Pulmonary Complications of Cystic Fibrosis

2019 ◽  
Vol 40 (06) ◽  
pp. 804-809 ◽  
Author(s):  
Bryan Garcia ◽  
Patrick A. Flume
Keyword(s):  
Cystic Fibrosis ◽  
Respiratory Failure ◽  
Lung Disease ◽  
Airflow Obstruction ◽  
Pulmonary Complications ◽  
Extrinsic Factors ◽  
Hypercapnic Respiratory Failure ◽  
Increased Risk ◽  
Impaired Lung Function ◽  
Intrinsic And Extrinsic Factors

AbstractCystic fibrosis (CF) lung disease is characterized by the development of progressive bronchiectasis and impaired lung function with severe airflow obstruction. CF patients suffer from shortened life expectancy, primarily driven by respiratory failure. The mechanism by which CF lung disease develops is the result of an interplay of multiple intrinsic and extrinsic factors including genotype, abnormalities in mucus composition and movement, chronic inflammation, and chronic airway infection. Although all CF patients are at increased risk for pulmonary complications including hemoptysis, pneumothorax, pulmonary hypertension, and chronic hypoxic and hypercapnic respiratory failure, the risk of developing these complications increases with progression of lung disease. The focus of this article is to summarize the pathophysiology, epidemiology, and management of these key pulmonary complications.

scholarly journals Prevalence and burden of chronic bronchitis symptoms: results from the BOLD study

2017 ◽  
Vol 50 (5) ◽  
pp. 1700621 ◽  
Author(s):  
Filip Mejza ◽  
Louisa Gnatiuc ◽  
A. Sonia Buist ◽  
William M. Vollmer ◽  
Bernd Lamprecht ◽  
...  
Keyword(s):  
Lung Function ◽  
Lung Disease ◽  
Chronic Bronchitis ◽  
Airflow Obstruction ◽  
Population Based ◽  
Normal Lung ◽  
Increased Risk ◽  
Potential Risk Factors ◽  
Definition Of ◽  
Disease Study

We studied the prevalence, burden and potential risk factors for chronic bronchitis symptoms in the Burden of Obstructive Lung Disease study.Representative population-based samples of adults aged ≥40 years were selected in participating sites. Participants completed questionnaires and spirometry. Chronic bronchitis symptoms were defined as chronic cough and phlegm on most days for ≥3 months each year for ≥2 years.Data from 24 855 subjects from 33 sites in 29 countries were analysed. There were significant differences in the prevalence of self-reported symptoms meeting our definition of chronic bronchitis across sites, from 10.8% in Lexington (KY, USA), to 0% in Ile-Ife (Nigeria) and Blantyre (Malawi). Older age, less education, current smoking, occupational exposure to fumes, self-reported diagnosis of asthma or lung cancer and family history of chronic lung disease were all associated with increased risk of chronic bronchitis. Chronic bronchitis symptoms were associated with worse lung function, more dyspnoea, increased risk of respiratory exacerbations and reduced quality of life, independent of the presence of other lung diseases.The prevalence of chronic bronchitis symptoms varied widely across the studied sites. Chronic bronchitis symptoms were associated with significant burden both in individuals with chronic airflow obstruction and those with normal lung function.

Airflow obstruction, impaired lung function and risk of sudden cardiac death: a prospective cohort study

Thorax ◽  
2021 ◽  
pp. thoraxjnl-2020-215632
Author(s):  
Yun-Jiu Cheng ◽  
Zhen-Guang Chen ◽  
Feng-Juan Yao ◽  
Li-Juan Liu ◽  
Ming Zhang ◽  
...  
Keyword(s):  
Sudden Cardiac Death ◽  
Lung Function ◽  
Cardiac Death ◽  
Airflow Obstruction ◽  
Partial Likelihood ◽  
Race And Gender ◽  
Increased Risk ◽  
Impaired Lung Function ◽  
And Gender ◽  
Lung Health

