The Association of Quality of Life with Psychosocial Factors in Adolescents with Tourette Syndrome.

Individuals with Tourette syndrome (TS) have poorer quality of life (QoL) than their peers, yet factors contributing to poor QoL in this population remain unclear. Research to date has predominantly focused on the impact of tics and psychiatric symptoms on QoL in TS samples. The aim of this cross-sectional, multi-informant study was to identify psychosocial variables that may impact adolescent QoL in TS. Thirty-eight adolescents aged 13 to 17 with TS and 28 age-matched controls participated with a caregiver. No group differences were found on QoL, although the TS group reported reduced QoL compared to population normative data. In the TS group, reduced QoL was associated with lower self-esteem, poorer family functioning, higher stress, and greater depression and anxiety; QoL was unrelated to tic severity. In regression analyses, after adjusting for covariates, family functioning was the strongest predictor of QoL. These results emphasize the need to further explore the influence of psychosocial factors, particularly family functioning, on QoL in adolescents with TS.

Impairments to executive function in emerging adults with Huntington disease.

BACKGROUND AND OBJECTIVES: The clinical diagnosis of Huntington disease (HD) is typically made once motor symptoms and chorea are evident. Recent reports highlight the onset of cognitive and psychiatric symptoms before motor manifestations. These findings support further investigations of cognitive function across the lifespan of HD sufferers. METHODS: To assess cognitive symptoms in the developing brain, we administered assessments from the National Institutes of Health Toolbox Cognitive Battery, an age-appropriate cognitive assessment with population norms, to a cohort of children, adolescents and young adults with (gene-expanded; GE) and without (gene-not-expanded; GNE) the trinucleotide cytosine, adenine, guanine (CAG) expansion in the Huntingtin gene. These five assessments that focus on executive function are well validated and form a composite score, with population norms. We modelled these scores across age, and CAP score to estimate the slope of progression, comparing these results to motor symptoms. RESULTS: We find significant deficits in the composite measure of executive function in GE compared with GNE participants. GE participant performance on working memory was significantly lower compared with GNE participants. Modelling these results over age suggests that these deficits occur as early as 18 years of age, long before motor manifestations of HD. CONCLUSIONS: This work provides strong evidence that impairments in executive function occur as early as the second decade of life, well before anticipated motor onset. Future investigations should delineate whether these impairments in executive function are due to abnormalities in neurodevelopment or early sequelae of a neurodegenerative process.

Mean-level correspondence and moment-to-moment synchrony in adolescent and parent affect: Exploring associations with adolescent age and internalizing and externalizing symptoms.

Interactions with parents are integral in shaping the development of children's emotional processes. Important aspects of these interactions are overall (mean level) affective experience and affective synchrony (linkages between parent and child affect across time). Respectively, mean-level affect and affective synchrony reflect aspects of the content and structure of dyadic interactions. Most research on parent-child affect during dyadic interactions has focused on infancy and early childhood; adolescence, however, is a key period for both normative emotional development and the emergence of emotional disorders. We examined affect in early to mid-adolescents (N = 55, Mage = 12.27) and their parents using a video-mediated recall task of 10-min conflict-topic discussions. Using multilevel modeling, we found evidence of significant level-2 effects (mean affect) and level-1 effects (affective synchrony) for parents and their adolescents. Level-2 and level-1 associations were differentially moderated by adolescent age and adolescent internalizing and externalizing symptoms. More specifically, parent-adolescent synchrony was stronger when adolescents were older and had more internalizing problems. Further, more positive adolescent mean affect was associated with more positive parent affect (and vice versa), but only for dyads with low adolescent externalizing problems. Results underscore the importance of additional research examining parent-child affect in adolescence.

COVID-19 stressors and symptoms of anxiety and depression in a community sample of children and adolescents.

