Research Papers and Summary proposals for possible Integrative treatment for Cystic Fibrosis*.
The following research herein is for information purposes only and is not intended to prevent, treat or cure any illness. Always consult your Doctor or health care professional before engaging in any changes to your medical regime*.
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Research Papers funded by The Tracie Lawlor Trust for Cystic Fibrosis*.
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PLAIN Language/Guidelines on normal Minute Ventilation in relation to Cystic Fibrosis, Click Here.
PLAIN Language summary of the Buteyko method and it’s possible significance, Click Here.
SUM of the use of Massage and it’s possible benefits in relation to Cystic Fibrosis, Click Here.
A small collection of some recent research of interest in relation to integrative medicine & Cystic Fibrosis.
A phase II study on safety and efficacy of high-dose N-acetylcysteine in patients with cystic fibrosis.
Dauletbaev N, Fischer P, Aulbach B, Gross J, Kusche W, Thyroff-Friesinger U, Wagner TO, Bargon J.
University Hospital, Frankfurt/Main, Germany.
(Eur J Med Res. 2009 Aug 12;14(8):352-8).
OBJECTIVE: We conducted a single-centre, randomised, double-blinded, placebo-controlled phase II clinical study to test safety and efficacy of a 12-week therapy with low-dose (700 mg/daily) or high-dose (2800 mg/daily) of NAC. METHODS: Twenty-one patients (DeltaF508 homo/heterozygous, FEV1>40% pred.) were included in the study. After a 3-weeks placebo run-in phase, 11 patients received low-dose NAC, and 10 patients received high-dose NAC. Outcomes included safety and clinical parameters, inflammatory (total leukocyte numbers, cell differentials, TNF-alpha, IL-8) measures in induced sputum, and concentrations of extracellular glutathione in induced sputum and blood. RESULTS: High-dose NAC was a well-tolerated and safe medication. High-dose NAC did not alter clinical or inflammatory parameters. However, extracellular glutathione in induced sputum tended to increase on high-dose NAC. CONCLUSIONS: High-dose NAC is a well-tolerated and safe medication for a prolonged therapy of patients with CF with a potential to increase extracellular glutathione in CF airways.
Glutathione exhibits antibacterial activity and increases tetracycline efficacy against Pseudomonas aeruginosa.
ZHANG Y, DUAN K.
(Sci China C Life Sci. 2009 Jun;52(6):501-5. Epub 2009 Jun 26).
Molecular Microbiology Laboratory, the College of Life Sciences, Northwest University, Xi’an 710069, China.
Glutathione (GSH) plays important roles in pulmonary diseases, and inhaled GSH therapy has been used to treat cystic fibrosis (CF) patients in clinical trials. The results in this report revealed that GSH altered the sensitivity of Pseudomonas aeruginosa to different antibiotics through pathways unrelated to the oxidative stress as generally perceived. In addition, GSH and its oxidized form inhibited the growth of P. aeruginosa.
Update on fat-soluble vitamins in cystic fibrosis.
Maqbool A, Stallings VA.
Division of Gastroenterology, Hepatology and Nutrition, The Children’s Hospital of Philadelphia, University of Pennsylvania School of Medicine, Philadelphia, PA 19104, USA. maqbool@email.chop.edu
PURPOSE OF REVIEW: We review and critique recent scientific advances in the understanding of fat-soluble vitamins and the care of people with cystic fibrosis. RECENT FINDINGS: A shift in the conceptual approach to fat-soluble vitamin status has occurred. Vitamin status in cystic fibrosis had previously been discussed in terms of sufficiency versus insufficiency as compared with healthy populations. The discussion of vitamin status has now shifted to that of suboptimal versus optimal with respect to health outcomes. This is best illustrated by advances in the study of vitamin D. Newer metabolic and immunological roles and biomarkers have been identified. With supplementation of water-miscible formulations of preformed vitamin A, increased serum retinol has been observed, and may increase the risk for toxicity. SUMMARY: A paradigm shift has occurred in defining fat-soluble vitamin status by utilizing different biomarkers and associations with health outcomes. Identification of additional biomarkers, redefining definitions of adequacy, optimal surveillance for toxicity as well as adequacy is needed for care of patients with cystic fibrosis. (Curr Opin Pulm Med. 2008 Nov;14(6):574-81).
Inhaled Mannitol Improves Lung Function in Cystic Fibrosis*
(Chest. 2008 Jun;133(6):1388-96. Epub 2008 Mar 13).
