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https://www.readbyqxmd.com/read/28737730/sildenafil-in-infants-and-children
#1
REVIEW
Larisa Simonca, Robert Tulloh
Pulmonary arterial hypertension (PAH) management has been transformed in recent times with the advent of cheap and effective diagnostic tools and therapy. Sildenafil, a phosphodiesterase-V inhibitor, has been at the centre of this treatment, and its success in treating PAH has led to its widespread uptake in adult and paediatric pulmonary hypertension (PH), as a first line treatment choice. This might apply to persistent pulmonary hypertension of the newborn (PPHN) or bronchopulmonary dysplasia, as well as to more complex diseases, such as idiopathic pulmonary hypertension...
July 24, 2017: Children
https://www.readbyqxmd.com/read/28734935/outcome-of-lung-transplantation-in-idiopathic-pulmonary-fibrosis-with-previous-anti-fibrotic-therapy
#2
Gabriela Leuschner, Florian Stocker, Tobias Veit, Nikolaus Kneidinger, Hauke Winter, René Schramm, Thomas Weig, Sandhya Matthes, Felix Ceelen, Paola Arnold, Dieter Munker, Friederike Klenner, Rudolf Hatz, Marion Frankenberger, Jürgen Behr, Claus Neurohr
BACKGROUND: Anti-fibrotic drugs may interfere with wound-healing after major surgery, theoretically preventing sufficient bronchial anastomosis formation after lung transplantation (LTx). The aim of this study was to assess the impact of previous treatment with pirfenidone and nintedanib on outcomes after LTx in patients with idiopathic pulmonary fibrosis (IPF). METHODS: All patients with IPF undergoing LTx at the University of Munich between January 2012 and November 2016 were retrospectively screened for previous use of anti-fibrotics...
July 5, 2017: Journal of Heart and Lung Transplantation
https://www.readbyqxmd.com/read/28733124/left-upper-lung-lobectomy-is-an-embolic-risk-factor-for-cerebral-infarction
#3
Yuya Kobayashi, Hiroyuki Yahikozawa, Ryota Takamatsu, Rie Watanabe, Kenichi Hoshi, Wataru Ishii, Shunichi Sato
Cerebral embolism is typically caused by a cardiogenic thrombus. The patent foramen ovale is a well-known cause of paradoxical embolism. However, some idiopathic cases of stroke have been reported. Such strokes are designated as embolic stroke of undetermined sources. Among them, lung lobectomy may be a new embolic risk factor for cerebral embolism. The risk of thrombus formation is high at the pulmonary vein stump after lung lobectomy, especially in the left upper lobe. Interestingly, the risk remains several years after surgery...
July 19, 2017: Journal of Stroke and Cerebrovascular Diseases: the Official Journal of National Stroke Association
https://www.readbyqxmd.com/read/28732835/idiopathic-pulmonary-fibrosis-clinical-behavior-and-aging-associated-comorbidities
#4
REVIEW
Ivette Buendía-Roldán, Mayra Mejía, Carmen Navarro, Moisés Selman
Idiopathic pulmonary fibrosis (IPF) is a progressive, irreversible and usually lethal lung disease of unknown etiology. Once considered as a relatively homogeneous, slowly progressive disease, is now recognized that the clinical behavior shows substantial heterogeneity, including an accelerated variant, and the presence of acute exacerbations. In addition, since IPF largely affects individuals over 60 years of age, the patients are at increased risk of several comorbidities that in turn have a remarkable clinical impact on the disease and increases mortality rate...
August 2017: Respiratory Medicine
https://www.readbyqxmd.com/read/28732832/current-approaches-to-the-management-of-idiopathic-pulmonary-fibrosis
#5
REVIEW
Ganesh Raghu, Luca Richeldi
Idiopathic pulmonary fibrosis (IPF) is a progressive and ultimately fatal lung disease associated with dyspnoea, cough and impaired quality of life. Currently, the aims of patient care are to improve outcomes for patients by slowing the progression of the disease, extending life, and improving quality of life. A prompt, accurate diagnosis is important to enable patients to receive treatment early in the course of the disease and to be considered for lung transplantation. Two anti-fibrotic drugs, nintedanib and pirfenidone, have been shown to reduce decline in lung function in patients with IPF...
