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Prior authorization criteria for Federal Drug Administration (FDA) approved immunotherapeutics, among the class of anti-amyloid monoclonal antibodies (mAbs), established by state drug formulary committees, are tailored for adults with late-onset Alzheimer's disease. This overlooks adults with Down syndrome (DS), who often experience dementia at a younger age and with different diagnostic assessment outcomes. This exclusion may deny DS adults access to potential disease-modifying treatments. To address this issue, an international expert panel convened to establish adaptations of prescribing criteria suitable for DS patients and parameters for access to Centers for Medicare & Medicaid Services (CMS) registries. The panel proposed mitigating disparities by modifying CMS and payer criteria to account for younger onset age, using alternative language and assessment instruments validated for cognitive decline in the DS population. The panel also recommended enhancing prescribing clinicians' diagnostic capabilities for DS and initiated awareness-raising activities within healthcare organizations. These efforts facilitated discussions with federal officials, aimed at achieving equity in access to anti-amyloid immunotherapeutics, with implications for national authorities worldwide evaluating these and other new disease-modifying therapeutics for Alzheimer's disease.
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Síndrome de Down , Humanos , Estados Unidos , Doença de Alzheimer/tratamento farmacológico , Adulto , Anticorpos Monoclonais/uso terapêutico , Imunoterapia/métodosRESUMO
Background: Executive function (EF) skills are important treatment targets for people with Down syndrome (DS); however, few EF measures have been evaluated for use with young children in this population. Methods: The present study evaluated preliminary psychometric properties of a measure of the EF component of inhibition. Participants were 73 children with DS between 2.5 and 8.67 years old who completed an adapted ability to delay task using a desirable toy. Results: Across two separate trials, latencies to touch the toys were significantly correlated. Latencies increased overall with chronological and mental age, with caveats for the youngest and oldest participants. Conclusion: Findings suggest that an adapted prohibition task is an appropriate method of measuring inhibition for children with DS between 4 and 7 years old, though many children in this chronological age range are at early stages of acquiring this skill set.
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BACKGROUND: Down syndrome is the most common genetic disorder associated with intellectual and developmental disabilities. Research to improve health care outcomes in Down syndrome lags significantly behind other disease categories. Among these reasons are funding, recruitment and availability of research studies being conducted. METHODS: We surveyed 228 parents of individuals with Down syndrome to understand their perceptions of research, study design, how they seek out information and topics they would like to see researched. RESULTS: Parents with children 18 years and younger responded to our survey. Parents indicated their willingness to participate in research (72%), yet few have (36%). Parents identified barriers to participation, research they feel would help their child, and interests in seeing new therapies and drug studies. CONCLUSION: These findings identify recommendations and insights from parents on future research agendas, studies and recruitment strategies that may help researchers improve outcomes for individuals with Down syndrome.
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Síndrome de Down , Deficiência Intelectual , Criança , Família , Humanos , Pais , Projetos de PesquisaRESUMO
Improved medical care of individuals with Down syndrome (DS) has led to an increase in life expectancy to over the age of 60 years. In conjunction, there has been an increase in age-related co-occurring conditions including Alzheimer's disease (AD). Understanding the factors that underlie symptom and age of clinical presentation of dementia in people with DS may provide insights into the mechanisms of sporadic and DS-associated AD (DS-AD). In March 2019, the Alzheimer's Association, Global Down Syndrome Foundation and the LuMind IDSC Foundation partnered to convene a workshop to explore the state of the research on the intersection of AD and DS research; to identify research gaps and unmet needs; and to consider how best to advance the field. This article provides a summary of discussions, including noting areas of emerging science and discovery, considerations for future studies, and identifying open gaps in our understanding for future focus.
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Doença de Alzheimer/complicações , Síndrome de Down/complicações , Doença de Alzheimer/metabolismo , Peptídeos beta-Amiloides/metabolismo , Síndrome de Down/metabolismo , HumanosRESUMO
Alzheimer's disease and related dementias (ADRDs) are a global crisis facing the aging population and society as a whole. With the numbers of people with ADRDs predicted to rise dramatically across the world, the scientific community can no longer neglect the need for research focusing on ADRDs among underrepresented ethnoracial diverse groups. The Alzheimer's Association International Society to Advance Alzheimer's Research and Treatment (ISTAART; alz.org/ISTAART) comprises a number of professional interest areas (PIAs), each focusing on a major scientific area associated with ADRDs. We leverage the expertise of the existing international cadre of ISTAART scientists and experts to synthesize a cross-PIA white paper that provides both a concise "state-of-the-science" report of ethnoracial factors across PIA foci and updated recommendations to address immediate needs to advance ADRD science across ethnoracial populations.
