Parent Proxy-report Research Articles

1004-P: Executive Dysfunction Predicts Lower Self-Care Readiness in Teens with Type 1 Diabetes

Aim: Management of type 1 diabetes (T1D) is a complex multi-step process in which executive function (EF) skills (planning, organization, problem solving) are critical for optimal control. In teens with T1D, EF may be important in transitioning from parent-supervised care to self-care. Methods: In an 18-month study, teens aged 13-17 years with T1D and parents completed the Diabetes Family Responsibility Questionnaire (DFR) and Readiness for Independent Self-Care Questionnaire (RISQ) every 6 months. Teen EF was assessed by parent proxy-report using Behavior Rating Inventory of Executive Function (BRIEF) . T-score ≥60 defined risk of executive dysfunction for the Global EF Composite (GEC) , Behavioral Regulation Index (BRI) and Metacognition Index (MI) . EF, demographic, and T1D treatment variables were used to predict change in RISQ over time using longitudinal mixed models with false discovery rate adjustment. Results: 169 teens (54% male) participated with baseline age 15.9±1.3 years, T1D duration 8.4±3.7 years, HbA1c 8.6±1.2%, 74.6% insulin pump use, and 21.3% continuous glucose monitor (CGM) use. 31.4% of teens had a parent-reported GEC score ≥60. After adjusting for age, gender, and parent-reported DFR (pDFR) , those with GEC ≥60 had parent-reported RISQ (pRISQ) 4.1 points lower than those with GEC <60, p=0.016. BRI (adjusted for age, CGM use, pDFR, and HbA1c) did not predict change in pRISQ while MI (adjusted for age, gender, and pDFR) did; those with MI ≥60 had pRISQ 5.3 points lower than those with MI <60, p<0.001. In models using teen-reported DFR and RISQ (tRISQ) , GEC and MI similarly predicted lower tRISQ. In all models, lower age (p<0.05) and greater parent involvement (p<0.001) also predicted lower RISQ. Conclusion: EF, especially metacognition, appears important for acquisition of self-care behaviors for teens with T1D. Evaluating EF during adolescence may identify teens with executive dysfunction who may need extra support during the transition process. Disclosure R.J.Vitale: None. L.J.Tinsley: None. L.K.Volkening: None. L.M.Laffel: Advisory Panel; Medtronic, Roche Diabetes Care, Consultant; Boehringer Ingelheim International GmbH, Dexcom, Inc., Dompé, Insulet Corporation, Janssen Pharmaceuticals, Inc., Lilly Diabetes, Novo Nordisk, Provention Bio, Inc. Funding NIH T32DK007529NIH P30DK036836NIH R01DK095273JDRF 2-SRA-2014-253-M-B

Development and Evaluation of Pediatric Versions of the Vanderbilt Fatigue Scale for Children With Hearing Loss.

Growing evidence suggests that fatigue associated with listening difficulties is particularly problematic for children with hearing loss (CHL). However, sensitive, reliable, and valid measures of listening-related fatigue do not exist. To address this gap, this article describes the development, psychometric evaluation, and preliminary validation of a suite of scales designed to assess listening-related fatigue in CHL: the pediatric versions of the Vanderbilt Fatigue Scale (VFS-Peds). Test development employed best practices, including operationalizing the construct of listening-related fatigue from the perspective of target respondents (i.e., children, their parents, and teachers). Test items were developed based on input from these groups. Dimensionality was evaluated using exploratory factor analyses (EFAs). Item response theory (IRT) and differential item functioning (DIF) analyses were used to identify high-quality items, which were further evaluated and refined to create the final versions of the VFS-Peds. The VFS-Peds is appropriate for use with children aged 6-17 years and consists of child self-report (VFS-C), parent proxy-report (VFS-P), and teacher proxy-report (VFS-T) scales. EFA of child self-report and teacher proxy data suggested that listening-related fatigue was unidimensional in nature. In contrast, parent data suggested a multidimensional construct, composed of mental (cognitive, social, and emotional) and physical domains. IRT analyses suggested that items were of good quality, with high information and good discriminability. DIF analyses revealed the scales provided a comparable measure of fatigue regardless of the child's gender, age, or hearing status. Test information was acceptable over a wide range of fatigue severities and all scales yielded acceptable reliability and validity. This article describes the development, psychometric evaluation, and validation of the VFS-Peds. Results suggest that the VFS-Peds provide a sensitive, reliable, and valid measure of listening-related fatigue in children that may be appropriate for clinical use. Such scales could be used to identify those children most affected by listening-related fatigue, and given their apparent sensitivity, the scales may also be useful for examining the effectiveness of potential interventions targeting listening-related fatigue in children. https://doi.org/10.23641/asha.19836154.

