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Idiopathic pulmonary disease

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https://www.readbyqxmd.com/read/28936704/extraction-of-the-subpleural-lung-region-from-computed-tomography-images-to-detect-interstitial-lung-disease
#1
Tae Iwasawa, Yuma Iwao, Tamiko Takemura, Koji Okudela, Toshiyuki Gotoh, Tomohisa Baba, Takashi Ogura, Mari S Oba
PURPOSE: To quantify lesions in the subpleural lung region (SubPL) on computed tomography (CT) images and to evaluate whether they are useful for detecting interstitial lung disease (ILD). MATERIALS AND METHODS: The subjects were 40 patients with idiopathic pulmonary fibrosis (IPF) diagnosed by multidisciplinary methods and 35 age-matched patients without ILDs. The lungs and SubPL were extracted from CT images using a Gaussian histogram normalized correlation system and evaluated for the mean CT attenuation value (CTmean) and the percentage of high attenuation area (%HAA), exceeding -700 Hounsfield units...
September 21, 2017: Japanese Journal of Radiology
https://www.readbyqxmd.com/read/28935667/upregulation-of-herv-k-is-linked-to-immunity-and-inflammation-in-pulmonary-arterial-hypertension
#2
Toshie Saito, Kazuya Miyagawa, Shih-Yu Chen, Rasa Tamosiuniene, Lingli Wang, Orr Sharp, Erik Samayoa, Daisuke Harada, Jan-Renier A J Moonen, Aiqin Cao, Pin-I Chen, Jan K Hennigs, Mingxia Gu, Caiyun G Li, Ryan D Leib, Dan Li, Christopher M Adams, Patricia A Del Rosario, Matthew A Bill, Francois Haddad, Jose G Montoya, William Robinson, Wendy J Fantl, Garry P Nolan, Roham T Zamanian, Mark R Nicolls, Charles Y Chiu, Maria E Ariza, Marlene Rabinovitch
Background -Immune dysregulation has been linked to occlusive vascular remodeling in pulmonary arterial hypertension (PAH) that is hereditary, idiopathic or associated with other conditions. Circulating autoantibodies, lung perivascular lymphoid tissue and elevated cytokines have been related to PAH pathogenesis but without clear understanding of how these abnormalities are initiated, perpetuated and connected in the progression of disease. We therefore set out to identify specific target antigens in PAH lung immune complexes as a starting point toward resolving these issues to better inform future application of immunomodulatory therapies...
September 21, 2017: Circulation
https://www.readbyqxmd.com/read/28934596/features-and-outcomes-of-methamphetamine-associated-pulmonary-arterial-hypertension
#3
Roham T Zamanian, Haley Hedlin, Paul Greuenwald, David M Wilson, Joshua I Segal, Michelle Jorden, Kristina Kudelko, Juliana Liu, Andrew Hsi, Allyson Rupp, Andrew J Sweatt, Rubin Tuder, Gerald J Berry, Marlene Rabinovitch, Ramona L Doyle, Vinicio De Jesus Perez, Steven M Kawut
RATIONALE: While amphetamines are recognized as "likely" agents to cause drugs and toxins associated pulmonary arterial hypertension (PAH), (meth)amphetamine associated PAH (Meth-APAH) has not been well described. OBJECTIVES: To prospectively characterize the clinical presentation, histopathology, and outcomes of Meth-APAH compared to those of idiopathic PAH (iPAH). METHODS AND MEASUREMENTS: We performed a prospective cohort study of Meth-APAH and iPAH patients presenting to the Stanford University Pulmonary Hypertension Program between 2003-2015...