Anna Jaques, BSc, MPH, Evangelia Daviskas, MBiomedE, PhD, James A. Turton, BSc (Hons), MBBS, MMedSc, Karen McKay, PhD, Peter Cooper, BSc, MB ChB, Robert G. Stirling, MB BCh (Hons), Colin F. Robertson, MD, Peter T. P. Bye, MBBS, PhD, FCCP, Peter N. LeSouëf, MD, Bruce Shadbolt, PhD, Sandra D. Anderson, PhD, DSc, and Brett Charlton, MBBS, PhD
Background: The airways in patients with cystic fibrosis (CF) are characterized by the accumulation of tenacious, dehydrated mucus that is a precursor for chronic infection, inflammation, and tissue destruction. The clearance of mucus is an integral component of daily therapy. Inhaled mannitol is an osmotic agent that increases the water content of the airway surface liquid, and improves the clearance of mucus with the potential to improve lung function and respiratory health. To this end, this study examined the efficacy and safety of therapy with inhaled mannitol over a 2-week period.
Methods: This was a randomized, double-blind, placebo-controlled, crossover study. Thirty-nine subjects with mild-to-moderate CF lung disease inhaled 420 mg of mannitol or placebo twice daily for 2 weeks. Following a 2-week washout period, subjects were entered in the reciprocal treatment arm. Lung function, respiratory symptoms, quality of life, and safety were assessed.
Results: Mannitol treatment increased FEV1 from baseline by a mean of 7.0% (95% confidence interval [CI], 3.3 to 10.7) compared to placebo 0.3% (95% CI, − 3.4 to 4.0; p < 0.001). The absolute improvement with mannitol therapy was 121 mL (95% CI, 56.3 to 185.7), which was significantly more than that with placebo (0 mL; 95% CI, − 64.7 to 64.7). The forced expiratory flow in the middle half of the FVC increased by 15.5% (95% CI, − 6.5 to 24.6) compared to that with placebo (increase, 0.7%; 95% CI, − 8.3 to 9.7; p < 0.02). The safety profile of mannitol was adequate, and no serious adverse events related to treatment were observed.
Conclusions: Inhaled mannitol treatment over a period of 2 weeks significantly improved lung function in patients with CF. Mannitol therapy was safe and well tolerated.
*Please click here for details on a possible conflict of interest in-relation to the above study.
A pilot study on the safety and efficacy of a novel antioxidant rich formulation in patients with cystic fibrosis.
Papas KA, Sontag MK, Pardee C, Sokol RJ, Sagel SD, Accurso FJ, Wagener JS.
Yasoo Health, Johnson City, TN, USA.
BACKGROUND: 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. METHODS: 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. RESULTS: 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. CONCLUSION: 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. (J Cyst Fibros. 2008 Jan;7(1):60-7. Epub 2007 Jun 13).
Choline related supplements improve abnormal plasma methionine – homocysteine metabolites and glutathione status in children with cystic fibrosis.
(American Journal of Clinical Nutrition, Vol. 85, No. 3, 702-708, March 2007). Sheila M Innis, A George F Davidson, Stepan Melynk and S Jill James. From the Department of Paediatrics, University of British Columbia, Vancouver, BC, Canada (SMI and AGFD), and the Department of Pediatrics, University of Arkansas for Medical Sciences, Little Rock, AR (SM and SJJ)
Liver triacylglycerol accumulation and oxidative stress are common in cystic fibrosis and also occur in choline deficiency. Previously, we showed an association between elevated plasma homocysteine, reduced ratios of S-adenosylmethionine to S-adenosylhomocysteine (SAM:SAH) and of phosphatidylcholine to phosphatidylethanolamine, and phospholipid malabsorption in children with cystic fibrosis. Children with cystic fibrosiswere assigned without bias to supplementation with 2 g lecithin/d (n = 13), 2 g choline per day (n = 12), or 3 g betaine per day (n = 10) for 14 d. Plasma concentrations of methionine, adenosine, cysteine, cysteinyl-glycine, glutathione, glutathione disulfide (GSSG), and fatty acids; SAM:SAH; and red blood cell phospholipids were measured within each group of children with cystic fibrosis before and after supplementation. Children with cystic fibrosis had higher plasma homocysteine, SAH, and adenosine and lower methionine, SAM:SAH, and glutathione:GSSG than did children without cystic fibrosis. Supplementation with lecithin, choline, or betaine resulted in a significant increase in plasma methionine, SAM, SAM:SAH, and glutathione:GSSG and a decrease in SAH. Supplementation with choline or betaine was associated with a significant decrease in plasma SAH and an increase in SAM:SAH, methionine, and glutathione:GSSG. Supplementation with lecithin or choline also increased plasma methionine and SAM. Conclusion: We showed that dietary supplementation with choline-related compounds improves the low SAM:SAH and glutathione redox balance in children with cystic fibrosis.