Translation and Validation of Urdu Version of Pediatric Quality of Life Inventory Generic Core Scale for Neoplase Children

Aim: To Translate and validate the Urdu version the Pediatric Quality of Life Inventory Generic Core Scale among neoplase children. Methods: The descriptive cross-cultural linguistic validation study in which PedsQL Generic core scale was conducted by following Bombardier, Beaton and Guillemin’s guidelines for cross cultural adaptation. The study was conducted in different phases i.e. translation and validation. The original English version of PedsQL was translated into Urdu by two individuals, one with an Urdu background and the other from a medical background, during the translation phase (T1, T2) (Forward translation). The T3, T4 Versions were then developed by two independent translators (one with an English background and the other with a medical background). The T1, T2, T3, and T4 versions were compared to the original English version and evaluated by a group of multilingual specialists, resulting in the T5-urdu version. Twenty pediatric rehab professionals were given the T5-urdu version to validate. Results: Chronbach’s alpha value of PedsQL of both child and parent reported after translation were 0.986 and 0.987 respectively and after testing on population were 0.956 and 0.910 respectively. P-values showed significant results which are <0.05 in all domains. The inter domain correlations of child and parent reports after translation and testing on population were >0.70 that interpreted good Pearson correlation. The interclass coeffieneint was ranged from 0.541-0.982. Conclusion: The Urdu version of PedsQL Generic Core Scale of Age 8-12 years both child and parent proxy report is valid for neoplase children. Keywords: Children, Health Related Quality of Life, Neoplase, Pediatric Quality of Life Inventory, Translation, Validation.

Addressing the Need for Better Measures of Positive Health.

Addressing the Need for Better Measures of Positive Health.

Prevalence and Impact of Fatigue in Children with Primary Immunodeficiency Disorders: a Quantitative Single-Center Study

Although fatigue is a common symptom in adult patients with primary immunodeficiencies (PID), data in pediatric patients are limited. The goal of this study is to estimate the prevalence and impact of fatigue in children with PID as reported by patients, parents, and health-care providers. A retrospective single-center observational study was performed. Prevalence of fatigue was measured by reviewing medical charts of 54 children in our department who are on immunoglobulin replacement therapy. Both prevalence and impact were also measured by the PedsQL-Multidimensional Fatigue Scale (MFS) in 27 patients and 32 of their parents. This is an age-appropriate questionnaire for self-report of fatigue symptoms in patients aged 5–18 years and for parent proxy reports for patients aged 2–18 years. General, cognitive, and sleep-rest fatigue was measured, and a total fatigue score was calculated. Means, standard deviation and Z scores were calculated using age-specific reference values. Intraclass correlation coefficients (ICC) were calculated for comparison of scores provided by parents vs children’s self-reported scores. Both chart review data and PedsQL-MFS showed fatigue rates of 65%. Pediatric PID patients of all ages had significantly lower scores on all subscales and total score of the PedsQL-MFS compared to healthy children, indicating greater perceived symptoms of fatigue. General fatigue was the most affected subscale in PID patients, suggesting that fatigue in these patients is mainly physical. Seventy-four percent of PID patients had a Z score lower than − 1 on the general fatigue subscale indicating severe fatigue. Child-parent concordance varied between 0.24 and 0.93. Our results show the feasibility of the PedsQL-MFS survey to evaluate the prevalence and severity of fatigue in children with PID and underscore the importance of this issue in our patient care.