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Doença de Alzheimer/etnologia , Doença de Alzheimer/epidemiologia , Etnicidade , Disparidades em Assistência à Saúde , Grupos Raciais , Idoso , Biomarcadores , Pesquisa Biomédica , HumanosRESUMO
PURPOSE: This pilot study measured effects of an adapted dance program on motor abilities and participation in children with Down syndrome (DS) and explored caregivers' qualitative feedback regarding its benefits. METHODS: Children with DS participated in 20 weekly 1-hour adapted dance sessions. The Canadian Occupational Performance Measure (COPM) and Gross Motor Function Measure (GMFM) Dimensions D and E were administered before and after the program. Caregivers completed a survey at the last session. RESULTS: Six children completed pre- and postassessments. Significant improvements were noted in GMFM-88 measures. Caregivers of 5 participants reported improved scores on the COPM that were not significant. Caregivers reported physical, cognitive, and emotional improvements. CONCLUSIONS: This study supports use of an adapted dance program to improve motor abilities and participation in children with DS. VIDEO ABSTRACT: For more insights from the authors, access Supplemental Digital Content 1, available at: http://links.lww.com/PPT/A231.
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Dançaterapia , Síndrome de Down/reabilitação , Atividade Motora/fisiologia , Modalidades de Fisioterapia , Adolescente , Canadá , Criança , Pré-Escolar , Síndrome de Down/fisiopatologia , Síndrome de Down/psicologia , Feminino , Humanos , Masculino , Avaliação de Resultados em Cuidados de Saúde , Projetos PilotoRESUMO
Adults with Down syndrome (DS) represent a unique population who are in need of clinical guidelines to address their medical care. The United States Preventive Service Task Force (USPSTF) has developed criteria for prioritizing conditions of public health importance with the potential for providing screening recommendations to improve clinical care. The quality of existing evidence needed to inform clinical guidelines has not been previously reviewed. Using the National Library of Medicine (NLM) database PubMed, we first identified 18 peer reviewed articles that addressed co-occurring medical conditions in adults with DS. Those conditions discussed in over half of the articles were prioritized for further review. Second, we performed detailed literature searches on these specific conditions. To inform the search strategy and review process a series of key questions were formulated a priori. The quality of available evidence was then graded and knowledge gaps were identified. The number of participating adults and the design of clinical studies varied by condition and were often inadequate for answering all of our key questions. We provide data on thyroid disease, cervical spine disease, hearing impairment, overweight-obesity, sleep apnea, congenital heart disease, and osteopenia-osteoporosis. Minimal evidence demonstrates massive gaps in our clinical knowledge that compromises clinical decision-making and management of these medically complex individuals. The development of evidence-based clinical guidance will require an expanded clinical knowledge-base in order to move forward.
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Síndrome de Down/epidemiologia , Adulto , Fatores Etários , Pesquisa Biomédica , Comorbidade , Atenção à Saúde , Gerenciamento Clínico , Síndrome de Down/terapia , Medicina Baseada em Evidências , Humanos , Guias de Prática Clínica como Assunto , PrevalênciaRESUMO
OBJECTIVE: To evaluate whether racial differences across a variety of medical factors collected in a longitudinal clinical database at a specialty clinical for children with Down syndrome provide insight into contributors to racial disparity in mortality. STUDY DESIGN: Comprehensive medical histories of 763 children receiving medical care at a Down syndrome specialty clinic were retrospectively reviewed regarding prenatal, postnatal, and medical issues, as well as subspecialty referrals. Frequency calculations and logistic regression were performed. The National Death Index was used to query death record databases to correlate medical histories with mortality data. RESULTS: Prenatal drug use and intubation were significantly more frequent, but hyperbilirubinemia was significantly less frequent, in black children compared with white children with Down syndrome. Among children with Down syndrome aged <5 years, significant increases in referral to cardiology were seen for black children compared with white children. Trends were seen in an increased incidence of congenital heart disease for black children. Correlations with death records did not demonstrate differences in rates of cardiac-related deaths. Minimal racial disparity was seen for all other measures investigated. CONCLUSION: Racial disparity in mortality exists, but the underlying cause remains unidentified despite use of a comprehensive, longitudinal database of individuals with Down syndrome and review of death records. Referrals to cardiology might be a clue to the underlying cause, perhaps as an indicator of access to care, but cardiac disease does not account for the disparity in mortality.
