FACTORS ASSOCIATED TO QUALITY OF LIFE IN CHILDREN AND ADOLESCENTS WITH CYSTIC FIBROSIS.

OBJECTIVE: To verify the association between quality of life, functional capacity and clinical and nutritional status in children and adolescents with cystic fibrosis (CF). METHODS: Cross-sectional study, including patients from eight to 18 years old with CF. Quality of life, functional capacity, nutritional status and clinical status were evaluated with the Cystic Fibrosis Questionnaire; the 6-minute walk test (6MWT) and manual gripping force (MGF); the height percentiles for age and body mass index for age and respiratory function test, respectively. Pearson and Spearman correlation tests and logistic regression were used to analyze the data. RESULTS: A total of 45 patients, 13.4±0.5 years old, 60% female, 60% colonized by Pseudomonas aeruginosa and 57.8% with at least one F508del mutation participated in the study. When assessing the perception of quality of life, the weight domain reached the lowest values, and the digestive domain, the highest. In the pulmonary function test, the forced expiratory volume of the first second was 77.3±3.3% and the 6MWT and MGF presented values within the normal range. There was an association between quality of life and functional capacity, nutritional status and clinical status of CF patients. CONCLUSIONS: The study participants had good clinical conditions and satisfactory values of functional capacity and quality of life. The findings reinforce that the assessment of quality of life may be important for clinical practice in the management of treatment.

Factors associated to quality of life in children and adolescents with cystic fibrosis / Fatores associados à qualidade de vida em crianças e adolescentes com fibrose cística

ABSTRACT Objective: To verify the association between quality of life, functional capacity and clinical and nutritional status in children and adolescents with cystic fibrosis (CF). Methods: Cross-sectional study, including patients from eight to 18 years old with CF. Quality of life, functional capacity, nutritional status and clinical status were evaluated with the Cystic Fibrosis Questionnaire; the 6-minute walk test (6MWT) and manual gripping force (MGF); the height percentiles for age and body mass index for age and respiratory function test, respectively. Pearson and Spearman correlation tests and logistic regression were used to analyze the data. Results: A total of 45 patients, 13.4±0.5 years old, 60% female, 60% colonized by Pseudomonas aeruginosa and 57.8% with at least one F508del mutation participated in the study. When assessing the perception of quality of life, the weight domain reached the lowest values, and the digestive domain, the highest. In the pulmonary function test, the forced expiratory volume of the first second was 77.3±3.3% and the 6MWT and MGF presented values within the normal range. There was an association between quality of life and functional capacity, nutritional status and clinical status of CF patients. Conclusions: The study participants had good clinical conditions and satisfactory values of functional capacity and quality of life. The findings reinforce that the assessment of quality of life may be important for clinical practice in the management of treatment.

RESUMO Objetivo: Verificar associação entre qualidade de vida (QV), capacidade funcional e estados clínico e nutricional em crianças e adolescentes com fibrose cística (FC). Métodos: Estudo transversal incluindo pacientes de oito a 18 anos de idade com FC. A QV, a capacidade funcional, o estado nutricional e o estado clínico foram avaliados por meio do Questionário de Fibrose Cística; do teste de caminhada dos 6 minutos (TC6M) e da força de preensão manual (FPM); dos percentis de estatura para a idade e do índice de massa corporal (IMC) para a idade; e da prova de função respiratória, respectivamente. Para a análise dos dados, utilizaram-se os testes de correlação de Pearson e de Spearman e a regressão logística. Resultados: Participaram do estudo 45 pacientes com 13,4±0,5 anos, sendo 60% do sexo feminino, 60% colonizados por Pseudomonas aeruginosa e 57,8% apresentando pelo menos uma mutação F508del. Ao avaliar a percepção da QV, o domínio peso alcançou os escores mais baixos e o digestório, os mais altos. Na prova de função pulmonar, o volume expiratório forçado do primeiro segundo médio foi 77,3±3,3%, e o TC6M e a FPM apresentaram valores na faixa de normalidade. Observou-se associação da QV com a capacidade funcional, o estado nutricional e o estado clínico dos pacientes com FC. Conclusões: Os participantes do estudo apresentaram boas condições clínicas e valores satisfatórios de capacidade funcional e QV. Os achados reforçam que a avaliação da QV pode ser importante para a prática clínica, no manejo do tratamento.

Adesão de adolescentes com fibrose cística a terapia de reposição enzimática: fatores associados / Adherence of adolescents with cystic fibrosis to enzyme replacement therapy: associated factors

Resumo O objetivo deste artigo é avaliar a prevalência e os fatores associados à adesão a terapia de reposição enzimática em adolescentes com Fibrose cística. Estudo transversal, descritivo e observacional. Foram coletados dados sociodemográficos e clínicos. Os instrumentos utilizados para avaliar adesão foram: questionário de Morisky-Green e a dispensação de medicação na farmácia e para os fatores associados, entrevista com questionário estruturado. Foram entrevistados 44 adolescentes. Segundo o método de análise de dispensação da farmácia e o questionário de Morisky-Green, encontramos uma adesão de 45,5% e 11,4%, respectivamente. A maior adesão foi observada naqueles com diagnóstico precoce e a menor nos adolescentes mais velhos e nas meninas. Os fatores com a maior prevalência de não adesão foram: não levar as enzimas ao comer fora de casa, tomar enzimas somente nas grandes refeições, função pulmonar normal, com obstrução grave e muito grave. A prevalência de adesão a enzima foi pequena. Informações relacionadas à doença e ao tratamento devem ser aprimoradas, principalmente em adolescentes mais velhos e com comprometimento da função pulmonar, com criação de estratégias e estudos longitudinais para identificar fatores que interferem na adesão.

