Hillman 2008 [] Tablets: 1,600 IU D3+500 mg calcium carbonate 2/d. placebo 6 months 15 Children and adolescents. 7 - 13 years. Serum minerals, bone turnover, BMC Gontijo-Amaral 2008 [] 300 mg oral (5-12 placebo 8 weeks 44 Stable clinical condition. Range: 7 - 19 years. Respiratory muscle strength, AE Drury 2008 [] 5 mg/day of vitamin K1. 1 mg/day of vitamin K1. 1 month 14 Pancreatic insufficient. Range: 8 - 18 years. Vitamin K status Watson 2008 [] Behavioral Nutrition Education (topics: increasing intake, managing appetite, etc). standard care 10 weeks 74 Understand written English. >16 Weight change, nutrition knowledge score, self-efficacy score, fat intake, health-related quality-of-life score Schnabel 2007 [] 0.070 mg/kg or 0.039 mg/kg body weight Somatropin. Max daily dosage: 3.4 mg. placebo 48 weeks, followed by an open treatment period of 24 weeks. 63 Bone age of 8-18 years, BMI 10th and/or weight 3rd percentile despite high caloric intake. Range: 9 - 20 years. Change in FEV1, height, weight, exercise tolerance. Hardin 2005 [] 1 year of GH (0.3 mg/kg per week) followed by a second year (injection) no GH for 1 year, followed by 1 year of GH 24 months 18 Received EN supplementation for≥2 years before enrollment. Range: 9 - 13 years. Pulmonary function, anthropometrics, hospitalizations, antibiotics, intake, protein turnover.
Oudshoorn 2007 [] 100 ml/d of a liquid micronutrient mixture. placebo 3 months 22 Predominantly mild lung disease. 12.9±2.5 years (mean±SD) Anthropometrics, pulmonary function, exercise performance, muscular strength, vitamins A and E. Poustie 2006 [] Oral protein energy supplements+ routine dietetic advice. Routine dietetic advice. 12 months 102 Moderately malnourished. Range: 2 - 15 years. Anthropometrics, intake, pulmonary function, habitual activity scale, gastrointestinal symptoms. Powers 2005 [] Nutrition counseling and child behavioral training. standard care 8 weeks 14 Mild to moderate lung disease, pancreatic insufficient Range: 8 - 20 years. Energy Intake, Weight, Height. Lloyd-Still 2006 [] Algal oil (50 mg/kg/d of DHA 1 to 4.2 g). placebo 6 months 20 Mild to moderate lung disease, pancreatic insufficient 8 - 20 years. FA, liver enzymes, lipid antioxidants, fecal FA, lung function, AE anthropometrics. Kalnins 2005 [] A supplement (150 kcal/100 mL; 20% protein, 30% fat, 50% carbohydrate). dietary counseling 3 months 13 <90% IBW or 5% reduction in IBW over a 3-month >10 years Anthropometrics, pulmonary function, REE, fecal fat output. Hardin 2006 [] Daily GH injections (0.3 mg/kg*wk). no treatment 24 months 57 Height and weight in 25th percentile or lower; Tanner I Range: 7 - 12 years. Anthropometrics, pulmonary function,
breast; testicular development≤ 3 cc. hospitalizations, antibiotic use, intake. Grey 2003 [] Whey protein isolate (20 g/d). casein placebo 3 months 24 Stable with mild to moderate lung disease, not severely malnourished. Adults Lymphocyte glutathione levels, height, weight, Pulmonary function. Homnick 2004 [] 8 mg/d Cyproheptadine for one week increased to 16 mg/d in the remaining weeks. placebo 12 weeks 18 IBW <100%, clinically stable. ≥5 years Appetite, weight, spirometry, antibiotic use. Darmaun 2004 [] GLN/rhGH combined. daily oral GLN (0.21 g/kg/d) or rhGH (0.3 mg/kg/wk) 4 9 Weight/height, <50th percentile or height <5th percentile 9.6±0.5 years (mean±SD) Protein Metabolism Ellis 1998 [] Hydrolyzed protein formula. Conventional cow’s milk–based formula 12 months 23 Newly diagnosed, pancreatic insufficient, fed infant formula. < 6 months Anthropometrics, dietary intake Wood 2003 [] 200 mg vitamin E, 300 mg vitamin C, 25 mg carotene, 90 g Se, and 500 g vitamin A Supplement: 10 mg vitamin E and 500 g vitamin A 8 weeks 46 Normal vitamin E plasma concentration. > 5 years Changes in plasma plasma FA status, clinical markers, antioxidant defenses