Health-related quality of life in Thai children with spinal muscular atrophy.

Spinal muscular atrophy (SMA) is a chronic genetic disease that causes varying degrees of disability. There is currently no data specific to the health-related quality of life (HRQoL) of Thai children and adolescents with SMA. Accordingly, this study aimed to evaluate the HRQoL of Thai pediatric SMA, and to identify factors significantly associated with HRQoL and other aspects of quality of life. The findings of this study will help to improve overall care in this vulnerable pediatric population. This prospective cross-sectional descriptive study was conducted at the Division of Neurology, Department of Pediatrics, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok, Thailand during 2015-2018. HRQoL was measured using the Thai language version of the Pediatric Quality of Life Inventory™ 4.0 Generic Core Scale (PedsQL™). The PedsQL™ was administered to SMA children aged 2-18 years and their parents. Disease severity was evaluated by pediatric neurologists, and patients were classified according to their SMA subtype. Forty-two Thai pediatric SMA (mean age: 9.8±5.0 years, 25 males) and their families were recruited. The mean PedsQL™ total score was 57.3±13.6 by child self-report, and 54.3±14.8 by parent proxy-report. The PedsQL™ scores of Thai SMA children were found to be significantly lower than those reported in healthy Thai children. The mean PedsQL™ total score among healthy Thai children was 78.7±9.3 by child self-report, and 79.0±12.8 by parent proxy-report. Factors independently associated with lower HRQoL were non-ambulation, household income less than 18,500 Thai baht/month, mechanical ventilation, and inability to attend school. HRQoL score was found to be an accurate reflection of patient and parent perception of SMA, which suggests the value of this tool for prioritizing interventions to improve the quality of life of Thai SMA. The results of this study also provide a baseline measurement of quality of life among Thai SMA.

The US population-level burden of cow's milk allergy

BackgroundCow's milk is a food allergen of public health importance both in the United States and globally. Its natural history and epidemiology have been most studied among infants and young children, but the public health burden it places on adults and older pediatric populations remains unclear. This study comprehensively characterizes the US population-level burden of cow's milk allergy (CMA), including its prevalence, severity, health care utilization, psychosocial impact, natural history, and other associated factors—including ability to tolerate extensively heated milk products—within a large, nationally-representative survey sample of US households. MethodsA cross-sectional survey was administered via web and telephone. Population level inference was based on data collected from participants recruited from National Opinion Research Center (NORC) at the University of Chicago's probability-based AmeriSpeak panel. Established dual-sample complex survey calibration methods were used to augment this sample with additional participants recruited through Survey Sampling International to increase the precision of the resulting estimates. In total, surveys were administered to a nationally representative sample of 51 819 US households from October 1, 2015, through September 31, 2016. Self-report responses from 40 453 adults and parent-proxy report for 38 408 children were analyzed. ResultsAnalyzing survey responses from 78 851 individuals, an estimated 4.7% (95% CI, 4.4%–4.9%) of the US population reported current CMA, whereas 1.9% (95% CI, 1.8%–2.1%) met symptom-report criteria for convincing IgE-mediated allergy. An estimated 0.9% (95% CI, 0.8%–1.0%) had CMA that met convincing symptom-report criteria and was physician diagnosed. Female and White respondents were more likely to report outgrowing CMA relative to males and those reporting other races and ethnicities. Individuals with CMA who reported tolerance to baked milk products were less likely to report severe reaction histories (33.5% vs 42.7%; p = .03), a lifetime history of food allergy-related emergency department visits (43.4% vs. 55.8%; p = .005), and treating a milk-allergic reaction with epinephrine (10.6 vs. 18.9%; p = .003). These individuals also exhibited less psychosocial burden on the validated Food Allergy Independent Measure (FAIM) than their counterparts with CMA who were allergic to baked forms of milk. ConclusionThese data indicate a discrepancy in reported rates of allergy to cow's milk among the US general population ranging from approximately 1 in 20 with reported CMA to fewer than 1 in 50 with physician-confirmed CMA. However, they suggest a substantial population-level burden of CMA, including substantial healthcare utilization, psychosocial burden and nutritional impacts—particularly among the approximately 30% of individuals with CMA who cannot tolerate baked forms of milk.

