Abstract To date, hydroxyurea is the only effective and safe drug that significantly reduces morbidity and mortality of individuals with Sickle cell disease. Twenty years of real-life experience has demonstrated that hydroxyurea reduces pain attacks, vaso-occlusive events, including acute chest syndrome, the number and duration of hospitalizations and the need for transfusion. The therapeutic success of hydroxyurea is directly linked to access to the drug, the dose used and adherence to treatment which, in part, is correlated to the availability of hydroxyurea. This consensus aims to reduce the number of mandatory exams needed to access the drug, prioritizing the requesting physician's report, without affecting patient safety. date disease reallife real life attacks vasoocclusive vaso occlusive events syndrome transfusion which part physicians physician s report safety

RESUMO Objetivo: Estimar as tendências da taxa de mortalidade e da idade média de morte e identificar os fatores sociodemográficos associados ao óbito precoce em pacientes com doença falciforme (DF). Métodos: Estudo ecológico e transversal realizado com dados do Sistema de Informações sobre Mortalidade. Foram incluídos todos os eventos de óbitos de pacientes residentes no estado de São Paulo de 1996 a 2015, que continham pelo menos um Código Internacional de Doenças para DF, em qualquer campo do atestado de óbito. As tendências foram estimadas por meio da regressão linear simples. Para a identificação dos fatores associados ao óbito precoce, foram realizadas análises de sobrevida, por meio da regressão de Cox simples e múltipla. Resultados: A taxa de mortalidade, padronizada pela idade, por milhão de habitantes, aumentou 0,080 ao ano (R²=0,761; p<0,001). Quando os eventos foram estratificados por idade do óbito, naqueles que ocorreram com 20 anos ou mais, o aumento foi de 0,108 ao ano (R²=0,789; p<0,001) e, nos que ocorreram antes de 20 anos, foi de 0,023 ao ano (R²=0,188; p=0,056). A idade média ao morrer aumentou 0,617 ano por ano (R²=0,835; p<0,001). Os fatores associados ao óbito precoce identificados no modelo múltiplo foram: sexo masculino (hazard ratio — HR=1,30), raça branca (HR=1,16), morte dentro do hospital (HR=1,29) e moradia na Grande São Paulo (HR=1,13). Conclusões: Houve aumento da taxa de mortalidade e da idade média de óbito com DF nas duas últimas décadas estudadas. Os fatores sociodemográficos sexo, raça, local de ocorrência e município de residência estiveram associados com a faixa etária do óbito. Objetivo DF. . (DF) Métodos Mortalidade 199 2015 sobrevida múltipla Resultados habitantes 0080 0 080 0,08 R²=0,761 R²0761 R R² 761 (R²=0,761 p<0,001. p0001 p p<0,001 001 2 mais 0108 108 0,10 R²=0,789 R²0789 789 (R²=0,789 0023 023 0,02 R²=0,188 R²0188 188 (R²=0,188 p=0,056. p0056 p=0,056 056 p=0,056) 0617 617 0,61 R²=0,835 R²0835 835 (R²=0,835 hazard HR=1,30, HR130 HR HR=1,30 , 1 30 HR=1,30) HR=1,16, HR116 HR=1,16 16 (HR=1,16) HR=1,29 HR129 29 (HR=1,29 HR=1,13. HR113 HR=1,13 13 (HR=1,13) Conclusões estudadas (DF 19 201 008 08 0,0 R²=0,76 R²076 76 (R²=0,76 p000 p<0,00 00 010 10 0,1 R²=0,78 R²078 78 (R²=0,78 002 02 R²=0,18 R²018 18 (R²=0,18 p005 p=0,05 05 061 61 0,6 R²=0,83 R²083 83 (R²=0,83 HR13 HR=1,3 3 HR11 HR=1,1 (HR=1,16 HR=1,2 HR12 (HR=1,2 (HR=1,13 0, R²=0,7 R²07 7 (R²=0,7 p00 p<0,0 01 R²=0,1 R²01 (R²=0,1 p=0,0 06 6 R²=0,8 R²08 8 (R²=0,8 HR1 HR=1, (HR=1,1 (HR=1, R²=0, R²0 (R²=0, p0 p<0, p=0, HR=1 (HR=1 R²=0 (R²=0 p<0 p=0 HR= (HR= R²= (R²= p< p= (HR (R² (R

