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Idiopathic pulmonary disease

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https://www.readbyqxmd.com/read/29774214/the-expression-of-aqp1-is-modified-in-lung-of-patients-with-idiopathic-pulmonary-fibrosis-addressing-a-possible-new-target
#1
Ana Galán-Cobo, Elena Arellano-Orden, Rocío Sánchez Silva, José Luis López-Campos, César Gutiérrez Rivera, Lourdes Gómez Izquierdo, Nela Suárez-Luna, María Molina-Molina, José A Rodríguez Portal, Miriam Echevarría
Activation of the epithelial-mesenchymal transition process (EMT) by which alveolar cells in human lung tissue undergo differentiation giving rise to a mesenchymal phenotype (fibroblast/miofibroblasts) has been well recognized as a key element in the origin of idiopathic pulmonary fibrosis (IPF). Here we analyzed expression of AQP1 in lung biopsies of patients diagnosed with IPF, and compared it to biopsies derived from patients with diverse lung pneumonies, such as hypersensitivity pneumonitis, sarcoidosis or normal lungs...
2018: Frontiers in Molecular Biosciences
https://www.readbyqxmd.com/read/29773608/what-s-in-a-name-that-which-we-call-ipf-by-any-other-name-would-act-the-same
#2
Athol U Wells, Kevin K Brown, Kevin R Flaherty, Martin Kolb, Victor J Thannickal
Idiopathic pulmonary fibrosis (IPF) remains a truly idiopathic fibrotic disease, with a modest genetic predilection and candidate triggers but no overall explanation for the development of disease in non-familial cases. Agreement on terminology has contributed to major clinical and translational advances since the millennium. It is likely that the entity currently captured by the term "IPF" will be radically reclassified over the next decade, either through "splitting" (into IPF subgroups responding selectively to individual disease-modifying agents) or through "lumping" of IPF with other forms of progressive fibrotic lung disease (with shared pathogenetic mechanisms and IPF-like disease behaviour)...
May 2018: European Respiratory Journal: Official Journal of the European Society for Clinical Respiratory Physiology
https://www.readbyqxmd.com/read/29769446/ubiquitination-and-deubiquitination-emerge-as-players-in-idiopathic-pulmonary-fibrosis-pathogenesis-and-treatment
#3
REVIEW
Shuang Li, Jing Zhao, Dong Shang, Daniel J Kass, Yutong Zhao
Idiopathic pulmonary fibrosis (IPF) is a fatal fibrotic lung disease that is associated with aberrant activation of TGF-β, myofibroblast differentiation, and abnormal extracellular matrix (ECM) production. Proper regulation of protein stability is important for maintenance of intracellular protein homeostasis and signaling. Ubiquitin E3 ligases mediate protein ubiquitination, and deubiquitinating enzymes (DUBs) reverse the process. The role of ubiquitin E3 ligases and DUBs in the pathogenesis of IPF is relatively unexplored...
May 17, 2018: JCI Insight
https://www.readbyqxmd.com/read/29768342/mucosa-associated-lymphoid-tissue-lymphoma-of-the-trachea-associated-with-idiopathic-pulmonary-fibrosis-a-case-report-and-literature-review
#4
June Hong Ahn, Jin Hong Chung, Kyeong-Cheol Shin, Eun Young Choi, Hyun Jung Jin, Joon Hyuk Choi, Kwan Ho Lee
RATIONALE: Mucosa-associated lymphoid tissue (MALT) lymphoma of the trachea is a rare disease that has been shown to be associated with chronic antigenic stimulation. There have been few reports of MALT lymphoma of the trachea in association with idiopathic pulmonary fibrosis (IPF). PATIENT CONCERNS: A 73-year-old patient visited with a 2-year history of dyspnea on exertion and productive cough, which had worsened 1 month ago. DIAGNOSES: MALT lymphoma of the trachea associated with IPF...
May 2018: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29764379/identification-of-usual-interstitial-pneumonia-pattern-using-rna-seq-and-machine-learning-challenges-and-solutions
#5
Yoonha Choi, Tiffany Ting Liu, Daniel G Pankratz, Thomas V Colby, Neil M Barth, David A Lynch, P Sean Walsh, Ganesh Raghu, Giulia C Kennedy, Jing Huang
BACKGROUND: We developed a classifier using RNA sequencing data that identifies the usual interstitial pneumonia (UIP) pattern for the diagnosis of idiopathic pulmonary fibrosis. We addressed significant challenges, including limited sample size, biological and technical sample heterogeneity, and reagent and assay batch effects. RESULTS: We identified inter- and intra-patient heterogeneity, particularly within the non-UIP group. The models classified UIP on transbronchial biopsy samples with a receiver-operating characteristic area under the curve of ~ 0...
