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INTRODUCTION: Despite the high prevalence of sickle cell disease (SCD) in Brazil, no studies have described the validation of an SCD-specific health-related quality-of-life (HRQoL) instrument in children. We validated PedsQL 3.0 Sickle Cell Disease Module (PedsQL-SCD) for Brazilian Portuguese, and cross-validated it with PedsQL 4.0 Generic Core Scale (PedsQL-GCS) in children with SCD. METHODS: PedsQL-SCD was translated and culturally adapted using forward and reverse translations. PedsQL-SCD and PedsQL-GCS were tested in children and adolescents with SCD aged 2-18 years and their caregivers. Validity was assessed using the Pearson and intraclass correlation coefficients, and reliability measured with Cronbach's alpha. RESULTS: PedsQL-SCD was validated in 206 children with SCD (median age 14 years, range: 8-18) and 201 caregivers. Among patients and caregivers, the mean total score for PedsQL-SCD was 65.7 and 64.1, respectively. The mean total score for PedsQL-GCS was 73.1 and 68.9 among patients and caregivers, respectively. The internal consistency for PedsQL-SCD and PedsQL-GCS was good; Cronbach's alpha coefficients ranged from .59-.93 to .64-.83 among patients and from .60-.95 to .65-.85 among caregivers, respectively. Most intercorrelations between PedsQL-SCD and PedsQL-GCS, for patients and caregivers, had medium to large effect sizes (range: .23-.63 and .27-.64, respectively). Pain and pain impact domains of PedsQL-SCD and physical dimension of PedsQL-GCS had the highest cross-correlation (.63 and .6 for patients; .63 and .64 for caregivers, respectively), confirming convergent construct validity. CONCLUSION: PedsQL-SCD is a valid, culturally appropriate measure to assess HRQoL in children with SCD in Brazil and is well-correlated PedsQL-GCS.

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Implementation of HCT programs is a high priority in Brazil, given the growing survival rates for children with SCD. Our data highlights a well-connected community of healthcare workers, but also reveals that the current level of HCT activities for SCD in Brazil is low. This underscores the urgent need for transition intervention. The key facilitators for successful HCT implementation in Brazil are strong leadership and a motivated workforce. However, community participation and finances are weak points that require implementation strategies during program implementation. Compared to the US, both Brazilian institutions scored significantly lower on all components needed for transition, emphasizing the need for intervention. This project is the first to evaluate the readiness for HCT implementation in a low- and middle-income country. Designing a context-specific HCT program that suits low-resource settings will improve outcomes for individuals with SCD in the country. (AU)

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ABSTRACT Introduction: It is important to know if patients with hemoglobinopathy could be more susceptible to COVID-19. Objective: Analyze SARS-CoV-2 infection in pediatric patients with hemoglobinopathy. Methods: Using the online platforms LILACS, PUBMED and EMBASE, on 17- JUL-2020 a search was made for the terms COVID-19 and SARS-CoV-2 associated with "sickle cell", "thalassemia" and "hemoglobinopathy". Results: There were 623 pediatric and adult patients with sickle cell disease (SCD) or beta thalassemia (BT) and COVID-19. Total mortality rate was 6.42%. No pediatric patient with BT has been described. So, our analysis focused on children and adolescents with SCD: there were 121 pediatric patients, one adolescent died, prophylactic anticoagulation was prescribed to six patients, 11.76% needed intensive care unit, blood transfusion was prescribed in 29.70%. Vaso-occlusive crisis (VOC) and acute chest syndrome (ACS) were the main clinical manifestations in SCD. Discussion: Pediatric patients with SCD and COVID-19 have a low mortality rate when compared to adults, although is higher than the global pediatric population with COVID-19 (0−0.67%). The comorbidities associated with age and the long-term complications inherent to hemoglobinopathies may contribute to the increased mortality outside the pediatric age group. In SCD the clinical manifestations, both in children and adults, are VOC and ACS, and there was increase in blood requirement. Pediatric SCD patients with COVID-19 need more intensive care unit than the global pediatric population (3.30%). Conclusion: Despite pediatric population with SCD needs more intensive care, the outcome after infection by COVID-19 is favorable.

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INTRODUCTION: It is important to know if patients with hemoglobinopathy could be more susceptible to COVID-19. OBJECTIVE: Analyze SARS-CoV-2 infection in pediatric patients with hemoglobinopathy. METHODS: Using the online platforms LILACS, PUBMED and EMBASE, on 17- JUL-2020 a search was made for the terms COVID-19 and SARS-CoV-2 associated with "sickle cell", "thalassemia" and "hemoglobinopathy". RESULTS: There were 623 pediatric and adult patients with sickle cell disease (SCD) or beta thalassemia (BT) and COVID-19. Total mortality rate was 6.42%. No pediatric patient with BT has been described. So, our analysis focused on children and adolescents with SCD: there were 121 pediatric patients, one adolescent died, prophylactic anticoagulation was prescribed to six patients, 11.76% needed intensive care unit, blood transfusion was prescribed in 29.70%. Vaso-occlusive crisis (VOC) and acute chest syndrome (ACS) were the main clinical manifestations in SCD. DISCUSSION: Pediatric patients with SCD and COVID-19 have a low mortality rate when compared to adults, although is higher than the global pediatric population with COVID-19 (0-0.67%). The comorbidities associated with age and the long-term complications inherent to hemoglobinopathies may contribute to the increased mortality outside the pediatric age group. In SCD the clinical manifestations, both in children and adults, are VOC and ACS, and there was increase in blood requirement. Pediatric SCD patients with COVID-19 need more intensive care unit than the global pediatric population (3.30%). CONCLUSION: Despite pediatric population with SCD needs more intensive care, the outcome after infection by COVID-19 is favorable.

