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Fibrosis pulmonary

Ho-Fung Chan, Guilhem J Collier, Nicholas D Weatherley, Jim M Wild
PURPOSE: To compare in vivo lung morphometry parameters derived from theoretical gas diffusion models, the cylinder model and stretched exponential model, in a range of acinar microstructural length scales encountered in healthy and diseased lungs with 3 He and 129 Xe diffusion-weighted MRI. METHODS: Three-dimensional multiple b-value 3 He and 129 Xe diffusion-weighted MRI was acquired with compressed sensing at 1.5 T from 51 and 31 subjects, respectively, including healthy volunteers, ex-smokers, idiopathic pulmonary fibrosis, and chronic obstructive pulmonary disease patients...
December 4, 2018: Magnetic Resonance in Medicine: Official Journal of the Society of Magnetic Resonance in Medicine
Bugra Kerget, Omer Araz, Elif Yilmazel Ucar, Ozkan Aydin, Metin Akgun, Leyla Saglam
Sulfasalazine has been used in the treatment of inflammatory bowel disease for over 60 years. Although the drug is frequently associated with gastrointestinal adverse effects, pulmonary adverse effects are very rare. Herein, we report a case of interstitial fibrosis resulting from 4-month sulfasalazine therapy for ulcerative colitis in a patient under long-term follow-up in our clinic due to chronic obstructive pulmonary disease.
October 2018: Eurasian Journal of Medicine
Kenichiro Yamamura, Darren Yuen, Edward J Hickey, Xiaolin He, Rajiv R Chaturvedi, Mark K Friedberg, Lars Grosse-Wortmann, Kate Hanneman, Filio Billia, Michael E Farkouh, Rachel M Wald
OBJECTIVE: The relationship between right ventricular (RV) fibrosis and right heart reverse remodelling following pulmonary valve replacement (PVR) has not been well studied in adults with repaired tetralogy of Fallot (rTOF). Our aims were to histologically quantify RV fibrosis and to explore the relationship between fibrosis severity and cardiac remodelling post-PVR. METHODS: Adults with rTOF and pre-PVR cardiovascular (CMR) imaging were consented to procurement of RV muscle during PVR...
December 4, 2018: Heart: Official Journal of the British Cardiac Society
Xiaoli Li, Guoliang An, Yan Wang, Di Liang, Zhonghui Zhu, Lin Tian
BACKGROUND: Silicosis is a common occupational disease, characterized by silicotic nodules and diffuse pulmonary fibrosis. We demonstrated an anti-fibrotic effect of bone marrow mesenchymal stem cells (BMSCs) in silica-induced lung fibrosis. In the present study, we sought to clarify the homing ability of BMSCs and the specific mechanisms for their effects. METHODS AND RESULTS: The biodistribution of BMSCs was identified by near-infrared fluorescence (NIRF) imaging in vivo and in vitro...
December 4, 2018: Stem Cell Research & Therapy
Margarida C Gomes, Yara Tasrini, Sujatha Subramoni, Kirsty Agnoli, Joana R Feliciano, Leo Eberl, Pamela Sokol, David O'Callaghan, Annette C Vergunst
The opportunistic pathogen Burkholderia cenocepacia is particularly life-threatening for cystic fibrosis (CF) patients. Chronic lung infections with these bacteria can rapidly develop into fatal pulmonary necrosis and septicaemia. We have recently shown that macrophages are a critical site for replication of B. cenocepacia K56-2 and the induction of fatal pro-inflammatory responses using a zebrafish infection model. Here, we show that ShvR, a LysR-type transcriptional regulator that is important for biofilm formation, rough colony morphotype and inflammation in a rat lung infection model, is also required for the induction of fatal pro-inflammatory responses in zebrafish larvae...
December 4, 2018: PLoS Pathogens
Seidai Sato, Toyoshi Yanagihara, Martin Rj Kolb
Idiopathic pulmonary fibrosis (IPF) is an age-associated, progressive and irreversible fatal interstitial lung disease. Although many drugs have failed in clinical trials, these failures improved the understanding of the pathogenesis of IPF. Currently, there are two drugs approved for IPF that slow the progression of the disease. However, the prognosis for patients with IPF remains poor and the search continues for drugs that inhibit the pathogenic pathways active in IPF to further reduce or even halt the progression of the disease...
December 4, 2018: Expert Opinion on Investigational Drugs
Cheng Zhou, Bleddyn Jones, Mahmoud Moustafa, Bing Yang, Stephan Brons, Liji Cao, Ying Dai, Christian Schwager, Ming Chen, Oliver Jaekel, Longhua Chen, Juergen Debus, Amir Abdollahi
Background and purposes: Carbon ion radiotherapy (CIRT) with raster scanning technology is a promising treatment for lung cancer and thoracic malignancies. Determining normal tissue tolerance of organs at risk is of utmost importance for the success of CIRT. Here we report the relative biological effectiveness (RBE) of CIRT as a function of dose and fractionation for development of pulmonary fibrosis using well established fibrosis index (FI) model. Materials and Methods: Dose series of fractionated clinical quality CIRT versus conventional photon irradiation to the whole thorax were compared in C57BL6 mice...
