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.

Development of Height and Body Mass Index After Pediatric Kidney Transplantation: Experience of the Nephrology Pediatric Service at HCFMRP- USP, 2005-2014 / Evolução de Estatura e Índice de Massa Corporal Após Transplante Renal Pediátrico: Experiência do Serviço de Nefrologia Pediátrica do HCFMRP-USP, 2005-2014

Abstract Introduction: Chronic kidney disease in children often determines poor nutritional status. Although renal transplantation (RTx) resolves endocrine and metabolic disorders, growth continues to be suboptimal and excessive weight gain may result in obesity. Objectives: Evaluating the development of height and body mass index in renal transplanted children and adolescents and identifying associated factors with final nutritional status. Methods: We reviewed the medical records of 17 patients with regular follow-ups up to 24 months after RTx. Nutritional status was assessed by height-for-age (H/A) and body mass index-for-age (BMI/A). It was considered catch-up growth the increase in z-score H/A ≥ 0.5 standard deviation. Multiple linear regression was used to estimate the influence of factors clinical and demographic variables on anthropometric indicators at 24 months after RTx. Results: Mean age was 9.1 ± 4.1 years old. Twenty-four months after RTx the mean z-score H/A increased from -2.66 ± 1.66 to -1.93 ± 1.08 (p ≤ 0.05), 47.0% of the patients showed catch-up growth and the same proportion showed z-score H/A < -2. Mean z-score BMI/A increased from -0.48 ± 1.03 at RTx to 0.80 ± 0.94 at third month after RTx (p < 0.001) and remained unchanged up to 24 months. The frequency of weight excess increased from 5.9% at RTx to 41.2% at 24 months. Age (r = -0.66; p = 0.006) and z-score H/A (r = -0.72; p = 0.002) at RTx were inversely associated with growth. Conclusion: Twenty-four months after transplant it was verified inadequate growth to recovery from stunting and excessive weight gain. RTx promoted greater growth in the youngest patients and most stunted at RTx.

Resumo Introdução: A doença renal crônica em crianças geralmente determina o comprometimento do estado nutricional. Embora o transplante renal (TxR) resolva os distúrbios endócrinos e metabólicos, o crescimento continua a ser inadequado e o ganho de peso excessivo pode resultar em obesidade. Objetivos: Avaliar a evolução da estatura e do índice de massa corporal de crianças e adolescentes transplantados renais e identificar fatores associados com o estado nutricional final. Método: Foram revisados os prontuários de 17 pacientes com seguimento regular até 24 meses após o (TxR). O estado nutricional foi avaliado por estatura para idade (E/I) e índice de massa corporal para idade (IMC/I). Foi considerado catch-up de crescimento o aumento no escore-z E/I ≥ 0,5 desvios-padrão. A análise de regressão linear múltipla foi utilizada para estimar a influência de variáveis clínicas e demográficas na variação dos indicadores antropométricos aos 24 meses após TxR. Resultados: A média de idade foi 9,1 ± 4,1 anos. Após 24 meses de TxR, a média de escore-z E/I aumentou de -2,66 ± 1,66 para -1,93 ± 1,08 (p ≤ 0,05), 47,0% dos pacientes apresentaram catch-up de crescimento e a mesma proporção apresentou escore-z E/I < -2. A média do escore-z IMC/I aumentou de -0,48 ± 1,03 no TxR para 0,80 ± 0,94 no terceiro mês após TxR (p < 0,001) e manteve-se estável até 24 meses. A frequência de excesso de peso aumentou de 5,9% no TxR para 41,2% aos 24 meses. A idade (r = -0,66; p = 0,006) e o escore-z E/I no TxR (r = -0,72; p = 0,002) foram inversamente associados ao crescimento. Conclusão: Após 24 meses de TxR verificou-se crescimento insuficiente para recuperação do déficit estatural e ganho ponderal excessivo. O TxR promoveu maior crescimento nos pacientes mais jovens e com maior déficit estatural no TxR.

