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https://www.readbyqxmd.com/read/29342368/reply-to-a-standardized-diagnostic-ontology-for-fibrotic-interstitial-lung-disease
#1
Christopher J Ryerson, Simon L F Walsh, Harold R Collard
No abstract text is available yet for this article.
January 17, 2018: American Journal of Respiratory and Critical Care Medicine
https://www.readbyqxmd.com/read/29342365/a-standardized-diagnostic-ontology-for-fibrotic-interstitial-lung-disease
#2
Yuben Moodley
No abstract text is available yet for this article.
January 17, 2018: American Journal of Respiratory and Critical Care Medicine
https://www.readbyqxmd.com/read/29341228/bronchoalveolar-lavage-fluid-neutrophilia-is-associated-with-the-severity-of-pulmonary-lesions-during-equine-asthma-exacerbations
#3
M Bullone, P Joubert, A Gagné, J-Pierre Lavoie, P Hélie
BACKGROUND: The severe form of equine asthma is associated with pathological changes of the peripheral airways and pulmonary parenchyma that are only partly described. Also, the relationship between these structural alterations and the percentage of neutrophils found within the airway lumen, assessed by bronchoalveolar lavage fluid (BALF) cytology, remains ill-defined. OBJECTIVE: To examine the histological lesions associated with equine asthma during disease exacerbation and remission, and their relationship with lung function and BALF neutrophilia...
January 17, 2018: Equine Veterinary Journal
https://www.readbyqxmd.com/read/29338781/correlation-between-disease-activity-and-serum-ferritin-in-clinically-amyopathic-dermatomyositis-with-rapidly-progressive-interstitial-lung-disease-a-case-report
#4
Kazuhiro Yamada, Kazuhisa Asai, Atsuko Okamoto, Tetsuya Watanabe, Hiroshi Kanazawa, Mai Ohata, Masahiko Ohsawa, Kazuto Hirata
BACKGROUND: Clinically amyopathic dermatomyositis with anti-Melanoma Differentiation-Associated gene 5 (MDA5) antibody often presents with severe interstitial lung disease. Although serum ferritin level is known to reflect interstitial lung disease activity, there are few case reports describing this association. CASE PRESENTATION: A 58-year-old man was referred to our outpatient clinic with a 3-week history of cough and respiratory distress. He had erythema over the V area of the neck and a Gottron's sign...
January 16, 2018: BMC Research Notes
https://www.readbyqxmd.com/read/29336713/persistent-pulmonary-hypertension-without-underlying-cardiac-disease-as-a-presentation-of-pulmonary-interstitial-glycogenosis
#5
Gordon Gray Still, Shuo Li, Mark Wilson, Paul Sammut
INTRODUCTION: Pulmonary interstitial glycogenosis (PIG) is an idiopathic lung condition that remains clinically underrecognized despite a growing body of literature. CASE REPORT: We present a case of PIG with pulmonary hypertension without underlying cardiac disease. This patient presented with respiratory distress and spontaneous pneumothorax at 6 months of age. Laboratory and imaging investigations demonstrated nonspecific features, but refractory pulmonary hypertension was confirmed on cardiac catheterization...
January 16, 2018: Fetal and Pediatric Pathology
https://www.readbyqxmd.com/read/29336618/rheumatoid-arthritis-a-single-center-egyptian-experience
#6
B R Sakr, M M Elfishawi, M H ElArousy, A K Hatw, A N AbdulKarim, A B Tammam, A N Kotp, M E Hamed, I E Genedy, E D El Desouky, Z O Nawito
OBJECTIVE: Demonstration of rheumatoid arthritis (RA) characteristics in a large cohort of Egyptian patients. METHODS: Retrospective analysis of data of 3219 RA patients attending the Rheumatology outpatient clinic, Kasr Alainy Hospital, Cairo University; from January 1995 till December 2015. RESULTS: Mean age at disease onset was 36.1 ±13.4 years; 2774 (84%) were females and mean disease duration was 12.9 ±7.9 years. Regarding number of joint affected at disease onset; polyarticular pattern was found in 77...