May 9, 2018: BMC Genomics
https://www.readbyqxmd.com/read/29763900/c-prosp-b-a-possible-biomarker-for-pulmonary-diseases
#6
Nicolas Kahn, Anne-Kathrin Rossler, Katrin Hornemann, Thomas Muley, Ekkehard Grünig, Werner Schmidt, Felix J F Herth, Michael Kreuter
BACKGROUND: Detection of surfactant proteins A and D (SP-A and SP-D) in the serum of patients with pulmonary diseases is thought to reflect an injury of the alveolar epithelial barrier and as such serve as a biomarker for these diseases. However, the data for SP-B are limited. OBJECTIVES: The aim of this feasibility study was to assess whether immature SP-B pre-proteins might have value as a possible biomarker for pulmonary diseases. METHODS: In serum samples from patients with different chronic lung diseases (interstitial lung diseases [ILDs], chronic obstructive pulmonary disease, asthma, lung cancer, pulmonary hypertension, inflammation, patients on ventilator support; total n = 283), C-proSP-B was measured using an electrochemiluminescence immunoassay based on mouse monoclonal anti-C-proSP-B antibodies...
May 15, 2018: Respiration; International Review of Thoracic Diseases
https://www.readbyqxmd.com/read/29763424/prevalence-of-primary-sj%C3%A3-gren-s-syndrome-in-patients-undergoing-evaluation-for-pulmonary-arterial-hypertension
#7
Tatsuyuki Sato, Masaru Hatano, Yukiko Iwasaki, Hisataka Maki, Akihito Saito, Shun Minatsuki, Toshiro Inaba, Eisuke Amiya, Keishi Fujio, Masafumi Watanabe, Kazuhiko Yamamoto, Issei Komuro
BACKGROUND: The prevalence of pulmonary arterial hypertension (PAH) in primary Sjögren's syndrome (SS) had been reported to be rare. However, recent studies using echocardiography as a screening method showed conflicting results, and the true prevalence is still unclear. Since diagnosing primary SS is difficult because of its heterogeneous nature, a number of patients with primary-SS-associated PAH may be misdiagnosed with idiopathic PAH, losing their chance to undergo immunosuppressive therapy...
2018: PloS One
https://www.readbyqxmd.com/read/29761573/using-fat-to-fight-disease-a-systematic-review-of-non-homologous-adipose-derived-stromal-stem-cell-therapies
#8
REVIEW
Marjorie E Bateman, Amy L Strong, Jeffrey M Gimble, Bruce A Bunnell
OBJECTIVE: The objective of this review is to describe the safety and efficacy of ASC and SVF in treating common diseases and the next steps in research that must occur prior to clinical use. EVIDENCE REVIEW: Pubmed, Ovid Medline, Embase, Web of Science, and the Cochrane Library were searched for articles about use of SVF or ASC for disease therapy published between 2012 and 2017. One meta-analysis, 2 randomized controlled trials, and 16 case series were included, representing 844 human patients...
May 15, 2018: Stem Cells
https://www.readbyqxmd.com/read/29761229/idiopathic-pulmonary-fibrosis-among-young-patients-challenges-in-diagnosis-and-management
#9
Gabriela Leuschner, Fredrik Reiter, Florian Stocker, Alexander Crispin, Nikolaus Kneidinger, Tobias Veit, Friederike Klenner, Felix Ceelen, Gregor Zimmermann, Hanno Leuchte, Simone Reu, Julien Dinkel, Jürgen Behr, Claus Neurohr
INTRODUCTION: Idiopathic pulmonary fibrosis (IPF) is considered a disease of older patients, being rare in patients ≤ 50 years. Still, IPF can occur in younger patients, but this particular patient group is not well characterised so far. The aim of this study was to compare the diagnostic certainty, clinical features, comorbidities and survival in young versus older IPF patients. METHODS: We reviewed our medical records from February 2011 until February 2015, to identify IPF patients, who were then classified as young (≤ 50 years) or older IPF (> 50 years)...
May 14, 2018: Lung
https://www.readbyqxmd.com/read/29761224/cardiopulmonary-factors-affecting-6-min-walk-distance-in-patients-with-idiopathic-inflammatory-myopathies
#10
Naoki Mugii, Fujiko Someya
Idiopathic inflammatory myopathies involve skeletal muscles and can be associated with interstitial lung disease and/or heart dysfunction, which may reduce exercise capacity. We aimed to clarify cardiopulmonary factors affecting the 6-min walk distance in patients who were able to walk without leg pain or fatigue. Twenty-three patients with inactive adult idiopathic inflammatory myopathies, and 18 age- and gender-matched healthy controls were evaluated for hemodynamic responses using noninvasive impedance cardiography during the 6-min walk test...
