Research

Long-chain PUFA in Granulocytes, Mononuclear Cells, and RBC in Patients With Cystic Fibrosis: Relation to Liver Disease

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Abstract

Background and Aim: 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.

cystic fibrosis Cystic Fibrosis (MeSH) respiratory system disease genetic disease congenital disease digestive system disease diabetes mellitus Diabetes Mellitus (MeSH) endocrine disease/pancreas metabolic disease liver disease Liver Diseases (MeSH) digestive system disease Pseudomonas infection bacterial disease Gram-Negative Aerobic Rods and Cocci Eubacteria Bacteria Microorganisms (Bacteria Eubacteria Microorganisms) - Pseudomonadaceae [06508] Pseudomonas aeruginosa species pathogen Primates Mammalia Vertebrata Chordata Animalia (Animals Chordates Humans Mammals Primates Vertebrates) - Hominidae [86215] human common child preadolescent child adolescent host female CF transmembrane conductance regulator protein CFTR docosahexaenoic acid 32839-18-2 PUFA SonoVue 2551-62-4 ursodeoxycholic acid 128-13-2 02506 Cytology - Animal 02508 Cytology - Human 03508 Genetics - Human 10066 Biochemistry studies - Lipids 10067 Biochemistry studies - Sterols and steroids 13002 Metabolism - General metabolism and metabolic pathways 13020 Metabolism - Metabolic disorders 14006 Digestive system - Pathology 15002 Blood - Blood and lymph studies 15004 Blood - Blood cell studies 16006 Respiratory system - Pathology 17002 Endocrine - General 17008 Endocrine - Pancreas 25000 Pediatrics 25503 Development and Embryology - Pathology 31000 Physiology and biochemistry of bacteria 34502 Immunology - General and methods 36002 Medical and clinical microbiology - Bacteriology Allied Medical Sciences Human Medicine Medical Sciences granulocyte immune system blood and lymphatics lymphocyte immune system blood and lymphatics monocyte immune system blood and lymphatics mononuclear cell immune system blood and lymphatics plasma blood and lymphatics red blood cell blood and lymphatics density gradient centrifugation laboratory techniques gas chromatography laboratory techniques chromatographic techniques hepatic ultrasound clinical techniques diagnostic techniques multiple regression analysis mathematical and computer techniques Clinical Endocrinology Gastroenterology Medical Genetics Metabolism Pediatrics