Brazilian Guidelines for Nutrition in Cystic Fibrosis

ABSTRACT Objective To develop a scientific consensus on nutrition in cystic fibrosis. Methods Sixteen coordinators elaborated relevant questions on nutritional therapy in cystic fibrosis, which were divided into six sections: nutritional assessment, nutritional recommendations, nutritional intervention, dietary counseling, special situations and enzyme replacement, and gastrointestinal manifestations. Two to three specialists in the field were responsible for each section and obtaining answers formulated based on standardized bibliographic searches. The available literature was searched in the PubMed®/MEDLINE database, after training and standardization of search strategies, to write the best level of evidence for the questions elaborated. Issues related to disagreement were discussed until a consensus was reached among specialists, based on the current scientific literature. Results Forty-two questions were prepared and objectively answered, resulting in a consensus of nutritional therapy in cystic fibrosis. Conclusion This work enabled establishing a scientific consensus for nutritional treatment of cystic fibrosis patients.

Impacto de fatores clínicos e laboratoriais no ganho de peso em uma coorte de pacientes com Fibrose Cística diagnosticados pela triagem neonatal / Impact of clinical and laboratory weight gain in a cohort of patients with cystic fibrosis diagnosed by newborn screening factors

Introdução: A Fibrose Cística é uma doença genética autossômica recessiva de caráter progressivo que acomete as glândulas exócrinas. Vários fatores podem interferir no ganho de peso e determinar a qualidade de vida desses pacientes. Objetivo: Avaliar os fatores clínicos e laboratoriais que influenciem na evolução do ganho de peso dos pacientes com Fibrose Cística. Método: Coorte híbrida, retrospectiva de 2003 a 2009. Questionário padronizado foi elaborado para coleta de dados dos prontuários. O programa utilizado foi SPSS 16.0 para armazenamento e análises estatísticas. Por se tratar de dados longitudinais, as análises univariadas consistiram em desenvolver modelos preliminares da variação peso em função do tempo para cada uma das covariáveis separadamente (sexo, insuficiência pancreática, colonização crônica por Pseudomonas aeruginosa (PA), Stafilococcus aureus sensível (MSSA) e resistente à meticilina (MRSA), nível sérico de albumina, íleo meconial e mutação genética). Para essa análise longitudinal do peso, foi utilizado o software R. Nível de significância de 5%. Resultados: Foram acompanhados 43 pacientes com mediana de idade ao diagnóstico de 41 dias, sendo 80% com mutação ΔF508 (22,5% homozigotos). 58% sexo masculino. Das variáveis analisadas, insuficiência pancreática, colonização crônica por PA, MSSA, MRSA e hipoalbuminemia influenciaram, do ponto de vista estatístico, o ganho longitudinal de peso. Nesta coorte, as variáveis, íleo meconial, mutação genética e sexo não tiveram influência no ganho ponderal. Conclusão: A insuficiência pancreática, colonização crônica por PA, MSSA, MRSA e hipoalbuminemia tiveram influência negativa no ganho ponderal, reforçando a necessidade de maior atenção com a assistência destes pacientes.

Introduction: Cystic fibrosis is an autosomal recessive genetic disorder of progressive affecting the exocrine glands. Several factors can interfere with weight gain and determine the quality of life of these patients. Objective: To evaluate the clinical and laboratory factors that influence the evolution of weight gain in patients with Cystic Fibrosis. Methods: Hybrid Cohort, retrospective from 2003 to 2009 standardized questionnaire was designed to collect data from medical records. The program SPSS 16.0 was used for storage and statistical analyzes. Because it is longitudinal data, univariate analyzes consisted develop preliminary models of weight change versus time for each of the covariates separately (sex, pancreatic insufficiency, chronic colonization with Pseudomonas aeruginosa (PA), Staphylococcus aureus sensitive (MSSA) and methicillin-resistant (MRSA), serum albumin, meconium ileus and genetic mutation). For this longitudinal analysis of weight R. Software level of significance of 5% was used. Results: 43 patients were followed with a median age at diagnosis of 41 days, of which 80% DF508 mutation (22.5% homozygous). 58% male, the variables analyzed, pancreatic insufficiency, chronic colonization by PA, MSSA, MRSA and hypoalbuminemia influenced, the statistical point of view, the longitudinal weight gain. In this cohort, variables, meconium ileus, genetic mutation and sex had no influence on weight gain. Conclusion: Pancreatic insufficiency, chronic colonization by PA, MSSA, MRSA and hypoalbuminemia had a negative influence on weight gain, reinforcing the need for greater attention to the care of these patients.