August 2017: Respiratory Medicine
https://www.readbyqxmd.com/read/28732823/correlation-of-pulmonary-function-and-usual-interstitial-pneumonia-computed-tomography-patterns-in-idiopathic-pulmonary-fibrosis
#6
Antonella Arcadu, Suzanne C Byrne, Pietro Pirina, Thomas E Hartman, Brian J Bartholmai, Teng Moua
BACKGROUND: Little is known about presenting 'inconsistent' or 'possible' usual interstitial pneumonia (UIP) computed tomography (CT) patterns advancing to 'consistent' UIP as disease progresses in idiopathic pulmonary fibrosis (IPF). We hypothesized that if 'consistent' UIP represented more advanced disease, such a pattern on presentation should also correlate with more severe pulmonary function test (PFT) abnormalities. MATERIAL AND METHODS: Consecutive IPF patients (2005-2013) diagnosed by international criteria with baseline PFT and CT were included...
August 2017: Respiratory Medicine
https://www.readbyqxmd.com/read/28731277/tnf-%C3%AE-induced-nf-%C3%AE%C2%BAb-activation-promotes-myofibroblast-differentiation-of-lr-mscs-and-exacerbates-bleomycin-induced-pulmonary-fibrosis
#7
Jiwei Hou, Tan Ma, Honghui Cao, Yabing Chen, Cong Wang, Xiang Chen, Zou Xiang, Xiaodong Han
Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive, and irreversible lung disease of unknown cause. It has been reported that both lung resident mesenchymal stem cells (LR-MSCs) and tumor necrosis factor-α (TNF-α) play important roles in the development of pulmonary fibrosis. However, the underlying connections between LR-MSCs and TNF-α in the pathogenesis of pulmonary fibrosis are still elusive. In this study, we found that the pro-inflammatory cytokine TNF-α and the transcription factor nuclear factor kappa B (NF-κB) p65 subunit were both upregulated in bleomycin-induced fibrotic lung tissue...
July 21, 2017: Journal of Cellular Physiology
https://www.readbyqxmd.com/read/28729480/home-spirometry-for-idiopathic-pulmonary-fibrosis-ready-for-prime-time
#8
EDITORIAL
Toby M Maher
No abstract text is available yet for this article.
July 2017: European Respiratory Journal: Official Journal of the European Society for Clinical Respiratory Physiology
https://www.readbyqxmd.com/read/28728146/chronic-kidney-disease-predicts-survival-in-patients-with-idiopathic-pulmonary-fibrosis
#9
Kohei Ikezoe, Tomohiro Handa, Kiminobu Tanizawa, Hideki Yokoi, Takeshi Kubo, Kensaku Aihara, Akihiko Sokai, Yoshinari Nakatsuka, Seishu Hashimoto, Kiyoshi Uemasu, Susumu Sato, Shigeo Muro, Sonoko Nagai, Motoko Yanagita, Kazuo Chin, Toyohiro Hirai, Yoshio Taguchi, Michiaki Mishima
BACKGROUND: The prevalence of chronic kidney disease (CKD) increases with age as with idiopathic pulmonary fibrosis (IPF). OBJECTIVES: We assessed the prevalence of CKD (stages 3-5) and investigated the relationship of CKD to clinical features and outcomes in patients with IPF. METHODS: This study comprised 123 patients with IPF; 61 subjects with chronic obstructive pulmonary disease (COPD), which was reportedly associated with CKD, were also enrolled as a disease control...
July 21, 2017: Respiration; International Review of Thoracic Diseases
https://www.readbyqxmd.com/read/28726637/microrna-101-attenuates-pulmonary-fibrosis-by-inhibiting-fibroblast-proliferation-and-activation
#10
Chaoqun Huang, Xiao Xiao, Ye Yang, Amarjit Mishra, Yurong Liang, Xiangming Zeng, Xiaoyun Yang, Dao Xu, Michael R Blackburn, Craig A Henke, Lin Liu
Aberrant proliferation and activation of lung fibroblasts contribute to the initiation and progression of idiopathic pulmonary fibrosis (IPF). However, the mechanisms responsible for the proliferation and activation of fibroblasts are not fully understood. The objective of the current study was to investigate the role of miR-101 in the proliferation and activation of lung fibroblasts. miR-101 expression was determined in lung tissues from patients with IPF and mice with bleomycin-induced pulmonary fibrosis...