ABSTRACT Objective: To estimate trends in mortality rate and average age of death, and identify sociodemographic factors associated with early death in patients with sickle cell disease (SCD). Methods: An ecological and cross-sectional study was conducted using data from the Mortality Information System. All deaths of patients residing in the state of São Paulo from 1996 to 2015 with at least one International Disease Code for SCD in any field of the death certificate were included. Simple linear regression was used to estimate trends. The Log-rank test and multiple Cox regression were used to identify factors associated with early death. Results: The age-standardized mortality rate per million inhabitants increased by 0.080 per year (R2=0.761; p<0.001). When the events were stratified by age at death, the increase was 0.108 per year for those occurring at age 20 years or older, (R2=0.789; p<0.001) and 0.023 per year for those occurring before age 20 years old (R2=0.188; p=0.056). The average age at death increased by 0.617 years (7.4 months) per year (R2=0.835; p<0.001). Sociodemographic factors associated with early death identified were male gender (hazard ratio — HR=1.30), white race (HR=1.16), death occurring in the hospital (HR=1.29), and living in the Greater São Paulo (HR=1.13). Conclusions: The mortality rate and the average age of death in patients with SCD have increased over the last two decades. Sociodemographic factors such as gender, race, place of occurrence, and residence were found to be associated with early death. Objective SCD. . (SCD) Methods crosssectional cross sectional System 199 201 included Logrank Log rank Results agestandardized standardized 0080 0 080 0.08 R2=0.761 R20761 R R2 761 (R2=0.761 p<0.001. p0001 p p<0.001 001 0108 108 0.10 2 older R2=0.789 R20789 789 (R2=0.789 0023 023 0.02 R2=0.188 R20188 188 (R2=0.188 p=0.056. p0056 p=0.056 056 p=0.056) 0617 617 0.61 7.4 74 7 4 (7. months R2=0.835 R20835 835 (R2=0.835 hazard HR=1.30, HR130 HR HR=1.30 , 1 30 HR=1.30) HR=1.16, HR116 HR=1.16 16 (HR=1.16) HR=1.29, HR129 HR=1.29 29 (HR=1.29) HR=1.13. HR113 HR=1.13 13 (HR=1.13) Conclusions decades occurrence (SCD 19 008 08 0.0 R2=0.76 R2076 76 (R2=0.76 p000 p<0.00 00 010 10 0.1 R2=0.78 R2078 78 (R2=0.78 002 02 R2=0.18 R2018 18 (R2=0.18 p005 p=0.05 05 061 61 0.6 7. (7 R2=0.83 R2083 83 (R2=0.83 HR13 HR=1.3 3 HR11 HR=1.1 (HR=1.16 HR12 HR=1.2 (HR=1.29 (HR=1.13 0. R2=0.7 R207 (R2=0.7 p00 p<0.0 01 R2=0.1 R201 (R2=0.1 p=0.0 06 6 ( R2=0.8 R208 8 (R2=0.8 HR1 HR=1. (HR=1.1 (HR=1.2 R2=0. R20 (R2=0. p0 p<0. p=0. HR=1 (HR=1. R2=0 (R2=0 p<0 p=0 HR= (HR=1 R2= (R2= p< p= (HR= (R2 (HR (R

RESUMO Objetivo: Descrever dois casos de pacientes que apresentaram microangiopatia trombótica (MAT) associada à doença falciforme (DF). Descrição do caso: Ambos os pacientes iniciaram com crise dolorosa e apresentaram síndrome torácica aguda durante a internação. Eles apresentaram piora significativa da anemia hemolítica, com níveis muito elevados de lactato desidrogenase, trombocitopenia, rebaixamento do nível de consciência, lesão de órgãos e presença de esquistócitos no sangue periférico. Diante da possibilidade de MAT, apesar da associação muito rara com DF, eles foram tratados com reposição de plasma fresco congelado e plasmaférese, com boa resposta. Comentários: A MAT é uma doença grave e com risco de vida, caracterizada por anemia hemolítica microangiopática, trombocitopenia e danos a órgãos. A associação de DF e MAT é de difícil diagnóstico, pois as duas podem ter apresentação clínica semelhante, portanto reconhecer essa associação e instituir o tratamento prontamente pode ter grande impacto na sobrevida desses pacientes. Objetivo (MAT DF. . (DF) caso internação desidrogenase consciência periférico plasmaférese resposta Comentários vida microangiopática diagnóstico semelhante (DF

