Abstract

Objective: To determine and compare the effect of vitamin D3 supplementation on modifying the disease severity in cystic fibrosis (CF) and non-CF bronchiectasis pediatric patients. Methods: A randomized clinical trial evaluating the role of oral vitamin D3 supplementation for six months, was performed in forty patients with CF and non-CF bronchiectasis under the age of 18 years with vitamin D deficiency or insufficiency. The primary outcome was to reach the sufficient Vitamin D level, the secondary outcome was to reevaluate bronchiectasis severity by following up the frequency, severity of pulmonary exacerbations and lung function after vitamin D3 supplementation. Results: Forty patients completed the trial. The percentage of improvement of vitamin D level after vitamin D3 supplementation for six months was significantly higher in CF (88.3%) than non-CF bronchiectasis patients (59.82%) (P = 0.03). Additionally, moderate to severe pulmonary exacerbations significantly decreased by more than 60%, 45% (P = 0.001, 0.005) and frequent exacerbations decreased by 15%, 10% (P = 0.327, 0.490), while the forced expiratory volume in 1 (FEV1) significantly increased by 17% and 15% in non CF bronchiectasis and CF patients respectively (p 0.001). Conclusions: Vitamin D3 therapy was effective in decreasing the frequency and severity of pulmonary exacerbations and preserving lung function. Thereby, improving the disease severity even more in non-CF bronchiectasis than CF patients.

Highlights

  • Bronchiectasis is a chronic inflammatory respiratory disorder characterized by bronchial dilatation, and symptoms of productive cough, dyspnea and repeated respiratory infections requiring multiple courses of antibiotics [1]

  • There was a statistically significant difference between cystic fibrosis (CF) patients and non CF bronchiectasis patients as regard mean age, weight & height which were lower among CF patients than non-CF bronchiectasis patients (p = 0.016, 0.016, 0.007) respectively

  • The current study showed that the mean age, weight and height were significantly lower among CF patients than among non-CF bronchiectasis patients (Table 1) which may be due to earlier median age of diagnosis of CF patients which is 6 - 8 months; as two thirds of CF patients are diagnosed by 1 year of age

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Summary

Introduction

Bronchiectasis is a chronic inflammatory respiratory disorder characterized by bronchial dilatation, and symptoms of productive cough, dyspnea and repeated respiratory infections requiring multiple courses of antibiotics [1]. Vitamin D deficiency occurs commonly in patients with CF [3] & non-CF bronchiectasis. Reduced outdoor physical activity which is a common consequence of chronic lung diseases, can lead to reduced exposure to sunlight and vitamin-D deficiency [4]. Vitamin D down-regulates cytokines and chemokines that promote tissue destruction, that are found in abundance in CF and non-CF bronchiectatic lungs [5] [6]. It was shown that CF respiratory epithelial cells and macrophages incubated with 1.25(OH)2D showed a significant down-regulation in the neutrophil attracting chemokine, IL-8 [7], The anti-inflammatory activity of vitamin D may originate from the enhancement of anti-inflammatory/regulatory cytokines secretion, such as IL-10 [8]. Vitamin D, as it was aforementioned, may have anti-infective and anti-inflammatory properties and may play some role in the pathogenesis of bronchiectasis [9]

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