Nutritional care and support should be an integral part of management of CF. Obtaining a normal growth pattern in children and maintaining an adequate nutritional status in adults are major goals of multidisciplinary cystic fibrosis centers.

Objective: To evaluate the relation of clinical parameters and genotype with the serum phospholipid fatty acid (FA) composition in cystic fibrosis (CF) patients. Methods: A blood sample was taken from CF patients with stable pulmonary disease for the determination of phospholipid FA composition and vitamin E concentration who had been followed for at least 6 months at our Cystic Fibrosis Centre. Genotype, age, pancreatic function, nutritional status, caloric intake, pulmonary function and presence of Pseudomonas colonization, liver disease or diabetes mellitus were recorded. Patients were divided into two groups according to their genotype (group A: mutation class I, II, or III, group B: mutation class IV, V). Results: CF patients (group A and B together) have significantly lower docosahexaenoic acid (DHA) (p < 0.007) and linoleic acid (LA) (p < 0.0001) and higher dihomogammalinolenic acid (DHGLA) (p < 0.0001), oleic acid (OA) (p < 0.0001) and Mead acid (MA) (p < 0.0001), resulting in an increased ratio of arachidonic acid (AA)/DHA (p < 0.004), MA/AA (p < 0.0001) and OA/LA (p < 0.0001). Compared to group B, group A had a lower LA (p < 0.002) and a higher DHGLA (p < 0.002), 22:4ω–6 (p < 0.03), 22:5ω–6 (p < 0.03) and 20:3ω–9 (p < 0.04). There was however no significant difference between the groups for age, pulmonary function, nutritional status and vitamin E concentration. There was no relation of serum FA composition with nutritional status, caloric intake, pancreatic function, gender, pulmonary function, Pseudomonas colonization or diabetes mellitus. In CF with liver disease the DHA was lower than in the patients of the same genotype. Conclusion: FA disturbances are more pronounced in the severe CF genotypes and the presence of CF-related liver disease. Future studies on supplementation should take these parameters into account.

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T he European Society for Clinical Nutrition and Metabolism (ESPEN) launched a process of developing updated guidelines on nutrition care for infants, children, and adults with cystic fibrosis (CF). To that end, a group of experts systematically reviewed the medical literature to summarize current knowledge on epidemiology and pathophysiology, prevention, and treatment of CF-related undernutrition. The group included representatives from several European countries, physicians, dietitians, and educators, all experts in the field of CF, and the guidelines coordinator. The group recommended evidence-based guidelines on nutrition care for infants, children, and adults with CF. The experts followed the GRADE method, which was based on determinations of grade of evidence and strength of recommendation. The European Society for Paediatric Gastroenterology, Hepatology and Nutrition (ESP-GHAN) and the European Society for Cystic Fibrosis (ECFS), who are partners of these guidelines, reviewed the final manuscript, with an external review for ESPGHAN. The reader of the Journal is kindly invited to refer to the original article published in the June 2016 issue of Clinical Nutrition (1). This summary will outline some of the recommendations on nutrition care for infants and children with CF.

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