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.

[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.

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.

Prevalência de doença mineral óssea em adolescentes com fibrose cística / Prevalence of bone mineral disease among adolescents with cystic fibrosis

OBJETIVO: Avaliar a prevalência de doença mineral óssea em adolescentes com fibrose cística e associar os achados com as variáveis estudadas. MÉTODOS: Foram selecionados 37 adolescentes, dos quais foram avaliados: estado nutricional pelos índices de altura/idade e massa corporal/idade; densidade mineral óssea da coluna lombar e corpo inteiro por densitometria com emissão de raio X de dupla energia; ingestão dietética diária pelo registro alimentar de 3 dias; e prova de função pulmonar pelo volume expiratório forçado no primeiro segundo. RESULTADOS: A média de idade foi de 13,2 (±2,8) anos. O estado nutricional adequado foi de 70,3 e 75,7 por cento pelos índices de altura/idade e de massa corporal/idade, respectivamente; 54,1 por cento dos pacientes apresentaram redução da densidade mineral óssea para coluna lombar e 32,5 por cento para corpo inteiro. Houve correlação positiva entre densidade mineral óssea e índice de massa corporal (p = 0,04). A doença pulmonar e a insuficiência pancreática apresentaram correlação com a alteração da densidade mineral óssea. O inquérito alimentar revelou percentuais de adequação para o cálcio, fósforo e calorias, de acordo com a recomendação nutricional preconizada pelo Consenso Europeu de Fibrose Cística. Essas variáveis não se mostraram estatisticamente significantes na análise multivariada. CONCLUSÃO: A prevalência de doença mineral óssea é alta na adolescência. O estado nutricional adequado, a reposição de enzimas pancreáticas e o controle da doença pulmonar podem ter efeito protetor para a massa óssea.

OBJECTIVE: To evaluate the prevalence of bone mineral disease among adolescents with cystic fibrosis and to relate the findings with the variables studied. METHODS: The study enrolled 37 adolescents who were assessed for: nutritional status according to height/age and body mass/age ratios; bone mineral density of the lumbar spine and entire body by densitometry with dual emission X-ray; daily dietary intake according to a 3-day dietary recall; and pulmonary function by the forced expiratory volume in one second test. RESULTS: Mean age was 13.2 (±2.8) years. Nutritional status was adequate in 70.3 and 75.7 percent of patients according to the height/age and body mass/age indices, respectively; 54.1 percent of the patients exhibited reduced lumbar spine bone mineral density and 32.5 percent for the whole body. There was a positive correlation between bone mineral density and body mass index (p = 0.04). Lung disease and pancreatic insufficiency exhibited a correlation with altered bone mineral density. The dietary recall revealed adequate percentages of calcium, phosphorous and calories, according to the nutritional recommendations laid out in the European Cystic Fibrosis Consensus. The multivariate analysis indicated that these variables were not statistically significant. CONCLUSIONS: There is a high prevalence of bone mineral disease among adolescents. Good nutritional status, pancreatic enzyme replacement and control of lung disease may have a protective effect on bone mass.

Prevalence of bone mineral disease among adolescents with cystic fibrosis.

OBJECTIVE: To evaluate the prevalence of bone mineral disease among adolescents with cystic fibrosis and to relate the findings with the variables studied. METHODS: The study enrolled 37 adolescents who were assessed for: nutritional status according to height/age and body mass/age ratios; bone mineral density of the lumbar spine and entire body by densitometry with dual emission X-ray; daily dietary intake according to a 3-day dietary recall; and pulmonary function by the forced expiratory volume in one second test. RESULTS: Mean age was 13.2 (+/-2.8) years. Nutritional status was adequate in 70.3 and 75.7% of patients according to the height/age and body mass/age indices, respectively; 54.1% of the patients exhibited reduced lumbar spine bone mineral density and 32.5% for the whole body. There was a positive correlation between bone mineral density and body mass index (p = 0.04). Lung disease and pancreatic insufficiency exhibited a correlation with altered bone mineral density. The dietary recall revealed adequate percentages of calcium, phosphorous and calories, according to the nutritional recommendations laid out in the European Cystic Fibrosis Consensus. The multivariate analysis indicated that these variables were not statistically significant. CONCLUSIONS: There is a high prevalence of bone mineral disease among adolescents. Good nutritional status, pancreatic enzyme replacement and control of lung disease may have a protective effect on bone mass.