Life satisfaction, health-related quality of life and physical activity after treatment for valvular aortic stenosis.

To investigate health-related quality of life and life satisfaction in children and adolescents treated for isolated congenital valvular aortic stenosis compared to healthy peers. Our second aim was to investigate the relationship between objectively measured physical activity, health-related quality of life and life satisfaction in the same group. Forty-eight patients, 8-18 years of age, were recruited, as well as 43 healthy peers matched for age, gender and residential area. Health-related quality of life was assessed by the KIDSCREEN-52 self-report and parent proxy report, and life satisfaction was evaluated with the Satisfaction With Life Scale. Physical activity was measured with an accelerometer for 7days. Sports participation was self-reported. No differences in the health-related quality of life domains were found between patients and controls in the self-reports. In the proxy reports, parents of the adolescents rated their child's autonomy lower than did the parents of the healthy controls. A negative relationship was found between moderate to vigorous physical activity, sports participation, life satisfaction and the psychological well-being domain in adolescent patients. In children there was a positive relationship between moderate physical activity and the physical and psychological well-being domains. Overall, children and adolescents treated for valvular aortic stenosis reported similar life satisfaction and health-related quality of life as their healthy peers. The negative relationships between intense physical activity and sports participation with health-related quality of life and life satisfaction in adolescent patients might be explained by both physical and psychological factors in these teenagers with complex, lifelong heart disease.

Needs and Research Priorities for Young People with Spinal Cord Lesion or Spina Bifida and Their Caregivers: A National Survey in Switzerland within the PEPSCI Collaboration.

The aim of this study was to describe the needs and research priorities of Swiss children/adolescents and young adults (from here, “young people”) with spinal cord injury/disorder (SCI/D) or spina bifida (SB) and their parents in the health and life domains as part of the international Pan-European Pediatric Spinal Cord Injury (PEPSCI) collaboration. Surveys included queries about the satisfaction, importance, research priorities, quality of life (QoL), and characteristics of the young people. Fifty-three surveys with corresponding parent-proxy reports were collected between April and November 2019. The self-report QoL sum scores from young people with SCI/D and SB were 77% and 73%, respectively. Parent-proxy report QoL sum scores were lower, with 70% scores for parents of young people with SCI/D and 64% scores for parents of young people with SB. “Having fun”, “relation to family members”, and “physical functioning” were found to be highly important for all young people. “Physical functioning”, “prevention of pressure injuries”, “general health”, and “bowel management” received the highest scores for research priority in at least one of the subgroups. As parents tend to underestimate the QoL of their children and young people prioritized research topics differently, both young peoples’ and caregivers’ perspectives should be included in the selection of research topics.

Translation and validation of Indonesian version of Pediatric Quality of Life Inventory\u2122 (PedsQL\u2122) Neuromuscular Module

