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Fibrosis pulmonary

Daniel Valdivia, Markus Kamler, Clemens Aigner
Severe chest wall deformities are considered a contraindication for lung transplantation. A 38-year-old male with idiopathic pulmonary fibrosis and severe pectus excavatum with a Haller-Index of 4.3 was considered eligible and listed for lung transplantation. Bilateral sequential transplantation and simultaneous correction of the pectus excavatum was performed via anterolateral thoracotomies and Nuss-bar insertion with peripheral femoro-femoral veno-arterial ECMO support. TLC increased from 4.1l preoperative to 5...
October 13, 2018: Annals of Thoracic Surgery
Jeffrey J Swigris
No abstract text is available yet for this article.
October 15, 2018: Respirology: Official Journal of the Asian Pacific Society of Respirology
Biyun Li, Xiaoxi Huang, Zheng Liu, Xuefeng Xu, Huijuan Xiao, Xin Zhang, Huaping Dai, Chen Wang
Idiopathic pulmonary fibrosis (IPF) is a lethal idiopathic interstitial pulmonary disease characterized by progressive deterioration in lung function that commonly affects eldly people. The pathogenesis of the disease is incompletely understood and therefore lacking effective therapy. Ouabain a digitalis has been reported to be able to suppress lung fibroblast activation via downregulating TGF-β-smad signal pathway in vitro . Here, we investigated the effects of ouabain in pulmonary fibrosis in vivo. Pulmonary fibrosis was induced in C57/BL6 mice by a intratracheal instillation of bleomycin (2...
2018: American Journal of Translational Research
Abdurrahman Erdem Başaran, Ayşen Başaran, İbrahim Cemal Maslak, Gökhan Arslan, Ayşen Bingöl
OBJECTIVES: The aim of this study was to evaluate radiological, clinical, and demographic data of patients with noncystic fibrosis bronchiectasis and to compare high-resolution computed tomography (HRCT) scores based on the demographic and clinical characteristics. MATERIALS AND METHODS: A total of 34 patients (18 male, 16 female) were assessed in terms of age at symptom onset, age at diagnosis, annual attack frequency, cough severity score, physical examination findings, and pulmonary function test results...
September 13, 2018: Turkish Thoracic Journal
Robert J Morrison, Nicolas-George Katsantonis, Kevin M Motz, Alexander T Hillel, C Gaelyn Garrett, James L Netterville, Christopher T Wootten, Susan M Majka, Timothy S Blackwell, Wonder P Drake, Alexander Gelbard
Objective To characterize the phenotype and function of fibroblasts derived from airway scar in idiopathic subglottic stenosis (iSGS) and to explore scar fibroblast response to interleukin 17A (IL-17A). Study Design Basic science. Setting Laboratory. Subjects and Methods Primary fibroblast cell lines from iSGS subjects, idiopathic pulmonary fibrosis subjects, and normal control airways were utilized for analysis. Protein, molecular, and flow cytometric techniques were applied in vitro to assess the phenotype and functional response of disease fibroblasts to IL-17A...
October 16, 2018: Otolaryngology—Head and Neck Surgery
Yu Fujita, Tsukasa Kadota, Jun Araya, Takahiro Ochiya, Kazuyoshi Kuwano
It is currently thought that extracellular vesicles (EVs), such as exosomes and microvesicles, play an important autocrine/paracrine role in intercellular communication. EVs package proteins, mRNA and microRNA (miRNA), which have the ability to transfer biological information to recipient cells in the lungs. Depending on their origin, EVs fulfil different functions. EVs derived from mesenchymal stem cells (MSCs) have been found to promote therapeutic activities that are comparable to MSCs themselves. Recent animal model-based studies suggest that MSC-derived EVs have significant potential as a novel alternative to whole-cell therapies...
October 14, 2018: Journal of Clinical Medicine
Bing Tian, Koa Hosoki, Zhiqing Liu, Jun Yang, Yingxin Zhao, Hong Sun, Jia Zhou, Erik Rytting, Lata Kaphalia, William J Calhoun, Sanjiv Sur, Allan R Brasier
BACKGROUND: Frequent exacerbations of allergic asthma leads to airway remodeling and a decline in pulmonary function, producing morbidity. Cat dander is an aeroallergen associated with asthma risk. OBJECTIVE: We sought to elucidate the mechanism of cat dander-induced inflammation-remodeling. METHODS: We identified remodeling in mucosal samples from allergic asthma by Q-RT-PCR. We developed a model of aeroallergen-induced experimental asthma by repetitive cat dander extract exposure...