July 18, 2017: Journal of Biological Chemistry
https://www.readbyqxmd.com/read/28722352/serpine-1-induces-alveolar-type-ii-cell-senescence-through-activating-p53-p21-rb-pathway-in-fibrotic-lung-disease
#11
Chunsun Jiang, Gang Liu, Tracy Luckhardt, Veena Antony, Yong Zhou, A Brent Carter, Victor J Thannickal, Rui-Ming Liu
Senescence of alveolar type 2 (ATII) cells, progenitors of the alveolar epithelium, is implicated in the pathogeneses of idiopathic pulmonary fibrosis (IPF), an aging-related progressive fatal lung disorder with unknown etiology. The mechanism underlying ATII cell senescence in fibrotic lung diseases, however, remains poorly understood. In this study, we report that ATII cells in IPF lungs express higher levels of serpine 1, also known as plasminogen activator inhibitor 1 (PAI-1), and cell senescence markers p21 and p16, compared to ATII cells in control lungs...
July 19, 2017: Aging Cell
https://www.readbyqxmd.com/read/28719560/perioperative-and-delayed-major-complications-following-surgical-treatment-of-adolescent-idiopathic-scoliosis
#12
Carrie E Bartley, Burt Yaszay, Tracey P Bastrom, Suken A Shah, Baron S Lonner, Jahangir Asghar, Firoz Miyanji, Amer Samdani, Peter O Newton
BACKGROUND: Reporting accurate surgical complication rates to patients and their families is important in the management of adolescent idiopathic scoliosis (AIS). In this study, we report the rate of major complications following the surgical treatment of AIS both in the perioperative period and among patients with a minimum of 2 years of follow-up. METHODS: We reviewed the prospectively collected data of a multicenter registry of patients who underwent surgical treatment of AIS during the period of 1995 to 2014 in order to identify all complications...
July 19, 2017: Journal of Bone and Joint Surgery. American Volume
https://www.readbyqxmd.com/read/28718917/proliferation-of-elastic-fibers-in-idiopathic-pulmonary-fibrosis-a-whole-slide-image-analysis-and-comparison-with-pleuroparenchymal-fibroelastosis
#13
Yoshiaki Kinoshita, Kentaro Watanabe, Hiroshi Ishii, Hisako Kushima, Masaki Fujita, Kazuki Nabeshima
BACKGROUND: We occasionally encounter patients with idiopathic pulmonary fibrosis (IPF) who have similar imaging patterns to pleuroparenchymal fibroelastosis (PPFE) in the upper lung fields but are not diagnosed as PPFE clinically. The clinicopathological features and intrapulmonary distribution of elastic fibers and collagen fibers in these patients have not been fully elucidated. METHODS: We retrospectively reviewed the medical records of patients with a clinical diagnosis of IPF and selected the consecutive patients who received autopsy or pneumonectomy for lung transplantation...
July 18, 2017: Histopathology
https://www.readbyqxmd.com/read/28717640/lung-microbiome-and-host-immune-tone-in-subjects-with-idiopathic-pulmonary-fibrosis-treated-with-inhaled-interferon-%C3%AE
#14
Jing Wang, Melissa Lesko, Michelle H Badri, Bianca C Kapoor, Benjamin G Wu, Yonghua Li, Gerald C Smaldone, Richard Bonneau, Zachary D Kurtz, Rany Condos, Leopoldo N Segal
Therapies targeting inflammation reveal inconsistent results in idiopathic pulmonary fibrosis (IPF). Aerosolised interferon (IFN)-γ has been proposed as a novel therapy. Changes in the host airway microbiome are associated with the inflammatory milieu and may be associated with disease progression. Here, we evaluate whether treatment with aerosolised IFN-γ in IPF impacts either the lower airway microbiome or the host immune phenotype. Patients with IPF who enrolled in an aerosolised IFN-γ trial underwent bronchoscopy at baseline and after 6 months...
July 2017: ERJ Open Research
https://www.readbyqxmd.com/read/28717083/pirfenidone-induced-eosinophilic-pleurisy
#15
Isano Hase, Bunpei Yamaguchi, Hidenori Takizawa, Hiroaki Arakawa, Hideo Sakuma, Koichi Fujiu, Hideaki Miyamoto, Yoshiki Ishii
The patient was a 69-year-old man with idiopathic pulmonary fibrosis who was taking pirfenidone. After 7 weeks of treatment, he suffered from left-sided eosinophilic pleurisy. Medical thoracoscopy was performed and the histopathological examination of the parietal pleura revealed the massive infiltration of eosinophils and lymphoid follicles. After stopping pirfenidone therapy, the patient's pleural effusion disappeared without additional treatment, and never recurred. This is the first case report of pirfenidone-induced pleurisy...
