Dietary Intake Assessment in Children with Cystic Fibrosis Using 3-day Food Diaries: a Single-centre Study

Overview

Journal Front Pediatr

Specialty Pediatrics

Date 2023 May 25

PMID 37228437

Authors

Margaux Gaschignard

Fabien Beaufils

Pauline Gallet

Haude Clouzeau

Joris Menard

Aurelie Costanzo

Lucie Nouard

Laurence Delhaes

Candice Tetard

Thierry Lamireau

Michael Fayon

Stephanie Bui

Raphael Enaud

Affiliations

Soon will be listed here.

Abstract

Background: Malnutrition is both a feature and major cause of morbidity in cystic fibrosis (CF). Therefore, nutritional management is an essential element of patient care. In 2016, an international guideline for nutritional management in patients with CF was published. In light of these recommendations, the aim of this study was to investigate the dietary intake of children with CF at the University Hospital of Bordeaux.

Methods: We conducted a retrospective study at the Paediatric CF Centre of the University Hospital of Bordeaux. Patients aged 2-18 years with CF who completed a 3-day food diary at home between January 2015 and December 2020 were included.

Results: A total of 130 patients, with a median age of 11.8 [interquartile range (IQR): 8.3; 13.4] years, were included. The median Z-score for BMI was -0.35 (IQR: -0.9; 0.2) and 20% of the patients had a -score for BMI < -1. Recommended total energy intakes were achieved in 53% of the patients, particularly those with nutritional support. Recommended protein intake was met in 28% of the cases, while fat and carbohydrate intakes were met in 54%. Vitamin and micronutrient levels were normal in 80% of the patients, with the exception of vitamin K, which was within the therapeutic range in only 42% of the cases.

Conclusion: Recommended nutritional targets are difficult to achieve in patients with CF, and providing nutritional support during follow-up remains a challenge.

References

Keating D, Marigowda G, Burr L, Daines C, Mall M, McKone E . VX-445-Tezacaftor-Ivacaftor in Patients with Cystic Fibrosis and One or Two Phe508del Alleles. N Engl J Med. 2018; 379(17):1612-1620. PMC: 6289290. DOI: 10.1056/NEJMoa1807120. View

Culhane S, George C, Pearo B, Spoede E . Malnutrition in cystic fibrosis: a review. Nutr Clin Pract. 2013; 28(6):676-83. DOI: 10.1177/0884533613507086. View

Declercq D, Van Meerhaeghe S, Marchand S, Van Braeckel E, Van Daele S, De Baets F . The nutritional status in CF: Being certain about the uncertainties. Clin Nutr ESPEN. 2019; 29:15-21. DOI: 10.1016/j.clnesp.2018.10.009. View

Sullivan J, Mascarenhas M . Nutrition: Prevention and management of nutritional failure in Cystic Fibrosis. J Cyst Fibros. 2017; 16 Suppl 2:S87-S93. DOI: 10.1016/j.jcf.2017.07.010. View

Sinaasappel M, Stern M, Littlewood J, Wolfe S, Steinkamp G, Heijerman H . Nutrition in patients with cystic fibrosis: a European Consensus. J Cyst Fibros. 2004; 1(2):51-75. DOI: 10.1016/s1569-1993(02)00032-2. View

Brownell J, Bashaw H, Stallings V . Growth and Nutrition in Cystic Fibrosis. Semin Respir Crit Care Med. 2019; 40(6):775-791. DOI: 10.1055/s-0039-1696726. View

Bailey J, Rozga M, McDonald C, Bowser E, Farnham K, Mangus M . Effect of CFTR Modulators on Anthropometric Parameters in Individuals with Cystic Fibrosis: An Evidence Analysis Center Systematic Review. J Acad Nutr Diet. 2020; 121(7):1364-1378.e2. DOI: 10.1016/j.jand.2020.03.014. View

OSullivan B, Freedman S . Cystic fibrosis. Lancet. 2009; 373(9678):1891-904. DOI: 10.1016/S0140-6736(09)60327-5. View

Shepherd R, Holt T, Cleghorn G, Ward L, Isles A, Francis P . Short-term nutritional supplementation during management of pulmonary exacerbations in cystic fibrosis: a controlled study, including effects of protein turnover. Am J Clin Nutr. 1988; 48(2):235-9. DOI: 10.1093/ajcn/48.2.235. View

10.

