Table 1.
Characteristics of included studies.
| First author [reference], study design | Location | Cases; n and characteristics | Controls; n and characteristics | Biomarker of interest |
|---|---|---|---|---|
| AbdulWahab [69], cross-sectional | Doha, Qatar | 53 people with cystic fibrosis (CF) (M = 41.5%; 15.1 ± 9.1 y) from a kindred Arab tribe. 84.9% were I1234V homozygous and pancreatic sufficient (PS); 15.1% were non I1234V and pancreatic insufficient (PI); 35.8% had chronic P. aeruginosa colonisation; Mean ± SD forced expiratory volume in 1 s (FEV1) was 79.1 ± 22.2%. All those with PI were administered with pancreatic enzyme replacement and multivitamin supplements. 17.7% who were PS were receiving multivitamins. | 45 healthy control participants (HCP) (male (M) = not reported (NR); 20.4 ± 10.1 y) were recruited from the same kindred Arab tribe. | Plasma albumin (CF group only) and zinc. |
| Olveira [58], cross-sectional | Malaga, Spain | 36 people with CF (M = 56.3%; 27.2 ± 8.9 y). In those with frequent exacerbations, bacterial colonisation and/or FEV1 ≤ 50% predicted, 500 mg/day of azithromycin was administered (63.9%). Of those administered with azithromycin, mean ± SD FEV1 was 47.1 ± 21.3% and forced vital capacity (FVC) was 60.1 ± 21.8%; 69.6% were PI; 30.4% had CF-related diabetes (CFRD). Of those who were not administered with azithromycin, mean ± SD FEV1 was 77.1 ± 20.9% and FVC was 82.9 ± 17.1%; 61.5% were PI; 38.5% had CFRD. None were receiving dietary supplements with Omega-3 fatty acids. | 41 HCP (M = 41.5%; 29.0 ± 9.6) matched for nutritional status, sex and age. | Plasma glutathione peroxidase (GPx), total antioxidant capacity (TAC), catalase (CAT) and superoxide dismutase (SOD). Plasma or serum F2-isoprostane 8-iso-prostaglandin F2α (8-iso-PG2α), Thiobarbituric acid reactive substances (TBARS), vitamins A, D and E, and zinc. |
| Konstantinidis [139], cross-sectional | Thessaloniki, Greece | 58 people with CF; 27 with (CFwNP) (M = 48.1%; 28.5 ± 6.1 y) and 31 without (CFsNP) (M = 54.8%; 28.1 ± 6.5 y) nasal polyposis; 25.9% were F508del homozygous; 50.0% were F508del heterozygous; 24.1% were non-F508del. 32.8% had P. aeruginosa colonisation. All CF participants were supplemented with 3000 IU/day of Vitamin D3. | 62 people with chronic rhinosinusitis; 32 with (CRSwNP) (M = 53.1%; 29.3 ± 8.4 y) and 30 without (CRSsNP) (M = 46.7%; 29.5 ± 5.9 y) nasal polyposis. 32 HCP (M = 53.0%; 28.2 ± 7.9 y) from an outpatient clinic for elective nasal or oral surgery. | Serum 25(OH) VD3. |
| Antus [74], cross-sectional | Budapest, Hungary | 40 people with CF (M = 60.0%; 25.0 ± 0.9 y); 50% were F508del homozygous. Mean ± SD FEV1 was 54.4 ± 4.0% and FVC was 73.5 ± 3.4%; 52.5% had P. aeruginosa colonisation. All were taking vitamin supplements, pancreatic enzyme supplements, inhaled/nebulised β2-agonists. 31.1% were receiving inhaled corticosteroid and 75.6% were receiving nebulised DNase. 31.1% were receiving inhaled antibiotics at the time of sample collection. | 25 HCP (M = 52.0%; 35.8 ± 2.4 y) with no evidence of pulmonary disease. | Plasma malondialdehyde (MDA). |
| Lee [45], cross-sectional | Atlanta, USA | 25 people with CF (M = 56.0%; 18.5 ± 14.0 y); 88% were PI; 48.0% were administered with vitamin D supplements. | 28 HCP (M = 21.0%; 29.0 ± 6.0 y) on no medications which affect vitamin D concentration and metabolism. Vitamin D intake was limited to <1000 IU/day. | Serum albumin, total and free 25(OH)D and vitamin D binding protein. |