ABSTRACT Objective: To describe two cases of patients who had thrombotic microangiopathy (TMA) associated with sickle cell disease (SCD). Case description: Both patients started with a painful crisis and had acute chest syndrome during hospitalization. They showed significant worsening of hemolytic anemia, with very high levels of lactate dehydrogenase, thrombocytopenia, lowered level of consciousness, organ damage and the presence of schistocytes in peripheral blood. Due to the possibility of TMA, despite the very rare association with SCD, they were treated with fresh frozen plasma replacement and plasmapheresis, with good response. Comments: TMA is a serious, life-threatening disease, characterized by microangiopathic hemolytic anemia, thrombocytopenia, and organ damage. The association of SCD and TMA is difficult to diagnose, since they can share a similar clinical presentation. Recognizing this association and promptly instituting treatment may impact the survival of these patients. Objective (TMA SCD. . (SCD) description hospitalization anemia dehydrogenase thrombocytopenia consciousness blood plasmapheresis response Comments serious lifethreatening life threatening diagnose presentation (SCD

Abstract Objective Advances in medicine have increased the life expectancy of pediatric patients with chronic illnesses, and challenges with the guided transition of adolescents and young adults from pediatric clinics to adult clinics have grown. The aim of this study was to better understand readiness and factors related to this transition process in Brazil. Method In this cross-sectional study of 308 patients aged from 16 to 21 years under follow-up in pediatric specialties, the degree of readiness for transition was assessed using the Transition Readiness Assessment Questionnaire (TRAQ) and its domains. Associations with demographic data, clinical data, socio-economic level, medication adherence, family functionality, and parental satisfaction with health care were evaluated. Results The median TRAQ score was 3.7 (3.2 - 4.2). Better readiness was associated with female patients, socio-economic class A-B, current active employment, higher level of education, not failing any school year, attending medical appointments alone, functional family, and a good knowledge of disease and medications. A low correlation was observed between TRAQ and age. TRAQ presented good internal consistency (alpha-Cronbach 0.86). In the multiple linear regression, TRAQ score showed a significant association with female gender, advanced age, socio-economic class A-B, better knowledge of disease and medications, and independence to attend appointments alone. Conclusion TRAQ instrument can guide healthcare professionals to identify specific areas of approach, in order to support adolescents with chronic disease to set goals for their own personal development and improve their readiness to enter into the adult healthcare system. In this study, some factors were related to better TRAQ scores. illnesses grown Brazil crosssectional cross sectional 30 1 2 followup follow up specialties (TRAQ domains data socioeconomic socio economic adherence functionality evaluated 37 3 7 3. 3.2 32 (3. 4.2. 42 4.2 . 4 4.2) AB, AB B, B A-B employment education year alone medications age alphaCronbach alpha Cronbach 0.86. 086 0.86 0 86 0.86) regression gender approach system scores (3 4. 08 0.8 8 ( 0.

RESUMO Objetivo: Identificar diferenças clínicas e laboratoriais entre pacientes pediátricos hospitalizados com doença falciforme infectados ou não por SARS-CoV-2 e comparar o hemograma completo de pacientes com doença falciforme infectados por SARS-CoV-2 antes da hospitalização e durante a admissão. Métodos: Coorte prospectiva unicêntrica, cujos dados foram coletados em prontuários de pacientes pediátricos internados com doença falciforme, menores de 18 anos, infectados ou não com SARS-CoV-2, desde a primeira visita ao hospital até a alta e desde a última consulta médica. Todos os pacientes foram testados para SARS-CoV-2 pela transcrição reversa seguida de reação em cadeia da polimerase em tempo real. Resultados: Dos 57 pacientes pediátricos com doença falciforme internados de março a novembro de 2020 em um hospital universitário brasileiro, 11 (19,3%) apresentaram resultado positivo para SARS-CoV-2. Pacientes infectados pelo SARS-CoV-2 apresentaram maior prevalência de comorbidades do que aqueles não infectados (63,6 vs. 30,4%; p=0,046). Durante a internação hospitalar, não foram encontradas diferenças clínicas ou laboratoriais entre os grupos. Houve diminuição da contagem de eosinófilos na admissão hospitalar em pacientes com doença falciforme infectados pelo SARS-CoV-2 (p=0,008). Conclusões: Pacientes pediátricos hospitalizados com doença falciforme infectados pelo SARS-CoV-2 apresentaram mais comorbidades e diminuição da contagem de eosinófilos entre a admissão hospitalar e a última consulta médica. Objetivo SARSCoV2 SARSCoV SARS CoV 2 SARS-CoV- Métodos unicêntrica 1 anos SARSCoV2, 2, médica real Resultados 5 202 brasileiro 19,3% 193 19 3 (19,3% SARSCoV2. 2. 63,6 636 63 6 (63, vs 30,4% 304 30 4 p=0,046. p0046 p p=0,046 . 0 046 p=0,046) grupos p=0,008. p0008 p=0,008 008 (p=0,008) Conclusões SARS-CoV 20 19,3 (19,3 63, (63 30,4 p004 p=0,04 04 p000 p=0,00 00 (p=0,008 19, (19, (6 30, p00 p=0,0 (p=0,00 (19 ( p0 p=0, (p=0,0 (1 p=0 (p=0, p= (p=0 (p= (p

