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Pirfenidone

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https://www.readbyqxmd.com/read/28317233/pirfenidone-and-nintedanib-for-pulmonary-fibrosis-in-clinical-practice-tolerability-and-adverse-drug-reactions
#1
Jonathan A Galli, Aloknath Pandya, Michelle Vega-Olivo, Chandra Dass, Huaqing Zhao, Gerard J Criner
BACKGROUND AND OBJECTIVE: The real-world tolerability of pirfenidone and nintedanib in non-clinical trial patients is unknown. Many patients with pulmonary fibrosis have significant medical co-morbidities or baseline characteristics that exclude them from clinical trial participation. METHODS: We conducted a retrospective chart review study on subjects prescribed nintedanib or pirfenidone for pulmonary fibrosis treatment (any aetiology) from September 2014 to February 2016...
March 20, 2017: Respirology: Official Journal of the Asian Pacific Society of Respirology
https://www.readbyqxmd.com/read/28304388/pirfenidone-inhibits-post-traumatic-proliferative-vitreoretinopathy
#2
B N M K Khanum, R Guha, V P Sur, S Nandi, S K Basak, A Konar, S Hazra
PurposeThe purpose of the study was to evaluate the efficacy and safety of intravitreal pirfenidone for inhibition of proliferative vitreoretinopathy (PVR) in a model of penetrating ocular injury.Patients and methodsPenetrating trauma was induced on the retina of rabbit and treated either with 0.1 ml of phosphate-buffered saline (PBS) or 0.1 ml of 0.5% pirfenidone, and development of PVR was evaluated clinically and graded after 1 month. Histopathology and immunohistochemistry with transforming growth factor beta (TGFβ), alpha smooth muscle actin (αSMA), and collagen-1 were performed to assess the fibrotic changes...
March 17, 2017: Eye
https://www.readbyqxmd.com/read/28303974/antifibrotic-effect-of-pirfenidone-in-a-mouse-model-of-human-nonalcoholic-steatohepatitis
#3
Chikara Komiya, Miyako Tanaka, Kyoichiro Tsuchiya, Noriko Shimazu, Kentaro Mori, Shunsaku Furuke, Yasutaka Miyachi, Kumiko Shiba, Shinobu Yamaguchi, Kenji Ikeda, Kozue Ochi, Kazuhiko Nakabayashi, Ken-Ichiro Hata, Michiko Itoh, Takayoshi Suganami, Yoshihiro Ogawa
Non-alcoholic steatohepatitis (NASH) is characterized by steatosis with lobular inflammation and hepatocyte injury. Pirfenidone (PFD) is an orally bioavailable pyridone derivative that has been clinically used for the treatment of idiopathic pulmonary fibrosis. However, it remains unknown whether PFD improves liver fibrosis in a mouse model with human NASH-like phenotypes. In this study, we employed melanocortin 4 receptor-deficient (MC4R-KO) mice as a mouse model with human NASH-like phenotypes to elucidate the effect and action mechanisms of PFD on the development of NASH...
March 17, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28292522/guidelines-for-the-medical-treatment-of-idiopathic-pulmonary-fibrosis
#4
Antoni Xaubet, María Molina-Molina, Orlando Acosta, Elena Bollo, Diego Castillo, Estrella Fernández-Fabrellas, José Antonio Rodríguez-Portal, Claudia Valenzuela, Julio Ancochea
Idiopathic pulmonary fibrosis is defined as chronic fibrosing interstitial pneumonia limited to the lung, with poor prognosis. The incidence has been rising in recent years probably due to improved diagnostic methods and increased life expectancy. In 2013, the SEPAR guidelines for the diagnosis and treatment for idiopathic pulmonary fibrosis were published. Since then, clinical trials and meta-analyses have shown strong scientific evidence for the use of pirfenidone and nintedanib in the treatment of idiopathic pulmonary fibrosis...
