Vitamin ANEW2026-08

Vitamin A Status in Cystic Fibrosis in the Current Era of CFTR-Directed Therapies.

Sankararaman Senthilkumar, Schindler Terri, Vavrina Kay, Mascarenhas Maria — Nutrients

Summary

Historically, people with cystic fibrosis (CF) often struggled with low vitamin A levels due to their condition. However, new CFTR-directed therapies are changing this, with some patients now showing normal or even high vitamin A levels. This shift means doctors need to carefully monitor vitamin A to prevent both deficiency and potential toxicity.

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Key points

Historically, people with cystic fibrosis (CF) frequently experienced low vitamin A levels.
New CFTR-directed therapies are altering this trend, with some patients now having normal or even elevated vitamin A.
Monitoring vitamin A levels is crucial for CF patients to prevent both deficiency and potential toxicity.
Patients with certain conditions like advanced liver or kidney disease may be at higher risk for vitamin A toxicity.

What the study looked at

{ "What question did this study ask?": "This review examined how vitamin A status has changed in individuals with cystic fibrosis (CF) since the introduction of new CFTR-directed therapies.", "How was it studied?": "This was a comprehensive review paper, meaning the authors synthesized and summarized existing scientific literature and clinical observations rather than conducting a new experiment with participants. They detailed the basics of vitamin A metabolism and its implications in CF.", "What did it find?": "Historically, vitamin A deficiency was a common concern for people with CF, often due to issues with nutrient absorption. However, the review highlights a significant shift: with modern CFTR therapies, vitamin A deficiency is becoming less common. Instead, some patients are now showing normal or even elevated vitamin A levels, occasionally reaching potentially toxic ranges. This is particularly relevant for those with additional health complications like advanced liver disease or kidney problems. The paper emphasizes the critical need for healthcare providers to proactively monitor vitamin A levels in CF patients to manage both potential deficiencies and excesses, as both can lead to adverse health outcomes." }

Dietary takeaway

For the general public, this study underscores that while vitamin A is essential, both too little and too much can be harmful, especially for individuals with specific health conditions like cystic fibrosis. A balanced diet typically provides adequate vitamin A for most people, but those with chronic illnesses should always consult their doctor regarding dietary needs and supplements. Remember, this review focuses on a specific patient group, and its findings don't necessarily apply to the general healthy population.

Abstract

Vitamin A plays an important role in multiple homeostatic functions such as vision, immunity, epithelial cell integrity and differentiation, cell signaling, pulmonary function, reproduction, growth, and development. Vitamin A deficiency was described in people with cystic fibrosis (pwCF) due to a multitude of causes, such as suboptimally managed exocrine pancreatic insufficiency, advanced cystic fibrosis-related liver disease (aCFLD), and a history of intestinal resection, and is generally rare in contemporary practice. Apart from true vitamin A deficiency, low vitamin A levels may also be noted in various inflammatory states, as vitamin A is a negative acute-phase reactant. In the current era of cystic fibrosis (CF) transmembrane-conductance regulator (CFTR)-directed therapies, there is a paradigm shift in vitamin A status, with deficiency statuses becoming rarer, and instead higher serum vitamin levels (in some cases, even in the hypervitaminosis range) are increasingly reported. People with aCFLD, renal insufficiency, post-lung transplantation, and pregnancy are prone to vitamin A toxicity. Hence, CF clinicians should be proactive in evaluating these abnormalities and proficient in managing both deficiency and toxicity, as both these conditions can be associated with adverse outcomes. In this review, we detailed the basics of vitamin A metabolism, manifestations of both vitamin A deficiency and excess, and their clinical implications in pwCF.

View on PubMed →DOI: 10.3390/nu18162639