Abstract This article sets out to evaluate the prevalence and factors associated with adherence to enzyme replacement therapy among adolescents with cystic fibrosis. It is a cross-sectional, descriptive and observational study. Sociodemographic and clinical data were collected. The instruments used to assess adherence were: the Morisky-Green questionnaire and medication dispensation at the pharmacy, and interviews with structured questionnaires for the associated factors. Forty-four adolescents were interviewed. According to the method of the pharmacy medication dispensation analysis and the Morisky-Green questionnaire, the adherence of 45.5% and 11.4% was found, respectively. The higher adherence was observed in those with early diagnosis and the lowest in older adolescents and girls. The factors with the highest prevalence of non-adherence were: not taking enzymes when eating out of the home; only taking enzymes with major meals; normal lung function; with severe and very severe obstruction. The prevalence of adhesion to enzymes was low. Information related to the disease and treatment should be improved, especially among older adolescents and with impairment of lung function, with the creation of strategies and longitudinal studies to identify factors that interfere with adherence.

[Adherence of adolescents with cystic fibrosis to enzyme replacement therapy: associated factors]. / Adesão de adolescentes com fibrose cística a terapia de reposição enzimática: fatores associados.

This article sets out to evaluate the prevalence and factors associated with adherence to enzyme replacement therapy among adolescents with cystic fibrosis. It is a cross-sectional, descriptive and observational study. Sociodemographic and clinical data were collected. The instruments used to assess adherence were: the Morisky-Green questionnaire and medication dispensation at the pharmacy, and interviews with structured questionnaires for the associated factors. Forty-four adolescents were interviewed. According to the method of the pharmacy medication dispensation analysis and the Morisky-Green questionnaire, the adherence of 45.5% and 11.4% was found, respectively. The higher adherence was observed in those with early diagnosis and the lowest in older adolescents and girls. The factors with the highest prevalence of non-adherence were: not taking enzymes when eating out of the home; only taking enzymes with major meals; normal lung function; with severe and very severe obstruction. The prevalence of adhesion to enzymes was low. Information related to the disease and treatment should be improved, especially among older adolescents and with impairment of lung function, with the creation of strategies and longitudinal studies to identify factors that interfere with adherence.

O objetivo deste artigo é avaliar a prevalência e os fatores associados à adesão a terapia de reposição enzimática em adolescentes com Fibrose cística. Estudo transversal, descritivo e observacional. Foram coletados dados sociodemográficos e clínicos. Os instrumentos utilizados para avaliar adesão foram: questionário de Morisky-Green e a dispensação de medicação na farmácia e para os fatores associados, entrevista com questionário estruturado. Foram entrevistados 44 adolescentes. Segundo o método de análise de dispensação da farmácia e o questionário de Morisky-Green, encontramos uma adesão de 45,5% e 11,4%, respectivamente. A maior adesão foi observada naqueles com diagnóstico precoce e a menor nos adolescentes mais velhos e nas meninas. Os fatores com a maior prevalência de não adesão foram: não levar as enzimas ao comer fora de casa, tomar enzimas somente nas grandes refeições, função pulmonar normal, com obstrução grave e muito grave. A prevalência de adesão a enzima foi pequena. Informações relacionadas à doença e ao tratamento devem ser aprimoradas, principalmente em adolescentes mais velhos e com comprometimento da função pulmonar, com criação de estratégias e estudos longitudinais para identificar fatores que interferem na adesão.

Fatty acid profile in erythrocytes associated with serum cytokines in pediatric cystic fibrosis patients / Perfil de ácidos graxos nos eritrócitos associados com citocinas séricas em pacientes pediátricos com Fibrose Cística

ABSTRACT Objective To analyze erythrocyte fatty acid composition and its association with serum cytokine levels in pediatric cystic fibrosis patients. Methods A cross-sectional study was performed at a reference center in Rio de Janeiro, Brazil. We have included all pediatric patients aged 5-19 years with confirmed cystic fibrosis diagnosis. Erythrocyte fatty acid composition and serum cytokine (TNF-α, IL-1β, IL-6 and IL-8) and C-reactive protein levels were measured. The cut-off point to determine essential fatty acids deficiency was the linoleic acid concentration of <21%. Results Twenty-six children (<10 years old) and thirty-one adolescents were studied. Most patients were female and heterozygous for DF508 mutation and suffered from exocrine pancreatic insufficiency. Both children and adolescents had lower linoleic acid concentration (<21%). TNF-α was the only pro-inflammatory marker whose levels were increased; the increase was greater in children. An association between fatty acid composition in erythrocytes and cytokines IL-1β and IL-6 was observed (p<0.05). Conclusion The pediatric cystic fibrosis patients studied presented a deficiency of essential fatty acids, and an association between fatty acid profile in erythrocytes and serum pro-inflammatory cytokines was observed. These findings highlight the importance of this type of assessment that may open new possibilities for studying pathophysiology and treating cystic fibrosis patients, such as the dietary supplementation with n-3 fatty acids (eicosapentaenoic and docosahexaenoic acids). However, further longitudinal studies are needed for better clarification of the imbalance in lipid metabolism and inflammation in cystic fibrosis

