Pleiotropic effect of vitamin D in cystic fibrosis.

Teresa Grzelak,Klaudia Mikołajczyk

doi:10.5603/arm.a2018.0029

Abstract

Cystic fibrosis - CF - is the most common recessively autosomally and inherited disorder in the Caucasian population. It is incurable, multi-systemic disease with progressive course. CF is caused by CFTR gene mutation, the product of which is Cystic Fibrosis Transmembrane Conductance Regulator (CFTR). CF patients are exposed to fat-soluble vitamins deficiency, including vitamin D. It is due to the fat malabsorption (caused by exacerbation exocrine pancreatic insufficiency), decreased sun exposure (caused by receiving antibiotics photophobia), reduction of adipose tissue and insufficient supply with food. The discovery of vitamin D receptor (VDR) presence outside the skeletal system allowed to conclude, that vitamin D is responsible not only for mineral economy, but also for immunological processes, respiratory status, intestial microflora and cystic fibrosis - related diabetes (CFRD) course. Based on literature data, it is suggested that vitamin D plays an important role in the prevention of diseases coexisting with CF. The right dosage of vitamin D allows to maintain a better lung function and prevent chronic pulmonary infections. It has also been shown that normal levels of vitamin D may be important in increasing the chances of successful lung transplant surgery. Taking the wide spectrum of vitamin D effect into account, it is recommended to maintain serum concentrations above the minimum in patients with CF. In summary, maintaining the proper vitamin D levels in patients with CF is important because of its pleiotropic effect. It can be achieved through regular monitoring of vitamin D levels and individual supplementary dose for each patients.

Highlights

Cystic fibrosis (CF) is the most common monogenic disease among the Caucasian population, inherited in an autosomal recessive manner [1,2,3]
The ones suffering from CF, besides other irregularities related to nutrition, are exposed to a deficiency in fat-soluble vitamins, including vitamin D
Due to a prevalence of insufficient concentration of vitamin D in serum, it is recommended by the Cystic Fibrosis Foundation to examine its body resources annually, preferably in the winter season

Summary

Introduction

Cystic fibrosis (CF) is the most common monogenic disease among the Caucasian population, inherited in an autosomal recessive manner [1,2,3]. Available literature data indicate a positive correlation between age and severity of pulmonary disease and the level of advancement of skeletal changes and the vitamin D deficiency among these patients [9].

Results

Conclusion