September 21, 2017: American Journal of Respiratory and Critical Care Medicine
https://www.readbyqxmd.com/read/28933948/the-value-and-application-of-the-six-minute-walk-test-in-idiopathic-pulmonary-fibrosis
#4
A Whitney Brown, Steven D Nathan
The 6 minute walk test (6MWT) is a commonly employed assessment of performance ability in a variety of cardiopulmonary diseases. It provides important functional information that is not captured in standardized pulmonary function testing. The test may be influenced by factors other than the severity of lung disease, including frailty, deconditioning, and musculoskeletal issues. The primary output measure from the six minute walk test is the distance walked, which appears to confer prognostic information in many diverse disease states...
September 21, 2017: Annals of the American Thoracic Society
https://www.readbyqxmd.com/read/28933616/are-newly-launched-pharmacotherapies-efficacious-in-treating-idiopathic-pulmonary-fibrosis-or-is-there-still-more-work-to-be-done
#5
Riccardo Inchingolo, Carola Condoluci, Andrea Smargiassi, Annelisa Mastrobattista, Cristina Boccabella, Alessia Comes, Nicoletta Golfi, Luca Richeldi
Idiopathic pulmonary fibrosis (IPF) is a challenging and multifactorial disease that has been thought for some time to lack effective treatments. The approval of two drugs, nintedanib and pirfenidone, has heralded a new era in its management. Areas covered: Currently, there is a growing interest on therapeutic strategies. Many studies have been designed and performed, although few of them turned out to be successful. Nowadays, nintedanib and pirfenidone are considered disease modifying drugs, recommended treatments by current evidence-based guidelines...
September 21, 2017: Expert Opinion on Pharmacotherapy
https://www.readbyqxmd.com/read/28932114/spermidine-mediated-poly-lactic-co-glycolic-acid-nanoparticles-containing-fluorofenidone-for-the-treatment-of-idiopathic-pulmonary-fibrosis
#6
Jing Tang, Jianming Li, Guo Li, Haitao Zhang, Ling Wang, Dai Li, Jinsong Ding
Idiopathic pulmonary fibrosis is a progressive, fatal lung disease with poor survival. The advances made in deciphering this disease have led to the approval of different antifibrotic molecules, such as pirfenidone and nintedanib. An increasing number of studies with particles (liposomes, nanoparticles [NPs], microspheres, nanopolymersomes, and nanoliposomes) modified with different functional groups have demonstrated improvement in lung-targeted drug delivery. In the present study, we prepared, characterized, and evaluated spermidine (Spd)-modified poly(lactic-co-glycolic acid) (PLGA) NPs as carriers for fluorofenidone (AKF) to improve the antifibrotic efficacy of this drug in the lung...
2017: International Journal of Nanomedicine
https://www.readbyqxmd.com/read/28929225/prediction-of-survival-by-texture-based-automated-quantitative-assessment-of-regional-disease-patterns-on-ct-in-idiopathic-pulmonary-fibrosis
#7
Sang Min Lee, Joon Beom Seo, Sang Young Oh, Tae Hoon Kim, Jin Woo Song, Sang Min Lee, Namkug Kim
OBJECTIVES: To retrospectively investigate whether the baseline extent and 1-year change in regional disease patterns on CT can predict survival of patients with idiopathic pulmonary fibrosis (IPF). METHODS: A total of 144 IPF patients with CT scans at the time of diagnosis and 1 year later were included. The extents of five regional disease patterns were quantified using an in-house texture-based automated system. The fibrosis score was defined as the sum of the extent of honeycombing and reticular opacity...
September 19, 2017: European Radiology
https://www.readbyqxmd.com/read/28923239/a-novel-formulation-of-inhaled-sodium-cromoglicate-pa101-in-idiopathic-pulmonary-fibrosis-and-chronic-cough-a-randomised-double-blind-proof-of-concept-phase-2-trial
#8
Surinder S Birring, Marlies S Wijsenbeek, Sanjay Agrawal, Jan W K van den Berg, Helen Stone, Toby M Maher, Ahmet Tutuncu, Alyn H Morice
BACKGROUND: Cough can be a debilitating symptom of idiopathic pulmonary fibrosis (IPF) and is difficult to treat. PA101 is a novel formulation of sodium cromoglicate delivered via a high-efficiency eFlow nebuliser that achieves significantly higher drug deposition in the lung compared with the existing formulations. We aimed to test the efficacy and safety of inhaled PA101 in patients with IPF and chronic cough and, to explore the antitussive mechanism of PA101, patients with chronic idiopathic cough (CIC) were also studied...