RESUMO Objetivo Analisar a composição dos ácidos graxos nos eritrócitos e sua associação com os níveis séricos de citocinas em pacientes pediátricos com fibrose cística. Métodos Estudo transversal, realizado em um centro de referência no Rio de Janeiro/Brasil. Foram incluídos todos os pacientes pediátricos com diagnóstico confirmado de fibrose cística, entre cinco e 19 anos de idade. Foram analisadas a composição de ácidos graxos nos eritrócitos, as citocinas séricas (TNFα, IL-1, IL-6 e IL-8) e a proteína C reativa. O ponto de corte para determinar a deficiência de ácidos graxos essenciais foi a concentração de ácido linoleico <21%. Resultados Foram estudadas 26 crianças (<10 anos) e 31 adolescentes, sendo a maioria do sexo feminino, heterozigoto para a mutação DF508 e com insuficiência pancreática exócrina. Nas crianças e nos adolescentes as concentrações de ácido graxo linoleico eram baixas (<21%). O TNF-α foi o único marcador pró-inflamatório cujas concentrações estavam aumentadas, principalmente nas crianças. Uma associação entre a composição de ácidos graxos nos eritrócitos e as citocinas IL-1β e IL-6 foi observada (p<0,05). Conclusão Os pacientes pediátricos estudados apresentaram deficiência de ácidos graxos essenciais e foi observada associação entre o perfil de ácidos graxos nos eritrócitos com as citocinas pró-inflamatórias séricas. Os achados destacam a importância deste tipo de avaliação sobre a fisiopatologia e o tratamento de pacientes com fibrose cística, como a suplementação com ácidos graxos n-3 (eicosapentaenoico e docosahexaenoico). No entanto são necessários mais estudos longitudinais no esclarecimento entre o desequilíbrio do metabolismo lipídico e a inflamação na fibrose cística.

trans Fatty Acids in Colostrum, Mature Milk and Diet of Lactating Adolescents.

The purpose of this study was to investigate the trans fatty acids (TFA) content and distribution in colostrum, mature milk, and diet of adolescent mothers, after TFA declaration in food labels became mandatory in Brazil. Participants were healthy adolescents (n 54, 15-19 years, 1-90 days postpartum) practicing exclusive breastfeeding. Milk samples were collected 3 days after delivery (colostrum) and in the third month postpartum (mature milk) by hand expression. The fatty acid composition of the milk samples was determined by gas chromatography. TFA intake corresponded to 1.23 % of total energy value. Total 18:2 TFA accounted for less than 0.5 % of the energy intake. The amount of total 18:1 TFA (mean ± SEM) was 1.9 % ± 0.14 in colostrum and 1.5 % ± 0.2 in mature milk. The total content of n-3 PUFA was inversely correlated with the total content of 18:1 TFA in colostrum. Both in colostrum and in mature milk, vaccenic acid (11t-18:1) was found to be the most abundant 18:1 trans isomer, followed by elaidic acid (9t-18:1), whereas rumenic acid (9c,11t-18:2 CLA) was the predominant 18:2 trans isomer. In conclusion, the levels of TFA of industrial sources found in the mother's diet and breast milk (colostrum and mature milk) showed a decrease in relation to those observed in studies conducted prior to the TFA labeling resolution in Brazil. However, the current low intake levels of n-3 LCPUFA and DHA content in the milk of lactating adolescents may be insufficient for supporting adequate neurological development of the infants.

[Anemia in indigenous women and children in Brazil: a systematic review]. / Anemia em crianças e mulheres indígenas no Brasil: revisão sistemática.

Iron-deficiency anemia can be considered the most important nutritional deficiency in Brazil. The scope of this article was to conduct a systematic review of the literature in order to describe the frequency of anemia in indigenous women and children in Brazil, and gather information on associated factors. A search of the PubMed, Scopus and Lilacs databases was conducted. Seventeen studies were selected. The rates of prevalence of anemia ranged from 23.1%-74.6%; 42.9%-92.3% and 29.6%-84%, for children aged 6 to 119 months, 6 to 23 months and 6 to 59 months, respectively. Two studies were conducted with women of childbearing age and rates of 67% were found among the Suruí and 16.1% among the Xukuru of Ororubá. The majority of authors attribute the prevalence of anemia to inadequate sanitary conditions, diets deficient in iron and essential micronutrients and a lack of access to basic health services. The findings highlight the importance of anemia among the indigenous peoples studied, which has been reflected by a substantial increase in research over the last twenty years.