| Turowski [140], flaxseed supplement non-randomized and non-controlled trial | Philadelphia and Pennsylvania, USA | 10 people with CF (M = 30.0%; 31.9 ± 10.8 y; FEV1 76.8 ± 16.8% predicted; body mass index (BMI) 22.0 ± 1.8 kg/m2); 70.0% were PI, 80.0% taking pancrealipase. | 5 HCP (comparable in age). | Plasma enterodiol and enterolactone. |
| Yadav [59], cross-sectional | Chandigarh, India | 21 people with CF (M = 81.5%; 5.7 ± 2.5 y; this includes those with an acute respiratory exacerbation); 22.2% were receiving pancreatic enzyme supplementation. | 27 HCP (M = 70.4%; 7.4 ± 2.7 y) who were matched for age and sex. | Plasma 25(OH)D, and vitamins A and E. Serum copper, iron and zinc. |
| Sadowska-Bartosz [53], cross-sectional | Rzeszow, Poland | 22 people with CF; 12 with chronic P. aeruginosa (M = 30.0%; 12.8 ± 7.6 y; BMI 18.7 ± 2.9 kg/m2) and 10 with chronic S. aureus (M = 30.0%; 10.2 ± 3.6 y; BMI 18.6 ± 3.8 kg/m2) infections; 40.1% for F508del homozygous. Of those with P. aeruginosa colonisation, mean ± SD FEV1 was 71.0 ± 8.7% predicted and FEV1/FVC ratio was 79.7 ± 20.0%. Of those with S. aureus colonisation, mean ± SD FEV1 was 94.2 ± 11.4%, and FEV1/FVC ratio was 89.6 ± 17.8%. All were PI and receiving pancreatic replacement therapy and nebulised DNase. All were receiving multivitamin supplements, nutritional drinks, inhaled sodium chloride. Those with P. aeruginosa were receiving 250 mg azithromycin 3 days per week. | 11 HCP (M = 45.5%; 11.3 ± 4.5 y; BMI 16.7 ± 1.5 kg/m2) who were recruited as outpatients without chronic disease. FEV1 was 96.5 ± 12.1% predicted and FEV1/FVC ratio was 107.5 ± 5.9%. | Plasma advances glycation end-products (AGE), amadori products, advanced oxidation protein products (AOPP), dityrosine, formylkynurenine, kynurenine, protein carbonyls, thiol groups and tryptophan. Erythrocyte CAT, glutathione S-transferase (GST), SOD and TAC. |
| Ambroszkiewicz [60], cross-sectional | Warsaw, Poland | 35 people with CF (M = 48.6%; median (range), 7.0 (5–9) y); 60.0% were F508del homozygous; 29.0% were F508del heterozygous; 11.0% were non-F508del. Mean ± SD FEV1 was 89.6 ± 12.2% predicted. No participants had CFRD and 2.9% had hepatic insufficiency; 94.3% of participants were PI, and were receiving pancreatic enzyme replacement supplements (6000 U lipase/kg/day). No participants received inhaled or systemic corticosteroids 1 month prior to sample collection. All participants were supplemented with Vitamins A (2000 IU/day), E (200 IU/day) and D3 (400 IU/day) | 35 HCP (M = 48.6%; median (range), 7.0 (5–9) y) who were matched for age and sex. HCP were recruited from an outpatient clinic and presented with minor problems other than infections and diseases which may influence bone status. | Serum 25(OH)D, and vitamins A and E. |
| Olveira [52], cross-sectional | Malaga, Spain | 36 people with CF (M = 50.0%; 27.2 ± 8.9 y). Mean ± SD FEV1 was 57.8 ± 25.4% and FVC was 68.2 ± 22.8%; 86.1 were colonized with P. aeruginosa and mean ± SD exacerbations for the past year was 2.4 ± 2.0. None were receiving Omega-3 fatty acid supplements. | 50 HCP (M = 25.0%; 12.8 ± 7.6 y); 54 non-CF bronchiectasis (M = 29.7%; 47.4 ± 18.9 y). | Plasma or serum copper, selenium and vitamin C. Plasma 8-iso-PG2α, CAT, GPx, SOD, TAC and TBARS. Serum vitamins A, D, E:cholerterol and zinc. Neutrophil, lymphocyte, monocyte and total leukocyte peroxide, superoxide and reduced glutathione (GSH). |