Vitamins A and E and pulmonary exacerbations in patients with cystic fibrosis.
Hakim F, Kerem E, Rivlin J, Bentur L, Stankiewicz H, Bdolach-Abram T, Wilschanski M.
(J Pediatr Gastroenterol Nutr. 2007 Sep;45(3):347-53).
Department of Pediatrics, Rambam Medical Center, Haifa, Israel.
BACKGROUND: Increased levels of oxidative stress result in pulmonary damage contributing to the development of chronic lung disease in cystic fibrosis (CF). The aim of this study was to investigate the longitudinal effect of serum vitamin A and E levels on the incidence of pulmonary exacerbations in pancreatic insufficient (PI) and pancreatic sufficient (PS) patients with CF. MATERIALS AND METHODS: Patient records were retrospectively examined over a 3-year period and serum vitamin A and E levels were retrieved. Subsequently, levels of vitamin A and E were prospectively measured over a 2-year period at the onset of intravenous antibiotic therapy for acute exacerbation and at the first recovery visit. RESULTS: Retrospectively, 597 pulmonary exacerbations were identified in 102 patients, 74 PI and 28 PS, with a mean age of 11.1 +/- 6.4 years (range, 1.5-27 y). An increased number of exacerbations was directly correlated with lower vitamin A and E levels, even within the normal range. Prospectively, 62 exacerbations were analyzed (43 PI patients and 19 PS patients). At onset of exacerbation, vitamin A and E levels were reduced in the PI patients (P < 0.001; P < 0.001) and the PS patients (P < 0.005; P < 0.07). CONCLUSIONS: Reduced serum levels of vitamin A and E even in the normal range are associated with an increased rate of pulmonary exacerbations in CF. Further studies are required to confirm the necessity of supplementation of vitamins A and E to PS patients.
Omega-3 fatty acids (from fish oils) for cystic fibrosis.
McKarney C, Everard M, N’Diaye T.
(Cochrane Database Syst Rev. 2007 Oct 17;(4):CD002201).
Sheffield Children’s Hospital, Western Bank, Sheffield, UK, S10 2TH. colleen.mckarney@sch.nhs.uk
Update of:(Cochrane Database Syst Rev. 2002;(3):CD002201).
BACKGROUND: Epidemiological and other studies suggest that a diet rich in omega-3 essential fatty acids (derived from fish oil) may have beneficial anti-inflammatory effects for chronic conditions such as cystic fibrosis (CF). OBJECTIVES: To determine whether there is evidence that omega-3 polyunsaturated fatty acid supplementation reduces morbidity and mortality. To identify any adverse events associated with omega-3 polyunsaturated fatty acid supplementation. SEARCH STRATEGY: We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group’s Trials Register comprising references identified from comprehensive electronic database searches and handsearches of relevant journals and abstract books of conference proceedings. Authors and persons interested in the subject of the review were contacted. Most recent search: April 2007 SELECTION CRITERIA: Randomised controlled trials in people with CF comparing omega-3 fatty acid supplements with placebo. DATA COLLECTION AND ANALYSIS: Two authors independently selected the studies for inclusion in the review, independently extracted data and assessed the methodological quality of the studies. MAIN RESULTS: Searches identified seven studies; three of which, involving 48 participants, were eligible for inclusion in the review. Two studies compared omega-3 fatty acids to olive oil controls for a six-week treatment period. One study compared omega-3 fatty acids in the form of a liquid dietary supplement containing polyunsaturated fatty acids to a liquid dietary supplement control for six months. One short-term study (19 participants) comparing omega-3 to placebo reported a significant improvement in forced expiratory volume in one second, forced vital capacity and Shwachman score and a reduction in sputum volume in the omega-3 group. The longer-term study (17 participants) demonstrated a significant increase in essential fatty acid content in neutrophil membranes in study participants taking omega-3 supplements compared to placebo, weighted mean difference 0.90 (95% confidence interval 0.46 to 1.34). AUTHORS’ CONCLUSIONS: This review found that regular omega-3 supplements may provide some benefits for people with CF with relatively few adverse effects, although the evidence is insufficient to draw firm conclusions or to recommend routine use of supplements of omega-3 fatty acids in people with CF. This review has highlighted the lack of data for many of the outcomes likely to be meaningful to people with or making treatment decisions about CF. A large, long-term, multicentre, randomised controlled study is needed in order to determine if there is a significant therapeutic effect and to assess the influence of disease severity, dosage and duration of treatment. Future researchers should note the need for additional pancreatic enzymes.
