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

Jørgensen, Marianne H.*; Ott, Peter; Michaelsen, Kim F.; Porsgaard, Trine§; Jensen, Fleming||; Lanng, Susanne*

Journal of Pediatric Gastroenterology & Nutrition: July 2012 - Volume 55 - Issue 1 - p 76–81
doi: 10.1097/MPG.0b013e318249438c
Original Articles: Hepatology and Nutrition

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.

*Department of Paediatrics, Rigshospitalet, Copenhagen

Department of Hepatology and Gastroenterology, Aarhus University Hospital, Aarhus

Department of Human Nutrition, Faculty of Life Sciences, University of Copenhagen, Frederiksberg

§Biocentrum-DTU, Technical University of Denmark, Lyngby

||Ultrasound section, Rigshospitalet, Copenhagen, Denmark.

Address correspondence and reprint requests to Marianne H. Jørgensen, MD, PhD, Department of Paediatrics, 4072 JMC, University Hospital of Copenhagen, Rigshospitalet, Blegdamsvej 3, 2100 Copenhagen, Denmark (e-mail:

Received 29 March, 2011

Accepted 29 November, 2011

The present study received funding from the Danish Cystic Fibrosis Association Hyrdebakken 246 DK-8800 Viborg.

The authors report no conflicts of interest.

Copyright 2012 by ESPGHAN and NASPGHAN