BACKGROUND: The COVID-19 public health crisis has created abrupt and unparalleled disruptions to the daily lives of children and adolescents across the world, placing them at significant risk for developing symptoms of anxiety and depression. METHOD: The current study used two data collection periods to determine which types of COVID-19-related stressors were associated with the greatest risk of anxiety and depression symptoms in a community sample of children and adolescents in the United States (U.S.) from May-August 2020 (T1) to February-April 2021 (T2). Seventy-nine youth (ages 10-17; M = 13.41, SD = 2.10; 54.4% female) completed a battery of online standardized questionnaires about COVID-19 stress and psychiatric symptoms at T1 and 56 of these also participated at T2. RESULTS: The majority of children and adolescents reported experiencing the COVID-19-related stressors in multiple domains including daily routines, interpersonal relationships, education, finances, and health. A substantial proportion of the sample reported clinical levels of depression and anxiety symptoms at both T1 and T2. Multiple linear regression analyses revealed that, controlling for T1 anxiety and depression symptoms, T2 interpersonal stressors were significantly associated with elevated depression and anxiety scores at T2. CONCLUSIONS: The findings highlight the salience of social connection for children and adolescents, and may also underscore the risk associated with lockdown restrictions, social distancing, and school closures during the pandemic.

High prevalence of idiopathic (islet antibody-negative) type 1 diabetes among Indian children and adolescents.

OBJECTIVES: To study the prevalence and clinical characteristics of islet antibody-negative (idiopathic) type 1 diabetes mellitus (T1DM) among Indian children and adolescents at the time of diagnosis of illness. METHODS: In a hospital-based cross-sectional study, we studied 110 patients with T1DM aged ≤18 years. This included 61 patients with duration of diabetes ≤2 weeks (mean ± SD age of onset 9.9 ± 4.4 years) and 49 patients with duration 2 to 12 weeks. Antibodies against GAD65 (GADA), IA-2 (IA-2A) and zinc transporter 8 (ZnT8A), detected by radio-binding assay, were measured in all patients. Insulin autoantibody (IAA) was measured only in subjects with duration ≤2 weeks, using a competitive radio-binding assay. RESULTS: The prevalence of GADA, IA-2A, and ZnT8A was 53%, 34%, and 29% respectively, while IAA (measured in 61 patients) was detected in 31%. All four antibodies were absent in 17 of 61 (28%) patients. The prevalence of islet antibody-negative patients was similar among both sexes and in children with onset younger and older than 10 years. ZnT8A was the only antibody detected in four patients, and its measurement resulted in 6% reduction in islet antibody-negative patients. Patients with idiopathic T1DM did not differ in their clinical features or fasting plasma C-peptide at the onset and after follow-up of 1 year. Compared with idiopathic T1DM, antibody-positive patients had an increased allele frequency of HLA DRB1*0301 (46% vs 14%, OR = 5.10 [confidence interval = 1.61-16.16], P = .003). CONCLUSION: Nearly 30% of Indian patients were negative for all islet antibodies at the onset of T1DM. Patients with idiopathic T1DM had similar clinical features to antibody-positive subjects.

Neurobiological and Psychosocial Correlates of Communication Between Huntington's Disease Patients and Their Offspring.

OBJECTIVE: Huntington's disease (HD) is an autosomal dominant neurodegenerative disease that presents significant challenges to family communication. The investigators examined observations of communication between parents with HD and their offspring talking about the challenges of HD and explored potential correlates of their communication. METHODS: The sample included parents with HD and their adolescent and young-adult offspring (N=64). Parent communication and chorea were independently coded from video recordings. Parents and offspring completed working memory assessments and self-reports of neuropsychiatric symptoms, stress, and coping. RESULTS: Evidence was found for the association of observed parent-offspring communication with disease markers, psychosocial characteristics, and neurocognitive function. For parents, disease markers and working memory were correlates of communication, whereas offspring's psychiatric symptoms, stress, and coping were associated with their communication. CONCLUSIONS: These findings have potential implications for clinical interventions to enhance communication and quality of life for HD families.

Cognitive and Attentional Function in Children with Hypoplastic Left Heart Syndrome: A Pilot Study.

