The role of nutrition and pancreatic enzyme replacement therapy in children with cystic fibrosis

Main Article Content

Muzal Kadim
William Cheng

Abstract

Background Cystic fibrosis (CF) is an inherited genetic disorder with high mortality and morbidity. CF is strongly correlated with malnutrition due to higher energy losses, pancreatic insufficiency, chronic inflammation, higher resting energy expenditure, and feeding problems. Malnutrition in CF patients associated with worse survival. Thus, appropriate and prompt nutritional intervention should be addressed to reduced malnutrition in CF patients.
Methods The literature search was performed on 9 August 2021 in four major databases such as MEDLINE, EBSCOhost, Cochrane Reviews, and Web of Sciences to find the role of nutrition and pancreatic enzyme replacement therapy in pediatrics population with cystic fibrosis.
Recent findings In recent decades, early nutritional management and pancreatic enzyme replacement therapy (PERT) have been shown to improve CF patient’s outcomes. Nutrition should be given in higher calories compared to healthy individuals with close and regular nutritional status monitoring. High protein and fat diets are essential for CF patient’s overall survival. Adequate level of micronutrients should be ensured to avoid morbidity caused by micronutrients deficiency. Regular pancreatic insufficiency screening should be done annually in order to start PERT early.  Further research focusing on body composition, growth chart, protein intake, and PERT are needed to further improve the management of CF patient.
Conclusion Nutritional intervention and PERT play an important role in prolonging CF patient survival. Both treatments should be initiated early with nutritional status close monitoring and tailored to each individual. Collaboration with parents and children is critical to warrant that CF patients followed the dietary advice.

Downloads

Download data is not yet available.

Article Details

Section
Articles
Author Biography

Muzal Kadim, 1. Division of gastrohepatology, Child Health Department, Faculty of Medicine, Universitas Indonesia-Cipto Mangunkusumo Hospital, Jakarta, Indonesia

Gastrohepatology Division, Child Health Department, University of Indonesia RSCM Hospital

References

Naehrig S, Chao CM, Naehrlich L. Cystic Fibrosis. Dtsch Arztebl Int. 2017;114:564-74.

Cutting GR EJ, Zeitlin PL. Genetics and pathophysiology of cystic fibrosis. In: Kendig’s Disorders of the Respiratory Tract in Children. Elsevier. 2019:p. 757-68.

Park HW LM. Transepithelial bicarbonate secretion: lessons from the pancreas. Cold Spring Harb Perspect Med. 2012;2:1-7.

PB D. Cystic fibrosis since 1938. Am J Respir Crit Care Med. 2006;173(5):475–82.

Zolin A OA, Naehrlich L, Jung A, van Rens J et al. ECFS Patient Registry Denmark: European Cystic Fibrosis Society; 2018.

Li L SS. Digestive system dysfunction in cystic fibrosis: challenges for nutrition therapy. Dig Liver Dis. 2014;46(10):865–74.

Leung DH, Narkewicz MR. Cystic Fibrosis-related cirrhosis. J Cyst Fibros. 2017;16 Suppl 2:50-61.

Marshall B FA, Fink A, Loeffler D, Elbert A, O'Neil T et al. 2017 Patient Registry Annual Data Report. Bethesda, Maryland: Cystic Fibrosis Foundation; 2017.

Tridello G, Castellani C, Meneghelli I, Tamanini A, Assael BM. Early diagnosis from newborn screening maximises survival in severe cystic fibrosis. ERJ Open Res. 2018;4:1-5.

Sanders DB ZZ, Farrell PM, Lai HJ. Early life growth patterns persist for 12 years and impact pulmonary outcomes in cystic fibrosis. 2018;17(4):528–35.

Turck D, Braegger CP, Colombo C, Declercq D, Morton A, Pancheva R, et al. ESPEN-ESPGHAN-ECFS guidelines on nutrition care for infants, children, and adults with cystic fibrosis. Clin Nutr. 2016;35:557-77.

Charatsi AM DP, Freund R, Maruani G, Rossin H, Boulier A, et al. Bioelectrical impedance in young patients with cystic fibrosis: Validation of a specific equation and clinical relevance. J Cyst Fibros. 2016;15:825–33.

Declercq D, Van Meerhaeghe S, Marchand S, Van Braeckel E, van Daele S, De Baets F, et al. The nutritional status in CF: Being certain about the uncertainties. Clin Nutr ESPEN. 2019;29:15-21.

Calella P, Valerio G, Thomas M, McCabe H, Taylor J, Brodlie M, et al. Association between body composition and pulmonary function in children and young people with cystic fibrosis. Nutrition. 2018;48:73-6.

Engelen MP, Com G, Deutz NE. Protein is an important but undervalued macronutrient in the nutritional care of patients with cystic fibrosis. Curr Opin Clin Nutr Metab Care. 2014;17:515-20.

Kaminski BA, Goldsweig BK, Sidhaye A, Blackman SM, Schindler T, Moran A. Cystic fibrosis related diabetes: Nutrition and growth considerations. J Cyst Fibros. 2019;18 Suppl 2:S32-S7.

Maqbool A G-MR, Schall JI, Zemel BS, Stallings VA. Vitamin A intake and elevated serum retinol levels in children and young adults with cystic fibrosis. J Cyst Fibros. 2008;7:137–41.

Singh VK, Schwarzenberg SJ. Pancreatic insufficiency in Cystic Fibrosis. J Cyst Fibros. 2017;16:70-8.

Brownell JN, Bashaw H, Stallings VA. Growth and Nutrition in Cystic Fibrosis. Semin Respir Crit Care Med. 2019;40(6):775-91.

Gelfond D, Heltshe SL, Skalland M, Heubi JE, Kloster M, Leung DH, et al. Pancreatic Enzyme Replacement Therapy Use in Infants With Cystic Fibrosis Diagnosed by Newborn Screening. J Pediatr Gastroenterol Nutr. 2018;66:657-63.