Cognitive flexibility assessment in youth with Down syndrome: Reliability, practice effects, and validity.

BACKGROUND: Cognitive flexibility refers to the ability to switch between different mental sets, tasks, or strategies and is challenging for some individuals with Down syndrome (DS). The lack of reliable and valid cognitive flexibility measures for individuals with DS is a major barrier to clinical trials and intervention studies designed to address cognitive challenges specific to DS. To avoid measurement limitations that could confound interpretations of performance in clinical trials in children with DS, it is critical to use phenotype-sensitive and psychometrically sound measures of cognitive flexibility. AIM: This study aimed to evaluate the psychometric properties of three measures of cognitive flexibility including Rule-Shift, Weigl Sorting, and KiTAP Flexibility in a sample of 97 youth with DS aged 6-17 years old. METHOD: Data were collected at two time points with a two-week interval. Parents also completed adaptive behavior and cognitive flexibility questionnaires. Child cognitive and language abilities were also assessed. RESULTS: The Weigl Sorting met the most psychometric criteria, with adequate feasibility (≥ 80 %) and significant correlations with most of the broader developmental domains; however, the levels of test-retest reliability, practice effects, and convergent validity did not meet a priori criteria. Rule-Shift and KiTAP Flexibility measures did not have acceptable feasibility; although sensitivity and specificity analyses revealed that Rule-Shift may be appropriate for a subgroup of the participants. CONCLUSION: No evaluated measures met all psychometric study criteria and, therefore, additional evaluation of cognitive flexibility measures is needed for use among individuals with DS.

Musculoskeletal Ultrasound in Childhood Arthritis Limited Examination: A Comprehensive, Reliable, Time-Efficient Assessment of Synovitis.

OBJECTIVE: To develop and initially validate a comprehensive pediatric musculoskeletal ultrasound (MSUS) joint-specific scoring system, and to determine the minimum number of joints needed to identify active disease. METHODS: A semiquantitative scoring system was developed by consensus and initially validated by interrater reliability using intraclass correlation coefficients (ICCs). Subsequently, newly diagnosed juvenile idiopathic arthritis patients with an active joint count of >4 had a 42-joint MSUS performed at baseline and 3 months using this protocol. A minimum set of joints needed to identify all patients with synovitis on MSUS was obtained through a data reduction process. Spearman's correlation (rs ) was calculated to determine the association between MSUS findings and clinical Juvenile Arthritis Disease Activity Score in 10 joints (cJADAS10). Standardized response means (SMRs) were used to assess change over time. RESULTS: The final joint-specific scoring system revealed an excellent interrater reliability (ICC 0.81-0.96) for all joints. Thirty patients were enrolled. Scanning 5 joints bilaterally (wrists, second and third metacarpophalangeal joints, knees and ankles) captured 100% of children with B-mode synovitis and had moderate correlation with the cJADAS10 at baseline (rs  = 0.45). Mean ultrasound scores at baseline and follow-up were 28.3 and 22.3, with an SRM of 0.69 (P = 0.002) for 42 joints, and 36 and 27.7, with an SRM of 0.76 (P = 0.003) for the reduced joints, respectively. CONCLUSION: A limited MSUS examination called musculoskeletal ultrasound in childhood arthritis limited examination (MUSICAL) captures all patients with active synovitis, and our new joint-specific scoring system is highly reliable and sensitive to change.

Understanding racial disparities in childhood asthma using individual- and neighborhood-level risk factors.

BACKGROUND: Racial disparities in childhood asthma outcomes result from a complex interplay of individual- and neighborhood-level factors. OBJECTIVES: We sought to examine racial disparities in asthma-related emergency department (ED) visits between African American (AA) and European American (EA) children. METHODS: This is a retrospective study of patients younger than 18 years who visited the ED at Cincinnati Children's for asthma from 2009 to 2018. The outcome was number of ED visits during a year. We assessed 11 social, economic, and environmental variables. Mediation and mixed-effects analyses were used to assess relationships between race, mediators, and number of ED visits. RESULTS: A total of 31,114 children (46.1% AA, 53.9% EA) had 186,779 asthma-related ED visits. AA children had more visits per year than EA children (2.23 vs 2.15; P < .001). Medicaid insurance was associated with a 7% increase in rate of ED visits compared with commercial insurance (1.07; 95% CI, 1.03-1.1). Neighborhood socioeconomic deprivation was associated with an increased rate of ED visits in AA but not in EA children. Area-level particulate matter with diameter less than 2.5 µm, pollen, and outdoor mold were associated with an increased rate of ED visits for both AA and EA children (all P < .001). Associations between race and number of ED visits were mediated by insurance, area-level deprivation, particulate matter with diameter less than 2.5 µm, and outdoor mold (all P < .001), altogether accounting for 55% of the effect of race on ED visits. Race was not associated with number of ED visits (P = .796) after accounting for mediators. CONCLUSIONS: Racial disparities in asthma-related ED visits are mediated by social, economic, and environmental factors, which may be amenable to interventions aimed at improving outcomes and eliminating inequities.