January 2019: Clinical and Translational Radiation Oncology
Seungho Choi, Jong Kyu Woo, Yeong-Su Jang, Ju-Hee Kang, Jong-Ik Hwang, Je Kyung Seong, Yeo Sung Yoon, Seung Hyun Oh
The transmembrane nerve injury-induced protein 1 (Ninjurin1 or Ninj1) is involved in progressing inflammatory diseases. In this study, we aimed to investigate a novel function of Ninj1 in pulmonary fibrosis. We found that the expression of Ninj1 in a patient cohort was upregulated in the lung specimens of idiopathic pulmonary fibrosis patients as well as mice with bleomycin-induced pulmonary fibrosis. In addition, the BLM-injected Ninj1 KO mice exhibited a mild fibrotic phenotype, as compared to WT mice. Therefore, we hypothesized that Ninj1 would play an important role in the development of pulmonary fibrosis...
December 3, 2018: Scientific Reports
Torsten Witte
Primary Sjögren's syndrome (pSS) is an autoimmune disease affecting the salivary and lachrymal glands. The patients complain of symptoms of dry eyes and dry mouth, but up to 50 % may additionally develop extraglandular manifestations such as arthritis, vasculitis, polyneuropathy, pulmonary fibrosis or interstitial nephritis. Most therapeutic studies on the glandular manifestations of pSS failed to meet their primary endpoints, possibly since many of the patients had already advanced and irreversible disease...
December 2018: Deutsche Medizinische Wochenschrift
Anna Jakubczyc, Claus Neurohr
Interstitial lung diseases compromise a group of diffuse predominantly chronic inflammatory and fibrosing disorders of the lung parenchyma. This article reviews practice-relevant publications on the diagnosis and treatment of idiopathic pulmonary fibrosis - the most common form of idiopathic interstitial lung diseases. The new patient questionnaire of the German Society of Pneumology facilitates collection of a complex case history of diverse interstitial lung diseases in individual patients. The Fleischner Society White Paper emphasize the importance of high-resolution computer tomography in the diagnosis of IPF...
December 2018: Deutsche Medizinische Wochenschrift
Kalliopi Keramida, George Lazaros, Petros Nihoyannopoulos
Although hypertrophic cardiomyopathy (HCM) is the most common inherited cardiomyopathy worldwide, the criteria for its definition and most of the literature concern the left ventricle, confirming the theory that the right ventricle is the neglected one. Right ventricular (RV) involvement includes structural and functional changes with significant impact on clinical presentation and prognosis. The pattern of RV hypertrophy can be variable with possible dynamic obstruction. Histological findings suggest similar pathogenetic changes in both ventricles supporting the common myopathic process with sarcomeric mutations...
November 30, 2018: Hellenic Journal of Cardiology: HJC, Hellēnikē Kardiologikē Epitheōrēsē
Valerie D Nolt, Kyle D Pijut, Elizabeth B Autry, Whitney C Williams, David S Burgess, Donna R Burgess, Vaneet Arora, Robert J Kuhn
AIM: Pseudomonas aeruginosa (PsA) is a common pathogen in cystic fibrosis (CF). Management of an acute pulmonary exacerbation (APE) caused by PsA is dual anti-pseudomonal antibiotics, a beta-lactam plus aminoglycoside. Aminoglycoside dosing in CF differs from the general population due to altered pharmacokinetics. The primary objective of this study was to utilize pharmacokinetic data from adult CF patients that received amikacin to determine the probability of target attainment for APEs caused by PsA...
December 3, 2018: Pediatric Pulmonology
Huimei Wang, Qiqi Xie, Wen Ou-Yang, Mingwei Zhang
Idiopathic pulmonary fibrosis (IPF), characterized by irreversible scarring and progressive destruction of the lung tissue, is one of the most common types of idiopathic interstitial pneumonia worldwide. However, there are no reliable candidates for curative therapies. Hence, elucidation of the mechanisms of IPF genesis and exploration of potential biomarkers and prognostic indicators are essential for accurate diagnosis and treatment of IPF. Recently, efficient microarray and bioinformatics analyses have promoted an understanding of the molecular mechanisms of disease occurrence and development, which is necessary to explore genetic alternations and identify potential diagnostic biomarkers...
December 2, 2018: Journal of Cellular Biochemistry
Marlies Wijsenbeek, Elisabeth Bendstrup, Claudia Valenzuela, Michael T Henry, Catharina Moor, Monica Bengus, Andras Perjesi, Frank Gilberg, Klaus-Uwe Kirchgaessler, Carlo Vancheri
BACKGROUND/OBJECTIVES: This study will aim to characterise disease behaviour during the peri-diagnostic period in patients with suspected interstitial lung disease (ILD), including idiopathic pulmonary fibrosis (IPF), using daily home spirometry and accelerometry. Additionally, this study will aim to increase collaboration between secondary and tertiary centres using a digital collaboration platform. METHODS: The STARLINER study (NCT03261037) will enrol approximately 180 symptomatic patients aged 50 years or more with radiological evidence of ILD/IPF from community and tertiary centres in Canada and Europe...