BackgroundThe Pediatric Quality of Life™ 3.0 Neuromuscular Module is an instrument to assess health-related quality of life (HRQoL) among children with neuromuscular diseases (NMDs) aged 2–18 years. This study aimed to determine whether the PedsQL™ 3.0 Neuromuscular Module Indonesian Version is valid and reliable.MethodsThis study used the Indonesian translation of the PedsQL™ 3.0 Neuromuscular Module after getting formal permission from the inventor, and the translation process followed the Mapi linguistic translation guidelines. This study administered the PedsQL™ 3.0 Neuromuscular Module Indonesian Version to 84 parents and 71 children. In addition, we used the Rasch model to analyze the psychometric properties.ResultsThe reliability of the total scale of the PedsQL™ Neuromuscular Module Indonesian Version shows good to very good criteria. On the parent proxy-report, Cronbach alpha was 0.95, person reliability was 0.84, item reliability was 0.93, person separation was 2.32, item separation was 3.61, person strata separation was 4 levels, and item strata separation was 5 levels. On the child self-report, Cronbach alpha was 0.93, person reliability was 0.81, item reliability was 0.81, person separation was 2.08, item separation was 2.06, person strata separation was 3 levels, and item strata separation was 3 levels. The total scale of the PedsQL Neuromuscular Module Indonesian Version shows fair to good construct validity in parent proxy-report (explained variance 51.9%; unexplained variance 8.4%) and child self-report (explained variance 40.9%; unexplained variance 12.6%). There were no misfit items in the parent proxy-report (infit 0.66–1.49; outfit 0.51–1.81; point measure correlation 0.36–0.93) and child self-report (infit 0.53–1.65; outfit 0.50–1.73; point measure correlation 0.31–0.90) identified by the Rasch models.ConclusionsThe PedsQL™ 3.0 Neuromuscular Modul Indonesian Version is a valid and reliable instrument in measuring HRQoL in Indonesian children with neuromuscular diseases.

Health Related Quality of Life of Children Aged 2-18 Years With Familial Mediterranean Fever

Objective: We aimed to evaluate both child-self reported and parents proxy reported health related quality of life of children with familial Mediterranean fever (FMF) aged between 2-18 years old and to compare the scores with their healthy peers. Material and Methods: Fifty -one patients with FMF and fifty one gender and age matched controls were enrolled in the study. Children aged 8-18 years completed the PedsQL4.0 child form questionnaires. Parents of all cases completed the PedsQL4.0 parent form. Results: Lower scores in the FMF group, with no statistical significance, was found between the FMF cases and healthy volunteers aged 8-18 years for the physical health, social health, school functioning, psychosocial total and scale total score of PEDsQL4.0 child self-report. The parent proxy report PedsQL4.0 scores of all patients with FMF were lower than the healthy group for physical, emotional, social, psycho-social, school functioning scores and scale total score but the difference was not statistically significant. A significant correlation was present between PedsQL4.0 child self-report and parent PedsQL4.0 report in all subscales except for social health total score. There was not a significant correlation between the demographic variables and clinical features of the patients and the child subscales of PedsQL4.0. Conclusion: The scores of FMF cases from the scales were lower than those of the healthy volunteers from the questionnaires but this difference was not statistically significant. Considering the relatively small sample size of the study it is obvious that more comprehensive prospective studies are required on this subject.

Are children with unilateral hearing loss more tired?

ObjectiveTo determine whether children with unilateral sensorineural hearing loss (USNHL) and unilateral conductive hearing loss (UCHL) have higher levels of fatigue than literature reported normal hearing (LRNH) children. MethodsThis was a cross-sectional survey utilizing the PedsQL™ Multidimensional Fatigue Scale administered to children with unilateral hearing loss (UHL) and their parents at two tertiary care academic medical centers and a nationwide microtia/atresia conference. The PedsQL™ Multidimensional Fatigue Scale was used to compare child and parental proxy reports of fatigue among USNHL, UCHL, and LRNH children. ANOVA and post-hoc Tukey Honest Significant Difference testing were used for statistical analysis. ResultsOf 69 children included in the study, 42 had UCHL (61%) and 27 (39%) had USNHL. Children with USNHL reported more total fatigue (mean 69.1, SD 19.3) than LRNH children (mean 80.5, SD 13.3; difference −11.4; 95% CI: −19.98 to −2.84) and children with UCHL (mean 78.0, SD 14.5; difference −8.95; 95% CI: −17.86 to 0.04). Children with UCHL reported similar levels of fatigue compared to LRNH children (difference −2.5; 95% CI: −9.95 to 5.03).Parents of children with USNHL reported greater levels of fatigue (mean 67.6, SD 22.6) in their children than parents of LRNH children (mean 89.6, SD 11.4; difference −22.0; 95% CI: −29.8 to −14.3) and parents of children with UCHL (mean 76.2, SD 17.3; difference −8.6; 95% CI: −17.5 to 0.21). Parents of children with UCHL also report higher levels of fatigue than parents of LRNH children (difference −13.4; 95% CI: −19.98 to −6.84). ConclusionsChildren with USNHL reported greater levels of fatigue than LRNH children and children with UCHL. Results implicate cognitive load as an important consideration in children with hearing loss. The measurement of fatigue may be a useful indicator to determine the benefit of intervention (e.g., amplification) for these children.