Abstract Objective: The aim of this study was to identify clinical and complete blood count differences between pediatric hospitalized patients with sickle cell disease infected or not by SARS-CoV-2 and compare the complete blood count of patients with sickle cell disease infected by SARS-CoV-2 before hospitalization and on admission. Methods: This study was a single-center prospective cohort. Data were collected from medical records of pediatric inpatients with sickle cell disease under 18 years old infected or not with SARS-CoV-2 from the first visit to the hospital until discharge and from the last medical appointment. All patients were tested for SARS-CoV-2 by the real-time reverse transcription polymerase chain reaction. Results: Among 57 pediatric patients with sickle cell disease hospitalized from March to November 2020 in a Brazilian academic hospital, 11 (19.3%) had a positive result for SARS-CoV-2. Patients infected by SARS-CoV-2 had a higher prevalence of comorbidities than the ones who were not infected (63.6 vs. 30.4%; p=0.046). During hospital stay, no clinical or complete blood count differences between groups were found. There was a decrease in eosinophil count on hospital admission in patients with sickle cell disease infected by SARS-CoV-2 (p=0.008). Conclusions: Pediatric hospitalized patients with sickle cell disease infected by SARS-CoV-2 had more comorbidities and had a decrease in eosinophil count between hospital admission and the last medical appointment. Objective SARSCoV2 SARSCoV SARS CoV 2 SARS-CoV- Methods singlecenter single center cohort 1 appointment realtime real time reaction Results 5 202 19.3% 193 19 3 (19.3% SARSCoV2. 2. 63.6 636 63 6 (63. vs 30.4% 304 30 4 p=0.046. p0046 p p=0.046 . 0 046 p=0.046) stay found p=0.008. p0008 p=0.008 008 (p=0.008) Conclusions SARS-CoV 20 19.3 (19.3 63. (63 30.4 p004 p=0.04 04 p000 p=0.00 00 (p=0.008 19. (19. (6 30. p00 p=0.0 (p=0.00 (19 ( p0 p=0. (p=0.0 (1 p=0 (p=0. p= (p=0 (p= (p