March 11, 2017: Archivos de Bronconeumología
https://www.readbyqxmd.com/read/28287347/predicting-life-expectancy-for-pirfenidone-in-idiopathic-pulmonary-fibrosis
#5
Mark Fisher, Steven D Nathan, Christian Hill, Jade Marshall, Fred Dejonckheere, Per-Olof Thuresson, Toby M Maher
BACKGROUND: Conducting an adequately powered survival study in idiopathic pulmonary fibrosis (IPF) is challenging due to the rare nature of the disease and the need for extended follow-up. Consequently, registration trials of IPF treatments have not been designed to estimate long-term survival. OBJECTIVE: To predict life expectancy for patients with IPF receiving pirfenidone versus best supportive care (BSC) in a population that met the inclusion criteria of patients enrolled in the ASCEND and CAPACITY trials...
March 2017: Journal of Managed Care & Specialty Pharmacy
https://www.readbyqxmd.com/read/28287346/systematic-review-and-network-meta-analysis-of-idiopathic-pulmonary-fibrosis-treatments
#6
Kelly Fleetwood, Rachael McCool, Julie Glanville, Susan C Edwards, Sandro Gsteiger, Monica Daigl, Mark Fisher
BACKGROUND: The antifibrotics pirfenidone and nintedanib are both approved for the treatment of idiopathic pulmonary fibrosis (IPF) by regulatory agencies and are recommended by health technology assessment bodies. Other treatments such as N-acetylcysteine are used in clinical practice but have not received regulatory approval. No head-to-head trials have been conducted to directly compare the efficacy of these therapies in IPF. OBJECTIVE: To compare the efficacy of treatments for IPF...
March 2017: Journal of Managed Care & Specialty Pharmacy
https://www.readbyqxmd.com/read/28285699/pirfenidone-plays-a-biphasic-role-in-inhibition-of-epithelial-mesenchymal-transition-in-non-small-cell-lung-cancer
#7
Ayako Fujiwara, Yasushi Shintani, Soichiro Funaki, Tomohiro Kawamura, Toru Kimura, Masato Minami, Meinoshin Okumura
INTRODUCTION: Epithelial to mesenchymal transition (EMT) relates to both organ fibrosis and malignant behavior of cancer. Pirfenidone (PFD) is an anti-fibrotic agent for idiopathic pulmonary fibrosis and one of its functions may be to inhibit fibrotic EMT. This study aimed to investigate the possibility that PFD might exert an anti-tumor effect through inhibition of EMT in non-small cell lung cancer (NSCLC) cell lines in vitro and in vivo. METHODS: NSCLC cells (A549, NCI-H358) were used to evaluate PFD effects on TGF-β1 induced phenotypic changes...
April 2017: Lung Cancer: Journal of the International Association for the Study of Lung Cancer
https://www.readbyqxmd.com/read/28284377/treatment-of-renal-fibrosis-turning-challenges-into-opportunities
#8
REVIEW
Barbara M Klinkhammer, Roel Goldschmeding, Jürgen Floege, Peter Boor
Current treatment modalities are not effective in halting the progression of most CKD. Renal fibrosis is a pathological process common to all CKD and thereby represents an excellent treatment target. A large number of molecular pathways involved in renal fibrosis were identified in preclinical studies, some of them being similar among different organs and some with available drugs in various phases of clinical testing. Yet only few clinical trials with antifibrotic drugs are being conducted in CKD patients...
March 2017: Advances in Chronic Kidney Disease
https://www.readbyqxmd.com/read/28268982/acoustic-evaluation-of-pirfenidone-on-patients-with-combined-pulmonary-fibrosis-emphysema-syndrome
#9
Sonia Charleston-Villalobos, Norma Castaneda-Villa, Ramon Gonzalez-Camarena, M Mejia-Avila, H Mateos-Toledo, Tomas Aljama-Corrales
The combined pulmonary fibrosis emphysema syndrome (CPFES) overall has a poor prognosis with a 5-year survival of 35-80%. Consequently, to evaluate possible positive effects on patients of novel agents as pirfenidone is relevant. However, the efficacy of pirfenidone in CPFES patients is still not well-known. In this study we propose an alternative to evaluate the effects of pirfenidone treatment on CPFES patients via acoustic information. Quantitative analysis of discontinuous adventitious lung sounds (DLS), known as crackles, has been promising to detect and characterize diverse pulmonary pathologies...