Comprehensive Assessment of Quality of Life, Functioning, and Mental Health in Children With Juvenile Idiopathic Arthritis and Noninfectious Uveitis.

OBJECTIVE: Pediatric uveitis can lead to sight-threatening complications and can impact quality of life (QoL) and functioning. We aimed to examine health-related QoL, mental health, physical disability, vision-related functioning (VRF), and vision-related QoL in children with juvenile idiopathic arthritis (JIA), JIA-associated uveitis (JIA-U), and other noninfectious uveitis. We hypothesized that there will be differences based on the presence of eye disease. METHODS: A multicenter cross-sectional study was conducted at four sites. Patients with JIA, JIA-U, or noninfectious uveitis were enrolled. Patients and parents completed the Pediatric Quality of Life Inventory (PedsQL; health-related QoL), the Revised Childhood Anxiety and Depression Scale (RCADS; anxiety/depression), the Childhood Health Assessment Questionnaire (C-HAQ; physical disability), and the Effects of Youngsters' Eyesight on Quality of Life (EYE-Q) (VRF/vision-related QoL). Clinical characteristics and patient-reported outcome measures were compared by diagnosis. RESULTS: Of 549 patients, 332 had JIA, 124 had JIA-U, and 93 had other uveitis diagnoses. Children with JIA-U had worse EYE-Q scores compared to those with JIA only. In children with uveitis, those with anterior uveitis (JIA-U and uveitis only) had less ocular complications, better EYE-Q scores, and worse C-HAQ and PedsQL physical summary scores compared to those with nonanterior disease. In children with anterior uveitis, those with JIA-U had worse PedsQL physical summary and C-HAQ scores than anterior uveitis only. Further, EYE-Q scores were worse in children with bilateral uveitis and more visual impairment. There were no differences in RCADS scores among groups. CONCLUSION: We provide a comprehensive outcome assessment of children with JIA, JIA-U, and other uveitis diagnoses. Differences in QoL and function were noted based on underlying disease. Our results support the addition of a vision-specific measure to better understand the impact of uveitis.

Trends in Number of Women Speakers at the American Head and Neck Society Meetings, 2007-2019.

Importance: The number of female speakers at American Head and Neck Society (AHNS) conferences should ideally be consistent with the number of women entering head and neck surgery fellowships to ensure gender equity in the field. Yet the presence of women speakers at the annual AHNS meetings, which is specific to the field of head and neck cancer, endocrine and microvascular reconstructive surgery, has yet to be studied. Objective: To determine whether the proportion of female speakers at the AHNS has increased in a manner consistent with the numbers of women entering fellowships since 2007. Design, Setting, and Participants: This qualitative study assessed 13 final meeting programs from AHNS national/international conferences from 2007 to 2019. The number of male and female participants in different roles throughout the meeting were retrospectively tracked. Participants were male and female speakers at AHNS national/international conferences who took part in the roles of scientific session presenter, scientific session moderator, expert panelist, miscellaneous moderator, and named lecturers/keynote speaker. Gender of the speaker was determined by searching names on the internet and using available published pronouns. Main Outcomes and Measures: Number of speaking opportunities for men and women in different roles from 2007 to 2019 as well as number of men and women entering AHNS fellowships since 2007 and new active AHNS members since 2012. Results: In this qualitative study, from 2007 to 2019, 4059 speakers were identified. Of these speakers, 902 (22%) were women and 3157 (78%) were men. Overall, there was a strong correlation between increasing years and number of women speakers from 2007 to 2019 (ρ = 0.75; 95% CI, 0.72-0.78). There were 2096 invited speaking roles that excluded research presentations, of which 400 were offered to female participants (19.1%) across the study period. There were 131 different women that made up all 400 of the opportunities that were offered to women in the years surveyed. There was a strong correlation in the proportion of women as presenters for oral abstracts, expert panelists, and miscellaneous moderators between the years but no correlation in scientific session moderators and named lecturers/keynote speakers. Of the 45 named lecturers/keynote speakers in the programs tracked, only 2 were women. Conclusions and Relevance: In this study, from 2007 to 2019, the presence of women at ANHS has increased overall, reflecting the changing demographic characteristics of those entering in head and neck oncology and microvascular surgery fellowships. However, a strong disparity continues to exist for preeminent speaking opportunities.