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Abstract Objective: Sleep deprivation in adolescents has lately become a health issue that tends to increase with higher stress prevalence, extenuating routines, and new technological devices that impair adolescents' bedtime. Therefore, this study aimed to assess the excessive sleepiness frequency and the factors that might be associated to it in this population. Methods: The cross-sectional study analyzed 531 adolescents aged 10–18 years old from two private schools and one public school. Five questionnaires were applied: the Cleveland Adolescent Sleepiness Questionnaire; the Sleep Disturbance Scale for Children; the Brazilian Economic Classification Criteria; the General Health and Sexual Maturation Questionnaire; and the Physical Activity Questionnaire. The statistical analyses were based on comparisons between schools and sleepiness and non-sleepiness groups, using linear correlation and logistic regression. Results: Sleep deprivation was present in 39% of the adolescents; sleep deficit was higher in private school adolescents (p < 0.001), and there was a positive correlation between age and sleep deficit (p < 0.001; r = 0.337). Logistic regression showed that older age (p = 0.002; PR: 1.21 [CI: 1.07–1.36]) and higher score level for sleep hyperhidrosis in the sleep disturbance scale (p = 0.02; PR: 1.16 [CI: 1.02–1.32]) were risk factors for worse degree of sleepiness. Conclusions: Sleep deficit appears to be a reality among adolescents; the results suggest a higher prevalence in students from private schools. Sleep deprivation is associated with older age in adolescents and possible presence of sleep disorders, such as sleep hyperhidrosis.

Resumo Objetivo: A privação de sono na adolescência é um importante problema de saúde na atualidade e só tende a se agravar com o aumento do estresse, da rotina extenuante e do advento de novos aparelhos tecnológicos que parecem refletir negativamente no início do sono em adolescentes. O estudo objetiva avaliar a frequência da sonolência excessiva e quais fatores podem estar associados a ela nessa população. Métodos: O estudo transversal avaliou 531 adolescentes de 10 a 18 anos em duas escolas de ensino privado e uma de ensino público e aplicou para cada adolescente cinco questionários: Cleveland Adolescent Sleepiness Questionnaire; Sleep Disturbance Scale for Children; Critério de Classificação Econômica Brasil; Questionário Geral de Saúde e Maturação Sexual; Questionário de Atividade Física. Fizeram-se comparações entre as escolas e entre grupos com e sem sonolência por meio de correlação linear e regressão logística. Resultados: Observou-se privação de sono em 39% dos adolescentes, débito de sono maior para escolares do ensino privado (p < 0,001) e correlação positiva entre idade e débito do sono (p < 0,001; r = 0,337). Na regressão logística, apontaram-se como fatores para pior grau de sonolência maior faixa etária dos escolares (p = 0,002; RP: 1,21 [IC: 1,07-1,36]) e maior escore na variável hiperidrose do sono do questionário de distúrbios do sono (p = 0,02; RP: 1,16 [IC: 1,02-1,32]). Conclusões: O déficit de sono é uma realidade na população estudada e apresenta-se pior em escolares do ensino privado. A privação de sono está relacionada com a maior faixa etária dos adolescentes e possível presença de distúrbios do sono, como a hiperidrose do sono.

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OBJECTIVE: Sleep deprivation in adolescents has lately become a health issue that tends to increase with higher stress prevalence, extenuating routines, and new technological devices that impair adolescents' bedtime. Therefore, this study aimed to assess the excessive sleepiness frequency and the factors that might be associated to it in this population. METHODS: The cross-sectional study analyzed 531 adolescents aged 10-18 years old from two private schools and one public school. Five questionnaires were applied: the Cleveland Adolescent Sleepiness Questionnaire; the Sleep Disturbance Scale for Children; the Brazilian Economic Classification Criteria; the General Health and Sexual Maturation Questionnaire; and the Physical Activity Questionnaire. The statistical analyses were based on comparisons between schools and sleepiness and non-sleepiness groups, using linear correlation and logistic regression. RESULTS: Sleep deprivation was present in 39% of the adolescents; sleep deficit was higher in private school adolescents (p<0.001), and there was a positive correlation between age and sleep deficit (p<0.001; r=0.337). Logistic regression showed that older age (p=0.002; PR: 1.21 [CI: 1.07-1.36]) and higher score level for sleep hyperhidrosis in the sleep disturbance scale (p=0.02; PR: 1.16 [CI: 1.02-1.32]) were risk factors for worse degree of sleepiness. CONCLUSIONS: Sleep deficit appears to be a reality among adolescents; the results suggest a higher prevalence in students from private schools. Sleep deprivation is associated with older age in adolescents and possible presence of sleep disorders, such as sleep hyperhidrosis.

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