Timing of itch among children with atopic dermatitis

Itch is a subjective, defining symptom of atopic dermatitis (AD), which guides treatment decisions. 1 Fishbein AB Silverberg JI Wilson EJ Ong PY. Update on atopic dermatitis: diagnosis, severity assessment, and treatment selection. J Allergy Clin Immunol Pract. 2020; 8: 91-101 Abstract Full Text Full Text PDF PubMed Scopus (50) Google Scholar In pediatric patients, clinic visits center on patient or parent-proxy report of itch. One study of 100 adult and adolescent patients found self-report of itch worsening generally in the evening and night. 2 Yosipovitch G Goon AT Wee J Chan YH Zucker I Goh CL. Itch characteristics in Chinese patients with atopic dermatitis using a new questionnaire for the assessment of pruritus. Int J Dermatol. 2002; 41: 212-216 Crossref PubMed Scopus (164) Google Scholar Timing of itch is one feature of AD to which treatment in children could be targeted. Nevertheless, there have been no pediatric studies evaluating itch timing. Given the frequency of sleep disturbance in AD and our clinical experience, 3 Fishbein AB Knutson KL Zee PC. Circadian disruption and human health. J Clin Invest. 2021; 131 Crossref PubMed Scopus (6) Google Scholar we hypothesized that pediatric patients experience greater nighttime itch in AD.

Development of a Bowel Management Scoring Tool in Pediatric Patients with Constipation

To develop a reliable and valid scoring tool, the Pediatric Bowel Management Scoring Tool (PBMST), to better guide management of constipation in pediatric patients. The project comprised 2 stages, development of the questionnaire and construction of the bowel management score. Two questionnaires were created, one for children aged 8-18years to self-report and one parent proxy-report for children aged 4-8years. Questions regarding physical symptoms (n=6), emotional aspects (n=2), social activities/school (n=1), and treatment (n=1) were included. Patients (or parents of patients) with symptoms of constipation completed the questionnaire. The reproducibility of each question was computed using the Cohen weighted kappa coefficient (κ). A bowel management score was developed using logistic regression analysis, assessing the associations between the questions and impact on self-reported quality of life (QoL). Questions with adequate reproducibility and significantly associated with QoL were incorporated into the score. The questionnaire was completed by 385 patients. Six questions met the inclusion criteria and were incorporated into the score: stool shape (range, 0-3 points), anorectal pain (0-4 points), abdominal pain (0-3 points), frequency of fecal incontinence (0-3 points), assistance of caregivers (0-3 points), and interference with social activities (0-6 points). Differences in bowel management scores among patients reporting no, little, some, or major impact on QoL were statistically significant (P<.001). The newly developed and validated PBMST is a reliable tool for evaluating bowel management strategies in children with constipation.

