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Frontiers in Physiology 2020Lung diseases constitute a global health concern causing disability. According to WHO in 2016, respiratory diseases accounted for 24% of world population mortality, the... (Review)
Review
Lung diseases constitute a global health concern causing disability. According to WHO in 2016, respiratory diseases accounted for 24% of world population mortality, the second cause of death after cardiovascular diseases. The Kv7 channels family is a group of voltage-dependent K channels (Kv) encoded by genes that are involved in various physiological functions in numerous cell types, especially, cardiac myocytes, smooth muscle cells, neurons, and epithelial cells. Kv7 channel α-subunits are regulated by KCNE1-5 ancillary β-subunits, which modulate several characteristics of Kv7 channels such as biophysical properties, cell-location, channel trafficking, and pharmacological sensitivity. Kv7 channels are mainly expressed in two large groups of lung tissues: pulmonary arteries (PAs) and bronchial tubes. In PA, Kv7 channels are expressed in pulmonary artery smooth muscle cells (PASMCs); while in the airway (trachea, bronchus, and bronchioles), Kv7 channels are expressed in airway smooth muscle cells (ASMCs), airway epithelial cells (AEPs), and vagal airway C-fibers (VACFs). The functional role of Kv7 channels may vary depending on the cell type. Several studies have demonstrated that the impairment of Kv7 channel has a strong impact on pulmonary physiology contributing to the pathophysiology of different respiratory diseases such as cystic fibrosis, asthma, chronic obstructive pulmonary disease, chronic coughing, lung cancer, and pulmonary hypertension. Kv7 channels are now recognized as playing relevant physiological roles in many tissues, which have encouraged the search for Kv7 channel modulators with potential therapeutic use in many diseases including those affecting the lung. Modulation of Kv7 channels has been proposed to provide beneficial effects in a number of lung conditions. Therefore, Kv7 channel openers/enhancers or drugs acting partly through these channels have been proposed as bronchodilators, expectorants, antitussives, chemotherapeutics and pulmonary vasodilators.
PubMed: 32676036
DOI: 10.3389/fphys.2020.00634 -
Seminars in Respiratory and Critical... Jun 2015Occupational and environmental causes of bronchiolar disorders are recognized on the basis of case reports, case series, and, less commonly, epidemiologic... (Review)
Review
Occupational and environmental causes of bronchiolar disorders are recognized on the basis of case reports, case series, and, less commonly, epidemiologic investigations. Pathology may be limited to the bronchioles or also involve other components of the respiratory tract, including the alveoli. A range of clinical, functional, and radiographic findings, including symptomatic disease lacking abnormalities on noninvasive testing, poses a diagnostic challenge and highlights the value of surgical biopsy. Disease clusters in workplaces and communities have identified new etiologies, drawn attention to indolent disease that may otherwise have been categorized as idiopathic, and expanded the spectrum of histopathologic responses to an exposure. More sensitive noninvasive diagnostic tools, evidence-based therapies, and ongoing epidemiologic investigation of at-risk populations are needed to identify, treat, and prevent exposure-related bronchiolar disorders.
Topics: Animals; Bronchial Diseases; Bronchioles; Environmental Exposure; Humans; Occupational Diseases; Occupational Exposure
PubMed: 26024345
DOI: 10.1055/s-0035-1549452 -
Medicine Dec 2023To analyze the clinical-pathological characteristics of 3 cases of bronchiolar adenoma/pulmonary ciliary mucinous nodular papillary tumors, and to improve the... (Review)
Review
OBJECTIVE
To analyze the clinical-pathological characteristics of 3 cases of bronchiolar adenoma/pulmonary ciliary mucinous nodular papillary tumors, and to improve the understanding of bronchiolar adenoma (BA)/ciliated muconodular papillary tumors (CMPT) (bronchiolar adenoma/ciliated muconodular papillary tumor).
METHODS
Retrospective analysis was done on the clinical information, diagnosis, and treatment of 3 instances of BA/CMPT at the Second People's Hospital of Weifang City. By scanning the CNKI, Wanfang, VIP database, and Pubmed database using the English key words "bronchiolar adenoma, ciliated muconodular papillary tumor," respectively patients with comprehensive clinical data were gathered, and studies from January 2002 to August 2021 that were relevant to the patients were examined.