May 14, 2018: Rheumatology International
https://www.readbyqxmd.com/read/29758336/grand-rounds-hepatic-manifestations-of-telomere-biology-disorders
#11
REVIEW
Mrinal M Patnaik, Patrick S Kamath, Douglas A Simonetto
Clinical Case A 51 year old Caucasian male was referred for evaluation of variceal bleeding. Laboratory tests were remarkable for mild thrombocytopenia and moderate alkaline phosphatase elevation. Synthetic liver function was well preserved. Abdominal computed tomography scan revealed moderate splenomegaly, gastric varices, and normal hepatic contour. A transjugular liver biopsy was performed revealing findings of nodular regenerative hyperplasia (NRH) with no significant fibrosis or necroinflammatory activity...
May 11, 2018: Journal of Hepatology
https://www.readbyqxmd.com/read/29755982/clinical-genetics-in-interstitial-lung-disease
#12
REVIEW
Chad A Newton, Philip L Molyneaux, Justin M Oldham
Interstitial lung disease (ILD) comprises a heterogeneous group of diffuse parenchymal lung processes with overlapping clinical, radiographic, and histopathologic features. Among the most common and deadly ILDs are idiopathic pulmonary fibrosis (IPF) and chronic hypersensitivity pneumonitis (CHP). As the name implies, the cause of IPF remains elusive, but a variety of genetic and infectious risk factors have been identified. CHP results from chronic inhalation of an organic antigen, usually of avian or mold origin, and may occur in patients with a genetic predisposition...
2018: Frontiers in Medicine
https://www.readbyqxmd.com/read/29755070/-hemothorax-due-to-idiopathic-rupture-of-pulmonary-artery-with-defect-of-tunica-media
#13
Kazuyoshi Shimada, Masaki Kawamura
A 47-years-old man with hemodynamic shock was refered to our hospital by an ambulance. Chest computed tomography(CT)showed left hemothorax and the extravasation of contrast media in his left lung. Emergency operation was done. A lot of intrathoracic hematoma and pulsating bleeding from the lung was found, and lingular segmentectomy was performed. Pathologically, the rupture of pulmonary artery of 2.2 mm in diameter was found without the finding of imflammation nor degeneration due to any basal diseases. Around the lesion, some artery of 0...
March 2018: Kyobu Geka. the Japanese Journal of Thoracic Surgery
https://www.readbyqxmd.com/read/29751748/underlying-and-immediate-causes-of-death-in-patients-with-idiopathic-pulmonary-fibrosis
#14
Miia Kärkkäinen, Hanna Nurmi, Hannu-Pekka Kettunen, Tuomas Selander, Minna Purokivi, Riitta Kaarteenaho
BACKGROUND: The most common cause of death of patients with idiopathic pulmonary fibrosis (IPF) has been reported to be the lung disease itself and mortality from IPF appears to be increasing. However, the causes of death in patients with IPF taking into account differences between genders and smoking histories as well as disease progression, have not been previously explored. METHODS: Retrospective data from hospital register and death certificates from national database of IPF patients treated in Kuopio University Hospital (KUH) from 2002 to 2012 were collected...
May 11, 2018: BMC Pulmonary Medicine
https://www.readbyqxmd.com/read/29751154/long-term-clinical-outcomes-of-high-dose-mepolizumab-treatment-for-hypereosinophilic-syndrome
#15
Fei Li Kuang, Michael P Fay, JeanAnne Ware, Lauren Wetzler, Nicole Holland-Thomas, Thomas Brown, Hector Ortega, Jonathan Steinfeld, Paneez Khoury, Amy D Klion
BACKGROUND: Conventional therapies for hypereosinophilic syndromes (HES) have variable efficacy and carry significant long-term toxicities. Anti-IL5 (mepolizumab) therapy has a glucocorticoid (GC)-sparing effect in GC-sensitive HES, but the efficacy of mepolizumab in treatment-refractory HES patients with severe disease has not been examined to date. OBJECTIVE: To identify predictors of response to mepolizumab in subjects with severe treatment-refractory HES and compare long-term outcomes in these subjects to HES subjects treated with conventional therapies...