Borowitz D, Lubarsky B, Wilschanski M, Munck A, Gelfond D, Bodewes F . Nutritional Status Improved in Cystic Fibrosis Patients with the G551D Mutation After Treatment with Ivacaftor. Dig Dis Sci. 2015; 61(1):198-207. DOI: 10.1007/s10620-015-3834-2. View

11.

Turck D, Braegger C, Colombo C, Declercq D, Morton A, Pancheva R . ESPEN-ESPGHAN-ECFS guidelines on nutrition care for infants, children, and adults with cystic fibrosis. Clin Nutr. 2016; 35(3):557-77. DOI: 10.1016/j.clnu.2016.03.004. View

12.

Stallings V, Stark L, Robinson K, Feranchak A, Quinton H . Evidence-based practice recommendations for nutrition-related management of children and adults with cystic fibrosis and pancreatic insufficiency: results of a systematic review. J Am Diet Assoc. 2008; 108(5):832-9. DOI: 10.1016/j.jada.2008.02.020. View

13.

Engelen M, Com G, Deutz N . Protein is an important but undervalued macronutrient in the nutritional care of patients with cystic fibrosis. Curr Opin Clin Nutr Metab Care. 2014; 17(6):515-20. PMC: 5241003. DOI: 10.1097/MCO.0000000000000100. View

14.

. Poster Session Abstracts. Pediatr Pulmonol. 2016; 51(S45):S194-S485. PMC: 7159391. DOI: 10.1002/ppul.23576. View

15.

Rettammel A, Marcus M, Farrell P, Sondel S, Koscik R, Mischler E . Oral supplementation with a high-fat, high-energy product improves nutritional status and alters serum lipids in patients with cystic fibrosis. J Am Diet Assoc. 1995; 95(4):454-9. DOI: 10.1016/S0002-8223(95)00121-2. View

16.

Konstan M, McKone E, Moss R, Marigowda G, Tian S, Waltz D . Assessment of safety and efficacy of long-term treatment with combination lumacaftor and ivacaftor therapy in patients with cystic fibrosis homozygous for the F508del-CFTR mutation (PROGRESS): a phase 3, extension study. Lancet Respir Med. 2016; 5(2):107-118. DOI: 10.1016/S2213-2600(16)30427-1. View

17.

Poulimeneas D, Grammatikopoulou M, Devetzi P, Petrocheilou A, Kaditis A, Papamitsou T . Adherence to Dietary Recommendations, Nutrient Intake Adequacy and Diet Quality among Pediatric Cystic Fibrosis Patients: Results from the GreeCF Study. Nutrients. 2020; 12(10). PMC: 7602117. DOI: 10.3390/nu12103126. View

18.

Borowitz D, Durie P, Clarke L, Werlin S, Taylor C, Semler J . Gastrointestinal outcomes and confounders in cystic fibrosis. J Pediatr Gastroenterol Nutr. 2005; 41(3):273-85. DOI: 10.1097/01.mpg.0000178439.64675.8d. View

19.

Farrell P, Kosorok M, Rock M, Laxova A, Zeng L, Lai H . Early diagnosis of cystic fibrosis through neonatal screening prevents severe malnutrition and improves long-term growth. Wisconsin Cystic Fibrosis Neonatal Screening Study Group. Pediatrics. 2001; 107(1):1-13. DOI: 10.1542/peds.107.1.1. View

20.

Maqbool A, Schall J, Gallagher P, Zemel B, Strandvik B, Stallings V . Relation between dietary fat intake type and serum fatty acid status in children with cystic fibrosis. J Pediatr Gastroenterol Nutr. 2012; 55(5):605-11. PMC: 4699309. DOI: 10.1097/MPG.0b013e3182618f33. View