RESULTS
A total of 35 articles and 71 instances were found, including 3 cases in our hospital, for a total of 74 cases. There were 31 males and 43 females among them, ranging in age from 18 to 84 years (average 63 years), and 15 cases had a smoking history. The majority of them were discovered by physical examination and had no clinical symptoms. The majority of the imaging revealed solid nodules with variable forms, with some ground-glass nodules displaying vacuole and bronchial inflation signs. BA/CMPT are generally gray-white, gray-brown solid nodules with obvious boundaries but no envelope with a maximum dimension of 4 to 45 mm (average 10.6 mm) on gross examination. Acinar, papillary, and lepidic formations can be seen under the microscope at high magnification; the majority of these structures are made up of tripartite epithelial components, including basal cells, mucous cells, ciliated columnar cells, and alveolar epithelial cells, demonstrating a variety of combinations. An important basis for diagnosis in immunohistochemistry is the continuous positive basal cell layer that is shown by p63, p40, and CK5/6. BRAF and epidermal growth factor receptor are the genes that are most frequently mutated. All of the patients showed no signs of metastasis or recurrence during follow-up period.
CONCLUSION
BA/CMPT is a rare benign tumor of lung epithelium. Because imaging and intraoperative cryosection diagnosis are easy to be misdiagnosed as malignant, it is necessary to further improve understanding and improve immunohistochemistry and genetic examination.
Topics: Adolescent; Adult; Aged; Aged, 80 and over; Female; Humans; Male; Middle Aged; Young Adult; Adenoma; Bronchioles; Epithelial Cells; Lung Neoplasms; Neoplasms, Cystic, Mucinous, and Serous; Retrospective Studies; Case Reports as Topic
PubMed: 38115282
DOI: 10.1097/MD.0000000000036559 -
The European Respiratory Journal Oct 2006Diffuse panbronchiolitis (DPB) is an idiopathic inflammatory disease, well recognised in Japan and principally affecting the respiratory bronchioles, causing a... (Review)
Review
Diffuse panbronchiolitis (DPB) is an idiopathic inflammatory disease, well recognised in Japan and principally affecting the respiratory bronchioles, causing a progressive suppurative and severe obstructive respiratory disorder. If left untreated, DPB progresses to bronchiectasis, respiratory failure and death. It was first described in the early 1960s. Subsequently, in 1969, the disease was named DPB to distinguish it from chronic bronchitis. "Diffuse" refers to the distribution of the lesions throughout both lungs, and "pan-" refers to the involvement of inflammation in all layers of the respiratory bronchioles. The distinctive imaging and histological features, the coexisting sinusitis, and the isolation of Haemophilus influenzae and Pseudomonas aeruginosa in the sputum enhance disease recognition. Histologically, DPB is characterised by chronic inflammation, localised mainly in the respiratory bronchioles and adjacent centrilobular regions, with characteristic interstitial accumulation of foamy histiocytes, neutrophils and lymphocyte infiltration. Neutrophils and T-lymphocytes, particularly CD8+ cells, together with the cytokines interleukin-8 and macrophage inflammatory protein-1, are believed to play key roles in the development of DPB. A significant improvement in the prognosis of this potentially fatal disease has been recently reported thanks to the use of long-term therapy with macrolide antibiotics, the effect of which is attributed to an anti-inflammatory and immunoregulatory action.
Topics: Anti-Bacterial Agents; Erythromycin; Humans; Lung; Lung Diseases, Interstitial; Macrolides; Radiography; Respiratory Function Tests
PubMed: 17012632
DOI: 10.1183/09031936.06.00131805 -
American Journal of Respiratory and... Feb 2024
Topics: Humans; Bronchioles; Bronchiectasis; Fibrosis
PubMed: 38190706
DOI: 10.1164/rccm.202312-2275ED -
American Journal of Respiratory and... Feb 2019
Topics: Bronchioles; Epithelial Cells; Humans; Lung Neoplasms; Protein Deficiency; Respiratory Physiological Phenomena
PubMed: 30557514
DOI: 10.1164/rccm.201811-2117ED -
Journal of Thoracic Disease Oct 2018Non-cystic fibrosis bronchiectasis (bronchiectasis) is an abnormal dilatation of the bronchi and bronchioles, resulting from a prolonged recurrent infectious process due... (Review)
Review
Non-cystic fibrosis bronchiectasis (bronchiectasis) is an abnormal dilatation of the bronchi and bronchioles, resulting from a prolonged recurrent infectious process due to various causes and predisposing factors. This disease has several etiologies and affects a heterogeneous population of patients. The most important viewpoint for the surgical management of bronchiectasis is to select appropriate candidates for surgery and conduct suitable anatomic lung resections at the right time under appropriate conditions. The ideal candidates for lung resection are symptomatic patients with focal disease due to truly localized bronchial pathology regardless of optimal and absolute medical management.
