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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.
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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.
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.
Subject(s)
Humans , Child , Adolescent , Bone Marrow Transplantation , Anemia, Sickle Cell , Ultrasonography, Doppler, Transcranial , HydroxyureaABSTRACT
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.
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.
Subject(s)
Humans , Child , Renal Insufficiency, Chronic , Anemia, Sickle Cell , Angiotensins , Cross-Sectional Studies , Peptidyl-Dipeptidase A , Angiotensin-Converting Enzyme 2ABSTRACT
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.
Subject(s)
Humans , Male , Female , Infant, Newborn , Infant , Child, Preschool , Child , Adolescent , Adult , Middle Aged , Aged , Thalassemia , Child , Coronavirus , SARS-CoV-2 , COVID-19 , Hemoglobinopathies , Anemia, Sickle CellSubject(s)
Humans , Male , Female , Child , Adolescent , Child , Adolescent , Acute Chest Syndrome , COVID-19 , Anemia, Sickle CellABSTRACT
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.
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.
Subject(s)
Humans , Male , Female , Child, Preschool , Child , Child Behavior , Anemia, Sickle Cell/psychology , Parents , Socioeconomic Factors , Severity of Illness Index , Cross-Sectional Studies , Surveys and QuestionnairesABSTRACT
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.
Subject(s)
Humans , Child, Preschool , Child , Adolescent , Child , Ultrasonography, Doppler, Transcranial , Erythrocyte Transfusion , Stroke , Anemia, Sickle CellABSTRACT
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.
Subject(s)
Humans , Male , Female , Infant, Newborn , Infant , Child, Preschool , Child , Adolescent , Nutritional Status , Socioeconomic Factors , Anemia, Sickle Cell/physiopathologyABSTRACT
BACKGROUND: As it is difficult to know the content of iron added in the Brazilian iron fortification of wheat and corn flour, and if the compound has good or poor bioavailability, the objective was to assess the effect of the Brazilian iron fortification program, as it was carried out, on iron nutritional status of adolescents, 6 years after its implementation. METHODS: A cross-sectional retrospective study was conducted with adolescents aged 10 to 17 years, both sexes, treated at a primary health care center. Data were collected from medical records of patients and compared with those of a previous survey conducted at the same center in 2004. Anthropometry, iron biochemical indicators, food intake, pubertal stage, and transferrin saturation were assessed, with pubertal stage and transferrin saturation being the last two variables compared between 2004 and 2010. Mann-Whitney and chi-square tests were also used. RESULTS: In 2010, mean hemoglobin values and serum ferritin levels were within normal ranges for both sexes, and adolescents who consumed diets with low iron bioavailability constituted more than half of the sample (52.7%). In2004, a 10.3 and 18.6% prevalence of iron deficiency was observed, based on low transferrin saturation, in females and males, respectively. It was noted that during the 6-year period, this prevalence decreased significantly, 4 and10.4%. CONCLUSIONS: Anemia and iron deficiency are not prevalent in this population, probably due to the implementation of flour fortification with iron; it is not possible, however, to attribute such a result only to the implementation of this strategy.
Subject(s)
Humans , Male , Female , Child , Adolescent , Food, Fortified/statistics & numerical data , Iron, Dietary/administration & dosage , Iron, Dietary/therapeutic useABSTRACT
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 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.
Subject(s)
Humans , Male , Female , Child , Blood Flow Velocity/physiology , Cerebrovascular Circulation/physiology , Ultrasonography, Doppler, Transcranial , Stroke/prevention & control , Anemia, Sickle Cell/complications , Cross-Sectional Studies , Predictive Value of Tests , Prospective Studies , Risk Factors , Patient Selection , Stroke/etiology , Stroke/physiopathology , Stroke/diagnostic imaging , Anemia, Sickle Cell/physiopathologyABSTRACT
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.
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.