Anemia em crianças e mulheres indígenas no Brasil: revisão sistemática / Anemia in indigenous women and children in Brazil: a systematic review

Resumo A anemia ferropriva pode ser considerada a mais importante carência nutricional no Brasil. O objetivo deste artigo foi realizar uma revisão sistemática da literatura sobre anemia em mulheres e crianças indígena no Brasil, com vistas a reunir informações relevantes sobre os fatores associados, assim como a frequência do agravo nesta população. Realizou-se busca nas bases bibliográficas Pubmed, Scopus e Lilacs, além do Portal de Teses em Saúde Pública da BVS. Foram selecionados 17 estudos. As prevalências de anemia encontradas variaram de 23,1% a 74,6%; 42,9% a 92,3% e de 29,6% a 84% para crianças na idade seis a 119 meses, de seis a 23 meses e de seis a 59 meses, respectivamente. Os dois estudos que foram realizados especificamente com mulheres em idade fértil encontraram taxas de 67% nas Suruí e de 16,1% entre as Xukuru do Ororubá. A maioria dos autores relaciona a maior prevalência da anemia às condições sanitárias impróprias, às dietas insuficientes em ferro e micronutrientes essenciais e à falta de acesso aos serviços básicos de saúde. Houve um perceptível aumento substancial das pesquisas ao longo dos últimos vinte anos, cujos achados evidenciam a importância de se resolver o problema da anemia entre os povos indígenas investigados.

Abstract Iron-deficiency anemia can be considered the most important nutritional deficiency in Brazil. The scope of this article was to conduct a systematic review of the literature in order to describe the frequency of anemia in indigenous women and children in Brazil, and gather information on associated factors. A search of the PubMed, Scopus and Lilacs databases was conducted. Seventeen studies were selected. The rates of prevalence of anemia ranged from 23.1%-74.6%; 42.9%-92.3% and 29.6%-84%, for children aged 6 to 119 months, 6 to 23 months and 6 to 59 months, respectively. Two studies were conducted with women of childbearing age and rates of 67% were found among the Suruí and 16.1% among the Xukuru of Ororubá. The majority of authors attribute the prevalence of anemia to inadequate sanitary conditions, diets deficient in iron and essential micronutrients and a lack of access to basic health services. The findings highlight the importance of anemia among the indigenous peoples studied, which has been reflected by a substantial increase in research over the last twenty years.

Nutritional status and body fat distribution in children and adolescentes with Cystic Fibrosis.

OBJECTIVE: assessing the nutritional status and body fat distribution in children and adolescents with cystic fibrosis. METHOD: Fifty-six (56) 8-18 year old patients were assessed for fat distribution by dual energy X-ray absorptiometry, for nutritional status by height/age, and for body mass index to age and dietary intake by 24-hour dietary recall. RESULTS: Approximately 50% of the sample showed adequate nutritional status. Most of it showed inadequate caloric and lipid intake. BMI/age was the nutritional indicator that best showed the increased percentage of trunk fat, android/gynecoidratio and trunk fat/total fat ratio. Patients with Pancreatic Insufficiency and eutrophic individuals showed higher median android/gynecoidratio. CONCLUSION: Increased abdominal adiposity was evidenced by DXA. The BMI did not identify decreased lean body mass. However, when body mass was high, it was significant for abdominal adiposity. The anthropometric assessment of patients with cystic fibrosis should be associated with body composition and body fat distribution to obtain an earlier malnutrition and cardiometabolic risk factor diagnosis.

Estado nutricional e distribuição de gordura corporal em crianças e adolescentes com Fibrose Cística / Nutritional status and body fat distribution in children and adolescentes with Cystic Fibrosis / Nutritional status and body fat distribution in children and adolescentes with Cystic Fibrosis

O objetivo deste estudo é avaliar o estado nutricional e a distribuição da gordura corporal em crianças e adolescentes com fibrose cística. Foram realizadas avaliação da distribuição de gordura corporal por meio da absorciometria de duplo feixe de energia, do estado nutricional por estatura/idade e índice de massa corporal/idade e a ingestão dietética pelo recordatório alimentar de 24horas, em 56 pacientes com idade entre 8 e 18 anos. Aproximadamente 50% da amostra apresentou estado nutricional adequado. A maioria apresentou a ingestão calórica e de lipídios inadequadas. O IMC/I foi o indicador nutricional que melhor evidenciou o aumento do percentual de gordura do tronco, razão androide/ginecoide e razão gordura tronco/gordura total. Os pacientes com Insuficiência Pancreática e os eutróficos apresentaram razão mediana androide/ginecoide maior. O aumento da adiposidade abdominal foi evidenciado pela DXA. O IMC/I não identificou a diminuição da massa magra corporal, mas quando aumentado foi significativo para adiposidade abdominal. Pacientes com fibrose cística devem associar a avaliação antropométrica à composição corporal e à distribuição de gordura corporal para um diagnóstico mais precoce de desnutrição e fatores de risco cardiometabólico.

