title: Dietary and circulating polyunsaturated fatty acids in cystic fibrosis: are they related to clinical outcomes?
abstract: OBJECTIVE
  To assess the relationship between dietary intakes, plasma phospholipid (PL) fatty acid profile and clinical parameters in children with cystic fibrosis (CF) in comparison to healthy controls.
  
  
  METHODS
  A cross-sectional survey including 37 patients with CF (ages 8.0 +/- 2.9 yrs) and a reference group of 68 healthy children (ages 8.0 +/- 0.7 yrs) was carried out by means of a food-frequency questionnaire. At enrollment, all subjects underwent blood sampling for plasma PL fatty acids (FA). In CF patients, pulmonary function tests (forced expiratory volume in 1 second and forced vital capacity), anthropometric measurements and the Shwachman score were also determined.
  
  
  RESULTS
  In CF patients, mean z score for weight and height (-0.35 +/- 1.16 and -0.28 +/- 0.99) were lower than controls (0.83 +/- 1.73 and 0.55 +/- 1.11, respectively). Patients with CF showed higher energy intakes (110 +/- 43 kcal/d) compared with controls (75 +/- 22 kcal/d; P < 0.0001), with higher intake of total (saturated and monounsaturated) fats and lower intake of polyunsaturated FA (3.9 +/- 1.0% of total macronutrient intake vs 4.3 +/- 1.2%, P = 0.05). In CF patients, plasma and PL levels of linoleic and docosahexaenoic acids were lower, whereas those of arachidonic acid were similar compared with controls. The Shwachman score showed significant positive associations with plasma PL levels of arachidonic acid and total n-6 long-chain FA (r = 0.32, P = 0.05, and r = 0.35, P = 0.03, respectively).
  
  
  CONCLUSIONS
  The data give suggestions that fat intake and CF-associated biomechanisms are bound in a vicious circle, concurring to create the clinical and biochemical picture of CF. The quantity and quality of fat supplementation in CF need careful attention to balance the fat supply with polyunsaturated FA.
authors.affiliation: Department of Pediatrics, CF Center, Fondazione IRCCS Policlinico, Mangiagalli, Regina Elena, University of Milan, Milan, Italy. carla.colombo@unimi.it
authors.forename: Carla
authors.initials: C
authors.lastname: Colombo
authors.forename: Veronica
authors.initials: V
authors.lastname: Bennato
authors.forename: Diana
authors.initials: D
authors.lastname: Costantini
authors.forename: Lauretta
authors.initials: L
authors.lastname: Valmarana
authors.forename: Valeria
authors.initials: V
authors.lastname: Daccò
authors.forename: Laura
authors.initials: L
authors.lastname: Zazzeron
authors.forename: Diana
authors.initials: D
authors.lastname: Ghisleni
authors.forename: Maria Grazia
authors.initials: MG
authors.lastname: Bruzzese
authors.forename: Silvia
authors.initials: S
authors.lastname: Scaglioni
authors.forename: Enrica
authors.initials: E
authors.lastname: Riva
authors.forename: Carlo
authors.initials: C
authors.lastname: Agostoni
mesh_terms: D000886:Anthropometry; D002648:Child; D003430:Cross-Sectional Studies; D003550:Cystic Fibrosis / Q000097:blood* / Q000178:diet therapy / Q000503:physiopathology; D004042:Dietary Fats, Unsaturated / Q000008:administration & dosage*; D002149:Energy Intake; D005231:Fatty Acids, Unsaturated / Q000097:blood*; D005247:Feeding Behavior; D005260:Female; D006801:Humans; D008297:Male; D010743:Phospholipids / Q000097:blood; D012129:Respiratory Function Tests
chemical_list: D004042:Dietary Fats, Unsaturated; D005231:Fatty Acids, Unsaturated; D010743:Phospholipids