Vitamin D supplementation, early diagnosis and exclusive breastfeeding contribute to the adequacy of anthropometric indicators in patients with cystic fibrosis
DOI:
https://doi.org/10.12873/453dasilvaPalabras clave:
Fibrose Cística, Estado nutricional, Doença crônica, PediatriaResumen
Objective: To assess the nutritional status and associated factors in pediatric patients with cystic fibrosis.
Methods: This case series was conducted from June 2023 to February 2024 at the pediatric outpatient clinic of the
Professor Alberto Antunes University Hospital of the Federal University of Alagoas. A non-probabilistic convenience sampling was used, including patients aged 0 to 18 years diagnosed with cystic fibrosis. Demographic, socioeconomic, clinical, anthropometric, and breastfeeding variables were collected,
with a p-value <0.05 being used for statistical significance.
Results: Twenty-four patients participated in the study,
50% (n=12) female. Most were diagnosed before 6 months of age (91%; n=20), and of these, 45% (n=9) were diagnosed within the first month of life. Percentages of 9.5%, 13.6% and 19.0% of children with Z-scores below -2SD for BMI/A (¯x±SD=-2.5±0.19), W/A ¯x±SD=-3.15±0.73) and H/A (¯x±SD=-2.8±0.7), respectively, were identified. Exclusive breastfeeding until 6 months of age was reported by 23.8% of guardians (n=5). Vitamin D was used by 91.7% (n=22) of the sample and these had higher W/A scores (-0.01±1.4 vs. -3.19±1.0; p=0.013). A BMI/A ratio below -2 SD was associated with a longer median time to diagnosis (37.5 months vs. 1 month; p=0.046), and a H/A ratio below -2 SD was associated with a shorter duration of exclusive breastfeeding
(1 month vs. 3 months; p=0.014).
Conclusion: Significant percentages of children and ado
lescents with Z-scores below -2 SD for W/A, H/A, and BMI/A were found, demonstrating associations between anthropometric indicators and clinical and breastfeeding variables.
Continuous anthropometric monitoring of children living with cystic fibrosis is important, as is the identification of risk factors for nutritional status deviations, aiming to prevent or treat situations that may lead to a worse quality of life and prognosis of the disease.
Referencias
Grupo Brasileiro de Estudos de Fibrose Cística. Registro Brasileiro de Fibrose Cística (REBRAFC) ano 2021. GBEFC: São Paulo, 2021.
Castellani C, Linnane B, Pranke I, Cresta F, Sermet-Gaudelus I, Peckham D. Cystic Fibrosis Diagnosis in Newborns, Children, and Adults. Seminars in Respiratory and Critical Care Medicine. 2019 Nov 3;40(06):701–14.
Errante PR, Cintra HC. Aspectos Fisiopatológicos, diagnóstico e tratamento da fibrose cística. UNILUS Ensino e Pesquisa. 2018;14(37):166-178.
Hauschild DB, Rosa AF, Ventura JC, Barbosa E, Moreira EAM, Ludwig Neto N, Moreno YMF. Associação do estado nutricional com função pulmonar e morbidade em crianças e adolescentes com Fibrose Cística: Coorte de 36 meses. Revista Paulista de Pediatria. 2018;36:31-38.
Wilschanski M, Munck A, Carrion E, Cipolli M, Collins S, Colombo C, et al. ESPEN-ESPGHAN-ECFS guideline on nutrition care for cystic fibrosis. Clinical Nutrition. 2024;43(2):413-445.
Brasil. Saúde da criança: aleitamento materno e alimentação complementar. 2. ed. Brasília.: Ministério da Saúde, Secretaria de Atenção à Saúde, Departamento de Atenção Básica; 2015. p. 184.
Brighente LS. Associação de fatores dietéticos com o crescimento nos dois primeiros anos de vida em crianças com fibrose cística: uma revisão sistemática [trabalho de conclusão de curso]. Florianópolis: Universidade Federal de Santa Catarina; 2021.
Brownell JN, Bashaw H, Stallings VA. Growth and Nutrition in Cystic Fibrosis. Seminars in Respiratory and Critical Care Medicine. 2019 Oct 28;40(06):775–91.
Sanità D. WHO child growth standards : length/height-for-age, weight-for-age, weight-for-length, weight-for-height and body mass index-for-age : methods and development. Geneva: World Health Organization; 2006.
Martins JP, Forte GC, Simon MIS dos S, Epifanio M, Pinto LA, Marostica PJC. The role of neonatal screening in nutritional evolution in the first 12 months after diagnosis of cystic fibrosis. Revista da Associação Médica Brasileira. 2018 Nov;64(11):1032–7.
Strandvik B. Nutrition in Cystic Fibrosis—Some Notes on the Fat Recommendations. Nutrients. 2022 Jan 1;14(4):853.
Rana M, Wong-See D, Katz T, Gaskin K, Whitehead B, Jaffe A, et al. Fat-soluble vitamin deficiency in children and adolescents with cystic fibrosis. Journal of clinical pathology. 2014;67(7):605-608.
Mendonça M, Souza G, Roriz J, Calapodopulos N, Vírgina Weffort. Correlation between serum levels of vitamin D and BMI of children and adolescents attended in a university outpatient clinic. Residência Pediátrica. 2023 Jan 1 [cited 2025 Feb 22];13(2).
Neri L de CL, Simon MIS dos S, Ambrósio VLS, Barbosa E, Garcia MF, Mauri JF, et al. Brazilian Guidelines for Nutrition in Cystic Fibrosis. Einstein (São Paulo). 2022;20:eRW5686.
Sociedade Brasileira De Pediatria SBDP. Terapia Nutricional da Criança com Fibrose Cística.: Departamentos Científicos de Suporte Nutricional e Pneumologia (2019-2021); 2021.
Zirke AB. Associação entre o estado nutricional e complicações clínicas em pacientes com fibrose cística no primeiro ano de vida. Santa Catarina. Trabalho de Conclusão de Curso [Graduação em Nutrição] - Universidade Federal de Santa Catarina; 2022.
Colombo C, Costantini D, Zazzeron L, Faelli N, Russo MC, Ghisleni D, et al. Benefits of breastfeeding in cystic fibrosis: a single‐centre follow‐up survey. Acta Paediatrica. 2007;96(8):1228-1232.
Fuhrmann MSA. Influência da modalidade dietética e estado nutricional na hospitalização nos primeiros 2 anos de vida de crianças com fibrose cística. 2023.
Universidade Federal Do Rio De Janeiro UFRJ. Aleitamento materno: prevalência e práticas de aleitamento materno em crianças brasileiras menores de 2 anos 4: ENANI 2019. Universidade Federal do Rio de Janeiro; 2021.
Jonsdottir OH, Kleinman RE, Wells JC, Fewtrell MS, Hibberd PL, Gunnlaugsson G, Thorsdottir I. Exclusive breastfeeding for 4 versus 6 months and growth in early childhood. Acta Paediatrica. 2014;103(1):105-111.
Colombo C, Alicandro G, Daccò V, Consales A, Mosca F, Agostoni C, et al. Breastfeeding in Cystic Fibrosis: A Systematic Review on Prevalence and Potential Benefits. Nutrients. 2021 Sep 18;13(9):3263.
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