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Full Text Journal Articles about
Idiopathic Pulmonary Fibrosis

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Adult outcomes of childhood bronchiectasis.

Dawn Sibanda, Rosalyn Singleton, John Clark, Christine Desnoyers, Ellen Hodges, Gretchen Day, Gregory Redding,

Recent literature has highlighted the importance of transition from paediatric to adult care for children with chronic conditions. Non-cystic fibrosis bronchiectasis is an important cause of respiratory morbidity in low-income countries and in indigenous children from affluent countries; however, there is little information about adult outcomes of childhood bronchiectasis. We ... Read more >>

Int J Circumpolar Health (International journal of circumpolar health)
[2020, 79(1):1731059]

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Management of systemic sclerosis: the first five years.

David Roofeh, Dinesh Khanna,

PURPOSE OF REVIEW:This review provides a risk-stratified and evidence-based management for subsets of systemic sclerosis (SSc) patients in the first five years from disease onset. RECENT FINDINGS:Cardiopulmonary disease remains the primary cause of mortality in SSc patients. Morbidity and mortality in SSc-associated pulmonary arterial hypertension have improved with combination treatment, ... Read more >>

Curr Opin Rheumatol (Current opinion in rheumatology)
[2020, 32(3):228-237]

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Renoprotective effect of vildagliptin following hepatic ischemia/reperfusion injury.

Iman O Sherif, Alaa A Alshaalan, Nora H Al-Shaalan,

Remote renal injury is a drastic consequence of hepatic ischemia/reperfusion (IR) injury. Vildagliptin (V) is a dipeptidyl peptidase-4 inhibitor that has a hepatorenal protective effect against models of liver and renal IR. This research was done to explore the protective role of vildagliptin against renal injury following hepatic IR injury ... Read more >>

Ren Fail (Renal Failure)
[2020, 42(1):208-215]

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Long Noncoding RNA FENDRR Exhibits Antifibrotic Activity in Pulmonary Fibrosis.

Chaoqun Huang, Yurong Liang, Xiangming Zeng, Xiaoyun Yang, Dao Xu, Xuxu Gou, Roshini Sathiaseelan, Lakmini Kumari Senavirathna, Pengcheng Wang, Lin Liu,

Abnormal activation of lung fibroblasts contributes to the initiation and progression of idiopathic pulmonary fibrosis (IPF). The objective of the present study was to investigate the role of fetal-lethal noncoding developmental regulatory RNA (FENDRR) in the activation of lung fibroblasts. Dysregulated long noncoding RNAs in IPF lungs were identified by ... Read more >>

Am. J. Respir. Cell Mol. Biol. (American journal of respiratory cell and molecular biology)
[2020, 62(4):440-453]

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Screening for YAP Inhibitors Identifies Statins as Modulators of Fibrosis.

Daniela M Santos, Lorena Pantano, Gina Pronzati, Paula Grasberger, Clemens K Probst, Katharine E Black, Jillian J Spinney, Lida P Hariri, Royale Nichols, Yufei Lin, Michael Bieler, Peter Seither, Paul Nicklin, David Wyatt, Andrew M Tager, Benjamin D Medoff,

Idiopathic pulmonary fibrosis is a lung disease with limited therapeutic options that is characterized by pathological fibroblast activation and aberrant lung remodeling with scar formation. YAP (Yes-associated protein) is a transcriptional coactivator that mediates mechanical and biochemical signals controlling fibroblast activation. In this study, we developed a high-throughput small-molecule screen ... Read more >>

Am. J. Respir. Cell Mol. Biol. (American journal of respiratory cell and molecular biology)
[2020, 62(4):479-492]

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Occupational exposures and idiopathic pulmonary fibrosis.

Gareth I Walters,

PURPOSE OF REVIEW:A recent meta-analysis of data from international case-control studies reports a population attributable fraction of 16% for occupational factors in the cause of idiopathic pulmonary fibrosis (IPF). Smoking, genetic factors and other prevalent diseases only partly explain IPF, and so this review aims to summarize recent progress in ... Read more >>

Curr Opin Allergy Clin Immunol (Current opinion in allergy and clinical immunology)
[2020, 20(2):103-111]

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Alveolar Epithelial Type II Cells as Drivers of Lung Fibrosis in Idiopathic Pulmonary Fibrosis.

