Vitamin A status and its determinants in patients with cystic fibrosis

Sapiejka, Ewa; Krzyżanowska, Patrycja; Walkowiak, Dariusz; Wenska-Chyży, Ewa; Szczepanik, Mariusz; Cofta, Szczepan; Pogorzelski, Andrzej; Skorupa, Wojciech; Walkowiak, Jarosław

doi:10.17306/j.afs.0473

Cited by 6 publications

(8 citation statements)

References 27 publications

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“…Sapiejka et al reported a frequency of 16.3% vitamin A deficiency, and stated the ALT and GGT values as 24.0 ve 12.0 U/L, respectively, in 196 CF patients. 19 Similarly, in our study, 45.4% of patients with CF had vitamin D deficiency and 23.8% vitamin A deficiency. The mean values of ALT and GGT were reported as 27.95 AE 16.01 U/L, and 15.25 AE 14.48 U/L, respectively.…”

Section: Discussionsupporting

confidence: 73%

Molecular Heterogeneity in Cystic Fibrosis

Civan

Seyhan

2020

J Pediatr Genet

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We aimed to evaluate type, frequency, and variety of pathogenic variants according to clinical and demographic features of children diagnosed with cystic fibrosis (CF). Twenty-five CF patients were evaluated retrospectively. Patients' demographics, physical examination, imaging, laboratory, and molecular pathogenic variant analysis findings were evaluated. Phe508del was the most frequently (33.3%) detected pathogenic variant, followed by point pathogenic variants E92K, 1898 + lGA/7T/7T, and 2789 + 5GA, respectively. Statistically higher rates of pathogenic variants were detected in male patients. The most frequently detected pathogenic variant was Phe508del. The identification of nine additional pathogenic variants of Phe508del revealed the heterogeneous nature of the CF.

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Section: Discussionsupporting

confidence: 73%

Molecular Heterogeneity in Cystic Fibrosis

Civan

Seyhan

2020

J Pediatr Genet

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“…Fat-soluble vitamin deficiency remains a challenge for many CF patients without access to CFTR potentiators and modulators, as well as for those who exhibit insufficient vitamin levels despite such treatment [11][12][13][14]. Moreover, the problem is relevant to other diseases that involve fat maldigestion and malabsorption, such as chronic pancreatitis or biliary atresia.…”

Section: Discussionmentioning

confidence: 99%

“…The knowledge of the responsiveness of individual patients to each fat-soluble vitamin might potentially enrich the interpretation of study results. However, such responsiveness is difficult to define or measure despite much research [11,13,14,18] and in clinical practice, no specific assessment of fat-soluble vitamin responsiveness is used. Instead, the doses are adjusted in each CF patient until satisfactory results are obtained and thus reflect individual needs of the patient.…”

Section: Generalizabilitymentioning

confidence: 99%

Fat-Soluble Vitamin Supplementation Using Liposomes, Cyclodextrins, or Medium-Chain Triglycerides in Cystic Fibrosis: A Randomized Controlled Trial

et al. 2021

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Fat-soluble vitamin deficiency remains a challenge in cystic fibrosis (CF), chronic pancreatitis, and biliary atresia. Liposomes and cyclodextrins can enhance their bioavailability, thus this multi-center randomized placebo-controlled trial compared three-month supplementation of fat-soluble vitamins in the form of liposomes or cyclodextrins to medium-chain triglycerides (MCT) in pancreatic-insufficient CF patients. The daily doses were as follows: 2000 IU of retinyl palmitate, 4000 IU of vitamin D3, 200 IU of RRR-α-tocopherol, and 200 µg of vitamin K2 as menaquinone-7, with vitamin E given in soybean oil instead of liposomes. All participants received 4 mg of β-carotene and 1.07 mg of vitamin K1 to ensure compliance with the guidelines. The primary outcome was the change from the baseline of all-trans-retinol and 25-hydroxyvitamin D3 concentrations and the percentage of undercarboxylated osteocalcin. Out of 75 randomized patients (n = 28 liposomes, n = 22 cyclodextrins, and n = 25 MCT), 67 completed the trial (89%; n = 26 liposomes, n = 18 cyclodextrins, and n = 23 MCT) and had a median age of 22 years (IQR 19–28), body mass index of 20.6 kg/m2 [18.4–22.0], and forced expiratory volume in 1 s of 65% (44–84%). The liposomal formulation of vitamin A was associated with the improved evolution of serum all-trans-retinol compared to the control (median +1.7 ng/mL (IQR −44.3–86.1) vs. −38.8 ng/mL (−71.2–6.8), p = 0.028). Cyclodextrins enhanced the bioavailability of vitamin D3 (+9.0 ng/mL (1.0–17.0) vs. +3.0 ng/mL (−4.0–7.0), p = 0.012) and vitamin E (+4.34 µg/mL (0.33–6.52) vs. −0.34 µg/mL (−1.71–2.15), p = 0.010). Liposomes may augment the bioavailability of vitamin A and cyclodextrins may strengthen the supplementation of vitamins D3 and E relative to MCT in pancreatic-insufficient CF but further studies are required to assess liposomal vitamin E (German Clinical Trial Register number DRKS00014295, funded from EU and Norsa Pharma).