While survival for children with hypoplastic left heart syndrome (HLHS) has improved, compromised cardiac output and oxygen delivery persist, and children show cognitive deficits. Most research has assessed young children on broad cognitive indices; less is known about specific indices in older youth. In this pilot study, cognitive function and attention in youth ages 8 to 16 years with HLHS (n = 20) was assessed with the Wechsler Intelligence Scale for Children - Fifth Edition (WISC-V) and NIH Toolbox Cognition Battery (NTCB); parents completed the Child Behavior Checklist. Children scored significantly lower than normative means on the WISC-V Full Scale IQ, Verbal Comprehension, Visual Spatial, Working Memory, and Processing Speed indices, and the NTCB Fluid Cognition Composite; effect sizes ranged from medium to large. Attention problems had a large significant effect. Child age corresponded to lower visual spatial scores. Findings highlight the importance of assessing multiple cognitive indices for targeted intervention and investigating age and disease factors as potential correlates in larger samples.

Coping with COVID-19 stress: Associations with depression and anxiety in a diverse sample of U.S. adults.

The COVID-19 pandemic has brought unprecedented levels of stress to individuals in the U.S. and throughout the world. These high stress levels place individuals at risk for symptoms of anxiety, depression, and other psychiatric disorders. The current study applies a control-based model of coping to contribute to the development of evidence-based interventions to promote resilience. Data were collected online from April 22 through July 12, 2020. Data from two samples of U. S. community adults who completed an online battery of standardized questionnaires were combined (N = 709). More than a quarter reported moderate to severe levels of depression symptoms, and more than one-fifth reported moderate to severe levels of anxiety symptoms; symptom levels were higher among adults who reported more COVID-19-related stress. As hypothesized, multiple regression analyses indicated that greater use of primary and secondary control coping was associated with lower symptom levels, whereas greater use of disengagement coping was associated with higher symptom levels, above and beyond the association of stress with symptoms. Race and ethnicity emerged as important moderators of these associations, indicating that what constitutes adaptive coping varies according to characteristics of the individual. Implications for public health policy and clinical practice are discussed. SUPPLEMENTARY INFORMATION: The online version contains supplementary material available at 10.1007/s12144-021-02444-6.

Stress Exposure and Maternal Depression as Risk Factors for Symptoms of Anxiety and Depression in Adolescents.

Little research has explored the implications of stress inside and outside of the family as a risk factor for psychological symptoms in adolescents of depressed mothers. In a sample of 115 adolescents and their mothers with and without depression histories, adolescents' family and peer stress exposure was measured through the Responses to Stress Questionnaire, and adolescents' anxious/depressed symptoms were measured with the Youth Self Report and Child Behavior Checklist. Mothers reported their current depression symptoms on the Beck Depression Inventory-II. Results suggest that adolescents of mothers with depression histories and current depression symptoms experience more family and peer stress than adolescents of nondepressed mothers. In multiple linear regression analyses, current maternal depression symptoms moderated the relation between adolescent peer stress and adolescent anxious/depressed symptoms, such that peer stress was associated with anxious/depressed symptoms when maternal depression symptoms were average or high, but not when maternal depression symptoms were low.

Responsive Parenting Behaviors and Cognitive Function in Children With Sickle Cell Disease.

OBJECTIVE: Children with sickle cell disease (SCD) are at increased risk for cognitive impairment as a result in part from biological characteristics of the disease; however, limited research has explored possible social and contextual factors associated with risk for cognitive problems. The primary aim of the present study was to examine the relation between children's cognitive functioning and responsive parenting, a potentially important contextual factor in children with SCD, accounting for family socioeconomic disadvantage, child disease severity, and caregivers' perceived stress. METHODS: Forty-eight children completed standardized cognitive assessments and caregivers provided self-reports of general and disease-related stress. Parent-child dyads completed a video recorded puzzle-solving task and observed parenting was quantified using two coding systems. Bivariate Pearson correlations were used to assess preliminary hypotheses, and linear multiple regression analyses were used to assess the primary hypothesis. RESULTS: Results suggested that increased levels of parental stress were related to fewer observations of responsive parenting and provided evidence of an association between children's cognitive function and responsive parenting. Specifically, increased disease-related parent stress and reduced parental use of expansive language were associated with significantly lower cognitive functioning in children with SCD. CONCLUSIONS: Findings suggest that social environmental factors along with disease characteristics are sources of risk for cognitive problems with children with SCD. Further, these findings highlight the need to develop targeted interventions for parents of children with SCD to decrease levels of stress and enhance parenting skills, with the aim improving cognitive functioning in youth.