Subject(s)
Humans , Male , Female , Infant, Newborn , Infant , Adolescent , Adult , Young Adult , Breast Feeding/statistics & numerical data , Hemoglobins/analysis , Mothers , Weight Gain , Linear Models , Cross-Sectional Studies , Age Factors , Age Distribution , Ferritins/bloodSubject(s)
Humans , Infant, Newborn , Anemia, Sickle Cell , Hemoglobinopathies , Neonatal Screening , Osteomyelitis , Oxygen Inhalation TherapyABSTRACT
OBJECTIVE: To evaluate the frequency of infectious complications in children with sickle cell disease (SCD) after surgical splenectomy for acute splenic sequestration crisis. METHODS: Retrospective cohort of children with SCD who were born after 2002 and were regularly monitored until July 2013. Patients were divided into two groups: cases (children with SCD who underwent surgical splenectomy after an episode of splenic sequestration) and controls (children with SCD who did not have splenic sequestration and surgical procedures), in order to compare the frequency of invasive infections (sepsis, meningitis, bacteremia with positive blood cultures, acute chest syndrome and/or pneumonia) by data collected from medical records. Data were analyzed by descriptive statistical analysis. RESULTS: 44 patients were included in the case group. The mean age at the time of splenectomy was 2.6 years (1-6.9 years) and the mean postoperative length of follow-up was 6.1 years (3.8-9.9 years). The control group consisted of 69 patients with a mean age at the initial follow-up visit of 5.6 months (1-49 months) and a mean length of follow-up of 7.2 years (4-10.3 years).All children received pneumococcal conjugate vaccine. No significant difference was observed between groups in relation to infections during the follow-up. CONCLUSIONS: Surgical splenectomy in children with sickle cell disease that had splenic sequestration did not affect the frequency of infectious complications during 6 years of clinical follow-up.
OBJETIVO: Avaliar a frequência de complicações infecciosas em pacientes portadores de doença falciforme (DF) submetidos à esplenectomia cirúrgica, após episódio de sequestro esplênico (SE). MÉTODOS: Coorte retrospectiva de crianças com DF que nasceram após 2002 e que estavam em acompanhamento regular até julho de 2013. Os pacientes foram divididos em dois grupos, casos (constituído pelas crianças com DF que fizeram esplenectomia cirúrgica após sequestro esplênico) e controles (crianças com DF que não tiveram SE e não foram submetidas ao procedimento), a fim de comparar a frequência de infecções invasivas (sepse, meningite, bacteremia com hemocultura positiva, síndrome torácica aguda e/ou pneumonia) por meio de informações obtidas do prontuário. A análise estatística foi descritiva. RESULTADOS: Foram avaliados 44 pacientes com idade média no momento da esplenectomia de 2,6 anos (1-6,9 anos) e com tempo médio de seguimento após esplenectomia de 6,1 anos (3,8-9,9 anos). O grupo controle foi formado por 69 pacientes com idade média do início do seguimento de 5,6 meses (1-49 meses) e tempo de acompanhamento médio de 7,2 anos (4-10,3 anos). Todos receberam a vacina pneumocócica conjugada. Não foi observada diferença significativa entre os grupos em relação aos processos infecciosos durante o período de seguimento. CONCLUSÕES: A esplenectomia cirúrgica nas crianças com doença falciforme e que sofreram sequestro esplênico não se associou ao aumento na frequência de complicações infecciosas após seis anos de acompanhamento clínico.
Subject(s)
Humans , Infant , Child, Preschool , Child , Anemia, Sickle Cell , Splenectomy , InfectionsABSTRACT
Objectives: To analyze the frequency of βS-globin haplotypes and alpha-thalassemia, and their influence on clinical manifestations and the hematological profile of children with sickle cell anemia. Method: The frequency of βS-globin haplotypes and alpha-thalassemia and any association with clinical and laboratorial manifestations were determined in 117 sickle cell anemia children aged 371 months. The confirmation of hemoglobin SS and determination of the haplotypes were achieved by polymerase chain reaction-restriction fragment length polymorphism, and alpha-thalassemia genotyping was by multiplex polymerase chain reaction (single-tube multiplex-polymerase chain reaction). Results: The genotype distribution of haplotypes was 43 (36.7%) Central African Republic/Benin, 41 (35.0%) Central African Republic/Central African Republic, 20 (17.0%) Rare/atypical, and 13 (11.1%) Benin/Benin. The frequency of the α3.7 deletion was 1.71% as homozygous (−α3.7/−α3.7) and 11.9% as heterozygous (−α3.7/αα). The only significant association in respect to haplotypes was related to the mean corpuscular volume. The presence of alpha-thalassemia was significantly associated to decreases in mean corpuscular volume, mean corpuscular hemoglobin and reticulocyte count and to an increase in the red blood cell count. There were no significant associations of βS-globin haplotypes and alpha-thalassemia with clinical manifestations. Conclusions: In the study population, the frequency of alpha-thalassemia was similar to published data in Brazil with the Central African Republic haplotype being the most common, followed by the Benin haplotype. βS-globin haplotypes and interaction between alpha-thalassemia and sickle cell anemia did not influence fetal hemoglobin concentrations or the number of clinical manifestations...