Objective : assessing the nutritional status and body fat distribution in children and adolescents with cystic fibrosis. Method : Fifty-six (56) 8-18 year old patients were assessed for fat distribution by dual energy X-ray absorptiometry, for nutritional status by height/age, and for body mass index to age and dietary intake by 24-hour dietary recall. Results : Approximately 50% of the sample showed adequate nutritional status. Most of it showed inadequate caloric and lipid intake. BMI/age was the nutritional indicator that best showed the increased percentage of trunk fat, android/gynecoidratio and trunk fat/total fat ratio. Patients with Pancreatic Insufficiency and eutrophic individuals showed higher median android/gynecoidratio. Conclusion : Increased abdominal adiposity was evidenced by DXA. The BMI did not identify decreased lean body mass. However, when body mass was high, it was significant for abdominal adiposity. The anthropometric assessment of patients with cystic fibrosis should be associated with body composition and body fat distribution to obtain an earlier malnutrition and cardiometabolic risk factor diagnosis.

Association between quality of the diet and cardiometabolic risk factors in postmenopausal women.

BACKGROUND: Climateric is a phase of women's life marked by the transition from the reproductive to the non-reproductive period. In addition to overall weight gain, the menopause is also associated with the increase of abdominal fat. We used The Healthy Eating Index as a summary measure to evaluate the major components and the quality of women's diet after the onset of the menopause. This study aims at examining the association between the quality of the diet and cardiometabolic risk factors in postmenopausal women. METHODS: Cross-sectional study including 215 postmenopausal women attending a public outpatient clinic. The 24-hour dietary recall method was used to assess the food intake and to establish the Healthy Eating Index. Diets were then classified as appropriate diet (>80 points), diet "requiring improvement" (80-51 points), and poor diet (<51 points). Cardiometabolic risk factors included abdominal obesity, dyslipidemia, diabetes mellitus, and hypertension. The Fisher's exact test was utilized for the Statistical analysis. RESULTS: The analysis of the food intake showed that the average daily intake of lipids (36.7%) and sodium (2829.9 mg) were above the recommended. Only 8.8% of the women performed moderate or intense physical exercises on a regular basis. The diet was considered poor in 16.3%, "requiring improvement" in 82.8%, and appropriate for only 0.9% of the women. The study detected increased waist circumference in 92.1% of the participants. The mean concentration of triglycerides was of 183.3 mg/dl, and 130.7 mg/dl for cholesterol (Low Density Lipoprotein). CONCLUSION: Women consume a low quality diet, possibly due to the low intake of vegetables and fruits and excessive consumption of sodium. These inappropriate eating habits are associated with and, have a negative impact on the cardiometabolic risk factors such as abdominal obesity.

Severe phenotype in an apparent homozygosity caused by a large deletion in the CFTR gene: a case report.

BACKGROUND: Over 1900 mutations have been identified in the cystic fibrosis conductance transmembrane regulator gene, including single nucleotide substitutions, insertions, and deletions. Unidentified mutations may still lie in introns or in regulatory regions, which are not routinely investigated, or in large genomic deletions, which are not revealed by conventional molecular analysis. The apparent homozygosity for a rare, cystic fibrosis conductance transmembrane regulator mutation screened by standard molecular analysis should be further investigated to confirm if the mutation is in fact homozygous. We describe a patient presenting with an apparent homozygous S4X mutation. CASE PRESENTATION: A 13-year-old female patient of African descent with clinical symptoms of classic cystic fibrosis and a positive sweat test (97 mEq/L, diagnosed at age 3 years) presented with pancreatic insufficiency and severe pulmonary symptoms (initial lung colonization with Pseudomonas aeruginosa at age 4 years; forced vital capacity: 69%; forced expiratory volume: 51%; 2011). Furthermore, she developed severe acute lung disease and recurrent episodes of dehydration requiring hospitalization. The girl carried the CFTR mutation S4X in apparent homozygosity. However, further analysis revealed a large deletion in the second allele that included the region of the mutation. The deletion that we describe includes nucleotides 120-142, which correspond to a loss of 23 nucleotides that abolishes the normal translation initiation codon. CONCLUSION: This study reiterates the view that large, cystic fibrosis conductance transmembrane regulator deletions are an important cause of severe cystic fibrosis and emphasizes the importance of including large deletions/duplications in cystic fibrosis conductance transmembrane regulator diagnostic tests.

Excesso de peso entre adolescentes em zona rural e a alimentação escolar oferecida / Overweight among adolescents in rural area and school meals offered

