Pulmonary Complications in Cystic Fibrosis: Past, Present, and Future

Mingora, Christina; Flume, Patrick A.

doi:10.1016/j.chest.2021.06.017

Cited by 16 publications

(7 citation statements)

References 64 publications

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“…CF affects multiple organs, including the lung, gut, pancreas, and reproductive tract, with its impact on the respiratory system resulting in the greatest morbidity and mortality [ 3 ]. People with CF typically have recurrent pulmonary infections, which over time become chronic, leading to fibrosis, pulmonary failure, and death [ 1 ].…”

Section: Introductionmentioning

confidence: 99%

Essential Fatty Acid Deficiency in Cystic Fibrosis Disease Progression: Role of Genotype and Sex

et al. 2022

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Adequate intake of nutrients such as essential fatty acids (EFA) are critical in cystic fibrosis (CF). The clinical course of deterioration of lung function in people with CF has been shown to relate to nutrition. Independent of the higher energy consumption and malabsorption due to pancreatic insufficiency, EFA deficiency is closely associated with the risk of pulmonary infection, the most significant pathology in CF. This review will focus on the EFA deficiency identified in people with CF, as well as the limited progress made in deciphering the exact metabolic pathways that are dysfunctional in CF. Specifically, people with CF are deficient in linoleic acid, an omega 6 fatty acid, and the ratio of arachidonic acid (omega 6 metabolite) and docosahexaenoic acid (omega 3 metabolite) is increased. Analysis of the molecular pathways in bronchial cells has identified changes in the enzymes that metabolise EFA. However, fatty acid metabolism primarily occurs in the liver, with EFA metabolism in CF liver not yet investigated, indicating that further research is required. Despite limited understanding in this area, it is well known that adequate EFA concentrations are critical to normal membrane structure and function, and thus are important to consider in disease processes. Novel insights into the relationship between CF genotype and EFA phenotype will be discussed, in addition to sex differences in EFA concentrations in people with CF. Collectively, investigating the specific effects of genotype and sex on fatty acid metabolism may provide support for the management of people with CF via personalised genotype- and sex-specific nutritional therapies.

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Section: Introductionmentioning

confidence: 99%

Essential Fatty Acid Deficiency in Cystic Fibrosis Disease Progression: Role of Genotype and Sex

et al. 2022

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“…The complementary deoxyribonucleic acid identifies a 6.1 kb transcript with 4400 nucleotides which encodes a protein with 1480 amino acids and a molecular weight of 170 kDa, known as the CFTR protein. This term was coined when it was recognized as the protein responsible for the appearance of CF, a genetic disease of autosomal recessive inheritance that originates from the failure of this protein [12].…”

Section: Cftr: Structure and Functionmentioning

confidence: 99%

Dysfunction in the Cystic Fibrosis Transmembrane Regulator in Chronic Obstructive Pulmonary Disease as a Potential Target for Personalised Medicine

et al. 2021

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In recent years, numerous pathways were explored in the pathogenesis of COPD in the quest for new potential therapeutic targets for more personalised medical care. In this context, the study of the cystic fibrosis transmembrane conductance regulator (CFTR) began to gain importance, especially since the advent of the new CFTR modulators which had the potential to correct this protein’s dysfunction in COPD. The CFTR is an ion transporter that regulates the hydration and viscosity of mucous secretions in the airway. Therefore, its abnormal function favours the accumulation of thicker and more viscous secretions, reduces the periciliary layer and mucociliary clearance, and produces inflammation in the airway, as a consequence of a bronchial infection by both bacteria and viruses. Identifying CFTR dysfunction in the context of COPD pathogenesis is key to fully understanding its role in the complex pathophysiology of COPD and the potential of the different therapeutic approaches proposed to overcome this dysfunction. In particular, the potential of the rehydration of mucus and the role of antioxidants and phosphodiesterase inhibitors should be discussed. Additionally, the modulatory drugs which enhance or restore decreased levels of the protein CFTR were recently described. In particular, two CFTR potentiators, ivacaftor and icenticaftor, were explored in COPD. The present review updated the pathophysiology of the complex role of CFTR in COPD and the therapeutic options which could be explored.

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“…Although a few studies examined the impact of pulmonary atelectasis on the prognosis of CF, the number of included patients was small. [3][4][5][6] Given that CF occurs with other pulmonary complications associated with poor quality of life, morbidity, and prognosis deterioration, [7][8][9][10] it is important to determine whether pulmonary atelectasis is a poor prognostic factor in CF. The published studies on pulmonary atelectasis in CF identified no clear factors that could predict the onset of atelectasis and its effect on CF.…”

Section: Introductionmentioning

confidence: 99%

Should atelectasis be considered a pulmonary complication and indicator of poor prognosis in cystic fibrosis?

Martínez Redondo,

Carpio Segura,

Zamarrón De Lucas

et al. 2024

J Int Med Res

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Objective This study examined whether bronchoscopy leads to clinicoradiological improvement in cystic fibrosis (CF) and the predictive factors. The study also investigated whether pulmonary atelectasis is a poor prognostic factor in CF. Methods This multicenter, case–control, observational, retrospective study included two groups of patients with CF: a case group (patients with persistent atelectasis who were followed-up at least for 2 years) and a control group (patients without atelectasis matched 1:1 by sex and age [±3 years]). We recorded demographic data, lung function test results, pulmonary complications, comorbidities, treatments (including bronchoscopies, surgery and transplantation), and deaths. Results Each group included 55 patients (case group: 20 men, mean age 25.4 ± 10.4 years; control group: 20 men, mean age 26.1 ± 11.4 years). Bronchoscopy did not lead to clinicoradiological improvement. Allergic bronchopulmonary aspergillosis (ABPA) was more frequent in the case group. Patients in the case group more frequently used inhaled steroids, their pre-atelectasis lung function was statistically worse, and they had more exacerbations during follow-up. Conclusion Moderate-to-severe pulmonary disease and ABPA can favor atelectasis. Pulmonary atelectasis can be a poor prognostic factor in CF because it increases exacerbations. Despite our results, we recommend enhancing treatment, including bronchoscopy, to prevent persistent atelectasis.

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Pulmonary Complications in Cystic Fibrosis: Past, Present, and Future

Cited by 16 publications

References 64 publications

Essential Fatty Acid Deficiency in Cystic Fibrosis Disease Progression: Role of Genotype and Sex

Essential Fatty Acid Deficiency in Cystic Fibrosis Disease Progression: Role of Genotype and Sex

Dysfunction in the Cystic Fibrosis Transmembrane Regulator in Chronic Obstructive Pulmonary Disease as a Potential Target for Personalised Medicine

Should atelectasis be considered a pulmonary complication and indicator of poor prognosis in cystic fibrosis?

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