Avaliação clínica, nutricional e espirométrica de pacientes com fibrose cística após implantação de atendimento multidisciplinar / Clinical, nutritional and spirometric evaluation of patients with cystic fibrosis after the implementation of multidisciplinary treatment

OBJETIVO: A fibrose cística (FC) é uma doença hereditária, multissistêmica e crônica, para a qual é importante uma abordagem multidisciplinar. O objetivo deste estudo foi mostrar a evolução de um grupo de pacientes com FC após a implantação desse tipo de atendimento. MÉTODOS: Foram analisados retrospectivamente 19 pacientes (idades entre 6 e 29 anos) em acompanhamento clínico no Hospital das Clínicas da Faculdade de Medicina de Ribeirão Preto da Universidade de São Paulo, na cidade de Ribeirão Preto (SP). Os pacientes foram divididos em dois grupos: grupo 6-12 (6-12 anos) e grupo 13+ (> 12 anos). Dados referentes a índice de massa corporal (IMC), escore Z, escore de Shwachman (ES), número de exacerbações/ano, colonização crônica por Pseudomonas aeruginosa e medidas espirométricas (CVF, VEF1, VEF1 por cento e FEF25-75 por cento) foram obtidos. Os dados foram coletados em dois momentos (antes e após a implantação de atendimento multidisciplinar) e foram analisados com o teste dos postos sinalizados de Wilcoxon. RESULTADOS: A mediana de idade de início de sintomas foi de 10 meses. No grupo 6-12, houve somente um aumento significativo do IMC e da CVF. As outras medidas espirométricas aumentaram, mas não significativamente. No grupo 13+, não houve diferenças significativas entre os dois momentos, sendo que o ES apresentou uma diminuição com significância limítrofe. Houve diminuição não significativa das medidas espirométricas. Entretanto, o número de pacientes com volumes e fluxos alterados diminuiu nos dois grupos. CONCLUSÕES: Apesar do grupo pequeno, a manutenção dos parâmetros espirométricos pode significar estabilidade clínica e funcional. Os pacientes estudados mantiveram estabilidade clínica, funcional e nutricional durante o período, e a implantação de abordagem multidisciplinar pode ter contribuído nesse sentido.

OBJECTIVE: Cystic fibrosis (CF) is a chronic multisystemic hereditary disease for which a multidisciplinary approach must be taken. The objective of this study was to show the evolution of a group of patients with CF after the implementation of multidisciplinary treatment. METHODS: A retrospective study involving 19 patients (6-29 years of age) under clinical follow-up treatment at the University of São Paulo at Ribeirão Preto School of Medicine Hospital das Clínicas, located in the city of Ribeirão Preto, Brazil. The patients were divided into two groups: 6-12 (6-12 years of age) and 13+ (> 12 years of age). We collected data regarding body mass index (BMI), Z score, Shwachman score (SS), number of exacerbations/year, chronic colonization by Pseudomonas aeruginosa, and spirometric measurements (FVC, FEV1, FEV1 percent, and FEF25-75 percent). Data were collected at two different time points (before and after the implementation of the multidisciplinary treatment) and were analyzed with the Wilcoxon signed rank test. RESULTS: The median age at the onset of symptoms was 10 months. In the 6-12 group, only BMI and FVC increased significantly. Although the other spirometric values increased, the differences were not significant. In the 13+ group, there were no significant differences between the two time points. There was a borderline significant decrease in SS and less than significant decreases in the spirometric measurements. However, the number of patients with alterations in volumes and flows decreased in both groups. CONCLUSIONS: Although our patient sample was small, the lack of changes in the spirometric parameters might reflect clinical and functional stability. In all of the patients evaluated, clinical, functional, and nutritional parameters remained stable throughout the study period. The implementation of a multidisciplinary approach might have contributed to this result.