Tanyalak Parimon, Changfu Yao, Barry R Stripp, Paul W Noble, Peter Chen,

: Alveolar epithelial type II cells (AT2) are a heterogeneous population that have critical secretory and regenerative roles in the alveolus to maintain lung homeostasis. However, impairment to their normal functional capacity and development of a pro-fibrotic phenotype has been demonstrated to contribute to the development of idiopathic pulmonary fibrosis ... Read more >>

Int J Mol Sci (International journal of molecular sciences)
[2020, 21(7):]

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Thrombomodulin Did Not Benefit Acute Exacerbation of Idiopathic Pulmonary Fibrosis in a Trial.

Nobuyuki Horita, Kaneko Takeshi,

Am. J. Respir. Crit. Care Med. (American journal of respiratory and critical care medicine)
[2020, :]

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Air Space Distension Precedes Spontaneous Fibrotic Remodeling and Impaired Cholesterol Metabolism in the Absence of Surfactant Protein C.

Jannik Ruwisch, Kirsten Sehlmeyer, Nuria Roldan, Begoña Garcia-Alvarez, Jesus Perez-Gil, Timothy E Weaver, Matthias Ochs, Lars Knudsen, Elena Lopez-Rodriguez,

Surfactant protein (SP)-C deficiency is found in samples from patients with idiopathic pulmonary fibrosis, especially in familial forms of this disease. We hypothesized that SP-C may contribute to fibrotic remodeling in aging mice and alveolar lipid homeostasis. For this purpose, we analyzed lung function, alveolar dynamics, lung structure, collagen content, ... Read more >>

Am. J. Respir. Cell Mol. Biol. (American journal of respiratory cell and molecular biology)
[2020, 62(4):466-478]

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Nanoengineered immunosuppressive therapeutics modulating M1/M2 macrophages into the balanced status for enhanced idiopathic pulmonary fibrosis therapy.

Xin Chang, Lei Xing, Yi Wang, Tian-Jiao Zhou, Li-Jun Shen, Hu-Lin Jiang,

Effective treatment in clinic for idiopathic pulmonary fibrosis (IPF) remains a challenge due to low drug accumulation in lungs and imbalanced polarization of pro/anti-inflammatory macrophages (M1/M2 macrophages). Herein, a novel endogenous cell-targeting nanoplatform (PNCE) is developed for enhanced IPF treatment efficacy through modulating M1/M2 macrophages into the balanced status to ... Read more >>

Nanoscale (Nanoscale)
[2020, :]

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Longitudinal functional changes with clinically significant radiographic progression in idiopathic pulmonary fibrosis: are we following the right parameters?

Marco Mura, Nada Taha, Dejanira D'Amato, Karishma Hosein, Tiziana Ranalli, Gianluigi Sergiacomi, Maurizio Zompatori,

<title>Abstract</title> <p> Background . Progression of the disease in idiopathic pulmonary fibrosis (IPF) is difficult to predict, due to its variable and heterogenous course. The relationship between radiographic progression and functional decline in IPF is unclear. We sought to confirm that a simple HRCT fibrosis visual score is ... Read more >>

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Reply to: Thrombomodulin Did Not Benefit Acute Exacerbation of Idiopathic Pulmonary Fibrosis in a Trial.

Yasuhiro Kondoh, Arata Azuma, Jun Tagawa, Sakae Homma,

Am. J. Respir. Crit. Care Med. (American journal of respiratory and critical care medicine)
[2020, :]

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[German Guideline for Idiopathic Pulmonary Fibrosis].

J Behr, A Günther, F Bonella, J Dinkel, L Fink, T Geiser, K Geißler, S Gläser, S Handzhhiev, D Jonigk, D Koschel, M Kreuter, G Leuschner, P Markart, A Prasse, N Schönfeld, J C Schupp, H Sitter, J Müller-Quernheim, U Costabel,

Idiopathic pulmonary fibrosis (IPF) is a severe and often fatal disease. Diagnosis of IPF requires considerable expertise and experience. Since publication of the international IPF guideline in the year 2011 and Update 2018 several studies and technical advances occurred, which made a new assessment of the diagnostic process mandatory. In ... Read more >>

Pneumologie (Pneumologie (Stuttgart, Germany))
[2020, :]

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Pirfenidone: Molecular Mechanisms and Potential Clinical Applications in Lung Disease.

