Índice de massa corporal, nutrição e atividade física em crianças e adolescentes com síndrome de Down / Body-mass index, nutrition and physical activity in children and adolescents with Down syndrome

Objetivo: Descrever e comparar hábito nutricional, prática de atividade física e índice de massa corporal (IMC) de crianças e adolescentes com síndrome de Down acompanhados em ambulatório especializado de um hospital terciário no sul do Brasil. Método: Estudo transversal realizado a partir da análise de prontuário de pacientes com síndrome de Down em idade escolar e adolescentes acompanhados em ambulatório especializado do Complexo Hospital de Clínicas da Universidade Federal do Paraná. A alimentação foi considerada adequada se consistisse em alimentos de todos os grupos nas três refeições principais e um ou dois lanches e água nos intervalos. Sedentarismo foi definido como tempo de atividade física inferior a 300 minutos por semana. O estado nutricional foi avaliado usando as curvas de IMC da Organização Mundial da Saúde, 2007.Resultados: O estudo incluiu 755 pacientes, sendo 236 (31,3%) crianças e 519 (68,7%) adolescentes. Sobrepeso e obesidade foram observados em 10,7% e 14,8% da população, respectivamente, sem diferença significativa entre os gêneros. Alimentação inadequada foi observada em 34,6% e sedentarismo em 23,7% dos pacientes. IMC elevado foi observado em 20,3% das crianças e 27,9% dos adolescentes (p=0,026). Observou-se sedentarismo em 29,5% dos adolescentes e 11% das crianças (p<0,001). Alimentação inadequada também foi mais prevalente em adolescentes, porém sem diferença estatística. Pacientes com IMC elevado, em comparação com eutróficos, tiveram maior prevalência de alimentação inadequada e sedentarismo, com significância estatística. Nesse subgrupo, o sedentarismo foi observado em 25% das crianças e 57,2% dos adolescentes (p<0,001). Conclusão: Adolescentes com síndrome de Down apresentam maiores taxas de IMC elevado e sedentarismo comparados com crianças. Estudos específicos em educação em saúde para essa população são necessários com o objetivo de promover hábitos de vida saudáveis e prevenir a obesidade com efetividade. [au]

Objective: To describe and compare the nutritional habits, physical activity, and body mass index (BMI) of children and adolescents with Down syndrome followed up in the specialized outpatient clinic of a tertiary hospital in southern Brazil. Method: Cross-sectional study conducted from the analysis of medical records of patients with Down syndrome at school age and adolescents followed up at the Complexo Hospital de Clínicas of the Universidade Federal do Paraná. The diet was considered adequate if it consisted of foods from all groups in the three main meals and one or two snacks and water in the intervals. A sedentary lifestyle was defined as physical activity time of fewer than 300 minutes per week. Nutritional status was assessed using BMI curves from the World Health Organization, 2007. Results: The study included 755 patients, of whom 236 (31.3%) were children and 519 (68.7%) were adolescents. Overweight and obesity were observed in 10.7% and 14.8% of the population, respectively, without significant gender differences. Inadequate diet was observed in 34.6% and sedentarism in 23.7% of the patients. High BMI was observed in 20.3% of children and 27.9% of adolescents (p=0.026). Sedentarism was observed in 29.5% of adolescents and 11% of children (p<0.001). Inadequate diet was also more prevalent in adolescents but without statistical difference. Patients with high BMI, compared to eutrophic patients, had a higher prevalence of inadequate diet and sedentarism, with statistical significance. In this subgroup, sedentarism was observed in 25% of children and 57.2% of adolescents (p<0.001). Conclusion: Adolescents with Down syndrome have higher rates of high BMI and sedentarism compared to children. Specific studies in health education for this population are necessary to promote healthy lifestyle habits and effectively prevent obesity. [au]

Respiratory and otolaryngological disorders in Down syndrome from one center in Brazil.

