Relationships among nutritional status and skeletal and respiratory muscle function in cystic fibrosis: does early dietary supplementation make a difference? Effect of an organized lipid matrix on lipid absorption and clinical outcomes in patients with cystic fibrosis. Bioavailability of a Novel, Water-Soluble Vitamin E Formulation in Malabsorbing Patients Oral protein energy supplements for children with cystic fibrosis: CALICO multicentre randomised controlled trial Bioavailability and safety of a high dose of docosahexaenoic acid triacylglycerol of algal origin in cystic fibrosis patients: a randomized, controlled study. Dietary supplementation with multiple micronutrients: no beneficial effects in pediatric cystic fibrosis patients. Copper enzyme activities in cystic fibrosis before and after copper supplementation plus or minus zinc. Energy Supplements Rich in Linoleic Acid Improve Body Weight and Essential Fatty Acid Status of Cystic Fibrosis Patients Improved glutathione status in young adult patients with cystic fibrosis supplemented with whey protein. Do infants with cystic fibrosis need a protein hydrolysate formula? A prospective, randomized, comparative study. Failure of conventional strategies to improve nutritional status in malnourished adolescents and adults with cystic fibrosis. Glycemic response to dietary supplements in cystic fibrosis is dependent on the carbohydrate content of the formula. Effect of Lactobacillus GG supplementation on pulmonary exacerbations in patients with cystic fibrosis: a pilot study. 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.
Relationships among nutritional status and skeletal and respiratory muscle function were examined in 16 children with cystic fibrosis ( CF ) and mild lung disease ( FEV1 95 + /- 16 % predicted ) . Subjects were r and omly assigned to receive ( or not ) noninvasive nutritional supplementation at 25 % of normal energy recommendations for 6 mo . Skeletal muscle strength and power were similar to those of healthy children as were respiratory muscle strength and endurance . Stepwise-regression analysis indicated that changes in skeletal muscle strength and energy intake correlated significantly with growth [ weight ( kg ) = 1.90 - 0.60 ( Tanner Stage ) + 0.49 ( maximum voluntary strength ( Nm ) + 0.03 ( energy intake , % RNI ) , r = 0.76 , P < 0.05 ] , though body composition , protein biochemistry , muscle power , respiratory muscle strength , and use of dietary supplements did not . Thus , changes in skeletal muscle strength may be a functional index of changes in nutritional status in CF . Dietary supplementation per se was not associated with functional improvement OBJECTIVES To compare the absorption of a lysophosphatidylcholine , monoglyceride , and fatty acid matrix ( organized lipid matrix , OLM ) with that of a triacylglycerol (TG)-based fat meal in patients with cystic fibrosis ( CF ) . STUDY DESIGN Five adolescents with CF and 3 control patients were given fat meals supplemented with retinyl palmitate of either OLM or TG at a 2-week interval . In a clinical trial , 73 patients with CF were r and omly assigned to nutritional supplements containing either OLM or TG for a 1-year double-blind trial followed by a 6-month observation period . RESULTS The peak increases and areas under the curve for TG and retinyl palmitate after the fat meal were 10-fold higher after OLM than after the TG fat load and did not differ from values obtained in control patients . OLM led to better clinical outcomes in terms of energy intake from the diet , weight-for-age Z score , essential fatty acid status , vitamin E , and retinol binding protein . Height-for-age Z score and FEV(1 ) only reached statistical significance at the end of the 6-month observation period . CONCLUSIONS These results suggest that OLM is a readily absorbable source of fat and energy in CF and is an effective nutritional supplement In cystic fibrosis ( CF ) , pancreatic insufficiency and a diminished bile acid pool cause malabsorption of important nutrients and dietary components leading to deficiency , poor nutritional status , and oxidative stress . Of particular significance is the malabsorption of fat-soluble nutrients and antioxidants , which are important for normal immune and neurologic function . Patients with CF often are deficient in these compounds despite supplementation with the current st and ard of care therapy . The objective was to compare the pharmacokinetic profile of this water-soluble vitamin E formulation ( Aqua-E ) with an oil-based softgel formulation in a malabsorbing patient population . Patients with CF who had documented malabsorption were recruited for participation in this pharmacokinetic study . Patients who met inclusion and exclusion criteria discontinued vitamin E supplementation , except for that in a