September 8, 2017: Lancet Respiratory Medicine
https://www.readbyqxmd.com/read/28922731/shikonin-suppresses-pulmonary-fibroblasts-proliferation-and-activation-by-regulating-akt-and-p38-mapk-signaling-pathways
#9
Yunjuan Nie, Yaoyao Yang, Jian Zhang, Guowei Cai, Yanhua Chang, Gaoshang Chai, Chongyong Guo
Fibroblast is believed to be the primary effector in idiopathic pulmonary fibrosis (IPF), a progressive lung disorder characterized by aberrant tissue remodeling and the formation of fibroblastic foci. Due to the complicated etiology and mechanism, there are few effective drugs for this fatal disease. Shikonin (SHI), which is the major ingredient isolated from the plant Lithospermum Erythrorhizon, has long been used as traditional medicine for many diseases including inflammation and cancer. The roles of SHI in attenuating skin scar and renal fibrosis by reducing TGFβ1-stimulated fibroblast activation are also reported...
September 14, 2017: Biomedicine & Pharmacotherapy, Biomédecine & Pharmacothérapie
https://www.readbyqxmd.com/read/28921394/combination-therapy-with-pirfenidone-plus-prednisolone-ameliorates-paraquat-induced-pulmonary-fibrosis
#10
Rokhsana Rasooli, Fatemeh Pourgholamhosein, Younes Kamali, Fatemeh Nabipour, Ali Mandegary
Pirfenidone is known to slow the decline in vital capacity and increase survival in idiopathic pulmonary fibrosis (IPF). Besides, administration of glucocorticoids, e.g., prednisolone has been the conventional strategy to the treatment of patients with this disease, although their efficacy is under debate. Since multiple coactivated pathways are involved in the pathogenesis of IPF, combination therapy is a foundation strategy to cover many more synergetic mechanisms and increase response. The aim of the present study was to compare the therapeutic efficacy of prednisolone plus pirfenidone with pirfenidone alone in PQ-induced lung fibrosis...
September 18, 2017: Inflammation
https://www.readbyqxmd.com/read/28918442/the-role-of-periostin-in-lung-fibrosis-and-airway-remodeling
#11
REVIEW
David N O'Dwyer, Bethany B Moore
Periostin is a protein that plays a key role in development and repair within the biological matrix of the lung. As a matricellular protein that does not contribute to extracellular matrix structure, periostin interacts with other extracellular matrix proteins to regulate the composition of the matrix in the lung and other organs. In this review, we discuss the studies exploring the role of periostin to date in chronic respiratory diseases, namely asthma and idiopathic pulmonary fibrosis. Asthma is a major health problem globally affecting millions of people worldwide with significant associated morbidity and mortality...
September 16, 2017: Cellular and Molecular Life Sciences: CMLS
https://www.readbyqxmd.com/read/28915874/unmet-needs-in-the-treatment-of-idiopathic-pulmonary-fibrosis-insights-from-patient-chart-review-in-five-european-countries
#12
Toby M Maher, Maria Molina-Molina, Anne-Marie Russell, Francesco Bonella, Stéphane Jouneau, Elena Ripamonti, Judit Axmann, Carlo Vancheri
BACKGROUND: Two antifibrotic drugs, pirfenidone and nintedanib, are approved by the European Medicines Agency and the US Food and Drug Administration for the treatment of idiopathic pulmonary fibrosis (IPF). In this analysis, treatment patterns of European patients with IPF were investigated to understand antifibrotic prescribing and identify unmet needs in IPF treatment practice. METHODS: Between February and March 2016, respiratory physicians from France, Germany, Italy, Spain, and the UK participated in an online questionnaire designed to collect information on IPF treatment patterns in patients under their care...