Resumo Introdução: A nefropatia falciforme começa na infância e apresenta aumentos precoces na filtração glomerular, que, em longo prazo, podem levar à insuficiência renal crônica. Várias doenças têm aumentado a atividade da enzima conversora da angiotensina (ECA) urinária e circulante, mas há pouca informação sobre alterações na atividade das ECAs em crianças com doença falciforme (DF). Objetivo: Examinamos a atividade da ECA-1 circulante e urinária em crianças com DF. Métodos: Este estudo transversal comparou crianças que eram portadoras de DF com crianças que compunham um Grupo Controle (GC). As atividades séricas e urinárias da ECA foram avaliadas, assim como os fatores bioquímicos, a relação albumina/creatinina urinária e a taxa de filtração glomerular estimada. Resultados: A atividade urinária da ECA foi significativamente maior em pacientes com DF do que em crianças saudáveis (mediana 0,01; intervalo 0,00-0,07 vs mediana 0,00; intervalo 0,00-0,01 mU/mL·creatinina, p < 0,001. Não foi observada diferença significativa nas atividades séricas da ECA entre os grupos DF e GC (mediana 32,25; intervalo 16,2-59,3 vs mediana 40,9; intervalo 18,0-53,4) mU/mL·creatinina, p < 0,05. Conclusão: Nossos dados revelaram uma alta atividade urinária da ECA-1, diferente do nível plasmático, em pacientes com DF, sugerindo uma dissociação entre o Sistema Renina Angiotensina Aldosterona (SRAA) intra-renal e sistêmico. O aumento da atividade urinária da ECA-1 em pacientes com DF sugere níveis mais elevados de Ang II com predominância do eixo clássico do SRAA, que pode induzir lesão renal. Introdução prazo crônica (ECA . (DF) Objetivo ECA1 1 ECA- Métodos GC. (GC) avaliadas bioquímicos albuminacreatinina albumina creatinina estimada Resultados 0,01 001 0 01 0,000,07 000007 0,00 0,07 00 07 0,00-0,0 000 0,000,01 000001 mUmLcreatinina mU mL mU/mL·creatinina 0001 0,001 32,25 3225 32 25 16,259,3 162593 16,2 59,3 16 2 59 3 16,2-59, 40,9 409 40 9 18,053,4 180534 18,0 53,4 18 53 4 18,0-53,4 005 05 0,05 Conclusão ECA1, 1, plasmático SRAA (SRAA intrarenal intra sistêmico (DF (GC 0,0 0,000,0 00000 007 0,00-0, 32,2 322 259 16,259, 16259 162 16, 593 59, 5 16,2-59 40, 053 18,053, 18053 180 18, 534 53, 18,0-53, 0, 0,000, 0000 0,00-0 32, 16,259 1625 16,2-5 18,053 1805 18,0-53 0,000 0,00- 16,25 16,2- 18,05 18,0-5 18,0-

Abstract Introduction: Sickle cell nephropathy begins in childhood and presents early increases in glomerular filtration, which, over the long term, can lead to chronic renal failure. Several diseases have increased circulating and urinary angiotensin-converting enzyme (ACE) activity, but there is little information about changes in ACEs activity in children with sickle cell disease (SCD). Objective: We examined circulating and urinary ACE 1 activity in children with SCD. Methods: This cross-sectional study compared children who were carriers of SCD with children who comprised a control group (CG). Serum and urinary activities of ACE were evaluated, as were biochemical factors, urinary album/creatinine rates, and estimated glomerular filtration rate. Results: Urinary ACE activity was significantly higher in patients with SCD than in healthy children (median 0.01; range 0.00-0.07 vs median 0.00; range 0.00-0.01 mU/mL·creatinine, p < 0.001. No significant difference in serum ACE activities between the SCD and CG groups was observed (median 32.25; range 16.2-59.3 vs median 40.9; range 18.0-53.4) mU/m`L·creatinine, p < 0.05. Conclusion: Our data revealed a high urinary ACE 1 activity, different than plasmatic level, in SCD patients suggesting a dissociation between the intrarenal and systemic RAAS. The increase of urinary ACE 1 activity in SCD patients suggests higher levels of Ang II with a predominance of classical RAAS axis, that can induce kidney damage. Introduction which term failure angiotensinconverting angiotensin converting (ACE . (SCD) Objective Methods crosssectional cross sectional CG. (CG) evaluated factors albumcreatinine album creatinine rates rate Results 0.01 001 0 01 0.000.07 000007 0.00 0.07 00 07 0.00-0.0 000 0.000.01 000001 mUmLcreatinine mU mL mU/mL·creatinine 0001 0.001 32.25 3225 32 25 16.259.3 162593 16.2 59.3 16 2 59 3 16.2-59. 40.9 409 40 9 18.053.4 180534 18.0 53.4 18 53 4 18.0-53.4 m L mU/m`L·creatinine 005 05 0.05 Conclusion level axis damage (SCD (CG 0.0 0.000.0 00000 007 0.00-0. 32.2 322 259 16.259. 16259 162 16. 593 59. 5 16.2-59 40. 053 18.053. 18053 180 18. 534 53. 18.0-53. 0. 0.000. 0000 0.00-0 32. 16.259 1625 16.2-5 18.053 1805 18.0-53 0.000 0.00- 16.25 16.2- 18.05 18.0-5 18.0-

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.