October 12, 2018: Journal of Allergy and Clinical Immunology
Diptiman Chanda, Eva Otoupalova, Kenneth P Hough, Morgan L Locy, Karen Bernard, Jessy S Deshane, Ralph D Sanderson, James A Mobley, Victor J Thannickal
Extracellular vesicles (EVs) are endosome and plasma membrane-derived nanosized vesicles that participate in intercellular signaling. Although EV cargo may signal via multiple mechanisms, how signaling components on the surface of EVs mediate cellular signaling is less well understood. In this study, we show that fibroblast-derived EVs carry fibronectin on the vesicular surface, as evidenced by mass spectrometry-based proteomics (sequential windowed acquisition of all theoretical peptides; SWATH) and flow cytometric analyses...
October 15, 2018: American Journal of Respiratory Cell and Molecular Biology
Lurdes Planas-Cerezales, Elena G Arias-Salgado, Ivette Buendia-Roldán, Ana Montes-Worboys, Cristina E López, Vanesa Vicens Zygmunt, Patricio L Hernaiz, Roger L Sanuy, Virginia Leiro-Fernandez, Eva B Vilarnau, Ernest S Llinás, Jordi D Sargatal, Rosario P Abellón, Moisés Selman, Maria Molina-Molina
BACKGROUND AND OBJECTIVE: The abnormal shortening of telomeres is a mechanism linking ageing to idiopathic pulmonary fibrosis (IPF) that could be useful in the clinical setting. The objective of this study was to identify the IPF patients with higher risk for telomere shortening and to investigate the outcome implications. METHODS: Consecutive Spanish patients were included at diagnosis and followed up for 3 years. DNA blood samples from a Mexican cohort were used to validate the results found in Spanish sporadic IPF...
October 15, 2018: Respirology: Official Journal of the Asian Pacific Society of Respirology
Xiaohong Wen, Yuan Liu, Yu Bai, Mingwei Li, Qiang Fu, Yi Zheng
A previous study demonstrated that Lysyl oxidase‑like 2 (LOXL2) serves an essential role in matrix remodeling and fibrogenesis, thus indicating its involvement in fibrosis‑associated diseases. Our previous studies revealed a novel association between LOXL2 expression and pulmonary fibrosis in mice. However, the exact role and mechanisms of LOXL2 in interstitial lung disease remain poorly understood. The present study aimed to detect LOXL2 expression in mice with bleomycin (BLM)‑induced pulmonary fibrosis, and explore the effects of silencing LOXL2 on the proliferation, activation and fibrosis process of mouse lung fibroblasts (MLFs)...
October 11, 2018: International Journal of Molecular Medicine
Saundray Raj Soni, Bibhas K Bhunia, Nimmy Kumari, Subhashis Dan, Sudipta Mukherjee, Biman B Mandal, Animesh Ghosh
The present study was conducted to develop therapeutically effective controlled release formulation of pirfenidone (PFD) and explore the possibility to reduce the total administered dose and dosing regimen. For this purpose, pH-sensitive biomaterial was prepared by inducing carboxymethyl group on pullulan by Williamson ether synthesis reaction, and further, interpenetrating polymeric network microspheres were prepared by glutaraldehyde-assisted water-in-oil (w/o) emulsion cross-linking method, which showed higher swelling ratio in acidic and basic pH...
September 30, 2018: ACS Omega
Ilianna Barbayianni, Ioanna Ninou, Argyrios Tzouvelekis, Vassilis Aidinis
Idiopathic Pulmonary Fibrosis (IPF) is a fatal disease characterized by exuberant deposition of extracellular matrix components, deterioration of lung architecture and impairment of lung functions. Its etiopathogenesis remains incompletely understood, as reflected in the lack of an appropriate therapy. Modeling the human disease in mice via the administration of bleomycin (BLM), despite the inherent limitations, has provided valuable insights into the underlying pathogenetic mechanisms, and has been instrumental for the development and validation of new pharmacologic interventions...
2018: Frontiers in Medicine
Lifang Sun, Minjie Mao, Zhisheng Yan, Cuiyun Zuo, Xiaojie Zhang
Pulmonary fibrosis is a chronic and fatal disease of lung tissue with high incidence and mortality in the world. The exploration of effective treatment for pulmonary fibrosis remains an urgent challenge. In our study, Qingfei Xieding was investigated as a novel Chinese traditional patent medicine against pulmonary fibrosis. A pulmonary fibrosis mouse model was constructed by injecting with bleomycin sulfate. Following Qingfei Xieding administration, lung samples were collected to assess pulmonary phenotype changes by analyzing lung coefficient, wet/dry, and histopathologic section...
2018: Canadian Respiratory Journal: Journal of the Canadian Thoracic Society
Tingting Yuan, Thomas Volckaert, Diptiman Chanda, Victor J Thannickal, Stijn P De Langhe
The lung is morphologically structured into a complex tree-like network with branched airways ending distally in a large number of alveoli for efficient oxygen exchange. At the cellular level, the adult lung consists of at least 40-60 different cell types which can be broadly classified into epithelial, endothelial, mesenchymal, and immune cells. Fibroblast growth factor 10 (Fgf10) located in the lung mesenchyme is essential to regulate epithelial proliferation and lineage commitment during embryonic development and post-natal life, and to drive epithelial regeneration after injury...