May 8, 2018: Journal of Allergy and Clinical Immunology in Practice
https://www.readbyqxmd.com/read/29747640/fibrocytes-are-increased-in-lung-and-peripheral-blood-of-patients-with-idiopathic-pulmonary-fibrosis
#16
P Heukels, J A C van Hulst, M van Nimwegen, C E Boorsma, B N Melgert, L M van den Toorn, K A T Boomars, M S Wijsenbeek, H Hoogsteden, J H von der Thüsen, R W Hendriks, M Kool, B van den Blink
BACKGROUND: Fibrocytes are implicated in Idiopathic Pulmonary Fibrosis (IPF) pathogenesis and increased proportions in the circulation are associated with poor prognosis. Upon tissue injury, fibrocytes migrate to the affected organ. In IPF patients, circulating fibrocytes are increased especially during exacerbations, however fibrocytes in the lungs have not been examined. Therefore, we sought to evaluate if fibrocytes can be detected in IPF lungs and we compare percentages and phenotypic characteristics of lung fibrocytes with circulating fibrocytes in IPF...
May 10, 2018: Respiratory Research
https://www.readbyqxmd.com/read/29747538/protective-effect-of-standardized-extract-of-myrtus-communis-l-myrtle-on-experimentally-bleomycin-induced-pulmonary-fibrosis-biochemical-and-histopathological-study
#17
Mitra Samareh Fekri, Ali Mandegary, Fariba Sharififar, Hamid Reza Poursalehi, Mohammad Hadi Nematollahi, Atefe Izadi, Mohammad Mehdipour, Amir Asadi, Masoomeh Samareh Fekri
CONTEXT: Myrtle (Myrtus communis L) has been used widely in traditional medicine for different respiratory disorders. Idiopathic pulmonary fibrosis (IPF) is an inflammatory disease characterized by progressive loss of lung function with poor prognosis. The pathogenesis of disease has not been completely elucidated, but probably persistent epithelial damages are involved. OBJECTIVE: Evaluation of biochemical and histopathological effect of preventive and therapeutic doses of myrtle against bleomycin (BLM)-induced pulmonary fibrosis (PF) in animal model...
May 11, 2018: Drug and Chemical Toxicology
https://www.readbyqxmd.com/read/29744939/heart-transplant-outcomes-in-patients-with-chagas-cardiomyopathy-in-the-united-states
#18
Rodolfo D Benatti, Sadeer G Al-Kindi, Fernando Bacal, Guilherme H Oliveira
BACKGROUND: Chagas cardiomyopathy (CC) is one of the chronic manifestations of Trypanosoma cruzi (T. cruzi) infection and is among the leading reasons for heart transplantation (HT) in Latin America. Chagas disease is also present in areas with large Hispanic communities in the United States. Our objective is to evaluate the outcomes of cardiac allograft recipients with the diagnosis of CC in the United States. METHODS AND RESULTS: We identified 25 adult patients with CC and 15,930 with idiopathic dilated cardiomyopathy (IDCMP) who underwent HT between 1987 and 2015...
May 9, 2018: Clinical Transplantation
https://www.readbyqxmd.com/read/29743074/genetic-analyses-in-a-cohort-of-191-pulmonary-arterial-hypertension-patients
#19
Hang Yang, Qixian Zeng, Yanyun Ma, Bingyang Liu, Qianlong Chen, Wenke Li, Changming Xiong, Zhou Zhou
BACKGROUND: Pulmonary arterial hypertension (PAH) is a progressive and fatal disorder associated with high pulmonary artery pressure. Genetic testing enables early diagnosis and offers an opportunity for family screening. To identify genetic mutations and help make a precise diagnosis, we performed genetic testing in 191 probands with PAH and tried to analyze the genotype-phenotype correlation. METHODS: Initially, PAH samples (n = 119) were submitted to BMPR2 screening using Sanger sequencing...
May 9, 2018: Respiratory Research
https://www.readbyqxmd.com/read/29740120/the-clinical-characteristics-and-outcomes-of-follicular-bronchiolitis-in-chinese-adult-patients
#20
Ju Lu, Miao Ma, Qi Zhao, Fanqing Meng, Dongmei Wang, Hourong Cai, Mengshu Cao
Follicular bronchiolitis (FB) is a rare interstitial lung disease (ILD) and has been reported in diverse clinical contexts. Six FB patients demonstrated by surgical lung biopsy (SLB) were reviewed between 2009 and 2017 from Nanjing Drum Tower Hospital in China. The average age of subjects was 42 years old (range: 31-55 years). The clinical symptoms were very mild. The laboratory findings showed elevated Erythrocyte sedimentation rate (ESR) and serum globulin and anemia. Pulmonary function tests were normal in four cases...
May 8, 2018: Scientific Reports
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