ubiquinone and Cystic-Fibrosis

Since the identification of cystic fibrosis (CF) in the 1940s, nutrition care of patients who have CF has been a challenge. Through optimal caloric intake and careful management of malabsorption, patients are expected to meet genetic potential for growth. Yet factors beyond malabsorption, including nutrient activity at the cellular level, may influence growth and health. This article reviews nutrition topics frequently discussed in relationship to CF and presents intriguing new information describing nutrients currently being studied for their impact on overall health of patients who have CF.

Topics: Adolescent; Adult; Child; Child, Preschool; Choline; Cystic Fibrosis; Dietary Proteins; Energy Intake; Fatty Acids, Essential; Glutathione; Humans; Infant; Malabsorption Syndromes; Micronutrients; Minerals; Trace Elements; Ubiquinone; Vitamins; Young Adult

Despite supplementation with standard multivitamins and pancreatic enzymes, deficiencies of vitamins D and K and antioxidants are common in cystic fibrosis (CF).. In this non-randomized, open-label study, AquADEKs® softgels were given daily over 12 weeks to 14 CF subjects (mean age 15 years, range 10-23) without a preceding wash-out period. Both pancreatic sufficient and insufficient subjects were enrolled. Plasma vitamin and antioxidant levels, urine 8-isoprostane levels, anthropometric measures, and pulmonary function were determined at baseline, 6 and 12 weeks.. Daily supplementation significantly increased plasma beta(β)-carotene, coenzyme Q10, and γ-tocopherol concentrations, decreased proteins induced in vitamin K absence (PIVKA-II) levels, but did not normalize vitamin D and K status in all subjects. Vitamin A levels did not exceed the normal range for any subject during the entire study period. Modest improvements in weight percentile and pulmonary function were observed. Change in plasma β-carotene concentrations weakly correlated with changes in weight and body mass index percentiles.. In this study, AquADEKs® increased systemic antioxidant levels, while maintaining vitamin A levels in the normal range, and improved but did not completely normalize vitamin D and K status. Increased β-carotene levels were associated with improved growth parameters. These results warrant further clinical evaluation in CF.

Topics: Adolescent; Antioxidants; Biomarkers, Pharmacological; Body Mass Index; Child; Cystic Fibrosis; Dietary Supplements; Dinoprost; Exocrine Pancreatic Insufficiency; Female; Humans; Male; Oxidative Stress; Respiratory Function Tests; Treatment Outcome; Ubiquinone; Vitamin A Deficiency; Vitamin D Deficiency; Vitamin K Deficiency; Vitamins; Young Adult

Pancreatic insufficiency and a diminished bile acid pool cause malabsorption of important essential nutrients and other dietary components in cystic fibrosis (CF). Of particular significance is the malabsorption of fat-soluble antioxidants such as carotenoids, tocopherols and coenzyme Q(10) (CoQ(10)). Despite supplementation, CF patients are often deficient in these compounds, resulting in increased oxidative stress, which may contribute to adverse health effects. This pilot study was designed to evaluate the safety of a novel micellar formulation (CF-1) of fat-soluble nutrients and antioxidants and to determine its efficacy in improving plasma levels of these compounds and reducing inflammatory markers in induced sputum.. Ten CF subjects, ages 8 to 45 years old, were given orally 10 ml of the CF-1 formulation daily for 56 days after a 21-day washout period in which subjects stopped supplemental vitamin use except for a standard multivitamin. Plasma obtained at -3, 0 (baseline), 1, 2, 4, and 8 weeks was assayed for beta-carotene, gamma-tocopherol, retinol, and CoQ(10) as well as for safety parameters (comprehensive metabolic panel and complete blood count). In addition, pulmonary function was measured and induced sputum was assayed for markers of inflammation and quantitative bacterial counts both prior and during dosing.. No serious adverse effects, laboratory abnormalities or elevated nutrient levels (above normal) were identified as related to CF-1. Supplementation with CF-1 significantly increased beta-carotene levels at all dosing time points when compared to screening and baseline. In addition, gamma-tocopherol and CoQ(10) significantly increased from baseline in all subjects. Induced sputum myeloperoxidase significantly decreased and there was a trend toward decreases in PMN elastase and total cell counts with CF-1. There was a significant inverse correlation between the antioxidant levels and induced sputum changes in IL-8 and total neutrophils. Lung function and sputum bacterial counts were unchanged.. The novel CF-1 formulation safely and effectively increased plasma levels of important fat-soluble nutrients and antioxidants. In addition, improvements in antioxidant plasma levels were associated with reductions in airway inflammation in CF patients.