Vitamin E status in children with cystic fibrosis and pancreatic insufficiency.
Huang SH, Schall JI, Zemel BS, Stallings VA.
(J Pediatr. 2006 Apr;148(4):556-559).
Division of Gastroenterology and Nutrition, The Children’s Hospital of Philadelphia, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania 19104-4399, USA.
Comment in:(J Pediatr. 2007 May;150(5):e85; author reply e85-6).
Vitamin E status was compared in 69 children (7.0-10.0 years) with cystic fibrosis and pancreatic with the National Health and Nutrition Examination Survey III sample (6.0-11.9 years). With median vitamin E intakes of 6 mg/day (dietary) and 224 mg/day (supplemental), children with cystic fibrosis had higher serum alpha-tocopherol:cholesterol ratios, higher alpha-tocopherol, and lower cholesterol levels than in the National Health and Nutrition Examination Survey.
A Controlled Trial of Long-Term Inhaled Hypertonic Saline in Patients with Cystic Fibrosis
Mark R. Elkins, M.H.Sc., Michael Robinson, Ph.D., Barbara R. Rose, Ph.D., Colin Harbour, Ph.D., Carmel P. Moriarty, R.N., Guy B. Marks, Ph.D., Elena G. Belousova, M.Appl.Sc., Wei Xuan, Ph.D., Peter T.P. Bye, Ph.D., for the National Hypertonic Saline in Cystic Fibrosis (NHSCF)
Background Inhaled hypertonic saline acutely increases mucociliary clearance and, in short-term trials, improves lung function in people with cystic fibrosis. We tested the safety and efficacy of inhaled hypertonic saline in a long-term trial.
Methods In this double-blind, parallel-group trial, 164 patients with stable cystic fibrosis who were at least six years old were randomly assigned to inhale 4 ml of either 7 percent hypertonic saline or 0.9 percent (control) saline twice daily for 48 weeks, with quinine sulfate (0.25 mg per milliliter) added to each solution to mask the taste. A bronchodilator was given before each dose, and other standard therapies were continued during the trial.
Results The primary outcome measure, the rate of change (slope) in lung function (reflected by the forced vital capacity [FVC], forced expiratory volume in one second [FEV1], and forced expiratory flow at 25 to 75 percent of FVC [FEF25–75]) during the 48 weeks of treatment, did not differ significantly between groups (P=0.79). However, the absolute difference in lung function between groups was significant (P=0.03) when averaged across all post-randomization visits in the 48-week treatment period. As compared with the control group, the hypertonic-saline group had significantly higher FVC (by 82 ml; 95 percent confidence interval, 12 to 153) and FEV1 (by 68 ml; 95 percent confidence interval, 3 to 132) values, but similar FEF25–75 values. The hypertonic-saline group also had significantly fewer pulmonary exacerbations (relative reduction, 56 percent; P=0.02) and a significantly higher percentage of patients without exacerbations (76 percent, as compared with 62 percent in the control group; P=0.03). Hypertonic saline was not associated with worsening bacterial infection or inflammation. (N Engl J Med. 2006 Jan 19;354(3):229-40).
Antioxidant deficiency in cystic fibrosis: when is the right time to take action?
(Am J Clin Nutr. 2004 Aug;80(2):374-84).
