ABSTRACT
BACKGROUND: Disarrangement in fatty acids and oxidative stress are features of cystic fibrosis. Cholesterol is very sensitive to oxidative stress. OBJECTIVES: The objectives were to examine whether cholesterol oxidation products are altered in cystic fibrosis and whether they are associated with fatty acids and with characteristics of the disease state. DESIGN: 7-Ketocholesterol and 7beta-hydroxycholesterol (prototype molecules of free radical-mediated cholesterol oxidation) and the fatty acid profile were assessed by mass spectrometry in patients and in sex- and age-matched control subjects. RESULTS: In a comparison with control subjects, mean (+/-SD) cholesterol oxidation was higher (7-ketocholesterol: 11.31 +/- 5.1 compared with 8.33 +/- 5.5 ng/mL, P = 0.03; 7beta-hydroxycholesterol: 14.5 +/- 6.8 compared with 9.7 +/- 4.1 ng/mL, P = 0.004), total saturated fatty acids were higher (31.90 +/- 1.93% compared with 30.31 +/- 0.98%, P < 0.001), monounsaturated fatty acids were higher (29.14 +/- 3.85% compared with 25.88 +/- 2.94%, P = 0.004), omega-6 (n-6) polyunsaturated fatty acids were lower (34.84 +/- 4.77 compared with 39.68 +/- 2.98%, P < 0.0001), and omega-3 (n-3) polyunsaturated fatty acids were comparable in patients with cystic fibrosis. Oxysterols were inversely associated with 24:0 and 18:2 omega-6 fatty acids but did not correlate with the increased oleic acid or with any of the omega-3 fatty acids. CONCLUSIONS: Cystic fibrosis is characterized by relevant cholesterol oxidation that is associated with an abnormal fatty acid profile. The interplay between oxysterols and fatty acids potentially provides insight into the biological mechanisms that underlie this complex disease.
Subject(s)
Cholesterol/metabolism , Cystic Fibrosis/metabolism , Fatty Acids/blood , Oxidative Stress , Adolescent , Adult , Biomarkers/blood , Case-Control Studies , Chi-Square Distribution , Fatty Acids/metabolism , Female , Humans , Hydroxycholesterols/blood , Ketocholesterols/blood , Male , Mass Spectrometry , Oxidation-Reduction , Statistics, Nonparametric , Young AdultABSTRACT
UNLABELLED: We used insulin pump therapy in three patients (two males and one female; age 5.5, 21 and 28.2 years, respectively) with CFRD and we reported the experience of 2 years of follow-up. In all the patients during the CSII treatment, the annual mean level of HbA1c reduced and the annual mean level of BMI increased, respected, the year before starting the CSII (in MDI treatment with 4 injections/day). The insulin requirements decreased during the CSII treatment and respected MDI treatment. During the CSII treatment, none of the patients exhibited episodes of DKA or severe hypoglycaemia. Only two episodes of lipohypertrophy and a slight local cutaneous inflammation were reported. CONCLUSIONS: The use of CSII in our patients with CFRD improves the metabolic control of diabetes and the nutritional status with no concomitant problems related to this treatment.