Topics: Adolescent; Adult; Antioxidants; beta Carotene; Biological Availability; Child; Cystic Fibrosis; Dietary Supplements; Exocrine Pancreatic Insufficiency; gamma-Tocopherol; Humans; Interleukin-8; Micelles; Middle Aged; Pilot Projects; Sputum; Ubiquinone

The ability to switch between different lifestyles allows bacterial pathogens to thrive in diverse ecological niches

Topics: Acute Disease; Anaerobiosis; Animals; Chronic Disease; Cystic Fibrosis; Genes, Bacterial; Humans; Oxygen; Pseudomonas aeruginosa; Pseudomonas Infections; RNA, Bacterial; Sepsis; Ubiquinone; Wounds and Injuries

Pandoraea species have been isolated from diverse environmental samples and are emerging important respiratory pathogens, particularly in people with cystic fibrosis (CF). In the present study, two bacterial isolates initially recovered from consecutive sputum samples collected from a CF patient and identified as Pandoraea pnomenusa underwent a polyphasic taxonomic analysis. The isolates were found to be Gram-negative, facultative anaerobic motile bacilli and subsequently designated as strains 6399

Topics: Bacterial Typing Techniques; Base Composition; Burkholderiaceae; Cystic Fibrosis; DNA, Bacterial; Fatty Acids; Genes, Bacterial; Humans; Phospholipids; Phylogeny; RNA, Ribosomal, 16S; Sequence Analysis, DNA; Sputum; Tasmania; Ubiquinone

To assess total serum levels of coenzyme Q(10) (Co-Q(10)), an important antioxidant, in children with cystic fibrosis (CF) and to investigate an association between Co-Q(10) level and clinical outcome.. Co-Q(10) levels were measured annually in a prospective cohort study of 381 children with CF. A total of 1092 serum levels of total Co-Q(10) were obtained by high-performance liquid chromatography and ultraviolet light detection. Associations of Co-Q(10) with demographic variables and clinical outcomes were investigated.. Of the 381 initial total serum Co-Q(10) measurements, 188 were in the deficient range. Low Co-Q(10) was significantly more prevalent in patients with pancreatic insufficiency (PI) (55%) compared with patients with pancreatic sufficiency (PS) (3%); 22% of the patients with PI exhibited persistently low Co-Q(10) levels. Low Co-Q(10) levels were significantly associated with Pseudomonas aeruginosa colonization in patients with PI and CF under age 24 months, but not with subsequent lung function or hospitalization rates. Low Co-Q(10) levels were related to other markers of nutritional status, including total lipids, beta-carotene, and alpha-tocopherol.. Persistently low total serum Co-Q(10) levels are common in children with CF and PI. A prospective study is indicated to determine whether Co-Q(10) supplementation in CF is beneficial.

Topics: Adolescent; Adult; Biomarkers; Child; Child, Preschool; Chromatography, High Pressure Liquid; Cystic Fibrosis; Disease Progression; Electron Transport Chain Complex Proteins; Female; Follow-Up Studies; Humans; Infant; Male; Prognosis; Prospective Studies; Severity of Illness Index; Ubiquinone

Coenzyme Q10 (CoQ10) is an effective lipophilic antioxidant and protects against lipid peroxidation by scavenging radicals. Patients with cystic fibrosis generally have fat malabsorption; thus, we hypothesized that overall plasma CoQ10 concentration in pediatric patients with cystic fibrosis might be diminished. Because these patients have increased oxidative stress due to chronic pulmonary inflammation, we also assumed that the oxidized form of CoQ10 might be relatively increased.. The total plasma CoQ10 levels and the oxidized and reduced form were measured by high-performance liquid chromatography in 30 children with cystic fibrosis (mean FEV1 % predicted = 88.5% +/- 18.7%) and 30 age-matched controls.. Total plasma CoQ10 levels were significantly lower in the cystic fibrosis group as compared with the control group (0.87 +/- 0.42 micromol/L and 1.35 +/- 0.39 micromol/L, respectively; P < 0.001). When correcting for the lower serum cholesterol level in patients with cystic fibrosis, this difference remained significant: the CoQ10/cholesterol ratio (micromol/mol) was 268.8 +/- 136.7 and 334.0 +/- 102.9 in patients and controls, respectively (P < 0.05). However, the CoQ10 redox status was identical in patients and controls (86.4% +/- 7.1% and 85.4% +/- 7.3%, respectively).. We found that the overall plasma CoQ10 concentration is lower in patients with cystic fibrosis, probably because of fat malabsorption. The CoQ10 redox status was not disturbed, indicating that CoQ10 could still be adequately regenerated in this group of patients with cystic fibrosis with mild-to-moderate pulmonary disease.

Topics: Adolescent; Child; Cholesterol; Chromatography, High Pressure Liquid; Coenzymes; Cystic Fibrosis; Female; Humans; Male; Oxidation-Reduction; Ubiquinone

Topics: Colitis, Ulcerative; Colon; Colonic Diseases; Colonic Diseases, Functional; Crohn Disease; Cystic Fibrosis; Diverticulum, Colon; Enterocolitis, Pseudomembranous; Glucosephosphate Dehydrogenase; Glycerolphosphate Dehydrogenase; Histocytochemistry; Humans; Intestinal Mucosa; Intestinal Polyps; Oxidoreductases; Rectal Neoplasms; Succinate Dehydrogenase; Ubiquinone