Little is known about age- and disease-related changes in prooxidant and antioxidant systems in patients with cystic fibrosis. We investigated changes in antioxidant concentrations and oxidative stress in plasma, buccal mucosal cells (BMCs), and breath condensate in patients with cystic fibrosis in relation to age and disease progression. We recruited 22 patients with cystic fibrosis as well as 35 healthy control subjects and conducted a cross-sectional study by dividing the participants into 4 age groups (/=18 y). We collected fasting blood samples, BMCs, and breath condensate. Carotenoids, alpha-tocopherol, vitamin C, protein carbonyls, and thiobarbituric acid-reactive substances were assessed. In patients with cystic fibrosis, plasma vitamin C concentrations, plasma and BMC alpha-tocopherol concentrations, and forced expiratory volume in 1 s (percentage predicted) decreased significantly with age. Plasma beta-carotene, beta-cryptoxanthin, and total lycopene were significantly lower in patients than in control subjects in all age groups. Furthermore, alpha-tocopherol and vitamin C plasma concentrations as well as alpha-tocopherol concentrations in BMCs were significantly lower in cystic fibrosis patients >/=18 y old, whereas all indicators of oxidative stress assessed were significantly higher than those same indicators in control subjects. Adult patients with cystic fibrosis in particular showed distinct vitamin deficits and elevated indicators of oxidative stress in plasma, BMCs, and breath condensate along with a progression of clinical status. We suggest that early in life dietary habits should be improved and that innovative supplementation strategies should be applied to optimize the antioxidant status of patients with cystic fibrosis.
Intestinal inflammation is a frequent feature of cystic fibrosis and is reduced by probiotic administration.
Bruzzese E, Raia V, Gaudiello G, Polito G, Buccigrossi V, Formicola V, Guarino A.
(Aliment Pharmacol Ther. 2004 Oct 1;20(7):813-9).
Department of Pediatrics, University of Naples Federico II, Naples, Italy.
AIMS: To assess the incidence of intestinal inflammation in children with cystic fibrosis and to investigate whether probiotics decrease it. STUDY DESIGN: In this two-phase, controlled, prospective study, faecal calprotectin was measured by enzyme-linked immunosorbent assay in 30 children with cystic fibrosis, 30 healthy controls and 15 children with active inflammatory bowel disease. Ten children with cystic fibrosis received Lactobacillus GG, and faecal calprotectin was re-measured 4 weeks later. Rectal nitric oxide production was measured with the rectal dialysis bag technique in 20 children with cystic fibrosis, 20 healthy controls and 15 children with inflammatory bowel disease. Five children with cystic fibrosis received Lactobacillus GG and nitric oxide was re-measured 4 weeks later. RESULTS: Mean faecal calprotectin was significantly higher in the two groups of patients than in controls. Abnormal values were detected in 27 of 30 cystic fibrosis and in 15 of 15 inflammatory bowel disease children. Also mean nitric oxide production was increased in both group of patients, and abnormal values were detected in 19 of 20 cystic fibrosis and in 15 of 15 inflammatory bowel disease children. Calprotectin and nitric oxide concentrations were reduced after probiotics administration. CONCLUSIONS: Intestinal inflammation is a major feature of cystic fibrosis and is reduced by probiotics. The latter finding suggests that intestinal microflora play a major role in intestinal inflammation in cystic fibrosis children.
Lutein, zeaxanthin, macular pigment, and visual function in adult cystic fibrosis patients.
(Am J Clin Nutr. 2004 Jun;79(6):1045-52).
Pancreatic insufficiency in cystic fibrosis, even with replacement pancreatic enzyme therapy, is often associated with decreased carotenoid absorption. Because the macular pigment of the retina is largely derived from 2 carotenoids, lutein and zeaxanthin, the decreased serum concentrations seen in cystic fibrosis may have consequences for ocular and retinal health Our aims were to determine plasma carotenoid concentrations, determine absorption and distribution of macular pigment, and assess retinal health and visual function in cystic fibrosis patients. In 10 adult cystic fibrosis patients (ages 21-47 y) and 10 age- and sex-matched healthy control subjects, we measured macular pigment density in vivo, measured serum lutein and zeaxanthin concentrations, and comprehensively assessed visual performance (including contrast sensitivity, color discrimination, and retinal function) under conditions of daylight illumination. Serum lutein and zeaxanthin were significantly reduced in cystic fibrosis patients compared with control subjects. Although macular pigment optical density was significantly lower in the cystic fibrosis group than in the control group, no significant differences in visual function were observed. Adults with cystic fibrosis have dramatically low serum and macular concentrations of carotenoids (lutein and zeaxanthin), but their ocular status and visual function are surprisingly good. The clinical implications of low plasma concentrations of carotenoids in cystic fibrosis are yet to be clarified.
Improved glutathione status in young adult patients with cystic fibrosis supplemented with whey protein.
(J Cystic Fibrosis. 2003 Dec;2(4):195-8).
