Standards of Nutritional Care for Patients with Cystic Fibrosis: A Methodological Primer and AGREE II Analysis of Guidelines

Maria G Grammatikopoulou; Tonia Vassilakou; Dimitrios G Goulis; Xenophon Theodoridis; Meletios P Nigdelis; Arianna Petalidou; Konstantinos Gkiouras; Dimitrios Poulimeneas; Olga Alexatou; Kyriaki Tsiroukidou; Georgios Marakis; Zoe Daniil; Dimitrios P Bogdanos

doi:10.3390/children8121180

Standards of Nutritional Care for Patients with Cystic Fibrosis: A Methodological Primer and AGREE II Analysis of Guidelines

Children (Basel). 2021 Dec 14;8(12):1180. doi: 10.3390/children8121180.

Authors

Maria G Grammatikopoulou^{1

2

3

4}, Tonia Vassilakou⁵, Dimitrios G Goulis³, Xenophon Theodoridis⁶, Meletios P Nigdelis³, Arianna Petalidou², Konstantinos Gkiouras^{2

6}, Dimitrios Poulimeneas⁷, Olga Alexatou⁷, Kyriaki Tsiroukidou⁴, Georgios Marakis⁸, Zoe Daniil⁹, Dimitrios P Bogdanos²

Affiliations

¹ Department of Nutritional Sciences & Dietetics, Faculty of Health Sciences, Alexander Campus, International Hellenic University, 57400 Thessaloniki, Greece.
² Department of Rheumatology and Clinical Immunology, Faculty of Medicine, School of Health Sciences, University of Thessaly, 41110 Larissa, Greece.
³ Unit of Reproductive Endocrinology, 1st Department of Obstetrics and Gynecology, Papageorgiou General Hospital, Medical School, Aristotle University of Thessaloniki, 56429 Thessaloniki, Greece.
⁴ 3rd Department of Pediatrics, Hippokration General Hospital of Thessaloniki, Aristotle University of Thessaloniki, 54124 Thessaloniki, Greece.
⁵ Department of Public Health Policy, School of Public Health, University of West Attica, Athens University Campus, 11521 Athens, Greece.
⁶ Medical School, Faculty of Health Sciences, Aristotle University of Thessaloniki, 54124 Thessaloniki, Greece.
⁷ Department of Nutrition & Dietetics, Harokopio University, 17676 Athens, Greece.
⁸ Nutrition and Food Standards Unit, Risk Assessment and Nutrition Directorate, Hellenic Food Authority, 11526 Athens, Greece.
⁹ Department of Respiratory Medicine, Faculty of Medicine, School of Health Sciences, University of Thessaly, 41110 Larissa, Greece.

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.

Keywords: clinical practice; clinical practice guidelines; critical appraisal; evidence-based dietetics; medical nutrition therapy; nutrition intervention; nutrition recommendation; pulmonary function; pulmonology.