Saleela M Ruwanpura, Belinda J Thomas, Philip G Bardin,

Pirfenidone (PFD) is a pharmacological compound with therapeutic efficacy in idiopathic pulmonary fibrosis. It has been chiefly characterized as an antifibrotic agent, although it was initially developed as an antiinflammatory compound because of its ability to diminish the accumulation of inflammatory cells and cytokines. Despite recent studies that have elucidated ... Read more >>

Am. J. Respir. Cell Mol. Biol. (American journal of respiratory cell and molecular biology)
[2020, 62(4):413-422]

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Active mTOR in Lung Epithelium Promotes Epithelial-Mesenchymal Transition and Enhances Lung Fibrosis.

Minako Saito, Akihisa Mitani, Taro Ishimori, Naoya Miyashita, Hideaki Isago, Yu Mikami, Satoshi Noguchi, Megumi Tarui, Takahide Nagase,

RATIONALE:The mTOR pathway is one of the key signal cascades in the pathogenesis of idiopathic pulmonary fibrosis. Previous studies have mainly focused on this pathway in the fibroblasts and/or myofibroblasts, but not in the epithelial cells. OBJECTIVES:To investigate the role of the mTOR pathway in lung epithelial cells in lung ... Read more >>

Am. J. Respir. Cell Mol. Biol. (American journal of respiratory cell and molecular biology)
[2020, :]

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Mitochondrial 8-Oxoguanine DNA Glycosylase Mitigates Alveolar Epithelial Cell PINK1 Deficiency, Mitochondrial DNA Damage, Apoptosis and Lung Fibrosis.

Seok-Jo Kim, Paul Cheresh, Renea P Jablonski, Lyudmilla Rachek, Anjana Yeldandi, Raul Piseaux-Aillon, Mark J Ciesielski, Karen M Ridge, Cara J Gottardi, Anna P Lam, Annie Pardo, Moisés Selman, Viswanathan Natarajan, David W Kamp,

Alveolar epithelial cell (AEC) apoptosis arising from mitochondrial dysfunction and mitophagy defects are important in mediating idiopathic pulmonary fibrosis (IPF). Our group established a role for the mitochondrial (mt) DNA base excision repair enzyme, 8-oxoguanine-DNA glycosylase 1 (mtOGG1), in preventing oxidant-induced AEC mtDNA damage and apoptosis and showed that OGG1-deficient ... Read more >>

Am. J. Physiol. Lung Cell Mol. Physiol. (American journal of physiology. Lung cellular and molecular physiology)
[2020, :]

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Biomarkers and their potential functions in idiopathic pulmonary fibrosis.

Shanshan Ni, Hong Peng, Min Song, Wei Guo, Ting Guo, Qinxue Shen,

Introduction: Idiopathic pulmonary fibrosis (IPF) is a chronic, devastating, and progressive lung disease which is characterized by fibrosis and respiratory failure. IPF holds high morbidity and poor prognosis and still faces with considerable problems of reliable diagnosis and valid prognosis. A growing body of literatures have reported changes in the ... Read more >>

Expert Rev Respir Med (Expert review of respiratory medicine)
[2020, :]

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Efficacy of lower dose Pirfenidone for idiopathic pulmonary fibrosis in real practice: a retrospective cohort study

Jong Park, Hyeontaek Hwang, Jung-Kyu Lee, Sun Choi, Yeon Lee, Young-Jae Cho, Ho Yoon, Jae Lee, Choon-Taek Lee, Young Kim,

<title>Abstract</title> <p> Background Pirfenidone slows the progression of idiopathic pulmonary fibrosis. We investigated the efficacy and safety of pirfenidone by dose and disease severity in real-world patients with idiopathic pulmonary fibrosis.Methods This multi-centre retrospective cohort study investigated 338 patients treated with pirfenidone between July 2012 and March 2018. ... Read more >>

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­­Assessment of Viral RNA in Idiopathic Pulmonary Fibrosis Using RNA-seq

Joseph Lasky, Qinyan Yin, Michael Strong, Yan Zhuang, Erik Flemington, Naftali Kaminski, Joao Andrade,

<title>Abstract</title> <p> Background Numerous publications suggest an association between herpes virus infection and idiopathic pulmonary fibrosis (IPF). These reports have employed immunohistochemistry, in situ hybridization and/or PCR, which are susceptible to specificity artifacts. Methods We investigated the possible association between IPF and viral RNA expression using next-generation sequencing, ... Read more >>

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Forefront: MiR-34a-Knockout Mice with Wild Type Hematopoietic Cells, Retain Persistent Fibrosis Following Lung Injury.

