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Expert Rev Respir Med. 2019 Jun;13(6):533-544. doi: 10.1080/17476348.2019.1614917. Epub 2019 May 16.

Challenges with optimizing nutrition in cystic fibrosis.

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a Department of Pathophysiology and Transplantation , Università degli Studi di Milano , Milan , Italy.
b Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Cystic Fibrosis Centre , Milan , Italy.
c Department of Clinical Sciences and Community Health , Università degli Studi di Milano , Milano , Italy.


Introduction. Optimizing nutrition remains the cornerstone of therapy for patients with cystic fibrosis (CF) since it is associated with better pulmonary function and survival. However, a significant proportion of patients still fail to achieve normal growth and nutritional status. Areas covered. This review describes the current challenges in providing effective nutritional therapy in CF with a focus on the current issues related to energy imbalance, dietary composition, adherence to nutritional recommendations, pancreatic enzyme replacement therapy, and the effects of modulators of the CF transmembrane conductance regulator. Expert opinion. CF is a multisystemic disease that requires a personalized nutritional approach with accurate evaluation of energy balance. There is an urgent need for evidence-based recommendations on the dietary composition, in consideration of the increasing prevalence of overweight, diabetes and the potential effects of fatty acids on inflammation and immune response. More research into new pancreatic enzyme formulations is also required.


Cystic fibrosis; adherence; diet; new therapies; nutrition

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