Abstract
Although many Clinical Practice Guidelines (CPGs) have been published for the care of patients with Cystic Fibrosis (CF), including a variety of nutrition recommendations, the quality of these CPGs has never been evaluated. The aim of this study was to compare, review, and critically appraise CPGs for the nutritional management of CF, throughout the lifespan. We searched PubMed, Guidelines International Network (GIN), ECRI Institute, and Guidelines Central for CPGs, with information on the nutritional management of CF. Retrieved CPGs were appraised by three independent reviewers, using the Appraisal of Guidelines, Research and Evaluation II (AGREE II) instrument and checklist. A total of 22 CPGs (seven solely nutrition oriented), by 14 different publishers, were retrieved. The Thoracic Society of Australia and New Zealand CPGs scored the highest overall quality (94.4%), while the Paediatric Gastroenterology Society/Dietitians Association of Australia CPGs had the lowest score (27.8%). Great variation in AGREE II domain-specific scores was observed in all CPGs, suggesting the existence of different strengths and weaknesses. Despite the availability of several CPGs, many appear outdated, lacking rigor, transparency, applicability, and efficiency, while incorporating bias. Considering that CPGs adherence is associated with better outcomes and the need for improving life expectancy in patients with CF, the development of CPGs of better quality is deemed necessary.
Highlights
Cystic fibrosis (CF) is an autosomal recessive disease that is caused by mutations in the gene for the CF trans-membrane conductance regulator (CFTR), which encodes an ion channel protein, with more than 2000 mutations identified to date [1,2,3]
The present study revealed that many CF-Clinical Practice Guidelines (CPGs) incorporate medical nutrition therapy (MNT) information, while seven have CF-MNT as their main aim
The highest quality was demonstrated by the Thoracic Society of Australia and New Zealand (TSANZ) [22] CPGs, whereas the lowest score was received by the Pediatric Gastroenterology Society (PGS)/Dietitians Association of Australia (DAA) [42] CPGs, based on the AGREE II scoring system
Summary
Cystic fibrosis (CF) is an autosomal recessive disease that is caused by mutations in the gene for the CF trans-membrane conductance regulator (CFTR), which encodes an ion channel protein, with more than 2000 mutations identified to date [1,2,3]. CF affects appetite, nutritional status, and pulmonary function in a progressive manner, with all synergistically resulting in poor health outcomes [4]. Malabsorption, gastrointestinal dysfunction, genetic modifiers, and chronic and progressive pulmonary infection compromise nutritional status, resulting in growth failure [5,6,7]. As far as nutritional status and pulmonary function are concerned, a two-way relationship exists, with each one affecting the other [8]. Recent advances in medicine and supplements [9], multidisciplinary care, the use of more holistic treatment approaches, and adherence to specific dietary protocols have all levelled up the delivery of nutrition intervention among CF patients, reducing growth failure [10,11]
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