| Bernardi [39], cross-sectional | Sao Paulo, Brazil | 44 people with CF (M = 50.0%; 8.4 ± 3.2 y); 31.8% were F508del homozygous; 68.2% for F508del heterozygous; 100% were PI; 6.8% had CFRD; 38.6% had hepatic disease. Furthermore, 25.0% were colonized with S. aureus and 6.8% were colonized with P. aeruginosa. | 16 HCP (M = 25.0%; 8.3 ± 2.6 y). | Erythrocyte GSH. |
| Sadowska-Woda [72], AquADEK supplementation non-randomized controlled trial | Rzeszow, Poland | 50 people with CF (M = 60.0%; 9.6 ± 3.7 y; BMI 19.3 ± 2.8 kg/m2). Mean ± SD FEV1 was 57.8 ± 25.4% predicted and FVC was 68.2 ± 22.8%; 100.0% were PI and receiving pancreatic enzyme replacement supplements (Creon or Lapancrea). Children were receiving daily inhaled DNase. | 21 HCP (M = 66.7%; 9.6 ± 3.1 y; BMI 20.1 ± 5.2 kg/m2) who were not receiving multivitamin supplementation. Mean ± SD FEV1 104.2 ± 12.3% predicted and FEV1/FVC was 90.0 ± 12.7%. | Plasma hydroperoxides, MDA, thiol groups and TAC. Erythrocyte CAT, SOD and thiol groups. |
| Cobanoglu [66], cross-sectional | Ankara, Turkey | 16 people with CF (M = 50.0%; 6.1 ± 1.5 y and pre-pubertal); 6.3% were F508del homozygous; 43.8% were F508del heterozygous; 50.0% were non-F508del; 100.0% were PI and receiving pancreatic enzyme replacement supplements and 800 IU/day of vitamin D. Non-had CFRD or liver disease. None were receiving anti-epileptic drugs, calcium supplements, systemic or inhaled steroids. | 16 HCP (M = 50.0%; 6.1 ± 1.4 y) admitted to hospital for minor issues other than infection and were pre-pubertal. | Serum 25(OH)D. |
| Durieu [43], intravenous fish oil n-3 emulsion open-pilot observation | Lyon, France | 13 people with CF (M = NR; 19.4 ± 11.1 y) prior to treatment with intravenous n-3 fatty acids. Mean ± SD FEV1 was 81.5 ± 12.8% and 51.0 ± 15.6% for children and adults, respectively. | 21 HCP (M = NR; age range, 20–55 y) from a related study [141]. | Plasma hydroperoxides. Plasma vitamins A and E, carotenoids and MDA in CF group only. |
| van Biervliet [142], cross-sectional | Ghent, Belgium | 104 people with CF; separated into two groups, (A) class I, II, III (M = 50.6%; median (IQR), 15.0 (13.4) y), and (B) class IV, V or unknown CFTR genotypes (M = 56.0%; median (IQR), 16.0 (20.0) y). Group A: median (IQR) FEV1 was 80.4 (40.4)% predicted and FVC 89.7 (21.3)% predicted; 100% were PI; 11.4% had CFRD; 16.5% had liver disease. Group B: median (IQR) FEV1 was 75.0 (41.4)% and FVC 86.7 (21.3)% predicted; 32.0% were PI; 19.0% had CFRD; 8.0% had liver disease. None received polyunsaturated fat supplements. PI participants received pancreatic enzyme replacement and multivitamin supplements (1000 IU cholecalciferol, 100 mg α-tocopherolacetate, 1 mg phytoimenadion and 10000 IU retionolacetate). | 44 HCP (M = NR; median (range), 18.0 (1–47) y). | Serum α-linolenic acid, arachidonic acid, docosahexaenoic acid, linoleic acid and oleic acid. |
| Rovner [67], cross-sectional | Pennsylvania, USA | 101 people with CF (M = 50.0%; 14.8 ± 4.2 y). FEV1 was 84 ± 19% predicted. None had CFRD. All participants were receiving vitamin D and pancreatic enzyme supplements. | 177 HCP (M = 42.0; 12.5 ± 3.5 y). | Serum 1,25(OH)2D and 25(OH)D. |