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Negro ou Afro-Americano/estatística & dados numéricos , Síndrome de Down/etnologia , Síndrome de Down/mortalidade , Disparidades nos Níveis de Saúde , População Branca/estatística & dados numéricos , Adolescente , Criança , Pré-Escolar , Bases de Dados Factuais , Feminino , Humanos , Estudos Longitudinais , Masculino , Estudos Retrospectivos , Índice de Gravidade de Doença , Adulto JovemRESUMO
BACKGROUND: Living with a child with Down syndrome (DS) influences the entire family, including caregivers. AIMS: This study examined positive and negative caregiver feelings about parenting youth with DS and to what extent children's demographic, cognitive, behavioral characteristics, and co-occurring medical conditions are associated with those parental feelings. Specifically, the mediatory role of child behavioral challenges on the relationship between child executive functioning (EF) and parent feelings about parenting a child with DS was examined in a mediation analysis model. METHODS AND PROCEDURES: Parents of 113 youth with DS aged 6 to 17 year rated their positive and negative feelings about parenting, and their child's behavioral challenges and EF. OUTCOMES AND RESULTS: Externalizing and Internalizing behavioral challenges and emotional and behavioral regulations of EF were significantly associated with positive and negative parent feelings. Child behavioral challenges fully mediated the relationship between child EF and caregiver feelings about parenting, after controlling for identified covariates of child demographics. CONCLUSIONS AND IMPLICATIONS: Findings have implications for understanding the role of EF, through its impact on behavioral challenges, on the feelings of caregivers about parenting a child with DS. These findings play a role in understanding outcomes of interventions targeted at EF and behavioral challenges, in the context of other child variables.
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Síndrome de Down , Poder Familiar , Criança , Humanos , Adolescente , Poder Familiar/psicologia , Função Executiva , Pais/psicologia , DemografiaRESUMO
OBJECTIVE: The primary aim of this study was to assess the feasibility of a developmentally tailored neurocognitive assessment in survivors of childhood acute leukemia with Down syndrome (DS-leukemia). A secondary aim was to compare outcomes in the DS-leukemia group to a historical comparison group of individuals with DS and no history of childhood cancer. METHODS: Survivors of DS-leukemia (n = 43; 56% male, mean [SD] age at diagnosis = 4.3 [4.5] years; age at evaluation = 15 [7.9] years) completed a neurocognitive assessment battery that included direct measures of attention, executive function, and processing speed, and proxy ratings of attention problems and executive dysfunction. Direct assessment outcomes were compared to a historical comparison cohort of individuals with DS and no history of childhood cancer (DS-control; n = 117; 56% male, mean [SD] age at evaluation = 12.7 [3.4] years). RESULTS: Rates of valid task completion ranged from 54% to 95%, suggesting feasibility for most direct assessment measures. Compared to the DS-control group, the DS-leukemia group had significantly lower completion rates on measures of executive function (p = 0.008) and processing speed (p = 0.018) compared to the DS-control group. There were no other significant group differences in completion rates. Compared to the DS-control group, the DS-leukemia group had significantly more accurate performance on two measures of executive function (p = 0.032; p = 0.005). Compared to the DS-control group, the DS-leukemia group had significantly more problems with executive function as identified on proxy ratings (6.5% vs. 32.6%, p = <0.001). CONCLUSION: Children with Down syndrome (DS) are at increased risk for developing acute leukemia compared to the general population but are systematically excluded from neurocognitive outcome studies among leukemia survivors. This study demonstrated the feasibility of evaluating neurocognitive late effects in leukemia survivors with DS using novel measures appropriate for populations with intellectual developmental disorder.