Coping and Stress Reactivity as Moderators of Maternal Depressive Symptoms and Youth's Internalizing and Externalizing Symptoms.

Youth's responses to stress are a central feature of risk and resilience across development. The current study examined whether youth coping and stress reactivity moderate the association of current maternal depressive symptoms with youth's internalizing and externalizing symptoms. Mothers (Mage = 41.58, SD = 6.18) with a wide range of depressive symptoms and their children ages 9-15 (Mage = 12.25, SD = 1.89, 45.3% girls) completed measures of youth symptoms and coping and automatic responses to stress. Mothers also completed a self-report measure of depressive symptoms. Youth's primary and secondary control coping, stress reactivity, and involuntary disengagement moderated the association between current maternal depressive symptoms and youth symptoms. Maternal depressive symptoms were associated with youth's internalizing and externalizing symptoms when youth used low as opposed to high levels of primary and secondary control coping. Conversely, maternal depressive symptoms were associated with youth symptoms for youth with high levels of stress reactivity and involuntary disengagement. The findings suggest interventions focused on improving the use of primary and secondary control coping skills and reducing reactivity and involuntary disengagement to stress may benefit youth with mothers who are experiencing high levels of depressive symptoms.

In vitro reconstitution of Rab GTPase-dependent vesicle clustering by the yeast lethal giant larvae/tomosyn homolog, Sro7.

Intracellular traffic in yeast between the Golgi and the cell surface is mediated by vesicular carriers that tether and fuse in a fashion that depends on the function of the Rab GTPase, Sec4. Overexpression of either of two Sec4 effectors, Sro7 or Sec15, results in the formation of a cluster of post-Golgi vesicles within the cell. Here, we describe a novel assay that recapitulates post-Golgi vesicle clustering in vitro utilizing purified Sro7 and vesicles isolated from late secretory mutants. We show clustering in vitro closely replicates the in vivo clustering process as it is highly dependent on both Sro7 and GTP-Sec4. We also make use of this assay to characterize a novel mutant form of Sro7 that results in a protein that is specifically defective in vesicle clustering both in vivo and in vitro. We show that this mutation acts by effecting a conformational change in Sro7 from the closed to a more open structure. Our analysis demonstrates that the N-terminal propeller needs to be able to engage the C-terminal tail for vesicle clustering to occur. Consistent with this, we show that occupancy of the N terminus of Sro7 by the t-SNARE Sec9, which results in the open conformation of Sro7, also acts to inhibit vesicle cluster formation by Sro7. This suggests a model by which a conformational switch in Sro7 acts to coordinate Rab-mediated vesicle tethering with SNARE assembly by requiring a single conformational state for both of these processes to occur.

Re-conceiving building design quality: A review of building users in their social context.

Considerable overlap exists between post-occupancy research evaluating building design quality and the concept of 'social value', popularised by its recent application to issues of the public realm. To outline this potential research agenda, the paper reviews design quality research on buildings in relation to users and their social context where the term 'social context' refers to building user group dynamics, a combination of organisational cultures, management strategies, and social norms and practices. The review is conducted across five key building types, namely housing, workplaces, healthcare, education, and the retail/service sector. Research commonalities and gaps are identified in order to build a more comprehensive picture of the design quality literature and its handling of users in their social context. The key findings concerning each building type are presented visually. It is concluded that the design quality field comprises a patchwork of relatively isolated studies of various building types, with significant potential for theoretical and empirical development through interdisciplinary collaboration. Users tend to be conceived as anonymous and autonomous individuals with little analysis of user identity or interaction. Further, the contextual impact of user group dynamics on the relationship between building design and building user is rarely addressed in the literature. Producing a more nuanced understanding of users in situ is proposed as an important area for future design quality research.

Reducing youth internalizing symptoms: effects of a family-based preventive intervention on parental guilt induction and youth cognitive style.