Down syndrome (DS) is the most common chromosomal condition. Anatomical and functional variations in the upper and lower airways are component manifestations of the syndrome and increase the risk of various medical problems. The objective of this study was to determine the prevalence of otorhinolaryngological and respiratory diseases in a DS outpatient clinic over a 3-year period. Medical records data from 1207 patients were retrospectively reviewed. Newborn Hearing Screening was positive in 7.1% of patients. Brainstem auditory evoked potential was performed in 1101 children and showed a hearing loss of 19.8% in the first year. It was positive in 21% of 1021 exams. Audiometry was altered in 64 of 994 exams (6.4%), showing a conductive loss in 90%. Adenotonsillectomy was performed in 308 (25.5%) patients, and 169 (14.0%) required serous otitis ventilation tubes. Asthma was observed in 140 (11.6%) patients, and allergic rhinitis in 544 (56.6%). There were hospitalizations for invasive infection in 480 (39.8%) children, and two (0.2%) patients had severe septicemia from pulmonary focus. Five (0.4%) infants had laryngotracheomalacia, and one patient had anomalous right tracheal bronchus. Recognizing the prevalence of respiratory and otorhinolaryngological disorders in patients with DS allows the promotion of optimal follow-up and early treatment, preventing the development of sequelae.

Ophthalmological abnormalities in Down syndrome among Brazilian patients.

Down syndrome is the most common chromosomal disorder, affecting 1/700 live births. Among the clinical findings, one constant concern is the high prevalence of visual disorders that, if left untreated, can negatively affect child development. The aim of this study was to determine the prevalence of ophthalmological findings among patients who attended an outpatient clinic for patients with Down syndrome in southern Brazil between 2005 and 2016. A cross-sectional study including 1,207 patients medical records were done, which 492 (40.8%) had some ophthalmological disorder. These data were subjected to descriptive analysis using Statistica software. Among the 492 patients with any ophthalmological disease, the need for glasses was found in 434 (36%) patients, keratoconus in 254 (42.1%), congenital cataract in 27 (15.1%), nasolacrimal duct obstruction in 25 (2.0%), strabismus in 22 (1.9%), nystagmus in four (0.3%), and juvenile cataract in two (0.2%). Two young adults with keratoconus underwent corneal transplantation. Although the prevalence of an ophthalmological disease among the present sample (40.8%) was lower than described in the current literature, it still reinforced the importance of routine and early evaluations in infants. These should begin at 6 months of age and be repeated half-year until 2 years old, annually until 7 years old, biennial in adolescents, and triennial in adults and elderly. Our findings of a high frequency of keratoconus support a detailed corneal study in such patients for early detection and treatment.

Zika Virus Infection at Different Pregnancy Stages: Anatomopathological Findings, Target Cells and Viral Persistence in Placental Tissues.

Zika virus (ZIKV) infection in humans has been associated with congenital malformations and other neurological disorders, such as Guillain-Barré syndrome. The mechanism(s) of ZIKV intrauterine transmission, the cell types involved, the most vulnerable period of pregnancy for severe outcomes from infection and other physiopathological aspects are not completely elucidated. In this study, we analyzed placental samples obtained at the time of delivery from a group of 24 women diagnosed with ZIKV infection during the first, second or third trimesters of pregnancy. Villous immaturity was the main histological finding in the placental tissues, although placentas without alterations were also frequently observed. Significant enhancement of the number of syncytial sprouts was observed in the placentas of women infected during the third trimester, indicating the development of placental abnormalities after ZIKV infection. Hyperplasia of Hofbauer cells (HCs) was also observed in these third-trimester placental tissues, and remarkably, HCs were the only ZIKV-positive fetal cells found in the placentas studied that persisted until birth, as revealed by immunohistochemical (IHC) analysis. Thirty-three percent of women infected during pregnancy delivered infants with congenital abnormalities, although no pattern correlating the gestational stage at infection, the IHC positivity of HCs in placental tissues and the presence of congenital malformations at birth was observed. Placental tissue analysis enabled us to confirm maternal ZIKV infection in cases where serum from the acute infection phase was not available, which reinforces the importance of this technique in identifying possible causal factors of birth defects. The results we observed in the samples from naturally infected pregnant women may contribute to the understanding of some aspects of the pathophysiology of ZIKV.

Down syndrome: Prevalence and distribution of congenital heart disease in Brazil.