| Oudshoorn [63], cross-sectional | Utrecht, The Netherland | 30 people with CF (M = 53.3; 11.9 ± 2.6 y, range 8–18 y). FEV1 was 88.5 ± 18.7% predicted. All received a mean ± SD 120 ± 75 mg/day α-tocopherol supplementation. | 30 outpatients (M = 43.3; 11.3 ± 2.9 y) who underwent ear, nose or throat surgery. | Plasma α-tocopherol, coenzyme Q10:cholesterol ratio, total, oxidized, reduced coenzyme Q10. |
| Tirouvanziam [40], non-randomized trial of N-acetylcysteine | Stanford, USA | 18 people with CF (sex and age NR) prior to treatment with N-acetylcysteine. | 9 HCP (sex and age NR). | Neutrophil GSH. |
| Nicolaidou [44], cross-sectional | Athens, Greece | 25 people with CF (M = 50.0; range 6–17 y, median (p25, p75), M = 15.0 (14.0, 16.0), F = 14.5 (11.0, 15.0) y) without a vitamin K intervention. Median (p25, p75) FEV1 was 71.5 (45, 85)% predicted for males and 75.5 (62, 104)% predicted for females. No group specific genotype data was available. 100.0% were PI and receiving pancreatic enzyme replacement supplements. None were receiving antibiotics or had ever received corticosteroids. All were receiving 800 IU/day vitamin D | 25 HCP (M = 52.0; range 8–17 y, median (p25, p75), M = 14.0 (12.0, 16.0), F = 11.0 (9.0, 14.0) y). | Serum 25(OH)D and vitamin K. |
| Back [42], cross-sectional | Tubingen, Germany | 22 people with CF (M = 40.9%); separated into 3 groups for analysis: those who are 7 6–11 y (median (IQR), 9.4 (7.7–11.1) y), 7 12–17 y (median (IQR), 15.0 (14.0–16.7) y) or 7 ≥ 18 y (median (IQR), 23.6 (19.7–29.0) y); 27.3% were F508del homozygous; 45.5% were F508del heterozygous; 13.6% were non-F508del. 86.4% were PI and 18.2% had CFRD. Median (IQR) FEV1 was 90.0 (84.5–102.0)% predicted in those 6–11 y, 80.0 (63.0–82.0)% predicted in those 12–17 y and 65.0 (42.0–84.0)% predicted in those ≥18 y. FVC was 92.0 (87.0–95.0)% predicted in those 6–11 y, 81.0 (71.0–86.0)% predicted in those 12–17 y and 6588 (65.0–95.0)% predicted in those ≥18 y. | 30 HCP (M = 53.3); separated into 3 groups for analysis: 9 6–11 y (median (IQR), 8.4 (7.4–10.3) y), 5 12–17 y (median (IQR), 13.3 (12.6–16.4) y) and 16 ≥ 18 y (median (IQR), 27.6 (24.5–30.2) y). | Plasma vitamin E, β-carotene, β-cryptoxanthin, lycopene, protein carbonyls, TBARS and vitamin C. |
| Best [71], randomized controlled trial of copper supplementation | Columbus, USA | 38 people with CF (M = NR; 24.8 ± 8.0 y, range 12–48 y) before supplementation with copper and zinc; 100% had PI and were receiving pancreatic enzyme replacement and multivitamin supplements (ADEK). None had CFRD, renal failure or advanced lung disease. | 30 HCP (age and sex NR). | Erythrocyte SOD. Plasma ceruloplasmin and diamine oxidase |
| Lagrange-Puget [7], cross-sectional | Lyon, France | Up to 232 people (depending on biomarker studied) with CF (M = NR; mean (range), 13.0 (0.5–45.0) y). All had PI and were receiving pancreatic enzyme replacement supplements. Those with vitamin A and E deficiency received retinol or tocopherol supplements. | 53 HCP (M = NR; mean (range), 22.0 (1.0–40.0) y); including, 21 children admitted for orthopedic surgery (before treatment) and 32 healthy individuals attending a phase I clinical trial centre. | Plasma α-carotene, β-carotene, GSH, tGSH, lipid peroxides, lutein, lycopene, MDA, TBARS, vitamins A and E, and zeaxanthin. |
| Schupp [57], cross-sectional | California, USA | 10 people with CF (M = 90.0; 31.2 ± 10.0 y, range 21–47 y). Mean ± SD FEV1 was 45.0 ± 25.4% predicted. 100% were PI and receiving pancreatic enzyme replacement supplements. 30% had CFRD. All were receiving nutritional supplements, however, 10% were receiving multivitamin supplements containing lutein. | 10 HCP (age range 20–51 y) matched for age, sex and ethnicity. | Plasma lutein and zeaxanthin. |