September 15, 2017: BMC Pulmonary Medicine
https://www.readbyqxmd.com/read/28904266/clinical-impact-of-main-pulmonary-artery-dilatation-on-outcome-in-pediatric-idiopathic-and-heritable-pulmonary-arterial-hypertension
#13
Satoshi Ikehara, Shinichi Takatsuki, Tomotaka Nakayama, Kazuyuki Naoi, Hiroyuki Matsuura, Tsutomu Saji
BACKGROUND: Few studies have investigated the clinical impact of pulmonary artery (PA) dilatation on outcomes in pediatric pulmonary arterial hypertension (PAH).Methods and Results:This study investigated the clinical outcomes of idiopathic or heritable PAH in 66 children aged <18 years at diagnosis. Main PA/thorax (MPA/T) ratio was measured on chest radiography in PAH patients. Patients were divided into 2 groups based on MPA/T ratio, and compared with a control group of 166 age- and gender-matched healthy children...
September 13, 2017: Circulation Journal: Official Journal of the Japanese Circulation Society
https://www.readbyqxmd.com/read/28902721/hiv-associated-pulmonary-hypertension
#14
Harish Jarrett, Christopher Barnett
PURPOSE OF REVIEW: HIV-associated pulmonary arterial hypertension (HIV-PAH) is a well-recognized severe cardiovascular complication of HIV infection that confers an adverse prognosis irrespective of the stage of disease. This review will summarize the available data on HIV-PAH epidemiology and provide insights into the pathophysiology and therapeutic strategies currently available. RECENT FINDINGS: Patients with HIV are several thousand times more likely to develop HIV-PAH compared to the incidence of idiopathic PAH...
September 11, 2017: Current Opinion in HIV and AIDS
https://www.readbyqxmd.com/read/28890532/diffuse-idiopathic-pulmonary-neuroendocrine-cell-hyperplasia-a-case-report-and-review-of-the-literature
#15
Evangelos Koliakos, Theodoros Thomopoulos, Ziad Abbassi, Christophe Duc, Michel Christodoulou
BACKGROUND Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) is a rare pulmonary disorder that is characterized by diffuse hyperplasia of bronchiolar and bronchial pulmonary neuroendocrine cells. In this condition, when no other pathological pulmonary condition is detected, DIPNECH is considered to be an idiopathic lung disease. DIPNECH is a rare condition that can be difficult to distinguish from other forms of reactive pulmonary neuroendocrine cell hyperplasia (NECH). We present a case of DIPNECH and describe the approach to diagnosis of this rare condition...
September 11, 2017: American Journal of Case Reports
https://www.readbyqxmd.com/read/28890480/effect-of-treatment-with-epoprostenol-and-endothelin-receptor-antagonists-on-the-development-of-thyrotoxicosis-in-patients-with-pulmonary-arterial-hypertension
#16
Mari Satoh, Keiko Aso, Tomotaka Nakayama, Tsutomu Saji
Thyroid disease is known to be associated with pulmonary arterial hypertension (PAH). We investigated the prevalence of thyroid disease in patients with idiopathic PAH (IPAH) or heritable PAH (HPAH), and the factors affecting the pathogenesis of thyroid disease. We retrospectively evaluated 59 patients with IPAH or HPAH who had been diagnosed with PAH before the age of 20 years. Thyrotoxicosis was detected in 12 of the 59 patients (6 patients with Graves' disease, 3 with hashitoxicosis, and 3 with silent thyroiditis) after the start of PAH treatment...
