{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,7]],"date-time":"2026-07-07T09:19:24Z","timestamp":1783415964140,"version":"3.54.6"},"reference-count":37,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2012,7,1]],"date-time":"2012-07-01T00:00:00Z","timestamp":1341100800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["J. pediatr. gastroenterol. nutr."],"published-print":{"date-parts":[[2012,7]]},"abstract":"<jats:title>ABSTRACT<\/jats:title><jats:sec><jats:title>Background and Aim:<\/jats:title><jats:p>Patients with cystic fibrosis (CF) have low levels of n\u20103 long\u2010chain polyunsaturated fatty acids (n\u20103 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)\u2010regulated tissues such as granulocytes, monocytes, and lymphocytes. The aim of the present study was to investigate whether patients with CF\u2010related liver disease have lower n\u20103 LCPUFA level than patients with CF without liver disease.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods:<\/jats:title><jats:p>Twenty patients with known CF\u2010related 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.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results:<\/jats:title><jats:p>No significant differences were seen in either n\u20106 or n\u20103 LCPUFAs in any cell line when the 2 groups were compared. In a multiple regression analysis including HTT, age, <jats:italic>Pseudomonas aeruginosa<\/jats:italic> infection, diabetes mellitus, treatment with ursodeoxycholic acid, forced expiratory volume in 1 second (% of predicted value), and Williams' ultrasound scoring scale, only n\u20103 LCPUFA docosahexaenoic acid in mononuclear cell membranes was positively associated with HTT (<jats:italic>P<\/jats:italic> = 0.02). The arachidonic acid\/docosahexaenoic acid ratio within the mononuclear cells was negatively associated with both HTT (<jats:italic>P<\/jats:italic> = 0.003) and Williams' ultrasound scoring scale (<jats:italic>P<\/jats:italic> = 0.03). For RBC\u2010LCPUFAs, no significant associations were seen.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions:<\/jats:title><jats:p>These findings indicate that in patients with CF, the degree of liver disease was negatively associated with LCPUFA n\u20103 levels in CFTR\u2010expressing white blood cells but unrelated to those levels in CFTR\u2010negative RBC.<\/jats:p><\/jats:sec>","DOI":"10.1097\/mpg.0b013e318249438c","type":"journal-article","created":{"date-parts":[[2012,1,11]],"date-time":"2012-01-11T08:47:59Z","timestamp":1326271679000},"page":"76-81","source":"Crossref","is-referenced-by-count":5,"title":["Long\u2010chain PUFA in Granulocytes, Mononuclear Cells, and RBC in Patients With Cystic Fibrosis"],"prefix":"10.1002","volume":"55","author":[{"given":"Marianne H.","family":"J\u00f8rgensen","sequence":"first","affiliation":[{"name":"Department of Paediatrics, Rigshospitalet 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