multivitamin , for 7 to 21 days before the day of dosing . Patients were r and omized to a single dose of 20 ml of Aqua-E or three oil-based softgels , which contained equivalent amounts of tocopherols . Blood was drawn from patients at time 0 , 2 , 4 , 8 , 24 , 48 , and 168 hr and analyzed for tocopherols . Eight patients were enrolled in the study and r and omized to Aqua-E or softgels . The primary outcome , the absorption of γ-tocopherol in Aqua-E ( AUC=115 μg/ml*hr ) , was significantly greater than that of oil-based softgels ( AUC=25.3 μg/ml*hr ; P=0.013 ) . Total-tocopherols ( α+γ+δ ) in Aqua-E ( AUC=294 μg/ml*hr ) showed a strong trend toward increased absorption compared with that of oil-based softgels ( AUC=117 μg/ml*hr ; P=0.09 ) . In conclusion , this novel , water-soluble formulation showed a marked and statistically significant increase in absorption of γ-tocopherol in malabsorbing patients with CF compared with an oil-based formulation Abstract Objective To determine whether oral protein energy supplements , used long term in children with cystic fibrosis who are moderately malnourished , improve nutritional and other outcomes . Design Multicentre r and omised controlled trial . Setting Seven specialist paediatric cystic fibrosis centres and their associated shared care clinics and seven smaller paediatric cystic fibrosis clinics . Participants 102 children with cystic fibrosis , aged between 2 and 15 years , who were moderately malnourished . Interventions Oral protein energy supplements in addition to usual dietary advice compared with dietary advice alone , for 12 months . Main outcome measure Change in body mass index centile over one year . Results Use of supplements was not associated with a change in body mass index centile ( mean difference 2.99 centile points , 95 % confidence interval −2.70 to 8.68 ) or other nutritional and spirometric outcomes in this group of children . Conclusions Long term use of oral protein energy supplements did not result in an improvement in nutritional status or other clinical outcomes in children with cystic fibrosis who were moderately malnourished .  Oral protein energy supplements should not be regarded as an essential part of the management of this group of children . Trial registration IS RCT N : 95744468 OBJECTIVE Several studies have reported omega-3 and omega-6 fatty acid imbalances in patients with cystic fibrosis ( CF ) . Whether these imbalances contribute to or are manifestations of the pathophysiology of CF is unknown . The study objective was to determine bioavailability , tissue accretion , and safety of a large dose of an algal source of docosahexaenoic acid ( DHA ) triacylglycerol and to observe effects on lung function in patients with CF . METHODS Twenty subjects with CF ( 8 to 20 y of age ) were r and omly assigned to receive algal oil providing 50 mg of DHA per kilogram per day ( 1 to 4.2 g of DHA per subject per day ) or placebo for 6 mo . Fatty acids , liver enzymes , and lipid soluble antioxidants were measured in blood at baseline and at 1 , 3 , and 6 mo . Rectal biopsy specimens were collected at baseline and at 3 mo for fatty acid analysis . Lung function , anthropometrics , and adverse experiences were monitored throughout the study . RESULTS Compared with placebo , DHA supplementation increased plasma , erythrocyte , and rectal DHA levels four- to five-fold ( P < 0.001 ) with concomitant decreases in blood arachidonic acid levels and the ratio of arachidonic acid to DHA . Supplementation was well tolerated , with no treatment-related changes in liver enzymes , growth , or antioxidant status . DHA supplementation had no detectable effect on lung function during the course of this study . CONCLUSIONS Algal DHA triacylglycerol oil is readily absorbed , well tolerated , and increases blood and tissue DHA levels in patients with CF . No adverse developments were associated with this large dose of DHA oil . Larger studies of longer duration are needed to determine whether DHA supplementation results in any clinical ly significant benefits in patients with CF BACKGROUND Cystic fibrosis ( CF ) patients are subjected to increased oxidative stress due to chronic pulmonary inflammation and recurrent infections . Additionally , these patients have diminished skeletal muscle performance and exercise capacity . We hypothesize that a mixture of multiple micronutrients could have beneficial effects on pulmonary function and muscle performance . METHODS A double-blind , r and omized , placebo controlled , cross-over trial with a mixture of multiple micronutrients ( ML1 ) was performed in 22 CF patients ( 12.9+/-2.5 yrs ) with predominantly mild lung disease .  