Nutrition support in children and adolescents with cystic fibrosis: Is there a difference between pancreatic sufficient and insufficient patients?
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Agia Sofia Children’s Hospital, Athens, Greece
Department of Nutrition and Dietetics, Harokopio University, Athens, Greece
Publication date: 2022-05-27
Public Health Toxicol 2022;2(Supplement 1):A89
Cystic fibrosis (CF) is the most common genetically inherited disease among the Caucasian population. Achieving and maintaining of a good nutritional status is of vital importance as it has a great impact on improving lung function in these patients1. Current guidelines2 recommend children and adolescents with CF to achieve a BMI ≥50th percentile through a High-Fat, High-Calorie (HFHC) diet. Although most patients with CF are pancreatic insufficient (PI), approximately 15% of CF patients have pancreatic sufficiency (PS), due to mild CF mutation3. As PS patients are characterized by milder disease severity compared to PI ones, it is not known whether they have different dietary needs, if not lower. In the current presentation we will discuss about the overall nutritional support of patients with CF, based on the current guidelines. We will also focus on the existing evidence about the nutritional support, weight and metabolic profile of patients with PS. Based on recent epidemiological data, children and adolescents with PS seems to have better weight status and therefore better lung function compared to PI patients4. However, their metabolic status (e.g. fat and glucose levels) were worse compared to PI patients.
Nagy R, Gede N, Ocskay K, et al. Association of Body Mass Index With Clinical Outcomes in Patients With Cystic Fibrosis: A Systematic Review and Meta-analysis. JAMA Netw Open. 2022;5(3):e220740. doi:10.1001/jamanetworkopen.2022.0740
Turck D, Braegger CP, Colombo C, et al. 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
Walkowiak J, Lisowska A, Blaszczynski M. The changing face of the exocrine pancreas in cystic fibrosis: pancreatic sufficiency, pancreatitis and genotype. Eur J Gastroenterol Hepatol. 2008;20(3):164-168. doi:10.1097/MEG.0b013e3282f36d04
Madde A, Okoniewski W, Sanders DB, Ren CL, Weiner DJ, Forno E. Nutritional status and lung function in children with pancreatic-sufficient cystic fibrosis. J Cyst Fibros. 2021;S1569-1993(21)02173-1. doi:10.1016/j.jcf.2021.12.014