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.

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.

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.