Search results for: bronchiectasis

Authors: Mahsa Zargaran

Abstract:

Introduction: Bronchiectasis, a chronic respiratory ailment, has grown progressively widespread globally. Common Variable Immunodeficiency (CVID) has been recognized as a notable contributing factor for bronchiectasis. In order to effectively manage this condition, a thorough and focused strategy is necessary. Material and Methods: A systematic literature search was conducted in Web of Science, PubMed, and EMBASE from January 2000 to December 2023 using established keywords. In addition, we discovered randomized controlled trials (RCTs) by searching the Cochrane Airways Group Register of trials and online trials registries. Two reviewers autonomously retrieved and recorded data from the papers that were included, and evaluated the potential for bias in each study. Results: The majority of research have shown that the prevalence of bronchiectasis in individuals with CVID is 24.9%. Furthermore, bronchiectasis is the most commonly observed radiological abnormality in these patients. Also, there is a significant occurrence of bronchiectasis in the Granulomatous Lymphocytic Interstitial Lung Disease (GL-ILD) group, with a prevalence rate of 31.3%. Research indicates that individuals diagnosed with CVID who also have bronchiectasis have insufficient forced expiratory volume in one second (FEV1). Furthermore, patients with bronchiectasis experience a higher frequency of respiratory tract infections and a diminished quality of life. Conclusion: Bronchiectasis is the predominant radiological observation in individuals with CVID, resulting in a reduction in FEV1, as well as recurrent infections in the lower respiratory tract. Additionally, individuals diagnosed with bronchiectasis exhibited reduced levels of serum immunoglobulin A (IgA) and immunoglobulin M (IgM). This study offers a fresh outlook and emphasizes the significance of early diagnosis and the need for enhancements in treatment approaches.

Keywords: common variable immunodeficiency -, bronchiectasis, forced expiratory volume in one second (FEV1), respiratory tract infections

Procedia PDF Downloads 0

Authors: Nadzeya Marozkina, Joe Zein, Benjamin Gaston

Abstract:

Introduction: We have observed that many patients with Primary Ciliary Dyskinesia (PCD) benefit from asthma medications. In healthy airways, the ciliary function is normal. Antigens and irritants are rapidly cleared, and NO enters the gas phase normally to be exhaled. In the PCD airways, however, antigens, such as Dermatophagoides, are not as well cleared. This defect leads to oxidative stress, marked by increased DUOX1 expression and decreased superoxide dismutase [SOD] activity (manuscript under revision). H₂O₂, in high concentrations in the PCD airway, injures the airway. NO is oxidized rather than being exhaled, forming cytotoxic peroxynitrous acid. Thus, antigen stasis on PCD airway epithelium leads to airway injury and may predispose PCD patients to asthma. Indeed, recent population genetics suggest that PCD genes may be associated with asthma. We therefore hypothesized that PCD patients would be predisposed to having asthma. Methods. We analyzed our database of 18 million individual electronic medical records (EMRs) in the Indiana Network for Patient Care research database (INPCR). There is not an ICD10 code for PCD itself; code Q34.8 is most commonly used clinically. To validate analysis of this code, we queried patients who had an ICD10 code for both bronchiectasis and situs inversus totalis in INPCR. We also studied a validation cohort using the IBM Explorys® database (over 80 million individuals). Analyses were adjusted for age, sex and race using a 1 PCD: 3 controls matching method in INPCR and multivariable logistic regression in the IBM Explorys® database. Results. The prevalence of asthma ICD10 codes in subjects with a code Q34.8 was 67% vs 19% in controls (P < 0.0001) (Regenstrief Institute). Similarly, in IBM*Explorys, the OR [95% CI] for having asthma if a patient also had ICD10 code 34.8, relative to controls, was =4.04 [3.99; 4.09]. For situs inversus alone the OR [95% CI] was 4.42 [4.14; 4.71]; and bronchiectasis alone the OR [95% CI] =10.68 (10.56; 10.79). For both bronchiectasis and situs inversus together, the OR [95% CI] =28.80 (23.17; 35.81). Conclusions: PCD causes antigen stasis in the human airway (under review), likely predisposing to asthma in addition to oxidative and nitrosative stress and to airway injury. Here, we show that, by several different population-based metrics, and using two large databases, patients with PCD appear to have between a three- and 28-fold increased risk of having asthma. These data suggest that additional studies should be undertaken to understand the role of ciliary dysfunction in the pathogenesis and genetics of asthma. Decreased antigen clearance caused by ciliary dysfunction may be a risk factor for asthma development.

Keywords: antigen, PCD, asthma, nitric oxide

Procedia PDF Downloads 111

Authors: Sharique Ahmed, Khushtar Anwar Salman

Abstract:

Alpha-1-antitrypsin (AAT) is a major plasma serine protease inhibitor (SERPIN). Hereditary AAT deficiency is one of the common diseases in some part of the world. AAT is mainly produced in the liver and functions to protect the lung against proteolytic damage (e.g., from neutrophil elastase) acting as the major inhibitor for neutrophil elastase. α (1)-Antitrypsin (AAT) deficiency is an under recognized genetic condition that affects approximately 1 in 2,000 to 1 in 5,000 individuals and predisposes to liver disease and early-onset emphysema. Not only does α-1-antitrypsin deficiency lead to disabling syndrome of pulmonary emphysema, there are other disorders too which include ANCA (antineutrophilic cytoplasmic antibody) positive Wegener's granulomatosis, diffuse bronchiectasis, necrotizing panniculitis in α-1-antitrypsin phenotype (S), idiopathic pulmonary fibrosis and steroid dependent asthma. Augmentation therapy with alpha-1 antitrypsin (AAT) from human plasma has been available for specific treatment of emphysema due to AAT deficiency. Apart from this several observations have also suggested a role for endogenous suppressors of HIV-1, alpha-1 antitrypsin (AAT) has been identified to be one of those. In view of its varied important role in humans, serum from a mammalian source was chosen for the isolation and purification. Studies were performed on the homogeneous fraction. This study suggests that the buffalo serum α-1-antritrypsin has characteristics close to ovine, dog, horse and more importantly to human α-1-antritrypsin in terms of its hydrodynamic properties such as molecular weight, carbohydrate content, etc. The similarities in the hydrodynamic properties of buffalo serum α-1-antitrypsin with other sources of mammalian α-1-antitrypsin mean that it can be further studied and be a potential source for "augmentation therapy", as well as a source of AAT replacement therapy to raise serum levels above the protective threshold. Other parameters like the amino acid sequence, the effect of denaturants, and the thermolability or thermostability of the inhibitor will be the interesting basis of future studies on buffalo serum alpha-1 antitrypsin (AAT).

Keywords: α-1-antitrypsin, augmentation therapy , hydrodynamic properties, serine protease inhibitor

Procedia PDF Downloads 492