August 2016: Conference Proceedings: Annual International Conference of the IEEE Engineering in Medicine and Biology Society
https://www.readbyqxmd.com/read/28266906/pirfenidone-for-the-treatment-of-idiopathic-pulmonary-fibrosis
#10
Peter M George, Athol U Wells
Idiopathic pulmonary fibrosis (IPF) is a diffuse parenchymal lung disease with no cure. Up until recently, no treatment had been proven to alter its natural history as judged by rate of lung function decline. In 2014 however, the emergence of two novel anti-fibrotic agents, Pirfenidone and Nintedanib revolutionized the management of this condition. Both have demonstrated the ability to deliver a major reduction in the rate of chronic IPF progression. Areas Covered: This review article focuses on Pirfenidone - a pyridone derivative initially designed as an analgesic and anti-pyretic agent...
March 7, 2017: Expert Review of Clinical Pharmacology
https://www.readbyqxmd.com/read/28261880/pirfenidone-combined-with-corticosteroids-in-a-patient-with-systemic-lupus-erythematosus-associated-interstitial-lung-disease
#11
Bei-Bei Yang, Min Zheng, Xiao-Yong Man
A 54-year-old woman (weight 45 kg) presented to our clinic complaining of a dry cough and exertional dyspnea for 1 week. She had been diagnosed with SLE and lupus nephritis at age 52, with manifestations including oral ulcers, lipsotrichia, butterfly erythema, high anti-nuclear antibody titers (with a speckled pattern at a 1:1,000 dilution), positive anti-double stranded DNA, hypocomplementemia C3, and a renal biopsy that indicated type III(A) lupus nephritis. The patient was a non-smoker. This article is protected by copyright...
March 6, 2017: Journal of the European Academy of Dermatology and Venereology: JEADV
https://www.readbyqxmd.com/read/28257817/the-earlier-the-better-impact-of-early-diagnosis-on-clinical-outcome-in-idiopathic-pulmonary-fibrosis
#12
REVIEW
Marina Aiello, Giuseppina Bertorelli, Marialuisa Bocchino, Alfredo Chetta, Alfeo Fiore-Donati, Alessandro Fois, Stefano Marinari, Tiberio Oggionni, Biagio Polla, Elisabetta Rosi, Anna Stanziola, Francesco Varone, Alessandro Sanduzzi
BACKGROUND: Idiopathic pulmonary fibrosis (IPF) is a complex disease with a highly variable clinical course and generally poor prognosis. Classified as a rare disease, significant increases in incidence have been recorded worldwide in recent years. Left untreated IPF is extremely debilitating with substantial personal, social and economic implications. OBJECTIVES: To discuss how IPF is diagnosed and managed in real life clinical practice with particular reference to Italy and to determine how new and effective therapies can be incorporated into a patient-centred management approach in order to improve the lives of patients with IPF...
February 28, 2017: Pulmonary Pharmacology & Therapeutics
https://www.readbyqxmd.com/read/28257580/a-novel-antifibrotic-mechanism-of-nintedanib-and-pirfenidone-inhibition-of-collagen-fibril-assembly
#13
Larissa Knüppel, Yoshihiro Ishikawa, Michaela Aichler, Katharina Heinzelmann, Rudolf Hatz, Jürgen Behr, Axel Walch, Hans Peter Bächinger, Oliver Eickelberg, Claudia A Staab-Weijnitz
RATIONALE: Idiopathic pulmonary fibrosis (IPF) is characterized by excessive deposition of extracellular matrix, in particular collagens. Two IPF therapeutics, nintedanib and pirfenidone, decelerate lung function decline, but their underlying mechanisms of action are poorly understood. In this study we sought to analyze their effects on collagen synthesis and maturation at important regulatory levels. METHODS: Primary human fibroblasts from IPF patients and healthy donors were treated with nintedanib (0...