November 30, 2018: Advances in Therapy
Yaqiong Ma, Shikui Zhang, Lianping Zhao, Xing Zhou, Zeqing Mao, Huaxin Xu, Xiaorui Ru, Gang Huang
Background: This retrospective study aimed to investigate the computed tomography (CT) manifestations, short-term dynamic evolution features and quantitative lung CT analysis of inhalation lung injury induced by smoke bomb flare. Methods: Eleven pediatric patients (aged 11 to 13) who inhaled the smoke of smoke bombs underwent several low-dose chest CT scans. The image characteristics and their dynamic changes were observed and quantitative CT values were analyzed...
October 2018: Journal of Thoracic Disease
Bo Da Nam, Tae Jung Kim, Man Pyo Chung, Myung Jin Chung, Tae Sung Kim, Kyung Soo Lee
Background: Pulmonary alveolar proteinosis (PAP) is a rare disease of unknown origin, characterized by impaired surfactant metabolism. In some patients, residual disease is observed after treatment; notably, this may progress to end-stage pulmonary fibrosis. This study was performed to evaluate changes in lung abnormalities on serial CT scans performed on the lungs of patients diagnosed with PAP, as well as to identify factors that can be used to predict clinical improvement in PAP. Methods: Twenty-five patients (16 men, nine women) were diagnosed with PAP at a single tertiary hospital...
October 2018: Journal of Thoracic Disease
Signe Holm Nielsen, Nicholas Willumsen, Diana Julie Leeming, Samuel Joseph Daniels, Susanne Brix, Morten Asser Karsdal, Federica Genovese, Mette Juul Nielsen
OBJECTIVES: Remodeling of the extracellular matrix (ECM) is a key event in different lung disorders, such as fibrosis and cancer. The most common cell type in the connective tissue is fibroblasts, which transdifferentiate into myofibroblasts upon activation. All myofibroblasts express α-SMA, which has been found to be upregulated in lung fibrosis and cancer. We evaluated the potential of α-SMA as a noninvasive biomarker of activated fibroblasts in lung fibrosis and cancer. METHODS: A monoclonal antibody was raised against the N-terminal of α-SMA, and a novel competitive enzyme-linked immunosorbent assay (ELISA) measuring α-SMA was developed and technically characterized...
November 29, 2018: Translational Oncology
Cécile Clercx, Aline Fastrès, Elodie Roels
Canine idiopathic pulmonary fibrosis (CIPF) affects middle-aged to older dogs of a single breed, mainly the West Highland white terrier (WHWT), which is suggestive of a genetic predisposition. CIPF causes exercise intolerance, restrictive dyspnoea and coughing. Coarse crackles are heard on thoracic auscultation. Abnormal blood gas parameters and a shortened '6-min-walking test' distance are common; secondarily induced pulmonary hypertension and/or airway collapse are frequent. These features of CIPF mimic those of idiopathic pulmonary fibrosis (IPF) in humans and therefore identify CIPF as a possible spontaneously arising model for study of human IPF...
December 2018: Veterinary Journal
C Ravnholt, T Qvist, M Kolpen, T Pressler, M Skov, N Høiby
BACKGROUND: Early signs of Mycobacterium avium complex pulmonary disease can be missed in patients with cystic fibrosis due to subclinical infection or delays in mycobacterial culture. The aim of this study was to determine the diagnostic accuracy of a novel enzyme linked immunosorbent assay for immunoglobulin G against Mycobacterium avium complex, which could help stratify patients according to risk. METHODS: A retrospective cross sectional analysis of serum samples from the Copenhagen Cystic Fibrosis Center was performed...
November 29, 2018: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
Sanae Kanno, Seishiro Hirano, Toshiji Mukai, Ayako Ro, Hideaki Kato, Mamiko Fukuta, Yasuhiro Aoki
Paraquat (PQ) is one of the commonly used herbicides in the world, despite its high toxicity. The ingestion of PQ accidentally or intentionally causes severe damage in diverse organs including the lung. Pulmonary fibrosis triggered by PQ accumulation in the lung epithelial cells is one of the major causes of death. This study investigated the intracellular accumulation of PQ, reactive oxygen species (ROS) generation and mitochondrial injury using two lung epithelial cell lines A549 and BEAS-2B (BEAS). Although A549 exhibit greater resistance to oxidative stress than BEAS, a cytotoxicity assay for PQ demonstrated that EC50 for lethality in A549 was 7 times lower than that in BEAS...
November 22, 2018: Legal Medicine
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