Anthropometric measures , pulmonary function , exercise performance by bicycle ergometry , muscular strength and vitamins A and E were determined . RESULTS Analysis was performed using the paired Student t-test comparing the change in each parameter during ML1 and placebo . Plasma vitamin E and A levels increased during ML1 when compared to placebo . However , no significant difference between the effect of the ML1 or placebo was observed neither for FEV1 , FVC , anthropometry , nor for the parameters for muscle performance . CONCLUSIONS The micronutrient mixture was not superior to placebo with respect to changes in pulmonary function or muscle performance in pediatric CF patients , despite a significant increase in plasma vitamin E concentrations One laboratory reports low activities for 2 blood copper enzymes in subjects with cystic fibrosis ( CF ) , which suggests that moderate copper deficiency is common in this state . The present study attempted to confirm this proposition in 3 ways : repeat the measures for 1 of the 2 copper enzymes ( superoxide dismutase ) in a new group of CF patients ( males and females , N = 38 ) , add another copper enzyme measure ( plasma diamine oxidase ) that has high sensitivity to copper status , and test if copper enzyme activities in CF patients rise by copper supplementation . The last test was performed plus or minus zinc supplementation since poor zinc status may contribute to poor copper status . The results for the first 2 aims supported the idea of poor copper status , as low activities were found for CF subjects for 2 copper enzyme activities , erythrocyte superoxide dismutase and plasma diamine oxidase ( although normal activities were obtained for another copper enzyme , plasma ceruloplasmin , both as U/mL plasma or U/mg ceruloplasmin immunoreactive protein ) . For the last aim , copper enzyme activities were not altered by copper supplementation ( 6 weeks , 3 mg copper/d as copper-glycinate ) , plus or minus concurrent zinc supplementation ( 30 mg zinc/d as zinc-glycinate ) . Therefore , CF may cause a tendency to moderate copper deficiency , which may be due to abnormal copper metabolism not easily corrected by increased copper and /or zinc intake Background Patients with cystic fibrosis who have steatorrhea frequently are underweight and have essential fatty acid ( EFA ) depletion , which is associated with a poor clinical course . It has been stated that poor EFA status is difficult to correct in patients with cystic fibrosis , and an impaired EFA metabolism with reduced synthesis of long-chain polyunsaturated fatty acids has been proposed . In this study , the effects of an oral energy supplement rich in linoleic acid were investigated in patients with cystic fibrosis who had a body weight below 95 % of normal for height . Methods Thirty-six patients ( 16 girls ) more than 4 years of age were r and omized either to a control group ( n = 20 , age 13.3 ± 3.8 years , mean ± SD ) receiving intensive dietary counseling only , or an intervention group ( n = 16 , age , 10.4 ± 4.3 years ) treated for 3 months with dietary counseling plus 628 ± 254 mL ( = kcal ) per day of an energy supplement rich in fat ( 31 % of energy ) and linoleic acid ( 16 % of energy ) . Results In contrast to the control group , the patients with supplemented diets achieved significant increases of energy intake ( 2189 ± 731 kcal/day vs. 2733 ± 762 kcal/day ) , weight for height ( 82.8 % ± 8.6 % vs. 84.8 % ± 9.6 % of normal ) , and body fat ( 5.1 ± 1.7 kg vs. 5.8 ± 2.2 kg ) as well as the initially low values of plasma phospholipid linoleic acid ( 11.8 % ± 1.1 % vs. 17.6 % ± 1.6 % of total phospholipid fatty acids ) and its main metabolite arachidonic acid ( 4.4 % ± 0.4 % vs. 5.9 % ± 0.3 % ) . Conclusions Patients with cystic fibrosis with low body weight and poor EFA status benefit from EFA-rich energy supplements and can synthesize arachidonic acid from the precursor linoleic acid BACKGROUND 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 dem and 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 . METHODS Twenty-one patients who were in stable condition were r and omly 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 . RESULTS 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 ( P < 0.05 ) in the lymphocyte GSH levels in the supplemented group . No other changes were observed . CONCLUSION 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 OBJECTIVES We compared the nutritional benefits of a protein hydrolysate and a conventional infant formula in infants newly diagnosed with cystic fibrosis ( CF ) . STUDY DESIGN Twenty-three infants with CF ( < 6 months of age ) and pancreatic insufficiency were r and omized to receive a hydrolysate formula ( Alimentum ) or a cow 's milk-based formula ( Similac ) . Each patient was monitored at 1 month and then every 3 months for 1 year . RESULTS Eighteen patients ( 8 Alimentum , 10 Similac ) completed the study . At entry , the age distribution and