Depression, anxiety and health-related quality of life in paediatric intracranial germ cell tumor survivors

BackgroundLittle is known about depression and anxiety among paediatric intracranial germ cell tumour (iGCT) survivors. We aimed to evaluate the risk factors associated with depression, anxiety and health-related quality of life (HRQoL) in paediatric iGCT survivors.MethodsWe recruited 200 iGCT patients (and their parents) from Beijing Tiantan Hospital and assessed their HRQoL using the Paediatric Quality of Life Inventory (PedsQL) 4.0 Generic Core Scales. The Children’s Depression Inventory, Screen for Child Anxiety Related Emotional Disorders, and Symptom Checklist 90 were used to evaluate depression and anxiety. The results were analysed based on disease recurrence, tumour location and treatment strategies.ResultsSurvivors with recurrent tumours had worse HRQoL scores than those with non-recurrent tumours. Patients with tumours involving both the suprasellar and basal ganglia regions had the worst HRQoL scores. A large proportion of survivors had depression or anxiety. Both depression and anxiety scores were highly correlated with the HRQoL emotional functioning scores. The parent proxy-reports (PPR) and child self-reports were highly correlated in all domains.ConclusionsThis study demonstrated the clinical factors affecting paediatric iGCT survivors’ depression, anxiety, and HRQoL. Therefore, psychological interventions should be implemented. It also suggests that the PedsQL PPR would be helpful for routine screening.

Early-life activities mediate the association between family socioeconomic status in early childhood and physical fitness in early adolescence

The graded association between family socioeconomic status (SES) and physical fitness is evident, but little is known about the mechanism underlying this association. This study investigated the role of early-life activities as mediators of the longitudinal relationship between early-life SES and health-related physical fitness in 168 adolescents (51.2% boys; final mean age: 12.4 years old). In Wave 1 (2011–12), their parents completed questionnaires about family socioeconomic status (SES), parent–child activities, and child screen time. In Wave 2 (2014–15), participants’ physical activity levels were assessed through parent proxy-reports. In Wave 3 (2018–19), a direct assessment of handgrip strength, standing long-jump, and 6-min walk test (6MWT) performance was conducted. After controlling for demographic factors, results of mediation analyses revealed that (a) Wave 1 SES predicted Wave 3 long-jump and 6MWT performance; (b) child physical activity level in Wave 2 mediated the relation between Wave 1 SES and standing long-jump performance in Wave 3; and (c) recreational parent–child activities and child screen time in wave 1 mediated the relation between Wave 1 SES and 6MWT performance in Wave 3. Our findings suggest that the type and frequency of early-life activities play a role in the graded association between childhood SES and physical fitness in adolescence.

PedsQL™ Spinal Cord Injury Module: Reliability and Validity.

The objective was to report on the initial measurement properties of the Pediatric Quality of Life Inventory™ (PedsQL™) Spinal Cord Injury (SCI) Module in youth with SCI from the youth and parent perspectives. PedsQL™ SCI Module and PedsQL™ 4.0 Generic Core Scales SF15 were completed in a multisite national study by 166 youth ages 8 to 25 years and 128 parents of youth ages 5 to 23 years. Data from the PedsQL™ Generic Core Scales were compared with an age- and sex-matched healthy control sample. Factor analysis was conducted to determine the factor structure of the items. In addition to a Total Scale Score, nine unidimensional scales were derived measuring daily activities, mobility, bladder function, bowel function, pressure injury, pain interference, social participation, worry bladder bowel, and worry social. The PedsQL™ SCI Module evidenced excellent reliability for Total Scale Scores (youth self-report, α = 0.93; parent proxy-report, α = 0.93) and acceptable reliability for the nine individual scales (youth self-report, α = 0.71-0.83; parent proxy-report, α = 0.67-0.87). Intercorrelations with the Generic Core Scales supported construct validity with medium to large effect sizes (most ps < .001). Factor analysis supported the unidimensionality of the nine individual scales. PedsQL™ Generic Core Scales comparisons to healthy controls demonstrated significantly impaired generic health-related quality of life in youth with SCI with large effect sizes. The PedsQL™ SCI Module Scales demonstrated acceptable measurement properties and may be utilized as standardized scales to assess SCI-specific concerns and problems in clinical research and practice in youth with SCI.

