Elsevier

Journal of Cystic Fibrosis

Original Article

Vitamin D deficiency and its treatment in cystic fibrosis

Under a Creative Commons license

open access

Highlights

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Vitamin D deficiency is a common finding in CF.

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Vitamin D3 is a secosteroid endogenously produced by the skin.

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Daily or weekly supplementation with cholecalciferol is preferred.

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Adults and children with CF may require higher doses of vitamin D compared to individuals without CF.

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More studies are needed to support a role for vitamin D beyond calcium homeostasis and skeletal health in CF.

Abstract

Vitamin D deficiency is a common finding in individuals with cystic fibrosis (CF), despite routine supplementation. Hypovitaminosis D is often the result of fat malabsorption, but other contributors include increased latitude, poor nutritional intake, decreased sun exposure, impaired hydroxylation of vitamin D, and non-adherence to the prescribed vitamin D regimen. Vitamin D is critical for calcium homeostasis and optimal skeletal health, and vitamin D deficiency in CF can lead to skeletal complications of osteopenia and osteoporosis. Over time, our understanding of treatment regimens for vitamin D deficiency in CF has evolved, leading to recommendations for higher doses of vitamin D to achieve target levels of circulating 25-hydroxyvitamin D. There is also some evidence that vitamin D deficiency may have non-skeletal consequences such as an increase in pulmonary exacerbations. The exact mechanisms involved in the non-skeletal complications of vitamin D deficiency are not clearly understood, but may involve the innate immune system. Future clinical studies are needed to help address whether vitamin D has a role in CF beyond skeletal health.

Keywords

Cystic fibrosis

Vitamin D

Supplementation

Ergocalciferol

Cholecalciferol

Abbreviations

25(OH)D

25-hydroxyvitaminD

1,25(OH)2D

1,25-dihydroxyvitamin D

CFF

Cystic Fibrosis Foundation

DBP

vitamin D binding protein

© 2019 The Authors. Published by Elsevier B.V. on behalf of European Cystic Fibrosis Society.