human
***TASK*** the task is to summarize an input biomedical literature in six sentences 
    ***INPUT*** the input is a biomedical literature 
    ***OUTPUT*** the output is the summary of an input biomedical literature in six sentences 
    ***DOCUMENTATION*** 
    ***EXAMPLES*** 
    Input: 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 
    Output:
    
agent
***TASK*** the task is to summarize an input biomedical literature in six sentences 
    ***INPUT*** the input is a biomedical literature 
    ***OUTPUT*** the output is the summary of an input biomedical literature in six sentences 
    ***DOCUMENTATION*** 
    ***EXAMPLES*** 
    Input: 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 
    Output:
    