March 3, 2017: American Journal of Respiratory Cell and Molecular Biology
https://www.readbyqxmd.com/read/28255112/-salidroside-improves-pulmonary-fibrosis-by-down-regulation-of-cathepsin-b-and-nf-%C3%AE%C2%BAbp65-in-rats
#14
Xingren Liu, Yifeng Bai, Liang Liang, Jing Feng, Fei Deng
To investigate the mechanism of salidroside in improvement of pulmonary fibrosis in rats.
 Methods: SD rats were subjected to 6 groups: a blank group, a model group, a pirfenidone group, a high-dose salidroside group, a middle-dose salidroside group, and a low-dose salidroside group. The contents of ALB, ALP, LDH, PC-III and COL4, and the expression levels of cathepsin B (CB) and NF-κBp65 in the 6 groups were analyzed.
 Results: Lung coefficient and oxygen partial pressure in the blank group were lower than those in the model group (P<0...
February 28, 2017: Zhong Nan da Xue Xue Bao. Yi Xue Ban, Journal of Central South University. Medical Sciences
https://www.readbyqxmd.com/read/28254742/pirfenidone-ameliorates-murine-chronic-gvhd-through-inhibition-of-macrophage-infiltration-and-tgf-%C3%AE-production
#15
Jing Du, Katelyn Paz, Ryan Flynn, Ante Vulic, Tara M Robinson, Katie E Lineburg, Kylie A Alexander, Jingjing Meng, Sabita Roy, Angela Panoskaltsis-Mortari, Michael Loschi, Geoffrey R Hill, Jonathan S Serody, Ivan Maillard, David Miklos, John Koreth, Corey S Cutler, Joseph H Antin, Jerome Ritz, Kelli P MacDonald, Timothy W Schacker, Leo Luznik, Bruce R Blazar
Allogeneic hematopoietic stem cell transplantation is hampered by chronic graft-versus-host disease (cGVHD) resulting in multi-organ fibrosis and diminished function. Fibrosis in lung and skin leads to progressive bronchiolitis obliterans (BO) and scleroderma, respectively, for which new treatments are needed. We evaluated pirfenidone, a FDA approved drug for idiopathic pulmonary fibrosis, for its therapeutic effect in cGVHD mouse models with distinct pathophysiology. In a full MHC-mismatched, multi-organ system model with BO, donor T cell responses that support pathogenic antibody production are required for cGVHD development...
March 2, 2017: Blood
https://www.readbyqxmd.com/read/28251374/perioperative-management-of-hepatectomy-in-patients-with-interstitial-pneumonia-a-report-of-three-cases-and-a-literature-review
#16
REVIEW
Norio Kubo, Kenichiro Araki, Takahiro Yamanaka, Kouki Hoshino, Norihiro Ishii, Mariko Tsukagoshi, Takamichi Igarashi, Akira Watanabe, Keitaro Hirai, Fumiyoshi Saitoh, Hiroyuki Kuwano, Ken Shirabe
PURPOSE: Interstitial pneumonia (IP) is a progressive and irreversible fibrosis and can be fatal if acute exacerbation (AE) occurs. While a useful risk-scoring system has been established for lung surgery, no risk evaluation exists for AE of IP related to non-pulmonary surgery. The objective of this review is to describe the management for patients with IP. METHODS: We experienced three hepatectomy cases with IP. The first was a 72-year-old male patient diagnosed with hepatocellular carcinoma...