This study utilized structural equation modeling to examine the associations among parental guilt induction (a form of psychological control), youth cognitive style, and youth internalizing symptoms, with parents and youth participating in a randomized controlled trial of a family-based group cognitive-behavioral preventive intervention targeting families with a history of caregiver depression. The authors present separate models utilizing parent report and youth report of internalizing symptoms. Findings suggest that families in the active condition (family-based group cognitive-behavioral group) relative to the comparison condition showed a significant decline in parent use of guilt induction at the conclusion of the intervention (6 months postbaseline). Furthermore, reductions in parental guilt induction at 6 months were associated with significantly lower levels of youth negative cognitive style at 12 months. Finally, reductions in parental use of guilt induction were associated with lower youth internalizing symptoms 1 year following the conclusion of the intervention (18 months postbaseline).

Coping and Emotion Regulation from Childhood to Early Adulthood: Points of Convergence and Divergence.

Processes of coping with stress and the regulation of emotion reflect basic aspects of development and play an important role in models of risk for psychopathology and the development of preventive interventions and psychological treatments. However, research on these two constructs has been represented in two separate and disconnected bodies of work. We examine possible points of convergence and divergence between these constructs with regard to definitions and conceptualization, research methods and measurement, and interventions to prevent and treat psychopathology. There is clear evidence that coping and emotion regulation are distinct but closely related constructs in all of these areas. The field will benefit from greater integration of methods and findings in future research.

Feasibility and Validity of In-Home Self-Collected Capillary Blood Spot Screening for Type 1 Diabetes Risk.

Aims: Self-collection of a blood sample for autoantibody testing has potential to facilitate screening for type 1 diabetes risk. We sought to determine the feasibility and acceptability of this approach and the performance of downstream antibody assays. Methods: People living with type 1 diabetes and their family members (N = 97) provided paired capillary blood spot and serum samples collected, respectively, by themselves and a health worker. They provided feedback on the ease, convenience, and painfulness of blood spot collection. Islet antibodies were measured in blood spots by antibody detection by agglutination PCR (ADAP) or multiplex enzyme-linked immunoassay (ELISA), and in serum by radioimmunoassay (RIA) or ELISA. Results: Using serum RIA and ELISA to define antibody status, 50 antibody-negative (Abneg) and 47 antibody-positive (Abpos) participants enrolled, of whom 43 and 47, respectively, returned testable blood spot samples. The majority indicated that self-collection was easier, more convenient, and less painful than formal venesection. The sensitivity and specificity for detection of Abpos by blood spot were, respectively, 85% and 98% for ADAP and 87% and 100% for multiplex ELISA. The specificities by ADAP for each of the four antigen specificities ranged from 98% to 100% and areas under the receiver operator curve from 0.841 to 0.986. Conclusions: Self-collected blood spot sampling is preferred over venesection by research participants. ADAP and multiplex ELISA are highly specific assays for islet antibodies in blood spots with acceptable performance for use alone or in combination to facilitate screening for type 1 diabetes risk. Clinical Trial Registration number: ACTRN12620000510943.

Stress in Huntington's Disease: Characteristics and Correlates in Patients and At-Risk Individuals.

Background: Huntington's disease (HD) is a neurodegenerative disease that presents families with significant numbers of stressful events. However, relatively little empirical research has characterized the stressors encountered by members of HD-affected families and their correlations with psychological symptoms. Objective: This study examined frequencies of specific stressors in HD patients and at-risk individuals and the correlates of these stressors with demographics, disease characteristics, and symptoms of depression and anxiety. Methods: HD patients (n = 57) and at-risk individuals (n = 81) completed the Responses to Stress Questionnaire -Huntington's Disease Version to assess HD-related stressors. Participants completed measures of depression and anxiety symptoms. Patient health records were accessed to obtain information related to disease characteristics. Results: Patients endorsed a mean number of 5.05 stressors (SD = 2.74) out of the 10-item list. Demographics were not related to total stressors, but disease characteristics were significantly related to specific stressors. At-risk individuals endorsed a mean number of 3.20 stressors (SD = 2.65) out of the 11-item list. Age and sex were significantly related to specific stressors. Total number of stressors was significantly related to depression (ß=0.67, p < 0.001) and anxiety symptoms (ß=0.58, p < 0.001) in patients and at-risk individuals (ß=0.35, p = 0.003 and ß=0.32, p = 0.006, respectively). Conclusions: hese findings emphasize the significant burden of stress experienced by HD patients and at-risk individuals. We highlight a need for more specific stress-based measures and psychosocial support interventions for HD-affected families.