Subject(s)
Humans , Child , alpha-Thalassemia , Anemia, Sickle Cell , Beta-Globulins , Child , HaplotypesABSTRACT
Objective The objective was to determine the prevalence of iron deficiency and iron deficiency anemia among exclusively breastfed infants from one to six months of life and to identify associated risk factors. Methods This is a cohort study of the hemoglobin and serum ferritin levels of 102 healthy full-term infants, weighing more than 2500 grams (5.5 pounds) at birth, evaluated for growth development and supported to promote exclusive breastfeeding. Hemoglobin and ferritin levels were measured in the first, fourth, and sixth months of life. The hemoglobin and ferritin levels of the mothers were also measured in the first month postpartum. Results At four months, 5.7% presented iron deficiency and 3.4% had iron deficiency anemia. At six months, the percentage of children with iron deficiency increased more than four times, reaching 26.1%, while iron deficiency anemia was present in 23.9% of the infants studied. Iron deficiency at six months of age was significantly correlated to growth velocity. Conclusion According to the results of this study, exclusive breastfeeding protects infants from iron deficiency and iron deficiency anemia for the first four months of life. After this age, in accordance with the literature, the findings of this study demonstrated an increase in anemia and iron deficiency rates, adding to evidence that supports the monitoring of iron levels in exclusively breastfed children presenting higher weight gains beginning at four months of age. .
Objetivo Determinar a prevalência da deficiência de ferro e anemia ferropriva em crianças, de um aos seis meses de vida, alimentadas exclusivamente ao seio materno e identificar os fatores de risco associados. Métodos Estudo de coorte dos valores de hemoglobina e ferritina sérica de 102 lactentes a termo, saudáveis, nascidos com peso maior que 2500 g, acompanhados quanto ao crescimento e desenvolvimento e com apoio à promoção do aleitamento materno exclusivo. Hemoglobina e ferritina sérica foram dosadas no primeiro, quarto e sexto mês de vida. Hemoglobina e ferritina sérica também foram dosadas nas mães no primeiro mês pós-parto. Resultados Aos quatro meses, 5,7% dos lactentes apresentaram deficiência de ferro e 3,4%, anemia ferropriva. Aos seis meses, o percentual de crianças com deficiência de ferro aumentou mais de quatro vezes, atingindo a proporção de 26,1%, enquanto a anemia ferropriva esteve presente em 23,9% dos lactentes da amostra. A deficiência de ferro aos seis meses foi associada significativamente com maior ganho ponderal. Conclusão Neste estudo, o aleitamento materno exclusivo protegeu as crianças da deficiência de ferro e da anemia ferropriva nos primeiros quatro meses de vida. Após essa idade, concordando com dados da literatura, esse estudou mostrou aumento na taxa de anemia e da deficiência de ferro, o que fornece mais uma evidência para apoiar a vigilância do estado do ferro, a partir dos quatro meses, para crianças em aleitamento materno exclusivo e que apresentem ganho de peso acima da média. .