| Sidlova [88], cross-sectional | Prague, Czech Republic | 37 people with CF (M = 64.9; mean (range), 10.4 (1–28) y); 10.8% had hepatobiliary abnormalities. | 27 controls (M = 66.7; mean (range), 8.5 (2–17) y). | Serum GSTα |
| Aris [68], cross-sectional | North Carolina, USA | 50 people with CF (M = 46.0; 28.3 ± 7.8 y); 56% were F508del homozygous; 28% were F508del heterozygous; 16% were non-F508del. Mean ± SD FEV1 was 46.1 ± 18.6% predicted and FVC was 67.5 ± 17.9% predicted. 94% were PI and were receiving pancreatic enzyme replacement supplements; All were receiving multivitamin supplementation. | 53 HCP (M = 50.9; 28.9 ± 7.8 y) who were matched for age and sex. | Serum 1,25(OH)2D and 25(OH)D. |
| McGrath [54], cross-sectional | Belfast, UK | 11 people with CF (M = 72.7%; range, 18–37 y). All were receiving vitamin E supplementation (200 mg/day). None receiving any other antioxidants and all non-smokers. | 11 HCP (M = 72.7%; range, 20–35 y). All non-smokers. | Plasma vitamin E:cholesterol, MDA:cholesterol, protein carbonyls and protein thiols. |
| Wood [41], cross-sectional | Newcastle, Australia | 21 people with CF (M = 61.9; 14.8 ± 1.1 y); 57% were F508del homozygous; 38% were F508del heterozygous; 5% were non F508del. Mean ± SEM FEV1 was 85.3 ± 6.2% predicted and FVC was 90.1 ± 4.7% predicted. 91% were PI; 71% were receiving aerosol β2-agonists; 33% were receiving inhaled corticosteroids; 14% were receiving cromoglycate; 24% were receiving ipratropium; 19% were receiving DNase; 19% were receiving antibiotics. None received vitamin supplementation in the previous 4 wk. | 21 HCP (M = 61.9; 14.2 ± 1.1 y) matched for age and sex. | Erythrocyte SOD. Plasma 8-iso-PGF2α, β-carotene, GPx, selenium, zinc, copper, vitamins A, C and E. |
| Madarasi [61], cross-sectional | Budapest, Hungary | 21 people with CF (M = 57.1; mean (range), 8.7 (6–12) y). None had liver manifestations; 100.0% received pancreatic enzyme replacement and multivitamin supplements. | 24 HCP (M = 45.8; mean (range), 8.3 (6–12) y) matched for age. HCP were formally hospitalised children with minor ailments who were attending clinic for follow-up blood testing. | Erythrocyte CAT and SOD. Serum α-tocopherol, ascorbic acid, MDA, TAC, uric acid and vitamin A. Whole blood GPx. |
| Lands [73], cross-sectional | Montreal, Canada | 24 people with CF (M = 62.5; 11.4 ± 3.4 y), who were not hospitalised for an acute respiratory exacerbation. Mean ± SD FEV1 was 77.6 ± 17.4% predicted and RV/TLV was 31.1 ± 10.3%. | 17 HCP (M = 58.8; 23.8 ± 3.9 y) who were recruited as part of a study investigating oxidative stress and exercise. | Plasma trolox equivalent antioxidant capacity (TEAC). |
| Percival [70], cross-sectional | Florida, USA | 7 people with CF (M = 100.0; 24.5 ± 3.5 y, range 19–32 y). Mean ± SD FEV1 was 55.0 ± 28.8% (range 25–99%) and FVC was 75.8 ± 20.2% (51–105%) predicted. 85.7% were PI and receiving pancreatic enzyme replacement supplements. All were receiving multivitamin supplements. | 6 HCP (M = 100.0; 24.5 ± 5.5 y, range 20–30 y) who were matched for age. | Plasma, polymorphonuclear cell and mononuclear cell copper. Polymorphonuclear cell and mononuclear cell Copper–Zinc SOD. Plasma ceruloplasmin. |