BackgroundGrowing evidence suggests that compromised lung health may be linked to cardiovascular disease. However, little is known about its association with sudden cardiac death (SCD).ObjectivesWe aimed to assess the link between impaired lung function, airflow obstruction and risk of SCD by race and gender in four US communities.MethodsA total of 14 708 Atherosclerosis Risk in Communities (ARIC) study participants who underwent spirometry and were asked about lung health (1987–1989) were followed. The main outcome was physician-adjudicated SCD. Fine-Gray proportional subdistribution hazard models with Firth’s penalised partial likelihood correction were used to estimate the HRs.ResultsOver a median follow-up of 25.4 years, 706 (4.8%) subjects experienced SCD. The incidence of SCD was inversely associated with FEV1 in each of the four race and gender groups and across all smoking status categories. After adjusting for multiple measured confounders, HRs of SCD comparing the lowest with the highest quintile of FEV1 were 2.62 (95% CI 1.62 to 4.26) for white males, 1.80 (95% CI 1.03 to 3.15) for white females, 2.07 (95% CI 1.05 to 4.11) for black males and 2.62 (95% CI 1.21 to 5.65) for black females. The above associations were consistently observed among the never smokers. Moderate to very severe airflow obstruction was associated with increased risk of SCD. Addition of FEV1 significantly improved the predictive power for SCD.ConclusionsImpaired lung function and airflow obstruction were associated with increased risk of SCD in general population. Additional research to elucidate the underlying mechanisms is warranted.

scholarly journals Type-4 Phosphodiesterase (PDE4) Blockade Reduces NETosis in Cystic Fibrosis

2021 ◽  
Vol 12 ◽  
Author(s):  
Licia Totani ◽  
Concetta Amore ◽  
Antonio Piccoli ◽  
Giuseppe Dell’Elba ◽  
Angelo Di Santo ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Pseudomonas Aeruginosa ◽  
Lung Disease ◽  
Pde4 Inhibitor ◽  
Free Dna ◽  
Impaired Lung Function ◽  
Cellular Integrity ◽  
Net Release

Neutrophilic inflammation is a key determinant of cystic fibrosis (CF) lung disease. Neutrophil-derived free DNA, released in the form of extracellular traps (NETs), significantly correlates with impaired lung function in patients with CF, underlying their pathogenetic role in CF lung disease. Thus, specific approaches to control NETosis of neutrophils migrated into the lungs may be clinically relevant in CF. We investigated the efficacy of phosphodiesterase (PDE) type-4 inhibitors, in vitro, on NET release by neutrophils from healthy volunteers and individuals with CF, and in vivo, on NET accumulation and lung inflammation in mice infected with Pseudomonas aeruginosa. PDE4 blockade curbed endotoxin-induced NET production and preserved cellular integrity and apoptosis in neutrophils, from healthy subjects and patients with CF, challenged with endotoxin, in vitro. The pharmacological effects of PDE4 inhibitors were significantly more evident on CF neutrophils. In a mouse model of Pseudomonas aeruginosa chronic infection, aerosol treatment with roflumilast, a selective PDE4 inhibitor, gave a significant reduction in free DNA in the BALF. This was accompanied by reduced citrullination of histone H3 in neutrophils migrated into the airways. Roflumilast-treated mice showed a significant improvement in weight recovery. Our study provides the first evidence that PDE4 blockade controls NETosis in vitro and in vivo, in CF-relevant models. Since selective PDE4 inhibitors have been recently approved for the treatment of COPD and psoriasis, our present results encourage clinical trials to test the efficacy of this class of drugs in CF.

Cystic Fibrosis, Bronchiectasis, and Pleural Effusion

2016 ◽  
pp. 729-732
Author(s):  
Vivek N. Iyer
Keyword(s):  
Cystic Fibrosis ◽  
Central Nervous System ◽  
Nervous System ◽  
Respiratory Failure ◽  
Pleural Effusion ◽  
Neuromuscular Transmission ◽  
System Control ◽  
Hypoxemic Respiratory Failure ◽  
Hypercapnic Respiratory Failure ◽  
Alveolar Capillary

Effective functioning of the respiratory system requires 1) normal central nervous system control, 2) intact neuromuscular transmission and bellows function, 3) patent airways, and 4) normal gas exchange at the alveolar-capillary level. Respiratory failure may be caused by dysfunction at any of these levels, resulting in failure of oxygenation (hypoxemic respiratory failure) or ventilation (hypercapnic respiratory failure).