The lung disease of cystic fibrosis is associated with a chronic inflammatory reaction and an over abundance of oxidants relative to antioxidants. Glutathione functions as a major frontline defense against the build-up of oxidants in the lung. This increased demand for glutathione (GSH) in cystic fibrosis may be limiting if nutritional status is compromised. We sought to increase glutathione levels in stable patients with cystic fibrosis by supplementation with a whey-based protein. Twenty-one patients who were in stable condition were randomly assigned to take a whey protein isolate (Immunocal, 10 g twice a day) or casein placebo for 3 months. Peripheral lymphocyte GSH was used as a marker of lung GSH. Values were compared with nutritional status and lung parameters. At baseline there were no significant differences in age, height, weight, percent ideal body weight or percent body fat. Lymphocyte GSH was similar in the two groups. After supplementation, we observed a 46.6% increase from baseline in the lymphocyte GSH levels in the supplemented group. No other changes were observed. The results show that dietary supplementation with a whey-based product can increase glutathione levels in cystic fibrosis. This nutritional approach may be useful in maintaining optimal levels of GSH and counteract the deleterious effects of oxidative stress in the lung in cystic fibrosis.
Fatty acids composition of plasma phospholipids and triglycerides in children with cystic fibrosis. The effect of dietary supplementation with an olive and soybean oils mixture.
Caramia G, Cocchi M, Gagliardini R, Malavolta M, Mozzon M, Frega NG. Azienda Ospedaliera G. Salesi, Ancona.
Cystic fibrosis (CF) is characterized by abnormal levels of essential fatty acids (EFA) in plasma phospholipids. The reduced availability of EFA has been reported to alter patterns of circulating and tissue esterified acids and may determine profound changes in membrane fluidity and cell signaling mechanisms. In the current study, the results of a new strategy aimed at the realization of a practical, low cost integrator, for daily use in the dietary management of FC subjects, are reported. We investigated the plasma phospholipids and triglycerides fatty acids composition of CF patients subjected to a dietary supplement constituted of a mixture of 50% extra virgin olive oil and 50% soybean oil and studied the clinical effects of this supplementation. The study included fourteen young subjects, aged between 6 and 15 years, affected by cystic fibrosis, with pancreatic insufficiency and heterozygotes or homozygotes for the delta F508 mutation. The subjects were matched by age and randomly assigned to either an oil mixture supplemented (OM) group (n = 7), or to a control (C) group (n = 7). In contrast to the control group, the patients with supplemented diet achieved significant increases of the relative amount of C18:1 in the triglycerides as well as a significant decrease in saturated fatty acids (C 16:0, C 17:0, C 18:0, C 22:0). Moreover, the ratio between LA acid and AA significantly increased in the triglycerides of the OM group. In the phospholipids of the OM group, the relative amount of C 18:1 and of palmitic acid increased significantly whereas the relative amount of the most important polyunsaturated fatty acids (PUFA) decreased. These results show that oleic acid can be absorbed and incorporated into the plasma triglycerides of CF patients receiving pancreatic enzymes, whereas poor incorporation of LA occurs. Despite the reduction in the relative amounts of phospholipid PUFA, the supplemented subjects did not reported adverse effects There were no significant differences between groups in the clinical indexes recorded (height, weight, BMI, Schwachman-Kulczycki score and FEV 1s). The results of this study showed that the supplementation with a mixture of extravirgin olive and soybean oil was safe in seven CF patients treated during a 2-months period and no negative clinical effects were evident. However, further clinical trials will be necessary in order to better evaluate the consequence of the observed changes in plasma fatty acids composition in a longer testing period. (Pediatr Med Chir. 2003 Jan-Feb;25(1):42-9).
Vitamin therapy in cystic fibrosis–a review and rationale.
Peters SA, Rolles CJ.
Department of Child Health, Southampton General Hospital, U.K.
Vitamin supplements are routinely prescribed in cystic fibrosis, but published recommendations vary widely and there is little consistency in clinical practice. A review of the literature confirms that, while supplementation of the water-soluble vitamins (including B12 and folate) is unnecessary in uncomplicated cystic fibrosis, deficiency of the fat-soluble vitamins can lead to clinical problems. Supplements of these vitamins should be ensured for all patients with cystic fibrosis, while sparing them the unnecessary inconvenience of taking other vitamin supplements except where these are specifically indicated. J Clin Pharm Ther. (1993 Feb;18(1):33-8).