September 9, 2017: Endocrine Journal
https://www.readbyqxmd.com/read/28889759/nintedanib-with-add-on-pirfenidone-in-idiopathic-pulmonary-fibrosis-results-of-the-injourney-trial
#17
Carlo Vancheri, Michael Kreuter, Luca Richeldi, Christopher J Ryerson, Dominique Valeyre, Jan C Grutters, Sabrina Wiebe, Wibke Stansen, Manuel Quaresma, Susanne Stowasser, Wim A Wuyts
RATIONALE: Nintedanib and pirfenidone slow the progression of IPF, but the disease continues to progress. More data are needed on the safety and efficacy of combination therapy with nintedanib and add-on pirfenidone. OBJECTIVES: To investigate safety, tolerability, pharmacokinetic and exploratory efficacy endpoints in patients treated with nintedanib and add-on pirfenidone versus nintedanib alone. METHODS: Patients with IPF and FVC ≥50% predicted at screening who completed a 4-5 week run-in with nintedanib 150 mg bid without dose reduction or treatment interruption were randomized to nintedanib 150 mg bid with add-on pirfenidone (titrated to 801 mg tid), or nintedanib 150 mg bid alone, open-label for 12 weeks...
September 10, 2017: American Journal of Respiratory and Critical Care Medicine
https://www.readbyqxmd.com/read/28889145/efficacy-and-adverse-events-of-pirfenidone-in-treating-idiopathic-pulmonary-fibrosis
#18
Hui Ren, Kai Wang, Hao Yang, Lingyun Gao
To analyze the efficacy and adverse events  (AEs) of pirfenidone in idiopathic pulmonary fibrosis (IPF) trials. Methods: MEDLINE, Cochrane Library, and ClinicalTrials.gov were searched for studies published before June 2016. All studies of clinical trials with the key words IPF or idiopathic pulmonary fibrosis or lung fibrosis and pirfenidone or Esbriet were identified. Quality assessment and data extraction nwere conducted by 2 independent researchers. A meta-analysis of randomized controlled trials (RCTs) was performed, and relative risk (RR) and 95% confidence intervals (95% CIs) were calculated...
September 2017: Saudi Medical Journal
https://www.readbyqxmd.com/read/28887286/why-does-the-health-related-quality-of-life-in-idiopathic-normal-pressure-hydrocephalus-fail-to-improve-despite-the-favorable-clinical-outcome
#19
Junkkari Antti, Roine Risto P, Luikku Antti, Rauramaa Tuomas, Sintonen Harri, Nerg Ossi, Koivisto Anne M, Häyrinen Antti, Viinamäki Heimo, Soininen Hilkka, Jääskeläinen Juha E, Leinonen Ville
OBJECTIVE: Occasionally a favorable clinical disease-specific outcome does not reflect into improved generic health-related quality of life (HRQoL) in patients with idiopathic normal pressure hydrocephalus (iNPH) one year after the installation of the cerebrospinal fluid (CSF) shunt. Our aim was to identify factors causing this discrepancy. METHODS: The one-year HRQoL outcome of 141 iNPH patients was evaluated using the generic 15D instrument, in which the minimum clinically important change/difference on the 0-1 scale has been estimated to be ± 0...
September 5, 2017: World Neurosurgery
https://www.readbyqxmd.com/read/28886713/increased-activated-regulatory-t-cells-proportion-correlate-with-the-severity-of-idiopathic-pulmonary-fibrosis
#20
Ziliang Hou, Qiao Ye, Meihua Qiu, Yu Hao, Junyan Han, Hui Zeng
BACKGROUND: Regulatory T cells (Tregs) are crucial in maintaining immune tolerance and immune homeostasis, but their role in idiopathic pulmonary fibrosis (IPF) is unclear. This study was designed to explore the role of Tregs in IPF. METHODS: Percentages of Tregs and their subpopulations in peripheral blood (PB) and bronchoalveolar lavage (BAL) samples were determined by flow cytometry in 29 patients with IPF, 19 patients with primary Sjögren's syndrome-related interstitial pneumonia (pSS-IP), and 23 healthy controls (HCs)...
September 8, 2017: Respiratory Research
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