2018: Frontiers in Genetics
C Martin, L Regard, G Chassagnon, P-R Burgel
Bronchiectasis is defined by a permanent and irreversible increase in airway caliber. Computed tomography (CT) scan is required for diagnosis and allows differentiating localized (affecting only one lobe) from diffuse (affecting two or more lobes) bronchiectasis. Localized bronchiectasis is usually related to a local cause (e.g., tumor, compression by lymph nodes, foreign body) whereas there are multiple causes of diffuse bronchiectasis. Main causes of diffuse bronchiectasis can be grouped into 5 categories: (1) immune deficiencies; (2) genetic disorders responsible for abnormal mucociliairy clearance (e...
October 11, 2018: Revue de Pneumologie Clinique
Katherine S Duke, Elizabeth A Thompson, Mark D Ihrie, Alexia J Taylor-Just, Elizabeth A Ash, Kelly A Shipkowski, Jonathan R Hall, Debra A Tokarz, Mark F Cesta, Ann F Hubbs, Dale W Porter, Linda M Sargent, James C Bonner
The fiber-like shape of multi-walled carbon nanotubes (MWCNTs) is reminiscent of asbestos, suggesting they pose similar health hazards when inhaled, including pulmonary fibrosis and mesothelioma. Mice deficient in the tumor suppressor p53 are susceptible to carcinogenesis. However, the chronic pathologic effect of MWCNTs delivered to the lungs of p53 heterozygous (p53+/- ) mice has not been investigated. We hypothesized that p53+/- mice would be susceptible to lung tumor development after exposure to either tangled (t-) or rod-like (r-) MWCNTs...
October 14, 2018: Nanotoxicology
C D Zhu, M Z Guo, Q Cai, Y Y Li, K X Wu, M Huang
Objective: To investigate the roles of p38 mitogen-activated protein kinases (p38 MAPK) , extracellular regulated protein kinases (ERK) and c-Jun N-tenninal kinases (JNK) of MAPK signaling pathway in Paraquat-induced epithelial to mesenchymal transition (EMT) of type II alveolarepithelial cells. Methods: RLE-6NT cells were incubated with different concentrations of PQ (0, 25, 50, 100μmol/L) for 6, 12 and 24 h. Cell morphology alteration was observed under phase-contrast microscopy. Cell viability was determined using an MTT assay...
August 20, 2018: Chinese Journal of Industrial Hygiene and Occupational Diseases
Lingzhi Zhong, Haojie Hao, Deyun Chen, Qian Hou, Ziying Zhu, Wenjun He, Sujing Sun, Mengli Sun, Meirong Li, Xiaobing Fu
BACKGROUND: Tissue contraction and the extracellular matrix deposition are part of the pathogenesis of hypertrophic scars. The transcriptional factor NFE2L2 inhibits fibroblast differentiation in idiopathic pulmonary fibrosis and promotes myofibroblast dedifferentiation. Our previous study showed that the transcription factor NFE2L2 was strongly induced on treatment with arsenic trioxide (ATO). OBJECTIVE: The present study sought to investigate the effect of ATO on myofibroblast formation to determine its potential role in hypertrophic scar treatment...
October 14, 2018: Journal of Cellular Physiology
L Regard, H Lafoeste, C Martin, G Chassagnon, P-R Burgel
Cystic fibrosis (CF) is a genetic disease with pulmonary involvement being predominant and often leading to respiratory failure and premature death. Non-pulmonary complications related to CF transmembrane conductance regulator (CFTR) defect are numerous and account for significant morbidity (exocrine pancreatic insufficiency, diabetes, CF-related liver disease, chronic sinusitis, osteoporosis). Improvement in patients' care led to a continuous increase in life expectancy, with a subsequent increase in the number of adult CF patients worldwide...
October 10, 2018: Revue de Pneumologie Clinique
Yazeed Toukan, Michal Gur, Fahed H Hakim, Yuval Geffen, Ronen Bar-Yoseph, Vered Nir, Lea Bentur
INTRODUCTION: CF pulmonary guidelines recommend alternate therapy (one month on, one month off) with inhaled tobramycin for chronic Pseudomonas aeruginosa colonization in cystic fibrosis (CF). Tobramycin-inhaled powder (TIP™) is increasingly replacing time-consuming nebulizer therapy. It is unclear whether laboratory parameters change during the month off period compared with the month on therapy. PURPOSE: Our aim was to assess whether spirometry, lung clearance index and circulating inflammatory markers differ between on/off treatment periods...
September 30, 2018: Clinical and Investigative Medicine. Médecine Clinique et Experimentale
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