OBJETIVO: Avaliar a composição nutricional e a aceitabilidade da alimentação escolar, o estado nutricional e a segurança alimentar dos adolescentes das escolas municipais da zona rural de Carmo no Rio de Janeiro. MÉTODOS: Participaram 121 adolescentes de 7 escolas públicas em um município do interior do Rio de Janeiro. Foi utilizado o método de pesagem direta dos alimentos, calculando-se as porções médias oferecidas. A segurança alimentar e a aceitabilidade da alimentação escolar foram avaliadas a partir de questionários validados. O estado nutricional foi determinado pelo Índice de Massa Corporal por Idade (IMC/I) e estatura/idade e sexo. RESULTADOS: A prevalência de sobrepeso/obesidade foi de 28,9%, baixo peso 1,7% e baixa estatura por idade 0,8%. Todas as escolas ofereceram valores de nutrientes superiores à recomendação, exceto para fibras, vitamina C, magnésio e cálcio. A oferta de alimentos de elevada densidade calórica foi alta. A maioria dos alunos (92%) com insegurança alimentar consumia a alimentação escolar e nenhum destes possuía baixo peso ou estatura. CONCLUSÃO: Os alunos apresentaram um bom estado nutricional, mas sendo as prevalências de sobrepeso/obesidade próximas àquelas de áreas urbanas. É necessária maior ênfase na educação nutricional e planejamento da alimentação escolar sobre os grupos de alimentos oferecidos.

OBJECTIVE: To evaluate the nutricional composition and the acceptability of the school meals, nutritional status, and food security of adolescents in rural public schools in Carmo, Rio de Janeiro. METHODS: One hundred and twenty-one adolescents from the 7 public schools of a country side city of Rio de Janeiro, participated in the study. The method of food direct weighing was utilized, where the offered average portions were calculated. Food security and school meals acceptability were evaluate by validated questionnaires. The nutricional status was determined by Body mass index (BMI for age) and height for age and gender. RESULTS: The occurrence of overweight/obesity was 28.9%, underweight 1.7% and low height-for-age 0.8%. All school menus offered higher nutritional values than the recommended, except for fiber, vitamin C, magnesium, and calcium. The offered of high energetic density food was high. The majority of the students (92%) with food insecurity ate the meals offered by the school and none of them were low weight-for-age or low height-for-age. CONCLUSION: The students presented a good nutritional status, but the prevalence of overweight/obesity was similar to those in urban areas. It is required more emphasis on nutrition education, and planning of the school meals of the group of food to be selected.

Avaliação e recomendações nutricionais para crianças e adolescentes com fibrose cística / Nutritional assessment and recommendations for children and adolescents with cystic fibrosis

Revisar e discutir evidências sobre a avaliação do estado nutricional e as recomendações para o tratamento nutricional de crianças e adolescentes com fibrose cística. FONTES DE DADOS: Bancos de dados MEDLINE (versão PubMed) e Latin American and Caribbean Center on Health Sciences Information (LILACS), entre 1984 a 2009. Aplicou-se a combinação dos seguintes descritores: fibrose cística, estado nutricional, criança e adolescente - em inglês e português. SÍNTESE DOS DADOS: A fibrose cística é uma doença genética, de evolução crônica, progressiva e fatal. Resulta do defeito na proteína reguladora transmembrana que regula a condução de cloro e, consequentemente, o fluxo de sódio e água através da membrana apical das células epiteliais. Pacientes fibrocísticos são vulneráveis à desnutrição, que resulta do desequilíbrio entre ingestão alimentar, gasto e perdas energéticas. Novos conhecimentos sobre a fisiopatologia da doença, adquiridos nas últimas décadas, resultaram em mudanças significativas nas atuais recomendações energéticas e principalmente de lipídeos. A importância da nutrição no bem-estar e sobrevida dos fibrocísticos está bem estabelecida, assim como a associação entre a desnutrição e a deterioração da função pulmonar. Existem múltiplos fatores inter-relacionados que afetam o estado nutricional, tais como, mutação genética, insuficiência pancreática, ressecção intestinal, perda de sais e ácidos biliares, refluxo gastroesofágico, inflamação e infecções pulmonares, diabetes e condições emocionais. CONCLUSÕES: O monitoramento nutricional e o aconselhamento dietético são elementos chave no manejo de crianças e adolescentes com fibrose cística com o intuito de controlar a sintomatologia e a progressão da doença, proporcionando melhor qualidade de vida.

To review and discuss evidence on the nutritional status assessment and recommendations for nutritional management of children and adolescents with cystic fibrosis. DATA SOURCES: MEDLINE (PubMed version) and Latin American and Caribbean Center on Health Sciences Information (LILACS), from 1984 to 2009. The combination of the following keywords were applied: cystic fibrosis, nutritional status, child, and adolescent. DATA SYNTHESIS: Cystic fibrosis (CF) is a genetic disease, chronic, progressive, and fatal. It is caused by a defect in the transmembrane regulatory protein that regulates the flow of chlorine, sodium and water across the apical membrane of epithelial cells. CF patients are vulnerable to malnutrition resulting from the imbalance between food intake, energy expenditure and loss. New insights into the pathophysiology of the disease have resulted in significant changes in current recommendations for energy and especially lipids intake. There are many interrelated factors that affect the nutritional status of CF patients, such as genetic mutation, pancreatic insufficiency, bowel resection, loss of bile salts and acids, gastroesophageal reflux disease, lung inflammation and infections, diabetes, and emotional conditions. The importance of nutrition in the survival of CF patients is well established, as well as the association between malnutrition and deterioration of lung function. CONCLUSIONS: Nutritional monitoring and dietary counseling are key elements in the management of patients with CF aiming to control the symptoms and the progression of the disease in order to provide better quality of life.