Subject(s)
Female , Humans , Infant , Male , Anemia, Iron-Deficiency/epidemiology , Breast Feeding/statistics & numerical data , Iron/deficiency , Age Factors , Anemia, Iron-Deficiency/blood , Brazil/epidemiology , Child Development , Cohort Studies , Ferritins/blood , Hemoglobins/analysis , Iron/blood , Prevalence , Risk Factors , Weight GainABSTRACT
OBJETIVO: avaliar a capacidade funcional pulmonar (CF) para o exercício físico de crianças e adolescentes com doença falciforme (DF) pelo teste da caminhada de seis minutos (TC6'). MÉTODOS: estudo transversal prospectivo avaliando a CF pelo TC6' de 46 pacientes com DF. Foram avaliados: frequência cardíaca (FC), frequência respiratória (FR), saturação de pulso de oxigênio (SpO2), pico de fluxo expiratório (PFE), pressão arterial (PA) sistólica e diastólica, dispneia e cansaço em membros inferiores (escala de Borg modificada) em repouso, ao término e 10 minutos após o TC6' e a distância percorrida. Análise estatística: test t-Student pareado, análise de variância e comparações múltiplas de Bonferroni, significância p < 0,05. RESULTADOS: dos 46 pacientes, a média ± dp da idade foi 9,15 ± 3,06 anos, hemoglobina basal 9,4 ± 1,67 g/dL e distância percorrida 480,89 ± 68,70 m. Diagnóstico da DF: Grupo 1- HbSS (n = 20)/HbSβ0-talassemia (n = 3); e Grupo 2 - HbSC (n = 20)/ HbSβ+-talassemia (n = 3). O Grupo 1 apresentou menor distância percorrida do que o Grupo 2 (459,39 ± 57,19 vs 502,39 ± 73,60m; p = 0,032). Não houve diferença estatística em relação ao PFE. A SpO2 em ar ambiente e a SpO2 com O2 (1 L/min) após o teste foi maior no Grupo 2 (p < 0,001 e p = 0,002, respectivamente). A FR foi maior no Grupo 1 ao final do TC6' (p < 0,001). CONCLUSÃO: esta amostra apresentou CF para o exercício abaixo do predito para a faixa etária na literatura. Os pacientes com HbSS/Sβ0-talassemia apresentaram desempenho inferior na distância percorrida, FR e SpO2 após o TC6', comparativamente aos pacientes com HbSC/Sβ+-talassemia.
OBJECTIVE: to evaluate lung functional capacity (FC) for physical exercise in children and adolescents with sickle cell disease (SCD) through the six-minute walk test (6MWT). METHOD: a cross-sectional prospective study was performed to evaluate the FC of 46 patients with SCD through the 6MWT. The following parameters were assessed: heart rate (HR), respiratory rate (RR), peripheral pulse oxygen saturation (SpO2), peak expiratory flow (PEF), blood pressure (systolic and diastolic), dyspnea, and leg fatigue (modified Borg scale) at rest, in the end of the test, and ten minutes after the 6MWT. The total distance walked was also recorded. For statistical analysis, the parametric variables were analyzed using the paired Student's t-test, analysis of variance (ANOVA), and Bonferroni multiple comparisons, with a significance level set at p < 0.05. RESULTS: the 46 patients were aged age 9.15 ± 3.06 years, presented baseline Hb of 9.49 ± 1.67 g/dL, and walked 480.89 ± 68.70 m. SCD diagnosis was as follows: group 1- HbSS (n = 20)/HbSβ0-thalassemia (n = 3) and group 2 - HbSC (n = 20)/HbSβ+-thalassemia (n = 3). Regarding total distance walked, patients in group 1 walked a shorter distance than patients in group 2 (459.39 ± 57.19 vs. 502.39 ± 73.60 m; p = 0.032). There was no statistical difference regarding PEF in the three moments of evaluation. The SpO2 in ambient air and SpO2 with O2 differed between groups 1 and 2 (p < 0.001 vs. p = 0.002), as well as the RR (p = 0.001). CONCLUSION: these patients showed a lower FC for exercise than that predicted for the age range in the literature. Patients diagnosed with HbSS/Sβ0-thalassemia had a lower performance in the test than those with HbSC/Sβ+-thalassemia regarding total distance walked, RR, and SpO2 after the 6MWT.
Subject(s)
Adolescent , Child , Child, Preschool , Female , Humans , Male , Anemia, Sickle Cell/physiopathology , Exercise Test , Exercise/physiology , Walking/physiology , Analysis of Variance , Cross-Sectional Studies , Hyperbaric Oxygenation , Nutritional Status , Respiratory Function Tests , Task Performance and Analysis , Work Capacity EvaluationABSTRACT
OBJECTIVES To assess the food intake pattern and the nutritional status of children with cerebral palsy. METHODS Cross-sectional study with 90 children from two to 12.8 years with cerebral palsy in the following forms: hemiplegia, diplegia, and tetraplegia. Nutritional status was assessed by weight, height, and age data. Food intake was verified by the 24-hour recall and food frequency questionnaire. The ability to chew and/or swallowing, intestinal habits, and physical activity were also evaluated. RESULTS For 2-3 year-old age group, the mean energy intake followed the recommended range; in 4-6 year-old age group with hemiplegia and tetraplegia, energy intake was below the recommended limits. All children presented low intake of carbohydrates, adequate intake of proteins and high intake of lipids. The tetraplegia group had a higher prevalence of chewing (41%) and swallowing (12.8%) difficulties compared to 14.5 and 6.6% of children with hemiplegia, respectively. Most children of all groups had a daily intestinal habit. All children presented mild physical activity, while moderate activity was not practiced by any child of the tetraplegia group, which had a significantly lower height/age Z score than those with hemiplegia (-2.14 versus -1.05; p=0.003). CONCLUSIONS The children with cerebral palsy presented inadequate dietary pattern and impaired nutritional status, with special compromise of height. Tetraplegia imposes difficulties regarding chewing/swallowing and moderate physical activity practice. .