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Síndrome de Down , Leucemia Mieloide Aguda , Criança , Humanos , Masculino , Pré-Escolar , Feminino , Síndrome de Down/complicações , Função Executiva , Sobreviventes/psicologia , Atenção , Leucemia Mieloide Aguda/complicações , Leucemia Mieloide Aguda/epidemiologiaRESUMO
This study evaluated the appropriateness of scoring the Behavior Rating Inventory of Executive Function-Preschool (BRIEF-P) using age-equivalent scores generated from multiple measures of cognition and language among school-age children with Down syndrome (DS). Subscale T scores for 95 children with DS were contrasted using standard scoring on the Behavior Rating Inventory of Executive Function-Second edition (BRIEF-2; based on chronological age) to alternate scoring using the BRIEF-P (based on age-equivalent) for patterns of subscale intercorrelations, differences in mean scores, and agreement on findings from clinical cut-off scores. Results with children with DS suggested using (1) the BRIEF-P for children ages 2-5 years old, (2) the BRIEF-2 with chronological-age scoring or the BRIEF-P with age-equivalent scoring (with some caveats) for research on children ages 5-10 years old, and (3) the BRIEF-2 for children ages 11 and older.
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Síndrome de Down , Criança , Humanos , Pré-Escolar , Função Executiva , Cognição , Instituições AcadêmicasRESUMO
People with Down syndrome (DS) commonly experience challenges with sleep, executive functioning, everyday memory, and symptoms of attention deficit hyperactivity disorder (ADHD). A path analysis was conducted to determine if executive function mediated the relationship between sleep problems and both everyday memory and ADHD symptoms. Parents of 96 children and youth with DS completed questionnaires related to sleep, executive functioning, everyday memory, and ADHD symptoms. Results showed that executive functioning fully mediated the relation between sleep and both everyday memory and ADHD symptoms. Implications for education and intervention for children and youth with DS are discussed.
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Transtorno do Deficit de Atenção com Hiperatividade , Síndrome de Down , Humanos , Criança , Adolescente , Função Executiva , Transtorno do Deficit de Atenção com Hiperatividade/epidemiologia , Sono , PaisRESUMO
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.
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Síndrome de Down , Humanos , Adolescente , Síndrome de Down/psicologia , Reprodutibilidade dos Testes , Cognição , Inquéritos e Questionários , PsicometriaRESUMO
Introduction and Methods: This study provides preliminary data on the Social Skills Improvement System (SSiS) Rating Scales Parent Form to measure social skills in a sample of 124 children and adolescents with Down syndrome (DS) ages 6-17 years. Results: Overall, participants demonstrated relatively mild symptoms, with the sample's average standard score falling within 1 standard deviation from the mean of the normative sample for the social skills (M = 92, SD = 15) and problem behaviors (M = 104, SD = 12) domains (normative sample M = 100, SD = 15 for both domains). However, a wide range of scores was observed across the sample for the composite and subscale scores. Differential patterns were also observed by subscale. For some subscales (i.e., Cooperation, Assertion, Responsibility, Engagement, Externalizing, Hyperactivity/Inattention, and Autism Spectrum), a disproportionate number of participants scored in the below average (i.e., lower levels of social skills) or above average (i.e., more symptomatic in problem behaviors or autism spectrum) range relative to the normative sample; for other subscales (i.e., Communication, Empathy, Self-Control, Bullying, and Internalizing), participants' score distribution aligned more closely to that of the normative sample. SSiS composite scores correlated in the expected directions with standardized measures of autism characteristics, executive function, and expressive language. Discussion: This study provides some of the first evidence validating the use of the SSiS in youth with DS, filling a gap in standardized measures of social functioning in this population.