clinical characteristics of each group were comparable . Energy intake with each formula was the same at 1 and 3 months , but at 6 and 12 months the hydrolysate-fed infants had higher age-adjusted energy intake . There were no differences in fecal energy or fecal fat at entry or throughout the study . Although the hydrolysate-fed infants were slightly more malnourished at diagnosis , growth velocity and nutritional status of infants with CF in each group were the same throughout the study . CONCLUSIONS The results of this r and omized study fail to support the use of a hydrolyzed formula for the routine care of infants newly diagnosed with CF The use of oral dietary supplements was compared with dietary counseling in 13 malnourished patients ( 3 males , mean age 18.1 years ) with cystic fibrosis .  Energy intake and nutritional status were evaluated over 3 months . There was no significant change in energy intake or percent ideal body weight in either group BACKGROUND Enteral formula feedings are frequently prescribed to cystic fibrosis ( CF ) patients to boost caloric intake . A substantial number of these patients are glucose intolerant and have severe respiratory compromise . METHODS To determine the effect of the carbohydrate content on glucose tolerance and respiratory function in glucose-intolerant CF patients with poor lung function , we examined the response to bolus feedings of five dietary supplements ; a high-fat formula developed in our Clinical Research Center ( CRC ) , Pulmocare , a high-carbohydrate formula developed in our CRC , Ensure Plus , and sugar-free Sc and ishake . RESULTS Glucose excursion in response to the formulas with the lowest carbohydrate content was significantly less than that found in response to formulas with higher carbohydrate content . Insulin levels were also markedly lower in response to the low-carbohydrate high-fat formulas . Glucose excursion , expressed as a percent of the response to the CRC high-fat formula , was 111 % + /- 12 % for Pulmocare ( p = NS ) , 202 % + /- 34 % for Ensure Plus ( p < 01 ) , 227 % + /- 37 % for CRC high carbohydrate ( p = .001 ) , and 357 % + /- 33 % for sugar-free Sc and ishake ( p < .001 ) . CO2 production , O2 consumption , minute ventilation , and respiratory rate increased modestly but not significantly in response to all formulas . No significant differences were found between the formulas in regards to these parameters . There were no subjective complaints of dyspnea during any of the five studies . CONCLUSION The carbohydrate content of liquid dietary supplements appears to be an important determinant of hyperglycemia in glucose-intolerant adult CF patients BACKGROUND & AIMS Probiotics reduce intestinal inflammation in children with cystic fibrosis ( CF ) . We want to determine the effects of Lactobacillus GG ( LGG ) on pulmonary exacerbations in CF . METHODS A prospect i ve , r and omized , placebo-controlled , cross-over study was performed . Nineteen children received LGG for 6 months and then shifted to oral rehydration solution ( ORS ) for 6 months . In parallel nineteen received ORS and then shifted to LGG . Main outcome parameters were : incidence of pulmonary exacerbations and of hospital admissions , forced expiratory volume ( FEV1 ) , and modifications of body weight . RESULTS Patients treated with LGG showed a reduction of pulmonary exacerbations ( Median 1 vs. 2 , range 4 vs. 4 , median difference 1 , CI 95 % 0.5 - 1.5 ; p=0.0035 ) and of hospital admissions ( Median 0 vs. 1 , range 3 vs. 2 , median difference 1 , CI 95 % 1.0 - 1.5 ; p=0.001 ) compared to patients treated with ORS . LGG result ed in a greater increase in FEV1 ( 3.6 % + /- 5.2 vs. 0.9 % + /- 5 ; p=0.02 ) and body weight ( 1.5 kg + /- 1.8 vs. 0.7 kg + /- 1.8 ; p=0.02 ) . CONCLUSIONS LGG reduces pulmonary exacerbations and hospital admissions in patients with CF . These suggest that probiotics may delay respiratory impairment and that a relationship exists between intestinal and pulmonary inflammation 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 aim ed 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 r and omly 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
BACKGROUND Poor nutrition occurs frequently in people with cystic fibrosis ( CF ) and is associated with other adverse outcomes .
Oral calorie supplements are used to increase total daily calorie intake and improve weight gain .
However , they are expensive and there are concerns they may reduce the amount of food eaten and not improve overall energy intake .
OBJECTIVES To establish whether in people with CF , oral calorie supplements : increase daily calorie intake ; and improve overall nutritional intake , nutritional indices , lung function , survival and quality of life .
To assess adverse effects associated with using these supplements .