scholarly journals Acute effects of NPPV in interstitial lung disease with chronic hypercapnic respiratory failure

2010 ◽  
Vol 104 (2) ◽  
pp. 291-295 ◽  
Author(s):  
Dirk Koschel ◽  
Sabin Handzhiev ◽  
Bärbel Wiedemann ◽  
Gert Höffken
Keyword(s):  
Respiratory Failure ◽  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Acute Effects ◽  
Hypercapnic Respiratory Failure

Nocturnal Nasal IPPV Stabilizes Patients with Cystic Fibrosis and Hypercapnic Respiratory Failure

CHEST Journal ◽  
1992 ◽  
Vol 102 (3) ◽  
pp. 846-850 ◽  
Author(s):  
Amanda J. Piper ◽  
Sally Barker ◽  
Paul J. Torzillo ◽  
Colin E. Sullivan ◽  
Peter T P Bye
Keyword(s):  
Cystic Fibrosis ◽  
Respiratory Failure ◽  
Hypercapnic Respiratory Failure

scholarly journals Cardiovascular Risk in Patients with COPD: Cardiovascular Comorbidities in Patients with COPD Increase CAT and mMRC Dyspnea Scores

2021 ◽  
Vol 9 (B) ◽  
pp. 1772-1777
Author(s):  
Jagoda Stojkovic ◽  
Emilija Antova ◽  
Dragana Stojkovikj
Keyword(s):  
Respiratory Failure ◽  
Airflow Obstruction ◽  
Forced Expiratory Volume ◽  
Severe Exacerbation ◽  
Cardiovascular Comorbidity ◽  
Versus Group ◽  
Group I ◽  
Copd Patients ◽  
Increased Risk ◽  
Group Ii

BACKGROUND: Chronic obstructive pulmonary disease (COPD) is associated with a number of different comorbidities. Cardiovascular diseases (CVD) are the most frequent comorbidities in COPD. The economic burden associated with cardiovascular comorbidity (CVC) in this population of patients is considerable. The COPD patients are related to the increased systemic inflammation, reduced capacity for physical activity, and airflow obstruction. AIM: The aim of our investigation was to evaluate the dyspnea as a disabling symptom in COPD patients with cardiovascular comorbidity (CVC) especially heart failure. The main aim of this study is to evaluate its intensity in patients with COPD in stages II according to GOLD. METHODS: The investigation was conducted from December 2019 to January 2020, on pulmonology and allergology clinic and cardiology clinic of medical faculty in Skopje. We investigated 65 outpatients with COPD, 44 with different type of CVD, Group I, and 21 without CVD, Group II. All patients were with partial chronic respiratory failure (In type 1 respiratory failure hypoxemic). Patients, according GOLD initiative, were in COPD stadium II, 70% < forced expiratory volume in 1 s (FEV1)>50%. Heart condition was diagnosed on the basis of clinical examination, electrocardiography, and echocardiography of the heart. Included patients with CVD were with ejection fraction (EF) <65%. Dyspnea was measured with modified MRC (mMRC) dyspnea scale. RESULTS: The forced vital capacity and forced expiratory volume in 1 s were statically significantly higher in Group II with CVD. Dyspnea measured with Modified Medical Research Council (MRC) dyspnea scale showed statistically significantly higher values in Group I COPD patients with CVC (2.9 ± 1.4) versus Group II without CVC (1.7 ± 1.4), (p < 0.05). The perception of the higher dyspnea in Group I was associated with increased COPD assessment test-scores, in Group I: Group I (19.8 ± 9.1) versus Group II: (9.8 ± 9.1), (p < 0.001). The number of exacerbations and what is more important the number of severe exacerbation leading to hospitalizations was statistically higher in patients of Group I with CVC than in Group II without CVC (3.0 ± 1.1 vs. 1.0 ± 2.1), (p < 0.001) and the number of hospitalizations (1.0 ± 1.1 vs. 0.3 ± 2.1) (p < 0.001). CONCLUSION: We can conclude that patients with COPD who have CVC have an increased risk of high symptoms, which mean poor quality of life and increased morbidity.

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