Flexible multivariate joint model of longitudinal intensity and binary process for medical monitoring of frequently collected data.

A frequent problem in longitudinal studies is that data may be assessed at subject-selected, irregularly spaced time-points, resulting in highly unbalanced outcome data, inducing bias, especially if availability of data is directly related to outcome. Our aim was to develop a multivariate joint model in a mixed outcomes framework to minimize irregular sampling bias. We demonstrate using blood glucose monitoring throughout pregnancy and risk of preterm birth among women with type 1 diabetes mellitus. Blood glucose measurements were unequally spaced and intensity of sampling varied between and within individuals over time. Multivariate linear mixed effects submodel for the longitudinal outcome (blood glucose), Poisson model for the intensity of glucose sampling, and logistic regression model for binary process (preterm birth) were specified. Association between models is captured through shared random effects. Markov chain Monte Carlo methods were used to fit the model. The multivariate joint model provided better prediction, compared with a joint model with a multivariate linear mixed effects submodel (ignoring intensity of glucose sampling) and a two-stage model. Most association parameters were significant in the preterm birth outcome model, signifying improvement of predictive ability of the binary endpoint by sharing random effects between glucose monitoring and preterm birth. A simulation study is presented to illustrate the effectiveness of the multivariate joint modeling approach.

Female Representation in Otolaryngology Leadership Roles.

OBJECTIVES/HYPOTHESIS: Characterization of leadership within otolaryngology is key to evaluating trends and promoting program advancement. This study evaluates representation of women in otolaryngology holding residency and fellowship directorships, or chair positions, comparing sex differences in academic rank, years in practice, and scholarly activity. STUDY DESIGN: Cross sectional analysis. METHODS: A comprehensive list of otolaryngology residency and fellowship directors from Accreditation Council for Graduate Medical Education-accredited programs in 2017 to 2018 was compiled. Academic rank and years in practice were determined from departmental websites, with online search tools used as secondary resources. The h-index was utilized as a measure of research productivity. Regression analysis was performed to analyze these variables. RESULTS: Among the 306 directorships, women held 57 (18.6%) of these positions, 27 (26.5%) residency and 30 (14.7%) fellowship directorships. Of the 99 chair positions, five (5.1%) were held by women. The majority (53.6%) of male directors were full professors, whereas only 26.3% of females were full professors (P = .04). Mean years in practice for female directors (13.9 ± 6.8) was less than that of male directors (20.3 ± 9.4, P < .0001). Similarly, mean h-index for female directors (11 ± 7.2) was lower than for males (17.5 ± 12.5, P = .0001). After controlling for academic rank and years of practice in a multivariable regression, the h-index remained lower for women than for men (P = .03). CONCLUSIONS: Women are disproportionately underrepresented in positions of residency and fellowship directorships, and chair positions, consistent with other specialties. Women in leadership had lower academic ranks, fewer years of practice, and lower h-indices than their male counterparts. This may represent a shift in academic otolaryngology as female otolaryngologists seek early involvement in leadership. LEVEL OF EVIDENCE: 4 Laryngoscope, 130:1664-1669, 2020.

Comparison of fMRI data from passive listening and active-response story processing tasks in children.

PURPOSE: To use functional MRI (fMRI) methods to visualize a network of auditory and language-processing brain regions associated with processing an aurally-presented story. We compare a passive listening (PL) story paradigm to an active-response (AR) version including online performance monitoring and a sparse acquisition technique. MATERIALS AND METHODS: Twenty children (ages 11-13 years) completed PL and AR story processing tasks. The PL version presented alternating 30-second blocks of stories and tones; the AR version presented story segments, comprehension questions, and 5-second tone sequences, with fMRI acquisitions between stimuli. fMRI data was analyzed using a general linear model approach and paired t-test identifying significant group activation. RESULTS: Both tasks showed activation in the primary auditory cortex, superior temporal gyrus bilaterally, and left inferior frontal gyrus (IFG). The AR task demonstrated more extensive activation, including the dorsolateral prefrontal cortex and anterior/posterior cingulate cortex. Comparison of effect size in each paradigm showed a larger effect for the AR paradigm in a left inferior frontal region-of-interest (ROI). CONCLUSION: Activation patterns for story processing in children are similar in PL and AR tasks. Increases in extent and magnitude of activation in the AR task are likely associated with memory and attention resources engaged across acquisition intervals.

Functional magnetic resonance imaging assessment of cognitive function in childhood-onset systemic lupus erythematosus: a pilot study.