RESUMO Objetivo: Avaliar aspectos sociodemográficos e clínicos de crianças com doença falciforme (DF) e suas características comportamentais. Métodos: Aplicação de entrevista sobre aspectos socioeconômicos e outras condições de saúde e do questionário de capacidades e dificuldades (SDQ) em pais de pacientes de quatro a dez anos com DF, em um ambulatório de referência. Dados clínicos foram obtidos dos prontuários médicos. Critérios de exclusão: uso de hidroxiureia, diagnóstico prévio de acidente vascular cerebral, encefalopatia crônica e/ou deficiência intelectual. Resultados: Analisados 45 pacientes (19 meninas e 26 meninos). Mediana de idade=7 anos. Diagnóstico da DF=26 hemoglobinopatia SC; 19 hemoglobinopatia SS. Classe econômica (SES): D=24,4%; C2=44,4%; C1=28,8%; B2=2,2%. Antecedentes clínicos: síndrome torácica aguda=40%; transfusões=66,7%; internações=82,2%. Achados SDQ=88,9% alteração clínica (subescala emocional=68,9%); pontuação total=alterada em 48,9%. Não foi possível estabelecer relação entre gravidade da doença e os resultados do SDQ. Com relação à SES, entre indivíduos das classes B2 e C1, 21,4% tiveram alteração na pontuação total; nas classes C2 e D, esse percentual foi de 61,3%. Quanto à escolaridade do chefe da família, com no mínimo ensino fundamental completo, 39,3% das crianças tiveram alteração; para menor escolaridade, esse percentual foi 64,7%. Conclusões: Alterações comportamentais são altamente prevalentes em crianças com DF. Indivíduos das classes C2 e D tiveram mais alterações comportamentais em relação aos indivíduos das classes B2 e C1.

ABSTRACT Objective: To evaluate sociodemographic and clinical aspects of children with sickle cell disease (SCD) and their behavioral characteristics. Methods: Interview with parents of patients with SCD from four to ten years old, addressing socioeconomic aspects and other health conditions, and using the Strengths and Difficulties Questionnaire (SDQ). Clinical data were obtained from medical records. Exclusion criteria were the use of hydroxyurea, previous diagnosis of stroke, chronic encephalopathy and/or intellectual disability. Results: 45 patients (19 girls and 26 boys) were assessed. The median age was seven years. Diagnosis of SCD: 26 hemoglobinopathy SC; 19 hemoglobinopathy SS. Socioeconomic class: D: 24.4%; C2: 44.4%; C1: 28.9%; B2: 2.2%. Clinical history: acute chest syndrome: 40%; transfusions: 66.7%; hospitalizations: 82.2%. SDQ findings: 88.9% clinical impact (emotional subscale: 68.9%); total score: impact in 48.9%. It was not possible to establish a relation between the severity of the disease and the results of the SDQ. Regarding socioeconomic class: among individuals of classes B2 and C1, 21.4% had impact at the total score; in classes C2 and D, this percentage was 61.3%. Regarding the schooling of the head of the family, with Elementary School at least, 39.3% of the children had impacts; for fewer education, this percentage was 64.7%. Conclusions: Behavioral impacts are highly prevalent in children with SCD. Individuals in socioeconomic classes C2 and D suffered more behavioral impacts than individuals in classes B2 and C1.

Abstract Introduction About 10% of sickle cell anemia patients will have ischemic stroke. Adams showed stroke incidence reduction in children receiving monthly erythrocyte transfusions by reducing transcranial Doppler (TCD) velocities. Since then, chronic transfusion is recommended as primary stroke prophylaxis. This study aims to assess the effectiveness of chronic transfusions as stroke prophylaxis. Method Retrospective study, reviewing medical records from 15 sickle cell anemia patients undergoing chronic transfusion. Data collected were age, sex, adverse reactions, stroke, hemoglobin, reticulocytes, ferritin, HbS and TCD values (baseline, after 12 and 24 months of treatment). Results The mean age was 118.67 ± 41.40 months; six patients experienced allergic reactions. No stroke was recorded. One patient had alloimmunization. There was a decrease in the HbS rate and an increase in hemoglobin values in the first 12 months. Values were maintained after 24 months, but with no improvement of data. Before treatment, the mean HbS rate was 75.18%±11.69; after 12 months, 41.63 ± 14.99 and after 24 months, 43.78 ± 10.6. Thirteen patients initiated chelation after 12 months from the beginning of chronic transfusions and ferritin decline after 24 months. Pre-transfusional TCD velocities were 204.28 ± 9.41 cm/s (right) and 198.85 ± 33.37 cm/s (left). After a 12-month treatment, these values were 158.5 ± 28.89 cm/s and 157.62 ± 34.43 cm/s, respectively, and this reduction was statistically significant (p = 0.002 right and p = 0.02 left). After 24 months, these values were 149.63 ± 26.95 cm/s (right) and 143.7 ± 32.27 cm/s (left). Conclusion Significant reduction of TCD velocity occurred after treatment with chronic transfusion in sickle cell anemia patients, leading to a normal or conditional test and reducing stroke risk in all but one patient.