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“…The outcomes are illustrated in Figure 1. The assessment of the serum concentrations of all-trans-retinol and α-tocopherol (as well as the additional control assessments of β-carotene) was conducted using highperformance liquid chromatography (Hewlett Packard 1100 HPLC, Agilent, Waldbronn, Germany; Santa Clara, CA, USA) [9][10][11]. The concentrations of 25-hydroxyvitamin D3 were measured with chemiluminescence on the Architect (Abbott, Chicago, IL, USA).…”

Section: Methodsmentioning

confidence: 99%

Fat-Soluble Vitamins in Standard vs. Liposomal Form Enriched with Vitamin K2 in Cystic Fibrosis: A Randomized Multi-Center Trial

Nowak

Krzyżanowska-Jankowska

Drzymała-Czyż

et al. 2022

JCM

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Background: We aimed to assess a liposomal fat-soluble vitamin formulation containing vitamin K2 with standard treatment in cystic fibrosis (CF). Methods: A multi-center randomized controlled trial was carried out in 100 pancreatic-insufficient patients with CF. The liposomal formulation contained vitamin A as retinyl palmitate (2667 IU daily) and beta-carotene (1333 IU), D3 (4000 IU), E (150 IU), K1 (2 mg), and K2 as menaquinone-7 (400 µg). It was compared with the standard vitamin preparations in the closest possible doses (2500 IU, 1428 IU, 4000 IU, 150 IU, 2.14 mg, respectively; no vitamin K2) over 3 months. Results: Forty-two patients finished the trial in the liposomal and 49 in the control group (overall 91 pts: 22.6 ± 7.6 years, 62.6% female, BMI 19.9 ± 2.8 kg/m2, FEV1% 70% ± 30%). The main outcome was the change of vitamin status in the serum during the study (liposomal vs. standard): all-trans-retinol (+1.48 ± 95.9 vs. −43.1 ± 121.4 ng/mL, p = 0.054), 25-hydroxyvitamin D3 (+9.7 ± 13.4 vs. +2.0 ± 9.8 ng/mL, p = 0.004), α-tocopherol (+1.5 ± 2.5 vs. −0.2 ± 1.6 µg/mL, p < 0.001), %undercarboxylated osteocalcin (−17.2 ± 24.8% vs. −8.3 ± 18.5%, p = 0.061). The secondary outcome was the vitamin status at the trial end: all-trans-retinol (370.0 ± 116.5 vs. 323.1 ± 100.6 ng/mL, p = 0.045), 25-hydroxyvitamin D3 (43.2 ± 16.6 vs. 32.7 ± 11.5 ng/mL, p < 0.001), α-tocopherol (9.0 ± 3.1 vs. 7.7 ± 3.0 µg/mL, p = 0.037), %undercarboxylated osteocalcin (13.0 ± 11.2% vs. 22.7 ± 22.0%, p = 0.008). Conclusion: The liposomal fat-soluble vitamin supplement containing vitamin K2 was superior to the standard form in delivering vitamin D3 and E in pancreatic-insufficient patients with CF. The supplement was also more effective in strengthening vitamin K-dependent carboxylation, and could improve vitamin A status.

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Vitamin A status and its determinants in patients with cystic fibrosis

Cited by 6 publications

References 27 publications

Molecular Heterogeneity in Cystic Fibrosis

Molecular Heterogeneity in Cystic Fibrosis

Fat-Soluble Vitamin Supplementation Using Liposomes, Cyclodextrins, or Medium-Chain Triglycerides in Cystic Fibrosis: A Randomized Controlled Trial

Fat-Soluble Vitamins in Standard vs. Liposomal Form Enriched with Vitamin K2 in Cystic Fibrosis: A Randomized Multi-Center Trial

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