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OBJECTIVES: Obstructive sleep apnea (OSA) is highly prevalent among individuals with Down syndrome (DS), and the nonphysiological consequences of OSA require examination to inform treatment planning. This study aimed to investigate the association between OSA and aspects of language, executive functioning, behavioral, social abilities, and sleep problems in youth with DS aged 6 to 17 years. METHODS: Multivariate analysis of covariance was used to compare 3 groups adjusted for age, participants with DS with untreated OSA (n = 28), participants with DS without OSA (n = 38), and participants with DS with treated OSA (n = 34). To be eligible for the study, participants had to have an estimated mental age of 3 years. No children were excluded based on estimated mental age. RESULTS: After adjusting for age, participants with untreated OSA showed a common pattern of lower estimated marginal mean scores than those with treated OSA and those with no OSA in expressive and receptive vocabulary and higher estimated marginal mean scores with executive functions, everyday memory, attention, internalizing and externalizing behavior, social behavior, and sleep problems. However, only the group differences for executive function (emotional regulation) and internalizing behavior were statistically significant. CONCLUSION: Study findings corroborate and extend prior findings related to OSA and clinical outcomes for youth with DS. The study highlights the importance of OSA treatment in youth with DS and provides clinical recommendations for this population. Additional studies are necessary to control the effects of health and demographic variables.
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Síndrome de Down , Apneia Obstrutiva do Sono , Adolescente , Humanos , Função Executiva , Síndrome de Down/complicações , Síndrome de Down/epidemiologia , Polissonografia , Apneia Obstrutiva do Sono/complicações , Apneia Obstrutiva do Sono/epidemiologia , Apneia Obstrutiva do Sono/diagnóstico , IdiomaRESUMO
OBJECTIVE: To summarize the current state of the literature regarding emotion dysregulation (ED) in syndromic intellectual disabilities (S-IDs) in 6 of the most common forms of S-IDs-Down syndrome, fragile X syndrome (FXS), tuberous sclerosis complex, Williams syndrome, Prader-Willi syndrome, and Angelman syndrome-and to determine future research directions for identification and treatment of ED. METHOD: PubMed bibliographic database was searched from date of inception to May 2021. PRISMA 2020 guidelines were followed with the flowchart, table of included studies, list of excluded studies, and checklist provided. Filters applied included human research and English. Only original research articles were included in the final set, but review articles were used to identify secondary citations of primary studies. All articles were reviewed for appropriateness by 2 authors and summarized. Inclusion criteria were met by 145 articles (Down syndrome = 29, FXS = 55, tuberous sclerosis complex = 11, Williams syndrome = 18, Prader-Willi syndrome = 24, Angelman syndrome = 8). RESULTS: Each syndrome review was summarized separately and further subdivided into articles related to underlying neurobiology, behaviors associated with ED, assessment, and targeted intervention. FXS had the most thorough research base, followed by Down syndrome and Prader-Willi syndrome, with the other syndromes having more limited available research. Very limited research was available regarding intervention for all disorders except FXS. CONCLUSION: Core underlying characteristics of S-IDs appear to place youth at higher risk for ED, but further research is needed to better assess and treat ED in S-IDs. Future studies should have a standard assessment measure of ED, such as the Emotion Dysregulation Inventory, and explore adapting established curricula for ED from the neurotypical and autism spectrum disorder fields.
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Síndrome de Angelman , Transtorno do Espectro Autista , Síndrome de Down , Síndrome do Cromossomo X Frágil , Deficiência Intelectual , Síndrome de Prader-Willi , Esclerose Tuberosa , Síndrome de Williams , Criança , Adolescente , Humanos , Síndrome de Prader-Willi/complicações , Síndrome de Prader-Willi/psicologia , Síndrome de Angelman/complicações , Síndrome de Down/complicações , Transtorno do Espectro Autista/complicações , Deficiências do Desenvolvimento , Esclerose Tuberosa/complicações , Deficiência Intelectual/etiologia , Síndrome do Cromossomo X Frágil/complicações , Síndrome do Cromossomo X Frágil/psicologia , Síndrome de Williams/complicações , Síndrome de Williams/psicologia , EmoçõesRESUMO
Reliable and valid cognitive outcome measures, including examiner-administered and computer-facilitated assessments of processing speed and reaction time, are necessary for future clinical trials that include individuals with Down syndrome (DS). The current study evaluated the score distributions and psychometric properties of four examiner-administered and three computerized processing speed and reaction time measures. Participants included 97 individuals with DS, aged 6 to 17 (M = 12.6, SD = 3.3). Two examiner-administered measures (Differential Ability Scales-II Rapid Naming and Cat/dog Stroop Congruent) met most predetermined psychometric criteria. Other assessments demonstrated good test-retest reliability and had negligible practice effects but lacked adequate feasibility. Recommendations for using processing speed and reaction time assessments in research and suggestions for modifications of measures are discussed.