Renner 2001 [] 3 months β-carotene 1 mg/kg/d (maximum 50) + 3 months 10 mg/day. placebo 6 months 24 No specific inclusion. Range: 6.7-27.7 years. Plasma β-carotene, total antioxidant capacity, antibiotics, Shwachmann score, BMI, height, MDA, pulmonary function. Gottrand 1999 [] Enteral diet, "high fat" 33/67 non protein energy glucose to fat ratio. Enteral diet, "normal fat" 60/40 non protein energy glucose to fat ratio. 4 weeks 8 Receiving nighttime enteral nutrition for ≥1 month before the study. Range: 6-19 years. Pulmonary function, substrate oxidation, energy intake, tolerance Beker 1997 [] 5 mg/week oral vitamin K1. no supplementation 4 weeks 18 Stable, afebrile, without cholestasis/ overt liver disease, normal AST/ALT, normal albumin. Range: 13-35 years. Plasma vitamin K1, vitamin PIVKA-II, serum Osteocalcin, urinary GLA Van Egmond 1996 [] Predigested formula with 12% linoleic acid. Predigested formula with 7% linoleic acid. Not clear 76 Diagnosed by 9 mo, formula providing > 10% of intake ≥1 year Anthropometrics Stark 1995 [] Nutritional education+ mealtime behaviors. standard care 6 weeks 9 No specific inclusion. Range: 2-6 years. Calorie intake, weight gain Kurlandsky 1994 [] N-3 (menhaden oil 100-131 mg/kg/day, mean 112.8). N-6 (safflower oil mg/kg/day, mean 113.3). 6 weeks 14 Ability to pulmonary function + swallow capsules. Range: 6-16 years. Absorption and clinical effects of n-3 vs. n-6 fatty acids
Hanning 1993 [] Grocery foods - 25% of recommended energy intake. no supplement 6 months 20 Mild to moderate lung disease, no supplemental tube feeding or PN. 7-15 years. Skeletal and respiratory muscle function Marchand 2000 [] MA 10 mg/kg/d placebo 12 weeks 12 Loss/ plateau in weight>3 months, weight-for height < 85%, negative change in weight z score. Range: 21 months years. Anthropometrics, caloric intake, clinical assessment. Shepherd 1988 [] EN semi-elemental high nitrogen peptide formula (1000-1500 kcal/d more in intake). Standard diet 2-3 weeks 15 Acute pulmonary exacerbations. Range: 6.5 -17 years. Protein turnover, mean body weight, FEV1. Milla 1996 [] Equal caloric (355 kcal) and volume amount of one of five formulas with carbohydrate-to-fat ratios (0.5-1.7). carbohydrate-to-fat ratios of 0.5 2 months 5 Glucose intolerant Average age of 39 ± 3 years Glucose tolerance, dyspnea, CO2 retention. Kane 1990 [] Tube during admission for exacerbation: 1000 kcal, carbohydrate: low/medium/high. Low carbohydrate formula 8-10 days 10 Mild to moderate lung disease Median age 8-14 years. Respiratory function, nighttime oxygen therapy, energy metabolism during feedings.
and magnesium stearate (2 mg). taken antibiotics for at least two weeks. Fallahi 2013 [] One probiotic sachet each day. The powder used was 1 g Protexin Restor sachet, which contains of FOS and a mixture of 1×109 CFU/sachet bacteria. Placebo (maltodextrin) 4 weeks 47 All the patients had pancreatic insufficiency. No use of NSAID during the last two weeks. No antibiotic therapy during the study. All patients were pediatric cases referred to gastrointestinal clinic. > 4 years Status of intestinal inflammation (calprotectin level in fecal samples). Bertolaso 2014 [] Second generation LXS (LYM-X-SORB™- organized lipid matrix): 2 packets/day (64 g powder) for ages 5.0 to 11.9 yrs, and 3 packets/day (96 g powder) for ages 12.0 to 17.9 yrs. Placebo 12 months 58 All the patients had pancreatic insufficiency and mild to moderate lung disease. Range: 5-17.9 years Serum vitamins A, D, E, K and plasma proteins induced by vitamin K absence, dietary intake, supplemental vitamin intake, anthropometrics.
Gifford 2014 [] Ferrous sulfate 325mg taken orally once a day. Placebo 6 weeks 24 Patients with hypoferremic anemia adults Absolute change from baseline in hemoglobin concentration attributed to ferrous sulfate, iron related hematologic parameters, anthopometrics, sputum iron, Pulmonary Exacerbation Score (PES), sputum microbiome. Engelen 2013 [] 6.7g high-leucine EAA mixture (containing 41% leucine) + 15g carbohydrates, orally or enterally. 6.7g balanced mixture of total (EAA and non-essential) amino acids + 15g carbohydrates, orally or enterally. 1 day 15 Clinically stable inpatients after antibiotic treatment with no symptoms of pulmonary exacerbation. Pancreatic insufficient. Range: 10-21 years Whole body protein and Arginine metabolism, anthropometrics, lung function. Alicandro 2013 [] 100 mg/ kg/day of DHA during the first month and 1 g/day in the remaining 11 months as placebo. Placebo 12 months 41 Clinically stable, pancreatic insufficient, able to perform respiratory function Range: 6-10 years Percentage change in plasma AA:DHA ratio, number of pulmonary exacerbations compared to the previous year, lung function, anthropometrics, body composition, serum TNF-a, IL8 and CRP, serum and retinol.