March 1, 2017: Surgery Today
https://www.readbyqxmd.com/read/28231275/suppression-of-tgf-%C3%AE-pathway-by-pirfenidone-decreases-extracellular-matrix-deposition-in-ocular-fibroblasts-in-vitro
#17
Thomas Stahnke, Bhavani S Kowtharapu, Oliver Stachs, Klaus-Peter Schmitz, Johannes Wurm, Andreas Wree, Rudolf Friedrich Guthoff, Marina Hovakimyan
In glaucoma surgery, fibrotic processes occur, leading to impairment of liquid outflow. Activated fibroblasts are responsible for postoperative scarring. The transforming growth factor-β (TGF-β) pathway plays a key role in fibroblast function, differentiation and proliferation. The aim of this study was the characterization of the fibrotic potential of two subtypes of primary human ocular fibroblasts and the attempt to inhibit fibrotic processes specifically, without impairing cell viability. For fibrosis inhibition we focused on the small molecule pirfenidone, which has been shown to prevent pulmonary fibrosis by the decrease of the expression of TGF-β1, TGF-β2 and TGF-β3 cytokines...
2017: PloS One
https://www.readbyqxmd.com/read/28227192/acoustic-evaluation-of-pirfenidone-on-patients-with-combined-pulmonary-fibrosis-emphysema-syndrome
#18
Sonia Charleston-Villalobos, Norma Castaneda-Villa, Ramon Gonzalez-Camarena, M Mejia-Avila, H Mateos-Toledo, Tomas Aljama-Corrales, Sonia Charleston-Villalobos, Norma Castaneda-Villa, Ramon Gonzalez-Camarena, M Mejia-Avila, H Mateos-Toledo, Tomas Aljama-Corrales, Tomas Aljama-Corrales, Ramon Gonzalez-Camarena, Sonia Charleston-Villalobos, Norma Castaneda-Villa, M Mejia-Avila, H Mateos-Toledo
The combined pulmonary fibrosis emphysema syndrome (CPFES) overall has a poor prognosis with a 5-year survival of 35-80%. Consequently, to evaluate possible positive effects on patients of novel agents as pirfenidone is relevant. However, the efficacy of pirfenidone in CPFES patients is still not well-known. In this study we propose an alternative to evaluate the effects of pirfenidone treatment on CPFES patients via acoustic information. Quantitative analysis of discontinuous adventitious lung sounds (DLS), known as crackles, has been promising to detect and characterize diverse pulmonary pathologies...
August 2016: Conference Proceedings: Annual International Conference of the IEEE Engineering in Medicine and Biology Society
https://www.readbyqxmd.com/read/28148565/pirfenidone-exerts-antifibrotic-effects-through-inhibition-of-gli-transcription-factors
#19
Miroslava Didiasova, Rajeev Singh, Jochen Wilhelm, Grazyna Kwapiszewska, Lukasz Wujak, Dariusz Zakrzewicz, Liliana Schaefer, Philipp Markart, Werner Seeger, Matthias Lauth, Malgorzata Wygrecka
Pirfenidone is an antifibrotic drug, recently approved for the treatment of patients suffering from idiopathic pulmonary fibrosis (IPF). Although pirfenidone exhibits anti-inflammatory, antioxidant, and antifibrotic properties, the molecular mechanism underlying its protective effects remains unknown. Here, we link pirfenidone action with the regulation of the profibrotic hedgehog (Hh) signaling pathway. We demonstrate that pirfenidone selectively destabilizes the glioma-associated oncogene homolog (GLI)2 protein, the primary activator of Hh-mediated gene transcription...
February 1, 2017: FASEB Journal: Official Publication of the Federation of American Societies for Experimental Biology
https://www.readbyqxmd.com/read/28144054/current-trends-of-management-of-respiratory-diseases-by-pulmonologists-results-of-national-conference-of-pulmonary-disease-2015-survey
#20
Sheetu Singh, Nishtha Singh
CONTEXT: Respiratory diseases are a common problem in our country and these are associated with significant morbidity and mortality. AIMS: The aim of the paper was to analyze the pattern of diagnostic tests used and treatment prescribed for common respiratory diseases. SETTINGS AND DESIGN: A total of 1028 pulmonologists, either member of Indian Chest Society or delegate attending the National Conference of Pulmonary Diseases (NAPCON) 2015, participated in the online survey...
January 2017: Lung India: Official Organ of Indian Chest Society
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