RESUMO Objetivo: Descrever as características socioeconômicas e nutricionais de crianças e adolescentes com anemia falciforme. Fonte de dados: Estudo de revisão sistemática da literatura baseado em artigos publicados em revistas científicas. As buscas foram realizadas na base de dados eletrônica da National Library of Medicine, National Institutes of Health- PubMed.Foram realizadas duas buscas de artigos publicados nos últimos 20anos e sem limitação de idioma. Aprimeira partiu do Descritor em Ciências da Saúde “Anemia Falciforme” associado com “Fatores Socioeconômicos”; e a segunda partiu do descritor “Anemia Falciforme” associado com “Antropometria”. Asbuscas foram direcionadas para pesquisas realizadas em seres humanos na faixa etária de 0 a 18anos. Síntese dos dados: A seleção final foi composta por 11artigos referentes às características socioeconômicas e 21 referentes às características nutricionais. Asamostras estudadas foram de crianças e adolescentes de ambos os sexos, com idade de 0 a 18anos e com predomínio de populações negras. Famílias de crianças e adolescentes com anemia falciforme eram predominantemente de baixo nível socioeconômico. Ospais apresentaram níveis educacionais inferiores, quando comparados a pais de crianças e adolescentes saudáveis. Asmedidas corporais (peso e estatura) e os indicadores antropométricos de crianças com anemia falciforme foram frequentemente menores, quando comparados com os dos grupos saudáveis ou das populações de referência. Conclusão: Crianças e adolescentes com anemia falciforme apresentam limitações socioeconômicas e piores condições nutricionais, quando comparados às populações de referência. Essas limitações podem implicar pior crescimento e maior ocorrência de possíveis complicações, que podem prejudicar sua qualidade de vida.

ABSTRACT Objective: To describe the socioeconomic and nutritional characteristics of children and adolescents with sickle cell anemia. Data sources: The present study is a systematic literature review based on published scientific articles. The searches were carried out using the electronic database of the National Library of Medicine, National Institutes of Health- PubMed. Two searches of articles published in the last 20years and without limitation of language were carried out. Thefirst one started from the Medical Subject Headings term “Anemia, Sickle Cell” associated with “Socioeconomic Factors”; and the second started from the term “Anemia, Sickle Cell” associated with “Anthropometry”. Thesearches were directed to research conducted on humans in the age group from 0 to 18years. Data synthesis: The final selection was composed by 11 articles on socioeconomic characteristics and 21articles on nutritional characteristics. Allstudies included children and adolescents with sickle cells disease (age range 0-18years), both genders, and most of them of black ethnicity. Families of children and adolescents with sickle cell anemia were of predominantly low socioeconomic status. Parents had lower educational levels when compared to parents of healthy children and adolescents. Body measurements (weight and height) and anthropometric indicators of children with sickle cell anemia were often lower when compared to healthy groups or reference populations. Conclusions: Children and adolescents with sickle cell anemia have socioeconomic limitations and worse nutritional conditions, when compared to reference populations. These limitations may lead to worse growth and greater occurrence of possible complications that can impair their quality of life.

RESUMO Doppler transcraniano (DTC) é um componente estratégico da prevenção primária do acidente vascular cerebral (AVC) em crianças com doença falciforme (DF). Este estudo foi realizado para examinar as características do DTC de crianças com DF em nove centros médicos diferentes no Brasil. Métodos: DTC foi realizado de acordo com o protocolo de Stroke Prevention Trial in Sickle Cell Anemia Protocol (STOP). Resultados: Dos 396 pacientes avaliados, 69,5% eram homozigotos para hemoglobina SS. DTC foi anormal em 4,8%, condicional em 12,6%, inadequado em 5,3% e anormalmente baixo em 1%. As máximas velocidades de fluxo médio foram 121 ± 23,83cm/s e 124 ± 27,21 cm/s nas artérias cerebrais media esquerda e direita, respectivamente. Um total de 28,8% dos pacientes (média de 9,19 ± 5,92 anos) foram avaliados com o DTC pela primeira vez. Conclusões: Pacientes com DF foram avaliados com DTC numa idade considerada avançada, o que representa uma importante oportunidade perdida para a prevenção de AVC nessa população. Uma vez que a triagem com DTC em pacientes com DF é essencial para detectar aqueles com alto risco de AVC, recomenda-se que essa triagem seja amplamente disponível no país.

