为测量。
我们建议的多学科研究提供了一个首创的机会,以提供更多的
TRT对KS男童认知、行为和情绪影响的研究
青春期周围的时期。这项研究的结果旨在引导改进临床的发展。
KS男童认知行为和社会情绪症状的管理
关于男性青春期认知、行为和情绪的神经变化的常识。
项目成果
期刊论文数量(4)
专著数量(0)
科研奖励数量(0)
会议论文数量(0)
专利数量(0)
Executive Dysfunction in Klinefelter Syndrome: Associations With Brain Activation and Testicular Failure.
克兰费尔特综合征的执行功能障碍:与大脑激活和睾丸衰竭的关联。
- DOI:10.1210/clinem/dgad487
- 发表时间:2023
- 期刊:
- 影响因子:0
- 作者:Foland-Ross,LaraC;Ghasemi,Elnaz;LozanoWun,Vanessa;Aye,Tandy;Kowal,Karen;Ross,Judith;Reiss,AllanL
- 通讯作者:Reiss,AllanL
Multi-Table Differential Correlation Analysis of Neuroanatomical and Cognitive Interactions in Turner Syndrome.
- DOI:10.1007/s12021-017-9351-z
- 发表时间:2018-01
- 期刊:
- 影响因子:3
- 作者:Seiler C;Green T;Hong D;Chromik L;Huffman L;Holmes S;Reiss AL
- 通讯作者:Reiss AL
Cortical gray matter structure in boys with Klinefelter syndrome.
- DOI:10.1016/j.pscychresns.2021.111299
- 发表时间:2021-07-30
- 期刊:
- 影响因子:0
- 作者:Foland-Ross LC;Gil M;Shrestha SB;Chromik LC;Hong D;Reiss AL
- 通讯作者:Reiss AL
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Allan L Reiss其他文献
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{{ truncateString('Allan L Reiss', 18)}}的其他基金
Developmental trajectory of anxiety, avoidance, and arousal in girls with the FMR1 full mutation
FMR1 完全突变女孩的焦虑、回避和觉醒的发展轨迹
- 批准号:
10576763 - 财政年份:2022
- 资助金额:
$ 33.11万 - 项目类别:
Early life exposure to agricultural pesticides and functional brain imaging in young adults
年轻成人早期接触农业农药和功能性脑成像
- 批准号:
10303593 - 财政年份:2021
- 资助金额:
$ 33.11万 - 项目类别:
Early life exposure to agricultural pesticides and functional brain imaging in young adults
年轻成人早期接触农业农药和功能性脑成像
- 批准号:
10455703 - 财政年份:2021
- 资助金额:
$ 33.11万 - 项目类别:
Brain and Behavior during Puberty in Klinefelter Syndrome
克兰费尔特综合征青春期的大脑和行为
- 批准号:
10197985 - 财政年份:2018
- 资助金额:
$ 33.11万 - 项目类别:
Brain and Behavior during Puberty in Klinefelter Syndrome
克兰费尔特综合征青春期的大脑和行为
- 批准号:
9766339 - 财政年份:2018
- 资助金额:
$ 33.11万 - 项目类别:
Brain and Behavior during Puberty in Klinefelter Syndrome
克兰费尔特综合征青春期的大脑和行为
- 批准号:
10430045 - 财政年份:2018
- 资助金额:
$ 33.11万 - 项目类别:
Brain Development & Sex Chromosomes: Imaging of Turner and Klinefelter Syndromes
大脑发育
- 批准号:
8443566 - 财政年份:2013
- 资助金额:
$ 33.11万 - 项目类别:
Brain Development & Sex Chromosomes: Imaging of Turner and Klinefelter Syndromes
大脑发育
- 批准号:
8653989 - 财政年份:2013
- 资助金额:
$ 33.11万 - 项目类别:
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