Raanan Bulvik, Moshe Biton, Neville Berkman, Raphael Breuer, Shulamit B Wallach-Dayan,

MicroRNAs (miRs) are known to limit gene expression at the post-transcriptional level and have important roles in the pathogenesis of various conditions, including acute lung injury (ALI) and fibrotic diseases such as idiopathic pulmonary fibrosis (IPF). In this study, we found increased levels of miR-34 at times of fibrosis resolution ... Read more >>

Int J Mol Sci (International journal of molecular sciences)
[2020, 21(6):]

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Radiation induced apoptosis and pulmonary fibrosis: curcumin an effective intervention?

Shilpa Johnson, Sadiya B Shaikh, Fatheema Muneesa, Barki Rashmi, Yashodhar P Bhandary,

Idiopathic pulmonary fibrosis (IPF) is a fatal lung disease characterized by interstitial remodeling, leading to compromised lung function. Extra vascular fibrin deposition and abnormalities in the fibrinolysis are the major clinical manifestations of lung diseases such as acute lung injury (ALI) and its most severe form, acute respiratory distress syndrome ... Read more >>

Int. J. Radiat. Biol. (International journal of radiation biology)
[2020, :1-9]

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From an abdominal ultrasound to a lung disease passing through the diaphragm: a case of idiopathic pulmonary fibrosis.

Andrea Boccatonda, Imperia Liberatore, Giulio Cocco, Damiano D'Ardes, Stefano Marinari, Cosima Schiavone,

RATIONALE:Idiopathic pulmonary fibrosis (IPF) is a chronic lung disease characterized by abnormal and excessive deposition of collagen in the pulmonary interstitium (fibrosis) with minimal associated inflammation evolving into progressive and irreversible decline in lung function. PATIENT CONCERNS:Patient referred discomfort, bilateral upper quadrant abdominal pain, and progressive exertional dyspnea (shortness of ... Read more >>

J Ultrasound (Journal of ultrasound)
[2020, :]

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Metastasis-associated protein 1 promotes epithelial-mesenchymal transition in idiopathic pulmonary fibrosis by up-regulating Snail expression.

Weibin Qian, Xinrui Cai, Qiuhai Qian, Wei Zhang, Li Tian,

Idiopathic pulmonary fibrosis (IPF) is a progressive and usually fatal lung disease that lacking effective interventions. It is well known that aberrant activation of transforming growth factor-beta1 (TGF-β1) frequently promotes epithelial-mesenchymal transition (EMT) in IPF. Metastasis-associated gene 1 (MTA1) has identified as an oncogene in several human tumours, and aberrant ... Read more >>

J. Cell. Mol. Med. (Journal of cellular and molecular medicine)
[2020, :]

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Tolerability of nintedanib-related diarrhea in patients with idiopathic pulmonary fibrosis.

Yasutaka Hirasawa, Mitsuhiro Abe, Jiro Terada, Masashi Sakayori, Kenichi Suzuki, Keiichiro Yoshioka, Takeshi Kawasaki, Kenji Tsushima, Koichiro Tatsumi,

BACKGROUND:Nintedanib is an important drug for the treatment of idiopathic pulmonary fibrosis (IPF). However, the drug is discontinued in some patients who present with diarrhea. In this study, we aimed to assess the drug continuation rate in patients who developed diarrhea during nintedanib therapy and to evaluate if antidiarrheal drugs ... Read more >>

Pulm Pharmacol Ther (Pulmonary pharmacology & therapeutics)
[2020, :101917]

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Predictors of acute exacerbation in biopsy-proven idiopathic pulmonary fibrosis.

Tomoo Kishaba, Hironao Hozumi, Tomoyuki Fujisawa, Yuichiro Nei, Noriyuki Enomoto, Hiroaki Sugiura, Masashi Kitani, Takafumi Suda,

BACKGROUND:Acute exacerbation (AE) is a major cause of death in patients with idiopathic pulmonary fibrosis (IPF). Current evidence on AE-IPF has been largely based on clinical, rather than pathological, analyses. METHODS:We investigated AE incidence and its predictors using clinical, radiological, and pathological data of patients diagnosed with IPF by multi-disciplinary ... Read more >>

Respir Investig (Respiratory investigation)
[2020, :]

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