ABSTRACT Transcranial doppler (TCD) is a strategic component of primary stroke prevention in children with sickle cell disease (SCD). This study was conducted to examine the TCD characteristics of children with SCD in nine different medical centers in Brazil. Methods: Transcranial doppler was performed in accordance with the Stroke Prevention Trial in Sickle Cell Anemia Protocol. Results: Of the 396 patients, 69.5% had homozygous SS hemoglobin. The TCD result was abnormal in 4.8%, conditional in 12.6%, inadequate in 4.3% and abnormally low in 1% of patients. The highest mean flow velocities were 121±23.83cm/s and 124±27.21cm/s in the left and right middle cerebral artery respectively. A total of 28.8% patients (mean age 9.19±5.92 years) were evaluated with TCD for the first time. Conclusions: The SCD patients were evaluated with TCD at an older age, representing an important missed opportunity for stroke prevention. Since TCD screening in patients with SCD is important to detect those at high risk for stroke, it is recommended that this screening should be made more readily available.

Resumo Objetivo Avaliar a correlação entre os níveis de hemoglobina de mães e de seus filhos em aleitamento materno exclusivo, no primeiro semestre de vida. Métodos Estudo transversal com 221 binômios (mãe-filho) matriculados em programa de incentivo ao aleitamento materno, estratificados em seis grupos de acordo com a faixa etária das crianças. A amostra consistiu de crianças nascidas a termo, com peso normal, sem intercorrências neonatais e cujas mães não apresentavam doença infecciosa e anemia na época da coleta de dados. Foram feitas entrevistas com as mães, coleta de sangue por punção de veia periférica das mães e das crianças e antropometria das crianças. Foram calculados os coeficientes de correlação de Pearson entre os níveis de hemoglobina das mães e das crianças. Foram ajustados seis modelos de regressão linear múltiplos com estimativas de coeficientes de regressão, consideraram-se estatisticamente significantes associações com p ≤ 0,05. Resultados Os coeficientes de correlação dos níveis de hemoglobina das mães e das crianças variaram entre 0,253, aos três meses, e 0,601, aos cinco meses. O nível de hemoglobina das mães esteve mais correlacionado com o das crianças aos quatro meses (r = 0,578) e aos cinco meses (r = 0,601). Na regressão linear múltipla ajustada, os coeficientes de regressão foram maiores aos quatro meses (β = 1,134; p = 0,002) e aos cinco meses (β = 0,845; p < 0,001). Conclusão Esses achados permitem concluir que há correlação entre a hemoglobina de mães e a hemoglobina de seus filhos em aleitamento materno exclusivo no primeiro semestre de vida.

Abstract Objective: To evaluate the correlation between hemoglobin levels of mothers and their children on exclusive breastfeeding in the first six months of life. Methods: Cross-sectional study with 221 binomials (mother-child) enrolled in a breastfeeding support program, who were stratified into six groups according to the children's age group. The sample consisted of children born at term with normal weight, with no neonatal complications and whose mothers did not have anemia or infectious disease at the time of data collection. Interviews were carried out with the mothers, blood was collected by peripheral venipuncture from mothers and children, and children's anthropometric data were assessed. Pearson's correlation coefficients between the hemoglobin levels of mothers and children were calculated. Six multiple linear regression models were adjusted with regression coefficient estimates, considering as statistically significant associations with p ≤ 0.05. Results: The correlation coefficients of hemoglobin levels of mothers and children ranged from 0.253, at three months, to 0.601, at five months. The hemoglobin level of mothers was correlated with the hemoglobin level of their children at four months (r = 0.578) and at five months (r = 0.601). In the adjusted multiple linear regression, the regression coefficients were higher at four months (β = 1.134; p = 0.002) and at five months (β = 0.845; p < 0.001). Conclusion: These findings allow for the conclusion that there is a correlation between the hemoglobin of mothers and the hemoglobin of their children on exclusive breastfeeding in the first six months of life.