CONTEXT AND OBJECTIVE: Down syndrome is the most common genetic disorder, affecting 1/700 live births. Among the clinical findings, one constant concern is the high prevalence of congenital heart disease. The objective of this study was to determine the prevalence and profile of congenital heart disease among patients attended at a Down syndrome outpatient clinic in southern Brazil between 2005 and 2013. DESIGN AND SETTING: Cross-sectional study conducted in a referral center. METHODS: Data were retrospectively gathered from the medical files of 1,207 patients with Down syndrome, among whom 604 (50.0%) had been diagnosed with congenital heart disease. These data were subjected to descriptive analysis using the Statistica software. RESULTS: Among the 604 patients with congenital heart disease, 338 (55.8%) were male and 269 (44.5%) were female. The most common heart diseases were atrial septal defect in 254 patients (42.1%); total atrioventricular septal defect in 91 (15.1%); atrial septal defect and ventricular septal defect in 88 (14.6%); ventricular septal defect in 77 (12.7%); patent ductus arteriosus in 40 (6.6%); patent foramen ovale in 34 (5.6%) patients; tetralogy of Fallot in 12 (2%); and other diseases in 8 (1.3%). Pulmonary hypertension was present in 57 (9.4%). Out of the total, 150 patients (24.8%) underwent cardiac surgery. CONCLUSION: The high prevalence of congenital heart disease among the patients at the Down syndrome outpatient clinic (50%) was similar to findings from other studies and justifies investigation during the neonatal period, so as to decrease mortality and morbidity.

Down syndrome: Prevalence and distribution of congenital heart disease in Brazil / Síndrome de Down: prevalência e distribuição de cardiopatia congênita no Brasil

ABSTRACT CONTEXT AND OBJECTIVE: Down syndrome is the most common genetic disorder, affecting 1/700 live births. Among the clinical findings, one constant concern is the high prevalence of congenital heart disease. The objective of this study was to determine the prevalence and profile of congenital heart disease among patients attended at a Down syndrome outpatient clinic in southern Brazil between 2005 and 2013. DESIGN AND SETTING : Cross-sectional study conducted in a referral center. METHODS : Data were retrospectively gathered from the medical files of 1,207 patients with Down syndrome, among whom 604 (50.0%) had been diagnosed with congenital heart disease. These data were subjected to descriptive analysis using the Statistica software. RESULTS : Among the 604 patients with congenital heart disease, 338 (55.8%) were male and 269 (44.5%) were female. The most common heart diseases were atrial septal defect in 254 patients (42.1%); total atrioventricular septal defect in 91 (15.1%); atrial septal defect and ventricular septal defect in 88 (14.6%); ventricular septal defect in 77 (12.7%); patent ductus arteriosus in 40 (6.6%); patent foramen ovale in 34 (5.6%) patients; tetralogy of Fallot in 12 (2%); and other diseases in 8 (1.3%). Pulmonary hypertension was present in 57 (9.4%). Out of the total, 150 patients (24.8%) underwent cardiac surgery. CONCLUSION : The high prevalence of congenital heart disease among the patients at the Down syndrome outpatient clinic (50%) was similar to findings from other studies and justifies investigation during the neonatal period, so as to decrease mortality and morbidity.

RESUMO CONTEXTO E OBJETIVO : A síndrome de Down é a anomalia genética mais comum e afeta 1/700 nascidos vivos. Entre os achados clínicos, uma preocupação constante é a alta prevalência de cardiopatia congênita e o objetivo do presente estudo é determinar a prevalência e o perfil de cardiopatia congênita em pacientes atendidos em um ambulatório de síndrome de Down no período de 2005 a 2013 no sul do Brasil. TIPO DE ESTUDO E LOCAL : Estudo transversal conduzido em um centro de referência. MÉTODOS : Foi realizada coleta retrospectiva de dados de 1.207 prontuários de pacientes com síndrome de Down, com 604 (50,0%) diagnosticados com cardiopatia congênita. Os dados foram submetidos a análise descritiva, utilizando-se o programa Statistica. RESULTADOS : Entre os 604 pacientes com cardiopatia congênita, 338 (55,8%) eram do sexo masculino e 269 (44,5%) do feminino. A cardiopatia mais comum foi comunicação interatrial, observada em 254 (42,1%) pacientes, defeito de septo atrioventricular total, em 91 (15,1%), comunicação interatrial em conjunto com comunicação interventricular, em 88 (14,6%), comunicação interventricular, em 77 (12,7%), persistência do canal arterial, em 40 (6,6%), forame oval patente, em 34 (5,6%), tetralogia de Fallot, em 12 (2%) e outras cardiopatias observadas em oito (1,3%) pacientes. Hipertensão pulmonar esteve presente em 57 (9,4%). Do total, 150 (24,8%) pacientes foram submetidos a cirurgia cardíaca. CONCLUSÃO : A elevada prevalência das cardiopatias congênitas nos pacientes do ambulatório da síndrome de Down, de 50%, semelhante à encontrada em outros estudos, justifica a investigação no período neonatal, com diminuição da mortalidade e morbidade.