| Tauber [143], cross-sectional | Vienna, Austria | 28 people with CF (M = 51.1; 10.8 ± 6.5 y; this includes those with an acute respiratory exacerbation (n = 17)) who did not have an acute respiratory exacerbation; 4.4% of all (including those with an acute respiratory exacerbation) had chronic P. aeruginosa infections. | 175 children with bronchial asthma (BA) (M = 55.4; 9.8 ± 3.8 y); 87 HCP (M = 29.9; 10.2 ± 4.5 y); 23 non-asthmatic children with bacterial lower respiratory tract infection (LRTI) (M = 39.1; 8.6 ± 2.5 y). | Serum myeloperoxidase (MPO). |
| Collins [75], cross-sectional | New South Wales, Australia | 10 people with CF (sex NR; mean (range), 17.4 (5.8–37.8) y; this includes those with an acute respiratory exacerbation (n = 12)), who are not undergoing treatment for an acute respiratory exacerbation. | 9 HCP (sex NR; mean (range), 29.8 (22.8–46.1) y). | Plasma 8-iso-PGF2α. |
| Eichler [144], cross-sectional | Vienna, Austria | 23 people with CF (M = 57.1; median (range), 14.5 (8–17) y; this includes those with an acute respiratory exacerbation (n = 19)). In those who were clinically-stable, median (IQR) FEV1 was 81.2% (72.4–98.4) and FVC was 87.8% (79.6–102.3%) predicted. | 25 HCP (M = 56.0; median (range), 13.4 (3–16) y). | Serum lactoferrin and MPO. |
| Dominguez [64], cross-sectional | Barcelona, Spain | 101 people with CF (M = 54.5%; 11.5 ± 6.9 y). | 43-95 HCP (varies between biomarkers) of a similar age to the CF group (age and sex NR). | Erythrocyte SOD. Plasma α-tocopherol, glutathione reductase (GR), hydroperoxides, MDA and protein carbonyls. |
| Koller [145], cross-sectional | Vienna, Austria | 23 people with CF (M = 57.1; median (range), 14.5 (8–17) y; this includes those with an acute respiratory exacerbation (n = 19)), without an acute respiratory exacerbation. Median (quartile 1–3) FEV1 was 81.2 (72.4–98.4)% and FVC was 87.8 (79.6–102.3)%. | 25 HCP (M = 56.0; median (range), 13.4 (3–16) y). | Serum eosinophil peroxidase. |
| Hung [89], cross-sectional | Edinburgh, UK | 63 people with CF (M = 49.2; mean (range) 7.9 (0.5–16) y). All participants were receiving pancreatic enzyme replacement and multivitamin supplements; all participants were receiving flucloxacillin, inhaled steroids and/or inhaled steroid and bronchodilators. | 59 HCP (M = 62.7; mean (range), 7.2 (1–16) y). 96.6% were screened for CF and fragile X syndrome but had normal genotypes; 1.7% had bronchiolitis; 1.7% had an inguinal hernia. | Serum GSTα1. |
| Kearns [46], cross-sectional | Arkansas, USA | 15 people with CF (M = 46.7%; 13.1 ± 2.7 y, range 8.7–19.3 y); 7/15 had genotype analysis; 100% were F508del homozygous; 13.3% were receiving pancreatic enzyme replacement and multivitamin supplements. | 15 HCP (M = 46.7%; 14.5 ± 3.1 y, range 8.9–18.9 y). | Serum albumin and (γ-glutamyl transpeptidase) GGT. |
| Koller [146], cross-sectional | Vienna, Austria | 59 people with CF (M = 45.9%; 11.0 ± 7.7 y; this includes those with an acute respiratory exacerbation (n = 39)) without an acute respiratory exacerbation. | 85 HCP (M = NR; 10.8 ± 5.7 y). | Serum MPO. |
| Winklehofer-Roob [56], pre-β-alanine intervention | Zurich, Switzerland | Up to 32 people with CF (M = 48.6%; 10.8 ± 7.6 y); 100.0 were PI and receiving pancreatic enzyme replacement and multivitamin supplements. | Up to 40 HCP (M = 35.7%; 31.5 ± 8.0 y). | LDL α-tocopherol and β-carotene. Plasma α-tocopherol, β-carotene and MDA. |