Neurocognitive Functioning of Pediatric and Young Adult Patients with Gaucher Disease, Type 1

This exploratory study aimed to evaluate the cognitive and executive functioning of sixteen pediatric and fifteen young adult (ages 5 through 30) patients with Gaucher disease, Type 1. The neurocognitive functioning of children with Gaucher disease, Type 1 was based on self- and parent-proxy reports while neurocognitive functioning in young adults was based on self-report only. Results showed that pediatric participants with Gaucher disease, Type 1 on self-report demonstrated a trend toward weaker cognitive functioning than healthy children. Parent proxy-reports did not show significantly different cognitive functioning of children with Gaucher disease, Type 1 compared to healthy peers. Young adults with Gaucher disease, Type 1 self-reported no significant differences in cognitive functioning from healthy young adults. No group differences in executive functioning were observed for child or young adult samples. Parent-reported disease severity was not associated with cognitive outcomes among the pediatric or young adult samples. Results suggest possible mild cognitive weaknesses among children with Gaucher disease, Type 1 while cognition in young adults appears relatively well preserved. This is the first independent examination of cognitive functioning among children and young adults with GD1. Additional studies in younger patients with Gaucher disease, Type 1 are needed to identify possible cognitive dysfunction and elucidate underlying mechanisms.

Quality Of Life in Children With Home Mechanical Ventilation - A Scoping Review.

IntroductionHome mechanical ventilation is an established method to support children suffering from chronic respiratory insufficiency, still more research is needed regarding mechanically ventilated children's and adolescents’ quality of life (QoL). Therefore, the aim of this scoping review was to explore research regarding QoL and lived experience of children and adolescents with home mechanical ventilation.MethodsA scoping review with systematic searches for research studies published between year 2000–2020 was performed in Cinahl, Medline, and PubMed. Studies that met the inclusion criteria were quality assessed and a thematic analysis was performed.ResultsIn total, ten articles were quality assessed and included in the results. Four themes emerged: Children's self-reported QoL, Parents’ perception and parent-proxy report, Differences between the child's and parent's perception, and challenges in daily life. Children with home mechanical ventilation reported a lower QoL than healthy children and children with other chronic diseases. Generally, parents rate their child's QoL lower than the children themselves.ConclusionThis is the first literature review focusing on HMV in the paediatric population. It is clear that HMV does not only affect the treated child or adolescent but also the whole family. It is important to regularly measure and evaluate QoL in children and adolescents with HMV to provide person-centered care. More research is needed to improve these children's and adolescents’ QoL.

Impaired Health-Related Quality of Life in Brazilian Children with Chronic Abdominal Pain: A Cross-Sectional Study.

We compared the health-related quality of life (HRQOL) of children and adolescents with functional abdominal pain disorders (FAPDs) and organic abdominal pain disorders (ORGDs). This was a single-center, cross-sectional, observational study. The PedsQL 4.0 generic cores scales parent proxy-report was administered to parents/caregivers of 130 and 56 pediatric patients with FAPDs and ORGDs respectively on their first visit. The self-reported pain intensity in the patients was assessed using a visual analog scale (VAS) and facial affective scale (FAS). Irritable bowel syndrome was the most prevalent FAPDs, and the most prevalent ORGDs were reflux esophagitis (41.1%) and gastritis associated with Helicobacter pylori (21.4%). There was no difference in HRQOL among patients diagnosed with ORGDs and FAPDs (p>0.05). Patients with ORGDs and FAPDs had lower HRQOL Scale scores than healthy Brazilian and American children's references, with a high proportion of children at risk for impaired HRQOL (p<0.0001). There was no difference in the VAS and the FAS scores between the ORGDs and the FAPDs. FAPDs had a higher prevalence of girls' and couples' disagreement (p<0.02), although poor school performance (p<0.0007) and bullying (p<0.01) were higher in patients with ORGD. This study revealed that there was a difference in impaired HRQOL between patients with ORGDs and FAPDs. Thus, considering the high prevalence of chronic abdominal pain in children, a well-founded treatment plan is necessary for a multidisciplinary cognitive-behavioral Pain management program.