Prevalência em crianças de fatores de risco para as doenças cardiovasculares / Childhood prevalence of cardiovascular risk factors

Investigou-se a presença de fatores de risco para doenças cardiovasculares em estudo transversal em 356 crianças de 5 a 9 anos, atendidas em unidade básica de saúde de área de baixa renda da Região Metropolitana do Rio de Janeiro, Brasil. Foram avaliados: lipidograma, estado nutricional, hábitos alimentares e aspectos sócio-econômicos. Observaram-se 10,7 por cento de sobrepeso e 68,4 por cento com níveis alterados no lipidograma, sendo 18,6 por cento com LDL-colesterol alto. Para descrever o perfil alimentar as respostas ao questionário qualitativo de ingestão foram submetidas à classificação multivariada, obtendo-se seis grupos, resumidamente definidos como: da cultura tradicional brasileira; moderno (produtos diet e light); frituras; doces e refrescos (misturados com outros grupos); os demais pouco definidos. A alta prevalência dos fatores de risco para as doenças cardiovasculares desde a infância e a evidência de alimentação infantil inadequada indica a necessidade de desenvolver uma estratégia preventiva, procurando atingir toda a família, de forma a alterar os padrões de ingestão de alimentos das populações de baixa renda em direção à comportamentos mais saudáveis.

Cardiovascular risk factors were investigated in 356 children 5 to 9 years of age who were treated at a primary care center located in a low-income area in Greater Metropolitan Rio de Janeiro, Brazil. Lipid profile, nutritional status, food intake, and lifestyle were evaluated. 10.7 percent of the children were overweight, 68.4 percent had some type of dyslipidemia, and 18.6 percent showed high LDL-c. To describe the food intake pattern, the answers to the qualitative food questionnaire were submitted to multivariate cluster analysis, producing six basic groups: traditional Brazilian cooking; "modern" food (including diet and light products); fried food; sweets and soft drinks (mixed with other groups); and other poorly defined groups. The high prevalence of cardiovascular risk factors (beginning in childhood) and the evidence of inadequate dietary habits indicate that a preventive family-focused strategy is needed to change the dietary pattern of low-income groups towards healthier eating.

Exercício aeróbico, treinamento de força muscular e testes de aptidão física para adolescentes com fibrose cística: revisão da literatura / Exercise testing, aerobic and strength training for adolescents with cystic fibrosis: a literature review

São reconhecidos os benefícios da prática do exercício físico regular para pacientes com fibrose cística. Entretanto, poucos estudos envolvem pacientes adolescentes. O objetivo deste artigo foi revisar os efeitos da prática regular de exercícios aeróbicos e de força e resistência muscular para adolescentes com fibrose cística. Os principais testes de aptidão física para esta faixa etária e a importância deles para melhora do prognóstico e tratamento da doença também foram avaliados. As informações foram coletadas a partir de livro-texto e artigos publicados na literatura nacional e estrangeira nas seguintes bases de dados: LILACS, MEDLINE/PubMed, Biblioteca Cochrane e SciELO, abrangendo o período de 1994 a 2004. Foram utilizados os termos exercise e cystic fibrosis para seleção dos artigos. Esta pesquisa demonstrou que a prática de exercício aeróbico e treinamento de força muscular melhoram a desobstrução da árvore brônquica, diminuem a queda progressiva da função pulmonar, aumentam a massa muscular e a resistência ao exercício, promovem o desenvolvimento ósseo e melhoram a auto-estima e a qualidade de vida. Os melhores resultados foram obtidos com os programas de treinamento assistido, devido a sua maior regularidade.

The benefits of regular physical exercises for cystic fibrosis patients are well known. Nevertheless, few studies involve adolescent patients. The objective of this article was to review the effects of regular practice of aerobic exercises, strength exercises and muscular exercises in adolescents with cystic fibrosis. The main physical fitness tests for this age bracket and their value in improving prognosis and treatment were assessed as well. Information was collected from text books and articles published in the national and foreign literature in the following databases: LILACS, MEDLINE/PubMed, Cochrane Library and SciELO, comprising the period of 1994 to 2004. The terms exercise and cystic fibrosis were used to select articles. This survey demonstrated that aerobic exercises and muscular strength training help to relieve the bronchial tree, reduce progressive pulmonary function failure and improve self-esteem and quality of life. The best results were obtained with assisted training programs due to their regularity.