Motor delay in cystic fibrosis infants: an observational study.

OBJECTIVE: To verify the prevalence of delay in gross motor development in cystic fibrosis (CF) patients. STUDY DESIGN: This is a cross-sectional observational study. A total of 15 children with CF were included in the analyses. The selection criteria was age between 6 and 42months. Data on demographic, anthropometric, clinical characteristics and severity score (Shwachman) were obtained from patient records. The Bayley Scales of Infant and Toddler Development® - III Edition (BSITD-III) was used to assess motor abilities. RESULTS: Motor development delay was observed in 26.7% (n=4) of the children and, in 75% of these, there were statistically significant differences between gross and fine motor scores. Low stature, low weight and periods longer than 60days at hospital showed statistically significant association with motor delay (p=0.025, 0.032, 0.003, respectively). CONCLUSION: The prevalence of motor delay in the studied sample was high, suggesting that biological and ambient conditions of risk present in CF contribute to early motor deficits. Thus, the observation of the motor development in these patients is important for planning an adequate intervention.

Cohort study for monitoring cardiovascular risk factors in children using a primary health care service: methods and initial results.

An awareness of the presence of cardiovascular disease risk factors since childhood is essential to guide health promotion policies. The aim of this paper is to present, together with the main results, a methodological proposal to estimate both incidence and prevalence of these factors over time and their associated socio-demographic aspects in a population attending a primary health care unit, within Brazilian Unified National Health System. An open cohort design was adopted, starting in 2004 with a sample of 356 children aged between 5 and 9. In the second wave, in 2008, 126 children were enrolled and 205 were reevaluated. Socioeconomic variables, food intake, physical activity, anthropometric measures (weight, height and waist size), lipidogram and glycemia were collected. Results indicate a high prevalence of dyslipidemia, and an increase of excess weight and sedentary activity between both surveys. Some 55% of children were found to have lower values of HDL-cholesterol. The methodological proposal was considered adequate for the monitoring of cardiovascular disease predictors in poor urban populations.

Plasma levels of trans-fatty acids are low in exclusively breastfed infants of adolescent mothers.

The purpose of this study was to determine the levels of trans-octadecenoic acid (C18:1-trans) and trans-isomers of linoleic acid (18:2-trans), as well as long-chain polyunsaturated fatty acids (LC-PUFA), in the plasma from infants of adolescent mothers at 3 months of age, exclusively breastfed, and the relationship with the levels of the same isomers in plasma and milk of the mothers. Samples of blood and mature milk were obtained from 49 healthy adolescent mothers and their exclusively breastfed infants treated at the Instituto Fernandes Figueira-Fundação Oswaldo Cruz (IFF-FIOCRUZ) in Rio de Janeiro, Brazil. trans-Fatty acids (TFA) were analyzed by gas chromatography. The results of this study showed low levels of TFA in milk (1.53%), maternal plasma (0.50%), and plasma of infants (0.74%). The results show that, although TFA have been found in the plasma of the studied infants, the LC-PUFA levels are kept within normal limits. No association between TFA presence and parameters of nutritional status of the infants was observed, probably due to the low levels of these fatty acids found in this study.

Cohort study for monitoring cardiovascular risk factors in children using a primary health care service: methods and initial results / Estudo de coorte para vigilância dos fatores de risco cardiovascular em crianças na atenção básica de saúde: métodos e primeiros resultados

An awareness of the presence of cardiovascular disease risk factors since childhood is essential to guide health promotion policies. The aim of this paper is to present, together with the main results, a methodological proposal to estimate both incidence and prevalence of these factors over time and their associated socio-demographic aspects in a population attending a primary health care unit, within Brazilian Unified National Health System. An open cohort design was adopted, starting in 2004 with a sample of 356 children aged between 5 and 9. In the second wave, in 2008, 126 children were enrolled and 205 were reevaluated. Socioeconomic variables, food intake, physical activity, anthropometric measures (weight, height and waist size), lipidogram and glycemia were collected. Results indicate a high prevalence of dyslipidemia, and an increase of excess weight and sedentary activity between both surveys. Some 55 percent of children were found to have lower values of HDL-cholesterol. The methodological proposal was considered adequate for the monitoring of cardiovascular disease predictors in poor urban populations.

Conhecer a presença de fatores de risco para as doenças cardiovasculares desde a infância é essencial para orientar políticas de promoção da saúde. O objetivo deste artigo é apresentar, junto com os principais resultados, uma proposta metodólogica para estimar incidência e prevalência, ao longo do tempo, desses fatores e fatores sociodemográficos associados em população assistida em unidade básica, integrante do Sistema Único de Saúde do Brasil. Adotou-se o desenho de uma coorte aberta, iniciada em 2004, com amostra de 356 crianças de 5-9 anos. Na segunda onda, em 2008, 126 crianças aderiram, e 205 foram reavaliadas. Coletou-se dados socioeconômicos, alimentares, de atividade física, antropométricos (peso/altura/perímetro da cintura), lipidograma e glicemia. Os resultados apontam alta prevalência de dislipidemia, aumento do excesso de peso e de tempo sedentário entre ondas. Em 55 por cento das crianças, os valores de HDL-colesterol foram mais baixos. A proposta metodológica apresentada mostrou-se adequada para a vigilância dos preditores das doenças cardiovasculares em populações urbanas carentes.

