Vitamin E status and its determinants in patients with cystic fibrosis

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

doi:10.1016/j.advms.2018.04.001

Cited by 17 publications

(17 citation statements)

References 30 publications

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“…Similar to retinol, the risk of vitamin E deficiency increases with inflammation of the respiratory or digestive system [14,57]. On the other hand, excessive supplementation of vitamin E may lead to high serum levels of α-tocopherol and possible toxicity [58,59]. Overall, however, the evidence documenting the potential predictors of vitamin E levels in CF patients is sparse [10,58].…”

Section: Vitamin Ementioning

confidence: 99%

CFTR Modulator Therapy with Lumacaftor/Ivacaftor Alters Plasma Concentrations of Lipid-Soluble Vitamins A and E in Patients with Cystic Fibrosis

et al. 2021

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Rationale: Cystic fibrosis (CF), caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene, leads to impaired pancreatic function and therefore reduced intestinal absorption of lipids and fat-soluble vitamins especially in patients with CF developing pancreatic insufficiency (PI). Previous studies showed that CFTR modulator therapy with lumacaftor-ivacaftor (LUM/IVA) in Phe508del-homozygous patients with CF results in improvement of pulmonary disease and thriving. However, the effects of LUM/IVA on plasma concentration of the lipid soluble vitamins A and E remain unknown. Objectives: To investigate the course of plasma vitamin A and E in patients with CF under LUM/IVA therapy. Methods: Data from annual follow-up examinations of patients with CF were obtained to assess clinical outcomes including pulmonary function status, body mass index (BMI), and clinical chemistry as well as fat-soluble vitamins in Phe508del-homozygous CF patients before initiation and during LUM/IVA therapy. Results: Patients with CF receiving LUM/IVA improved substantially, including improvement in pulmonary inflammation, associated with a decrease in blood immunoglobulin G (IgG) from 9.4 to 8.2 g/L after two years (p < 0.001). During the same time, plasma vitamin A increased significantly from 1.2 to 1.6 µmol/L (p < 0.05), however, levels above the upper limit of normal were not detected in any of the patients. In contrast, plasma vitamin E as vitamin E/cholesterol ratio decreased moderately over the same time from 6.2 to 5.5 µmol/L (p < 0.01). Conclusions: CFTR modulator therapy with LUM/IVA alters concentrations of vitamins A and vitamin E in plasma. The increase of vitamin A must be monitored critically to avoid hypervitaminosis A in patients with CF.

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Section: Vitamin Ementioning

confidence: 99%

CFTR Modulator Therapy with Lumacaftor/Ivacaftor Alters Plasma Concentrations of Lipid-Soluble Vitamins A and E in Patients with Cystic Fibrosis

et al. 2021

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“…A plasma α‐tocopherol/cholesterol ratio of >5.4 mg/g is preferred in patients with CF . Vitamin E deficiency, once frequent in CF patients without supplementation, is now uncommon . In an Australian study involving CF infants diagnosed by newborn screening, 24% had low vitamin E levels, which normalized at 1 year of age with supplementation .…”

Section: Treatmentmentioning

confidence: 99%

“…With supplementation, there is a risk of toxicity . A recent Polish study involving young children and adults with CF identified vitamin E deficiency in 8% and elevated levels in 11.4% . Similar to vitamin A, elevated vitamin E levels are noted after lung transplantation in CF patients .…”

Section: Treatmentmentioning

confidence: 99%

Management of Exocrine Pancreatic Insufficiency in Children

2019

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The diagnosis of exocrine pancreatic insufficiency (EPI) can be difficult, as symptoms may be nonspecific. A delayed diagnosis of EPI can negatively impact health through poor weight gain, impaired growth, and malabsorption of nutrients. Because of active growth and development, children are more vulnerable to the consequences of untreated EPI. Pancreatic enzyme replacement therapy is the cornerstone of management and offers both symptomatic relief and improvement in clinical outcomes. Additionally, a high-energy diet with unrestricted fat and supplementation with fat-soluble vitamins is often required to optimize growth and prevent nutrition deficiencies. Cystic fibrosis (CF) is the most common condition in children that causes EPI, and improvement in nutrition management is associated with improved pulmonary function and increased survival. Currently, the management of other conditions leading to EPI in children is not well studied, and inferences from the CF literature are often necessary in caring for these patients. (Nutr Clin Pract. 2019;34(suppl 1):S27-S42)

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“…Due to central venous catheters, acquired thrombophilia due to chronic inflammation, deficiency of anticoagulant proteins due to vitamin K deficiency or liver dysfunction [1]. Vitamin E supplements are given to children with fat malabsorption such as in cystic fibrosis and cholestatic liver disease [2].…”

Section: Introductionmentioning

confidence: 99%