Risk factors leading to pulmonary exacerbation in patients with cystic fibrosis: A systematic review.

OBJECTIVE: To ascertain major risk factors associated with pulmonary exacerbation and pulmonary function decline in cystic fibrosis. METHODS: The systematic review was conducted at Aga Khan University, Karachi, in September 2018, and comprised electronic search of PubMed, Ovid, Science Direct and Cumulative Index of Nursing and Allied Health Literature databases of studies conducted from January 1990 to September 2018 which were categorised into 3 sets; 1990-98, 1999-2007 and 2008-18. Studies included for review focussed on articles with pulmonary exacerbation as the health outcome indicator, and had diagnosis of cystic fibrosis as the inclusion criteria, while risk factors were the exposure terms used in the search process. References in bibliographies of the included studies were also systematically searched for relevant documents. RESULTS: Of the 60 studies obtained, 31(51.7%) were selected; 2(6.45%) from 1990-98, 7(22.58%) from 1999-2007 and 22(70.96%) from 2008-18. Overall, 17(54.83%) were cohort studies, 7(22.5%) were cross-sectional studies, 3(9.6%) were case-control studies, 3(9.6%) were randomised controlled trials and 1(3.2%) was systematic review and meta-analysis. In terms of major risk factors, genetic mutations were cited by 4(12.9%) studies, infections and inflammatory biomarkers by 15(48.4%), nutritional deficiencies by 9(29%) and geographical and socioeconomic status by 3(9.6%) studies. CONCLUSIONS: Early identification and recognition of risk factors associated with pulmonary exacerbation can have an explicit impact on its management, leading to decreased morbidity and mortality burden in cystic fibrosis cases.

Vitamin D status and pulmonary exacerbations in children and adolescents with cystic fibrosis: Experience from a tertiary care center.

BACKGROUND: The function of Vitamin D in preventing inflammation and infection has been studied previously for different pathologies in different populations globally. Relationships between serum Vitamin D levels and its effect on pulmonary exacerbations in the cystic fibrosis (CF) population are not well studied in our part of the world. Therefore, we aimed to ascertain the Vitamin D status in pediatric and adolescent CF patients and its association with pulmonary exacerbations. MATERIALS AND METHODS: A retrospective study was conducted at The Aga Khan University Hospital from 2015 to 2018. Patients of CF with sweat chloride value >60 mmol/l and who had at least one measurement of 25 hydroxy Vitamin D (25 OHD) were included in the study. Annual serum Vitamin D levels were documented for enrolled patients and their past 1-year data were analyzed for pulmonary exacerbations, average length of stay, and tracheal/airway colonization with organisms. RESULTS: 69 patients were included in the study. 28 patients (40.57%) were found to be Vitamin D deficient, 22 patients (31.88%) were Vitamin D insufficient and 19 patients (27.53%) were labeled as Vitamin D insufficient. The average number of exacerbations per year was significantly high in Vitamin D deficient group (3.71 ± 0.96) in comparison with insufficient (3.18 ± 1.09) and sufficient groups (2.26 ± 0.93) (P < 0.001). CONCLUSION: Vitamin D deficiency is related to an increased number of annual pulmonary exacerbations and pseudomonas infections.