The Role of Nutrition and Pancreatic Enzyme Replacement Therapy in Children with Cystic Fibrosis: A Systematic Review

Abstract

Background: Cystic fibrosis (CF) is a genetic disease that seriously affects the respiratory and digestive tracts, predominantly on the nutritional and growth of the baby. Nutritional support and PERT are essential for achieving the expected growth and enhancing the quality of life of children with CF. It has been reported that several such interventions have been examined in different studies conducted during the recent years aimed at testing the effectiveness of these types of interventions for children and adolescents. Objective: This review will compare the effects of nutritional interventions and PERT on growth, nutritional status, digestive function, and general quality of life of children suffering from cystic fibrosis. The review also aims at making a contrast of various forms of nutritional support, doses, and formulation of PERT. Methodology: This synthesized work is oriented from the current literature database research that is from the period of January 2017 to January 2022. Specific criteria that were employed to define inclusion were that the studies had to contain children with CF aged between 0-18 years, in whom the intervention being practiced was either nutritional support and/or PERT. RCTs, cohort, case-control, and cross-sectional with publication language restricted to English only were included. Studies with the adult populations but without the separate analysis of the children population were excluded; studies conducted without any kind of intervention; articles with no access to full text. Results: The review also aimed at screening and organizing 30 studies for inclusion. These findings suggest that nutritional interventions and PERT have a positive effect in enhancing the growth features of a group of children with CF, most notably BMI and weight-for-age z-scores. Further, all these interventions identified were found to have a positive impact on the digestive function as well as the overall quality of life. It was found that different PERT formulations and dosages had been effective in enhancing the therapeutic results, and it established that an individualized PERT dosage regimen was preferable. Conclusion: Nutrition therapy and PERT play a critical role in the management of cystic fibrosis in children that comes with enhanced growth, nutritional well-being as well as improved quality of life. In these and related studies, it is evident that providing such interventions in a more person-centered way may help to promote their benefits. Future work is suggested to enhance these approaches and the investigation of long-term effects.