| Mocchegiani [47], cross-sectional | Ancona, Italy | 15 people with CF (M = 46.7%; mean (range), 6.7 (2–13) y); 90% were colonized with P. aeruginosa. All were receiving pancreatic enzyme replacement and vitamin supplementation. No individuals with signs of diabetes or heart failure were included. All were receiving antibiotics and bronchodilators. | 15 HCP (M = 60.0%; mean (range), NR (2-13) y) who were admitted to hospital for minor surgery. | Serum albumin. Plasma Zinc. |
| Koller [147], cross-sectional | Vienna, Austria | 42 people with CF (M = 47.6%; mean (range), 14.5 (0.8–28) y). Mean ± SD FEV1 was 67.8 ± 32.4% predicted (range 17.1–125.5% predicted), MEF50 was 52.0 (42.5)% predicted (range 4.4–130.2% predicted). 73.3% were colonized with P. aeruginosa. | 30 HCP (M = 53.3%; mean (range), 13.4 (4–32) y). | Serum MPO. |
| Mangione [51], cross-sectional | Pennsylvania, USA | 32 people with CF (age and sex NR). | 8 HCP who were healthy, active, non-smokers and age-matched to their CF-counterparts. | Erythrocyte GSH. |
| Vaisman [62], cross-sectional | Rehovot, Israel | 11 people with CF (mean (range), NR (4-14) y). “Most” were colonized with P. aeruginosa. All were receiving vitamin A and E supplements (100 IU) and multivitamin preparations. | 10 age-matched HCP (age and sex NR). Not receiving any vitamin supplementation. | Plasma tocopherol vitamin A. Blood neutrophil tocopherol. |
| James [38], cross-sectional | Cardiff, UK | 22 people with CF (M = 45.5%; median (range), 8.3 (1.6–16.5) y). | 9 HCP (M = 22.2%; median (range), 10.9 (4.7–16.4) y). | Plasma and erythrocyte α-tocopherol. Serum selenium. |
| Stead [50], cross-sectional | London, UK | 31 people with CF (24.5 y (range, 17–52 y)); 93.5% receiving pancreatic enzyme replacement and no evidence of liver disease. FEV1 was 41.5 ± 11.4% predicted. 96.8% taking vitamin D supplements (daily intake range: 0.6–54.3 μg [24–2172 IU)]. | 28 HCP (M = NR; age = NR). | Serum 25(OH)D, and 1,25(OH)2D. Plasma albumin. |
| Reiter [65], cross-sectional | Massachusetts and Arizona, USA | 20 people with CF (M = 50.0%; range, 12–25 y); 50% in Massachusetts and 50% in Arizona. All were receiving pancreatic enzyme replacement, vitamin supplementation and intermittent antibiotic therapy. | 8 HCP (M = 40.0%; 18.6 ± 8.1 y). | Serum carotene, albumin and 1,25(OH)2D. |
| Hahn [48], cross-sectional | St. Louis, USA | 21 people with CF (M = 57.1%; mean (range), 20.9 (12–36) y). All were PI. All were receiving pancreatic enzyme replacement (given as Cotazyme) and multivitamin supplements (containing 400 units of vitamin D/tablet). One patient had diabetes and was receiving oral hypoglycaemic agents. | 21 age- and sex-matched HCP (M = 57.1%; mean (range), 20.3 (12–34) y). | Serum 25(OH)D, albumin and carotene. |
| Jacob [49], cross-sectional | Chicago, USA | 18 people with CF (range, 6–17 y). All were receiving daily multivitamin supplements (Poly-vi-sol) containing 2500 IU of water miscible vitamin A per tablet. | 40 adolescent HCP (range, 11–17 y). | Plasma vitamin A, zinc and albumin. |
| Hubbard [55], cross-sectional | Tennessee, USA | 16 people with CF (M = 43.8%; range, 13–47 y); 68.8% were PI. Those with PI were receiving pancreatic enzyme replacement and multivitamin supplements. | 8 HCP (M = 50.0%; range, 19–32 y). | Serum vitamin A and carotene. Plasma vitamin E. |
Data is expressed as mean ± standard deviation (SD) unless otherwise stated.