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Síndrome de Down , Cognição , Síndrome de Down/psicologia , Testes Neuropsicológicos , Avaliação de Resultados em Cuidados de Saúde , Velocidade de Processamento , Tempo de Reação , Reprodutibilidade dos Testes , Humanos , Criança , AdolescenteRESUMO
BACKGROUND: Working memory involves the temporary storage and manipulation of information and is frequently an area of challenge for individuals with Down syndrome (DS). Despite the potential benefits of intervention, laboratory assessments of working memory that could capture intervention effects have not undergone rigorous evaluation for use with young children with DS. It is critical to evaluate assessments of working memory in young children with DS to ensure the reliable and accurate measurement of performance. AIM: This study evaluated an adapted laboratory measure of working memory for young children with DS 2-8 years old. METHOD: A self-ordered pointing task, the Garage Game, was administered to 78 children with DS (mean = 5.17 years; SD = 1.49). Adaptations were made to the task to minimize potential DS phenotype-related language and motor confounds. RESULTS: Results indicate that the measure is feasible, scalable, and developmentally sensitive, with minimal floor and practice effects for this population within this chronological and developmental age range. CONCLUSION: These findings demonstrate that the Garage Game is promising for use in studies of early working memory and treatment trials that aim to support the development of this critical dimension of executive functioning for children with DS.
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Síndrome de Down , Memória de Curto Prazo , Criança , Humanos , Psicometria , Cognição , Função ExecutivaRESUMO
BACKGROUND/OBJECTIVE: While positive airway pressure (PAP) is an efficacious intervention for the treatment of obstructive sleep apnea syndrome (OSAS) in children with Down syndrome (DS), implementation and consistent use can be difficult. Caregiver perspectives and experiences using PAP are described with the aim of informing clinical practice. METHODS: Qualitative semi-structured phone interviews were conducted with 40 caregivers (i.e., mothers) of children with DS and OSAS treated with PAP for at least 6 months. Content analysis was used to identify themes associated with adherence and non-adherence. RESULTS: Respondents indicated variability in caregiver experience with the adoption of PAP and observed benefits of PAP. Varied experiences were attributed to several themes including accessing supplies, interactions with the medical team and equipment company, and patients' unique needs and behaviors, including the child's willingness and ability to adapt to PAP, sensory sensitivities, keeping the mask on all night, and differences in daytime behavior. Many families reported that desensitization with a reward system and trust within the caregiver-patient relationship were helpful. Caregiver suggestions for improving PAP adherence for families of children with DS included improving communication with the medical team and medical equipment company, emphasizing patience, using visual supports, and social support and education for extended family. CONCLUSIONS: Although family experiences varied, several actionable strategies by both the medical team and families emerged for improving the experience of and adherence to PAP in children with DS.
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Síndrome de Down , Apneia Obstrutiva do Sono , Humanos , Criança , Cuidadores , Síndrome de Down/complicações , Pressão Positiva Contínua nas Vias Aéreas , Cooperação do PacienteRESUMO
BACKGROUND: Executive functioning (EF) is an area of challenge for individuals with Down syndrome (DS) associated with a variety of downstream difficulties. Verbal fluency performance is one potential downstream effect that is commonly assessed in individuals with DS due to the measure's utility as a predictor of dementia. Verbal fluency requires individuals to inhibit irrelevant responses, shift between groupings of related words, and monitor to prevent repetition, all skills related to EF. AIMS: This study aimed to determine the association between semantic verbal fluency performance and three EF subdomains (inhibition, shifting, and working memory) in youth with DS after taking into account vocabulary and cognitive ability. METHODS AND PROCEDURES: Neuropsychological assessments (verbal and visuospatial), and parent reports of EF, were completed at one time point by 69 youth with DS 6-17 years old and their caregivers. Expressive and receptive vocabulary skills and cognitive ability were also assessed. OUTCOMES AND RESULTS: The results revealed that verbal fluency performance was significantly associated with neuropsychological assessments of EF and parent report of inhibition even after controlling for the effects of vocabulary and cognitive ability. CONCLUSIONS AND IMPLICATIONS: The findings highlight the underlying importance of EF in verbal fluency tasks in youth with DS.