PubMed: 30505531
DOI: 10.21037/jtd.2018.08.128 -
Polish Archives of Internal Medicine Jun 2023Idiopathic pulmonary fibrosis (IPF) is a progressive and life‑threatening interstitial lung disease of familial or sporadic onset. The incidence and prevalence of IPF... (Review)
Review
Idiopathic pulmonary fibrosis (IPF) is a progressive and life‑threatening interstitial lung disease of familial or sporadic onset. The incidence and prevalence of IPF range from 0.09 to 1.3 and from 0.33 to 4.51 per 10 000 people, respectively. IPF has a poor prognosis, and death usually occurs within 2 to 5 years following the diagnosis due to secondary respiratory failure. Currently, there are 2 drugs available to treat IPF, pirfenidone and nintedanib. Both only slow the disease progression and, in addition, have unfavorable safety profiles. IPF bears the histology of usual interstitial pneumonia, which is characterized by bronchiolization of distal airspaces, honeycombing, fibroblastic foci, and abnormal epithelial hyperplasia. In the last years, alterations in metabolic pathways, in particular those associated with fatty acid (FA) metabolism have been linked with the pathogenesis of lung fibrosis. Changes in FA profiles have been reported in lung tissue, plasma, and bronchoalveolar lavage fluid of IPF patients, and have been found to correlate with the disease progression and outcome. In addition, they have been associated with the development of a profibrotic phenotype of epithelial cells, macrophages, and fibroblasts / myofibroblasts contributing to their (trans)differentiation and production of the disease‑relevant mediators. Furthermore, strategies focusing on the correction of FA profiles in experimental models of lung fibrosis brought advances in understanding tissue scarring processes and contributed to the transition of new molecules into clinical development. This review highlights the role of FAs and their metabolites in IPF and provides evidence for therapeutic potential of lipidome manipulations in the treatment of this disease.
Topics: Humans; Idiopathic Pulmonary Fibrosis; Lung Diseases, Interstitial; Lung; Bronchoalveolar Lavage Fluid; Disease Progression
PubMed: 37387676
DOI: 10.20452/pamw.16520 -
Archivos Argentinos de Pediatria Jun 2018Bronchiolitis obliterans is an uncommon and severe chronic lung disease due to lower respiratory tract injury. In our country, it is more frequent to observe it...
Bronchiolitis obliterans is an uncommon and severe chronic lung disease due to lower respiratory tract injury. In our country, it is more frequent to observe it secondary to a severe viral injury, especially by Adenovirus. Bronchiolitis obliterans is characterized by partial or total occlusion of the lumen of the respiratory and terminal bronchioles by inflammatory tissue and fibrosis producing chronic obstruction of the airway. This consensus discusses the current state of knowledge in the different areas of bronchiolitis obliterans due to an infectious lesion.
PubMed: 35786820
DOI: 10.5546/aap.2018.s48 -
The Quarterly Journal of Medicine Jan 1994We review current concepts about the clinical manifestations, diagnosis and treatment of patients with bronchiolitis obliterans (BO) with emphasis on... (Review)
Review
We review current concepts about the clinical manifestations, diagnosis and treatment of patients with bronchiolitis obliterans (BO) with emphasis on clinical/pathological correlations and recent developments. BO is a relatively rare disease, but its incidence is probably higher than generally believed and is continuously rising, partly because of better recognition, but also because of increased exposure to industrial fumes, and its occurrence in lung transplantation. BO is characterized histologically by varying degrees of obliteration of the lumen of the respiratory bronchioles by organizing connective tissue often extending into the alveoli ('proliferative' BO with organizing pneumonia--BOOP) or by more extensive fibrosis and scarring of the more proximal, conductive bronchioles ('constrictive' BO). Diverse clinical conditions have been associated with the development of BO, notably viral and mycoplasma infection, toxic fume exposure and immune reactions in the setting of a collagen vascular disease, drug reaction or organ transplantation. The clinical course and features of BO may vary considerably according to the aetiology, histological pattern and stage of the disease. The most common presentation is that of a progressive dry cough and dyspnea, associated with diffuse patchy interstitial lung infiltrates on chest X-ray. In the more advanced cases, lung function tests show either restrictive or obstructive defects, depending on the extent of alveolar involvement, and hypoxemia without CO2 retention. The diagnosis is often possible on clinical grounds, however, in a seriously ill patient uncertainty should be resolved by tissue diagnosis, preferably by open lung biopsy. Treatment is based on symptomatic therapy. The use of corticosteroids is controversial, but common. Patients with BOOP are exceptional, in that there may be no underlying condition ('idiopathic' BOOP or cryptogenic organizing pneumonia--COP), a restrictive ventilatory defect is usual and the response to corticosteroids often remarkable.
Topics: Adrenal Cortex Hormones; Bronchiolitis Obliterans; Humans
PubMed: 8140211
DOI: 10.1093/oxfordjournals.qjmed.a068855