title: Long-chain PUFA in granulocytes, mononuclear cells, and RBC in patients with cystic fibrosis: relation to liver disease.
abstract: OBJECTIVE
  Patients with cystic fibrosis (CF) have low levels of n-3 long-chain polyunsaturated fatty acids (n-3 LCPUFA) in plasma or red blood cells (RBC), as also seen in other chronic and acute liver diseases. The differences may be more pronounced in CF transmembrane conductance regulator protein (CFTR)-regulated tissues such as granulocytes, monocytes, and lymphocytes. The aim of the present study was to investigate whether patients with CF-related liver disease have lower n-3 LCPUFA level than patients with CF without liver disease.
  
  
  METHODS
  Twenty patients with known CF-related liver disease were matched with 20 CF patients without. Blood samples were analysed for liver biochemistry and haematology. Granulocytes, mononuclear cells, and RBC were separated by density gradient centrifugation, and fatty acid composition was measured by gas chromatography. Hepatic ultrasound was scored according to Williams et al. Hepatic transit time (HTT) was measured with the ultrasound contrast agent SonoVue.
  
  
  RESULTS
  No significant differences were seen in either n-6 or n-3 LCPUFAs in any cell line when the 2 groups were compared. In a multiple regression analysis including HTT, age, Pseudomonas aeruginosa infection, diabetes mellitus, treatment with ursodeoxycholic acid, forced expiratory volume in 1 second (% of predicted value), and Williams' ultrasound scoring scale, only n-3 LCPUFA docosahexaenoic acid in mononuclear cell membranes was positively associated with HTT (P = 0.02). The arachidonic acid/docosahexaenoic acid ratio within the mononuclear cells was negatively associated with both HTT (P = 0.003) and Williams' ultrasound scoring scale (P = 0.03). For RBC-LCPUFAs, no significant associations were seen.
  
  
  CONCLUSIONS
  These findings indicate that in patients with CF, the degree of liver disease was negatively associated with LCPUFA n-3 levels in CFTR-expressing white blood cells but unrelated to those levels in CFTR-negative RBC.
authors.affiliation: Department of Paediatrics, Rigshospitalet, Copenhagen, Denmark. mari@dadlnet.dk
authors.forename: Marianne H
authors.initials: MH
authors.lastname: Jørgensen
authors.forename: Peter
authors.initials: P
authors.lastname: Ott
authors.forename: Kim F
authors.initials: KF
authors.lastname: Michaelsen
authors.forename: Trine
authors.initials: T
authors.lastname: Porsgaard
authors.forename: Fleming
authors.initials: F
authors.lastname: Jensen
authors.forename: Susanne
authors.initials: S
authors.lastname: Lanng
mesh_terms: D000293:Adolescent; D000328:Adult; D000410:Alanine Transaminase / Q000097:blood; D000818:Animals; D016718:Arachidonic Acid / Q000032:analysis; D001219:Aspartate Aminotransferases / Q000097:blood; D001663:Bilirubin / Q000097:blood; D002648:Child; D002675:Child, Preschool; D003550:Cystic Fibrosis / Q000097:blood* / Q000150:complications; D004032:Diet; D004281:Docosahexaenoic Acids / Q000032:analysis; D015118:Eicosapentaenoic Acid / Q000032:analysis; D004912:Erythrocytes / Q000737:chemistry*; D015525:Fatty Acids, Omega-3; D005231:Fatty Acids, Unsaturated / Q000032:analysis*; D005260:Female; D005399:Fishes; D006098:Granulocytes / Q000737:chemistry*; D006801:Humans; D007963:Leukocytes, Mononuclear / Q000737:chemistry*; D008107:Liver Diseases / Q000097:blood* / Q000150:complications / Q000000981:diagnostic imaging; D008297:Male; D019301:Oleic Acid / Q000032:analysis; D011795:Surveys and Questionnaires; D014463:Ultrasonography; D055815:Young Adult
chemical_list: D015525:Fatty Acids, Omega-3; D005231:Fatty Acids, Unsaturated; D004281:Docosahexaenoic Acids; D016718:Arachidonic Acid; D019301:Oleic Acid; D015118:Eicosapentaenoic Acid; D001219:Aspartate Aminotransferases; D000410:Alanine Transaminase; D001663:Bilirubin