2017: Internal Medicine
https://www.readbyqxmd.com/read/28714804/cellular-proliferation-and-differentiation-induced-by-single-layer-molybdenum-disulfide-and-mediation-mechanisms-of-proteins-via-the-akt-mtor-p70s6k-signaling-pathway
#16
Wei Zou, Xingli Zhang, Mengyang Zhao, Qixing Zhou, Xiangang Hu
Single-layer molybdenum disulfide (SLMoS2) is a novel kind of 2D nanosheet that has attracted great attention regarding its use in biosensors, drug delivery, tissue engineering and therapy. However, our results demonstrated that SLMoS2 accelerated proliferation and promoted myogenic differentiation and epithelial-mesenchymal transition (EMT) in human embryonic lung fibroblasts (HELFs). The abnormal proliferation and differentiation of HELFs contribute to idiopathic pulmonary fibrosis. Specifically, SLMoS2 significantly stimulated the expression of myofibroblast- and mesenchymal-associated genes and proteins...
July 17, 2017: Nanotoxicology
https://www.readbyqxmd.com/read/28710188/leucocytoclastic-vasculitis-following-influenza-vaccination
#17
Sissi Cao, Dongmei Sun
BACKGROUND: Influenza vaccinations are commonly administered and effectively prevent influenza. Most vaccine reactions are mild and self-limiting with few reported cases of vasculitis. We present a case of vasculitis post-vaccination unique for its dramatic visual presentation and rapid response to treatment with the aim to emphasise possible causation and timely treatment. CASE PRESENTATION: This is a case of a 60-year-old man with chronic idiopathic pulmonary fibrosis, with fever, arthralgias and rash characterised by extensive erythematous plaques on his limbs and trunk 5 days after influenza vaccination...
July 14, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28709421/health-related-quality-of-life-in-patients-with-idiopathic-pulmonary-fibrosis-in-clinical-practice-insights-ipf-registry
#18
Michael Kreuter, Jeff Swigris, David Pittrow, Silke Geier, Jens Klotsche, Antje Prasse, Hubert Wirtz, Dirk Koschel, Stefan Andreas, Martin Claussen, Christian Grohé, Henrike Wilkens, Lars Hagmeyer, Dirk Skowasch, Joachim F Meyer, Joachim Kirschner, Sven Gläser, Felix J F Herth, Tobias Welte, Claus Neurohr, Martin Schwaiblmair, Matthias Held, Thomas Bahmer, Marion Frankenberger, Jürgen Behr
BACKGROUND: The INSIGHTS-IPF registry provides one of the largest data sets of clinical data and self-reported patient related outcomes including health related quality of life (QoL) on patients with idiopathic pulmonary fibrosis (IPF). We aimed to describe associations of various QoL instruments between each other and with patient characteristics at baseline. METHODS: Six hundred twenty-three IPF patients with available QoL data (St George's Respiratory Questionnaire SGRQ, UCSD Shortness-of-Breath Questionnaire SoB, EuroQol visual analogue scale and index EQ-5D, Well-being Index WHO-5) were analysed...
July 14, 2017: Respiratory Research
https://www.readbyqxmd.com/read/28709049/idiopathic-spontaneous-pulmonary-torsion-of-the-lingula-a-case-report
#19
Masatoshi Kanayama, Toshihiro Osaki, Natsumasa Nishizawa, Makoto Nakagawa, Tomoko So, Mantaro Kodate
INTRODUCTION: Spontaneous pulmonary torsion is an extremely rare event and is known to occur as a complication of thoracic surgery and traumatic injuries. PRESENTATION OF CASE: An 18-year-old man presented to our hospital with pain in the left back region. Clinical examination, computed tomography and bronchoscopy are crucial for diagnosis of pulmonary torsion. During thoracotomy, the lingula segment was observed to be bent on the head side and turned 180° counterclockwise; subsequently, lingulectomy was performed...
June 29, 2017: International Journal of Surgery Case Reports
https://www.readbyqxmd.com/read/28707969/ipf-moving-from-idiopathic-to-infectious-pulmonary-fibrosis
#20
Gisli Jenkins
No abstract text is available yet for this article.
July 15, 2017: American Journal of Respiratory and Critical Care Medicine
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