Anafilaxia associada à vacina contra sarampo, caxumba e rubéola / Anaphylaxis associated with the vaccine against measles, mumps and rubella

Estudo do tipo caso-controle cujo objetivo foi descrever os casos e as causas de anafilaxia associada à vacina contra sarampo, caxumba e rubéola. Foram estudados 22 casos notificados em crianças de Curitiba, PR, durante Campanha Nacional de Vacinação de 2004, que apresentaram manifestações mucocutâneas. Para o controle, foram selecionadas 66 crianças vizinhas dos casos, que não apresentaram sintomatologia após a aplicação vacinal. Foram realizadas dosagens séricas de anticorpos para os antígenos vacinais, IgE total e específicas para diversos alérgenos, e testes cutâneos. A resposta vacinal foi adequada, a dosagem de IgE específica e os testes cutâneos mostraram que os alérgenos potenciais constituintes da vacina e a atopia não foram relacionados às reações anafiláticas. Os testes cutâneos com a vacina e o dextrano foram positivos somente nos casos, sugerindo sensibilização por algum componente residual da vacina e possível reação cruzada com o dextrano.

Anaphylaxis associated with the vaccine against measles, mumps and rubella.

A case-control study was carried out aiming to describe the cases and causes of anaphylaxis associated with the vaccine against measles, mumps and rubella. A total of 22 reported cases in children who showed mucocutaneous manifestations, during the Campanha Nacional de Vacinação (Brazilian Vaccination Campaign), conducted in the city of Curitiba, Southern Brazil, in 2004, were studied. In addition, 66 children, who were next to these cases and did not show a symptomatology after the vaccine was applied, were selected. Serum measurements of antibodies for vaccine antigens and total IgE, specific IgE antibody measurements for several allergens, and skin tests were performed. Vaccine response was adequate, specific IgE measurement and skin tests showed that potential allergens in vaccines and atopy were not associated with anaphylactic reactions. Skin tests with the vaccine and dextran were positive in the cases exclusively, suggesting sensitization to certain residual components of the vaccine and possible cross-reaction with dextran.

Reações adversas à gelatina em imunobiológicos / Adverse reactions to gelatin-containing immunobiologic products

Objetivo: O objetivo desta revisão é despertar a atenção para reações à gelatina, um componente comum a alguns produtos farmacêuticos e vacinas, justificado por recente surto de reação durante campanha nacional de vacinação com a vacina tríplice viral. Fonte: O presente estudo foi realizado pela pesquisa de artigos disponíveis no banco de dados da Medline, Embase, Biosis, SciSearch e Chemical Abstract (1960 até 2005). Síntese: Entre os componentes de vacinas que causam reação anafilática estão a clara de ovo, a gelatina e os antibióticos. A gelatina adicionada como estabilizante pode estimular linfócitos a resposta tipo Th1 e Th2 e determinar reações do tipo imediatas e não imediatas. A sensibilização prévia ocorre pela exposição à gelatina contida em medicamentos, vacinas ou alimentos. Reações são mais freqüentes em orientais e possivelmente o risco de produzir IgE anti-gelatina está associado ao HLA-DR9. É apresentado um diagrama de avaliação de pacientes com reação prévia à vacina contendo gelatina. Conclusão: Os riscos de não vacinação superam os riscos de reações alérgicas à vacina, mas em todos os locais onde são administrados imunobiológicos deve haver recursos para tratamento de reações anafiláticas.