OBJECTIVE: To investigate changes in brain activation patterns detected by functional magnetic resonance imaging (FMRI), and the relationship between FMRI activation patterns and results of formal neuropsychological testing, in patients with childhood-onset systemic lupus erythematosus (SLE). METHODS: Ten patients with childhood-onset SLE underwent formal neuropsychological testing and FMRI using 3 paradigms: a continuous performance task (CPT) to evaluate attention, an N-Back task to assess working memory, and verb generation to evaluate language processing. Composite Z maps were generated to summarize the brain activation patterns for each FMRI paradigm in patients with childhood-onset SLE and to compare these patterns with those observed in healthy controls. Between-group comparison Z maps showing differences in activation between childhood-onset SLE patients and controls were generated, using a significance level of P < 0.05 in a general linear model. RESULTS: Compared with the control group, the childhood-onset SLE group showed statistically significant increased activation of brain areas involved in the CPT, N-Back, and verb generation tasks. In contrast, in the absence of active stimulus, e.g., during times of the paradigm control tasks, childhood-onset SLE patients consistently undersuppressed activity in the expected brain areas. Activation in selected cortical areas was found to correlate negatively with results of a subset of individual neuropsychological test scores. CONCLUSION: FMRI abnormalities are present in childhood-onset SLE, manifesting as an imbalance between active and inhibitory responses to an array of stimuli. Differences in brain activation patterns compared with those observed in controls suggest that childhood-onset SLE may be associated with abnormalities in white matter connectivity resulting in neuronal network dysfunction, rather than injury of specific gray matter areas.

Determinants of optimal breast-feeding in peri-urban Guatemala City, Guatemala.

OBJECTIVE: This survey was conducted to identify factors affecting early initiation of breast-feeding and exclusive breast-feeding among mothers in peri-urban Guatemala City, Guatemala. MATERIALS AND METHODS: In early November 1999 a census was begun in four communities of peri-urban Guatemala City, Guatemala, to identify all children < 6 months old as well as all pregnant women who were expected to deliver during the two-month data-gathering period. After the census was completed, a survey of breast-feeding practices was administered to all mothers of infants < 6 months of age. RESULTS: The most important determinant of early initiation of breast-feeding was place of delivery. Children born at home (odds ratio (OR) = 4.1, 95% confidence interval (CI): 1.2-13.3) and at Ministry of Health health centers (OR = 4.9, 95% CI: 1.6-15.0) were significantly more likely than children born at private hospitals to initiate breast-feeding early. The most important determinant of exclusive breast-feeding was whether or not the mother worked outside the home. After controlling for infant's age and sex and mother's ethnicity, women who did not work outside the home were 3.2 times as likely (95% CI: 1.6-6.4) to exclusively breast-feed as were women who worked outside the home. Lack of exclusive breast-feeding was often associated with giving a bottle. CONCLUSIONS: Our findings suggest that global efforts must be continued to improve breast-feeding practices in delivery hospitals. In addition, community-based support of breast-feeding is needed well after delivery. Mothers who work outside the home should be provided with options to maintain exclusive breast-feeding when they are physically separated from their infants due to the demands of work.

Determinants of optimal breast-feeding in peri-urban Guatemala City, Guatemala

Objective. This survey was conducted to identify factors affecting early initiation of breast-feeding and exclusive breast-feeding among mothers in peri-urban Guatemala City, Guatemala. Materials and Methods. In early November 1999 a census was begun in four communities of peri-urban Guatemala City, Guatemala, to identify all children < 6 months old as well as all pregnant women who were expected to deliver during the two-month data-gathering period. After the census was completed, a survey of breast-feeding practices was administered to all mothers of infants < 6 months of age. Results. The most important determinant of early initiation of breast-feeding was place of delivery. Children born at home (odds ratio (OR) = 4.1, 95 percent confidence interval (CI): 1.2- 13.3) and at Ministry of Health health centers (OR = 4.9, 95 percent CI: 1.6-15.0) were significantly more likely than children born at private hospitals to initiate breast-feeding early. The most important determinant of exclusive breast-feeding was whether or not the mother worked outside the home. After controlling for infant's age and sex and mother's ethnicity, women who did not work outside the home were 3.2 times as likely (95 percent CI: 1.6-6.4) to exclusively breast-feed as were women who worked outside the home. Lack of exclusive breast-feeding was often associated with giving a bottle. Conclusions. Our findings suggest that global efforts must be continued to improve breast-feeding practices in delivery hospitals. In addition, community-based support of breast-feeding is needed well after delivery. Mothers who work outside the home should be provided with options to maintain exclusive breast-feeding when they are physically separated from their infants due to the demands of work