Abstract

BackgroundVitamin D deficiency occurs frequently in cystic fibrosis (CF) and non-CF bronchiectasis patients. Yet, few studies have assessed the impact of vitamin D status on the clinical outcomes in pediatric bronchiectasis. This study is designed to assess vitamin D level and determine its effect on exacerbations, bacterial colonization, and lung function in pediatric patients with CF and non-CF bronchiectasis.ResultsThis cross-sectional case-control study assessing vitamin D level was performed in a total of sixty cases under the age of 18 years—forty cases with CF and non-CF bronchiectasis and twenty age- and sex-matched healthy controls. Associations between serum vitamin D and clinical and laboratory parameters were assessed in the patient groups. Vitamin D deficiency was more prevalent among CF and non-CF bronchiectasis patients (75%, 45%) compared to controls (10%) (P < 0.001). In addition, vitamin D deficiency was associated with more frequent and severe pulmonary exacerbations (66.7%, 46.7%) (P=0.033, < 0.001), chronic Pseudomonas infection (80%) (P=0.060) among CF patients, and with lower FEV1 (66%) (P= 0.071) among non-CF bronchiectasis. Moreover, a cutoff value of vitamin D level equal or less than 22.5 ng/ml was accurate in differentiating moderate from mild pulmonary exacerbations in both patients’ groups (AUC=0.809) (p=0.004).ConclusionsVitamin D deficiency is not uncommon in both CF and non-CF bronchiectasis. In this population, vitamin D deficiency is associated with more frequent pulmonary exacerbations, chronic Pseudomonas infection, and worse lung function.

Highlights

  • Vitamin D deficiency occurs frequently in cystic fibrosis (CF) and non-CF bronchiectasis patients

  • Subjects were excluded if (1) CF patients had liver disease based on Debray’s criteria [10], or renal disease based on estimated creatinine clearance using the Schwartz formula [11], because these might affect the metabolism of vitamin D (details of these are available in the; (2) CF patients had pancreatic sufficiency; (3) the CF patients were receiving vitamin D supplements exceeding 2000 IU per day; (4) the studied patients were receiving steroid therapy in the last 6 weeks; (5) the studied patients had chronic lung diseases other than CF and non CF bronchiectasis; (6) the studied patients had any other systemic illness; or (7) non CF bronchiectasis patients and controls were taking any vitamin D supplements during the last three months prior to the study

  • Positive consanguinity and family history of bronchiectasis were significantly higher among CF patients (75%, 60%) than controls (35%, 0%) (P= 0.036) (< 0.001)

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Summary

Introduction

Vitamin D deficiency occurs frequently in cystic fibrosis (CF) and non-CF bronchiectasis patients. This study is designed to assess vitamin D level and determine its effect on exacerbations, bacterial colonization, and lung function in pediatric patients with CF and non-CF bronchiectasis. Vitamin D deficiency occurs frequently in patients with CF [3] and non-CF bronchiectasis [4]. Vitamin D is known to be involved in a wide spectrum of significant immunomodulatory effects as downregulation of pro-inflammatory cytokines and chemokines [2]. It regulates the secretion of antimicrobial peptides like cathelicidin (LL-37) which has a potent antimicrobial activity against Pseudomonas aeruginosa [4]

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