Association between nutritional status measurements and pulmonary function in children and adolescents with cystic fibrosis.

OBJECTIVE: To evaluate the association between nutritional status measurements and pulmonary function in children and adolescents with cystic fibrosis. METHODS: We evaluated the nutritional status of 48 children and adolescents (aged 6-18 years) with cystic fibrosis based on body mass index (BMI) and body composition measurements-mid-arm muscle circumference (MAMC) and triceps skinfold thickness (TST)-at a referral center in the city of Rio de Janeiro, Brazil. Pulmonary function was assessed by means of spirometry, using FEV1 to classify the severity of airway obstruction. We used Student's t-tests for comparisons between proportions and linear regression analysis for associations between continuous variables. The level of significance was set at p < 0.05. RESULTS: The evaluation of nutritional status based on BMI identified a smaller number of malnourished patients than did that based on MAMC (14 vs. 25 patients, respectively). Most of the patients presented mild pulmonary disease. Mean FEV1 was 82.5% of predicted. Pulmonary function was found to correlate significantly with BMI, MAMC and TST (p = 0.001, p = 0.001 and p = 0.03, respectively). All subjects with moderate or severe pulmonary involvement were considered malnourished based on BMI and body composition parameters. Of the 25 patients considered malnourished based on body composition (MAMC), 19 were considered well-nourished based on their BMI. CONCLUSIONS: In the present study, all nutritional status measurements correlated directly with the pulmonary function of children and adolescents with cystic fibrosis. However, body composition measurements allowed earlier detection of nutritional deficiencies.

Associação entre medidas do estado nutricional e a função pulmonar de crianças e adolescentes com fibrose cística / Association between nutritional status measurements and pulmonary function in children and adolescents with cystic fibrosis

Objetivo: Avaliar a associação de medidas do estado nutricional com a função pulmonar de crianças e adolescentes com fibrose cística. Métodos: Foi avaliado o estado nutricional pelo índice de massa corpórea (IMC) e por medidas de composição corporal—circunferência muscular do braço (CMB) e medida da dobra cutânea triciptal (DCT)—de 48 crianças e adolescentes (6-18 anos) com fibrose cística em um centro de referência na cidade do Rio de Janeiro.A função pulmonar foi analisada por espirometria, e o parâmetro adotado para a classificação da obstrução das vias aéreas foi VEF1. Utilizou-se o teste t de Student para a comparação entre proporções e a regressão linear para associação entre variáveis contínuas. O nível de significância adotado foi p < 0,05. Resultados: A avaliação do estado nutricional através do IMC encontrou menos pacientes desnutridos do que a realizada através da CMB (14 vs. 25, respectivamente) A maioria dos pacientes apresentou doença pulmonar leve. A média do VEF1 foi de 82,5% do previsto. Houve associação do IMC, da CMB e da DCT com a função pulmonar (p = 0,001, p = 0,001 e p = 0,03, respectivamente). Todos os pacientes com comprometimento moderado e grave pulmonar eram desnutridos pela composição corporal (CMB). Entre os 25 pacientes considerados desnutridos através da composição corporal (CMB), 19 apresentavam peso adequado quando avaliados pelo IMC. Conclusões: Todas as medidas do estado nutricional apresentaram associação direta com a função pulmonar das crianças e adolescentes com fibrose cística no estudo. Entretanto, as medidas de composição corporal revelaram, de forma mais precoce, possíveis deficiências nutricionais.

Objective: To evaluate the association between nutritional status measurements and pulmonary functionin children and adolescents with cystic fibrosis. Methods: We evaluated the nutritional status of 48 children and adolescents (aged 6-18 years) with cystic fibrosis based on body mass index (BMI) and body composition measurements—mid-arm muscle circumference (MAMC) and triceps skinfold thickness (TST)—at a referral center in the city of Rio de Janeiro, Brazil. Pulmonary function was assessed by means of spirometry, using FEV1 to classify the severity of airway obstruction. We used Student’s t-tests for comparisons between proportions and linear regression analysis for associations between continuous variables. The level of significance was set at p < 0.05. Results: The evaluation of nutritional status based on BMI identified a smaller number of malnourished patients than did that based on MAMC (14 vs. 25 patients, respectively). Most of the patients presented mild pulmonary disease. Mean FEV1 was 82.5% of predicted. Pulmonary function was found to correlate significantly with BMI, MAMC and TST (p = 0.001, p = 0.001 and p = 0.03, respectively). All subjects with moderate or severe pulmonary involvement were considered malnourished based on BMI and body composition parameters. Of the 25 patients considered malnourished based on body composition (MAMC), 19 were considered well-nourished based on theirBMI. Conclusions: In the present study, all nutritional status measurements correlated directly with the pulmonaryfunction of children and adolescents with cystic fibrosis. However, body composition measurements allowed earlier detection of nutritional deficiencies.