OBJETIVO Evaluar el estándar alimentar y estado nutricional de niños con parálisis cerebral. MÉTODOS Estudio transversal con 90 niños de 2 a 12,8 años de edad, con parálisis cerebral de tipo hemiplejía, diplejía y tetraplejía. Se evaluaron el estado nutricional por medio de los datos de peso, altura y edad, el consumo alimentar por el Recordatorio de 24 horas y por el Cuestionario de Frecuencia Alimentar, la capacidad de masticar y/o deglutir, el hábito intestinal y la práctica de actividad física. RESULTADOS En el grupo de 2 a 3 años, el promedio de ingestión energética estaba conforme a la recomendación; en la franja de 4 a 6 años, los grupos con hemiplejía y con tetraplejía presentaban promedios inferiores al límite inferior de recomendación. El grupo como un todo presentó estándar dietético bajo en carbohidratos, adecuado en proteínas y alto en lípidos. El grupo con tetraplejía presentó mayor prevalencia de dificultad para masticación (41%) y deglución (12,8%), versus, respectivamente, 14,5 y 6,6% de los niños con hemiplejía. Se observó que la mayoría de los niños con cada tipo de parálisis cerebral presentaba hábito intestinal diario. Todos los niños estudiados tenían actividad física liviana, mientras que la actividad moderada no era practicada por ningún niño del grupo tetraplejía, que también presentó escore Z de -2,14 de la relación estatura/edad, significantemente menor respecto al grupo con hemiplejía (escore Z de -1,05; p=0,003). CONCLUSIONES Los niños presentaron estándar alimentar inadecuado, estado nutricional comprometido, principalmente la estatura. La tetraplejía impone dificultades de masticación/deglución y práctica de actividad física ...
OBJETIVO Avaliar o padrão alimentar e o estado nutricional de crianças com paralisia cerebral. MÉTODOS Estudo transversal com 90 crianças de dois a 12,8 anos de idade, com paralisia cerebral do tipo hemiplegia, diplegia e tetraplegia. Avaliaram-se o estado nutricional por meio dos dados de peso, altura e idade, o consumo alimentar pelo Recordatório de 24 horas e pelo Questionário de Frequência Alimentar, a capacidade de mastigar e/ou deglutir, o hábito intestinal e a prática de atividade física. RESULTADOS No grupo de dois a três anos, a média de ingestão energética estava de acordo com a recomendação; na faixa de quatro a seis anos, os grupos com hemiplegia e com tetraplegia apresentaram médias abaixo do limite inferior da recomendação. O grupo como um todo apresentou padrão dietético baixo em carboidratos, adequado em proteína e alto em lipídios. O grupo com tetraplegia apresentou maior prevalência de dificuldade para mastigação (41%) e para deglutição (12,8%) versus, respectivamente, 14,5 e 6,6% das crianças com hemiplegia. Observou-se que a maioria das crianças com cada tipo de paralisia cerebral apresentava hábito intestinal diário. Todas as crianças estudadas tinham atividade física leve, enquanto a atividade moderada não era praticada por nenhuma criança do grupo com tetraplegia, que também apresentou escore Z de -2,14 da relação estatura/idade, significantemente menor em relação ao grupo com hemiplegia (escore Z de -1,05; p=0,003). CONCLUSÕES As crianças apresentaram padrão alimentar inadequado, estado nutricional comprometido, o que afetou principalmente a estatura. A tetraplegia impõe dificuldade de mastigação/deglutição e da prática de atividade física moderada. .