Adolescents' Coping and Internalizing Symptoms: Role of Maternal Socialization of Coping and Depression Symptoms.

Parental emotion socialization, including processes of the socialization of coping and emotion regulation, is a key factor in shaping children's adjustment in response to acute and chronic stress. Given well-established links between parental depression and youth psychopathology, levels of parental depression symptoms are an important factor for understanding emotion socialization and regulation processes. The present study examined associations among maternal coping and depression symptoms with their adolescents' coping and internalizing problems. A sample of 120 adolescents (45% female, M = 12.27, SD = 1.90) and their mothers participated in a cross-sectional, multi-informant study. Mothers' depression symptoms and adolescents' coping were significantly related to adolescents' internalizing problems. Adolescents' coping moderated the association between maternal depression symptoms and adolescents' internalizing problems, where at low and moderate levels of primary control coping, maternal depression predicted greater internalizing symptoms in adolescents. Further, this study expanded on prior work, demonstrating that the relationship between adolescents' coping and internalizing symptoms was associated with the degree to which mothers model coping. Taken together, results suggest that maternal coping and adolescent coping serve as salient risk and protective factors in the context of family stress. Findings emphasize a need for researchers to further clarify the role of emotion socialization processes in adolescents' development of coping in the context of family stress.

Intrapersonal and Interpersonal Disengagement Coping: Associations with Emotions of Youth At-Risk for Huntington's Disease.

BACKGROUND: Families in which a parent has Huntington's disease (HD) are faced with significant stressors that can contribute to difficulties in communicating together about illness-related concerns. Family members who use more disengagement coping strategies, including denial and avoidance, to deal with illness-related stressors may have the greatest challenges to effective communication. OBJECTIVE: The current study examined the associations of intrapersonal and interpersonal disengagement coping responses with observed and reported emotions of adolescents and young adults (AYA) at genetic risk for HD. METHODS: Families included 42 AYA (n = 26 females) ages 10 to 34 (M = 19 years, 11 months; SD = 7 years, 6 months) and their parent with HD (n = 22 females, M age = 46 years, 10 months; SD = 9 years, 2 months). Dyads participated in observations of communication and completed questionnaires about disengagement coping and internalizing symptoms. RESULTS: Disengagement coping of AYA was unrelated to their observed and reported emotional difficulties (intrapersonal coping). However, there was evidence for the importance of interpersonal disengagement coping, as AYA's negative affect was observed and reported to be highest when both AYA and their parents reported using high levels of avoidance, denial, and wishful thinking to cope with HD-related stress. CONCLUSION: The findings underscore the importance of a family-oriented approach to coping and communication in families affected by HD.

Physiology and emotions: Within individual associations during caregiver-adolescent conflict.

Concordance between physiological and emotional responses is central to models of emotion and has been shown to correspond to effective responses and well-being in adults. A deeper understanding of physiological-emotional concordance during ecologically relevant scenarios is essential to then determine if these associations predict mental health problems or can serve as a helpful biomarker of risk or resilience in adults and youth. The present study assessed the minute-to-minute associations between sympathetic (i.e., skin conductance level [SCL]) and parasympathetic (i.e., respiratory sinus arrhythmia [RSA]) nervous system activity and self-reported emotions, assessed via video-mediated recall procedures, during a parent-adolescent conflict discussion task. Associations between emotion ratings and physiological activity were assessed in adolescents (N = 97; ages 10-15) and their adult caregivers (N = 97). Utilizing a multilevel modeling approach, findings demonstrated a significant positive association between SCL and emotion ratings for youth, suggesting that increased engagement and alertness contributed to more positive emotion. RSA was unrelated to emotion ratings. The presence of significant variability in associations indicated the presence of potential moderators. This could include clinically relevant processes (e.g., emotion regulation, relationship quality, and mental health). Future research should continue to build on findings to determine if, when, and for whom, physiological-emotional concordance occurs, and whether the degree of concordance predicts risk for mental and physical health problems.