January 16, 2018: Immunological Investigations
https://www.readbyqxmd.com/read/29335647/aged-polymorphonuclear-leukocytes-cause-fibrotic-interstitial-lung-disease-in-the-absence-of-regulation-by-b-cells
#7
Jung Hwan Kim, John Podstawka, Yuefei Lou, Lu Li, Esther K S Lee, Maziar Divangahi, Björn Petri, Frank R Jirik, Margaret M Kelly, Bryan G Yipp
Pulmonary immunity requires tight regulation, as interstitial inflammation can compromise gas exchange and lead to respiratory failure. Here we found a greater number of aged CD11bhiL-selectinloCXCR4+ polymorphonuclear leukocytes (PMNs) in lung vasculature than in the peripheral circulation. Using pulmonary intravital microscopy, we observed lung PMNs physically interacting with B cells via β2 integrins; this initiated neutrophil apoptosis, which led to macrophage-mediated clearance. Genetic deletion of B cells led to the accumulation of aged PMNs in the lungs without systemic inflammation, which caused pathological fibrotic interstitial lung disease that was attenuated by the adoptive transfer of B cells or depletion of PMNs...
January 15, 2018: Nature Immunology
https://www.readbyqxmd.com/read/29333842/an-adolescent-with-idiopathic-pleuroparenchymal-fibroelastosis-case-report
#8
Emine Atag, Nilay Bas Ikizoglu, Yasemin Gokdemir, Ela Erdem Eralp, Gursu Kiyan, Dilek Yilmazbayhan, Bulent Karadag
Idiopathic pleuroparenchymal fibroelastosis (IPPFE) is a rare disorder recently included in rare idiopathic interstitial pneumonias according to the updated American Thoracic Society/European Respiratory Society classification. IPPFE is characterized by pleural and subpleural parenchymal fibrosis causing volume loss predominantly in the upper lung lobes. Age of onset is variable, IPPFE mainly occurs in third and fourth decades. We present a 16 year old patient with a 2-year history of exertional dyspnea, nonproductive cough and weight loss...
February 1, 2018: Archivos Argentinos de Pediatría
https://www.readbyqxmd.com/read/29331337/elastin-in-lung-development-and-disease-pathogenesis
#9
REVIEW
Robert P Mecham
Elastin is expressed in most tissues that require elastic recoil. The protein first appeared coincident with the closed circulatory system, and was critical for the evolutionary success of the vertebrate lineage. Elastin is expressed by multiple cell types in the lung, including mesothelial cells in the pleura, smooth muscle cells in airways and blood vessels, endothelial cells, and interstitial fibroblasts. This highly crosslinked protein associates with fibrillin-containing microfibrils to form the elastic fiber, which is the physiological structure that functions in the extracellular matrix...
January 10, 2018: Matrix Biology: Journal of the International Society for Matrix Biology
https://www.readbyqxmd.com/read/29327719/the-histomorphological-spectrum-of-restrictive-chronic-lung-allograft-dysfunction-and-implications-for-prognosis
#10
Jan H von der Thüsen, Elly Vandermeulen, Robin Vos, Birgit Weynand, Erik K Verbeken, Stijn E Verleden
Chronic lung allograft dysfunction continues to be the main contributor to poor long-term allograft survival after lung transplantation. The restrictive phenotype of chronic lung allograft dysfunction carries a particularly poor prognosis. Little is known about the pathogenetic mechanisms involved in restrictive chronic lung allograft dysfunction. In this study, we performed histomorphological and immunohistochemical analysis of restrictive chronic lung allograft dysfunction lungs. Explant lung tissue from 21 restrictive chronic lung allograft dysfunction patients was collected and histopathologic patterns of rejection, fibrosis and vascular changes were scored after routine histochemical stains and additional immunohistochemistry for endothelial markers and C4d...
January 12, 2018: Modern Pathology: An Official Journal of the United States and Canadian Academy of Pathology, Inc
https://www.readbyqxmd.com/read/29327616/nintedanib-for-the-treatment-of-idiopathic-pulmonary-fibrosis
#11
Francesco Varone, Giacomo Sgalla, Bruno Iovene, Teresa Bruni, Luca Richeldi
Idiopathic Pulmonary Fibrosis (IPF) is an interstitial lung disease characterized by the progressive loss of pulmonary function, ultimately leading to respiratory failure and death. Two novel compounds, nintedanib and pirfenidone, have shown efficacy in reducing the rate of decline of lung function in IPF patients. The multiple tyrosine kinase inhibitor nintedanib has extensively being studied as a potential angiogenesis inhibitor in clinical against various neoplastic disorders. Afterwards, this compound was successfully tested in IPF...
January 12, 2018: Expert Opinion on Pharmacotherapy
https://www.readbyqxmd.com/read/29327393/pulmonary-hypertension-in-interstitial-lung-disease-limitations-of-echocardiography-compared-to-cardiac-catheterization
#12
Gregory J Keir, S John Wort, Maria Kokosi, Peter M George, Simon L F Walsh, Joseph Jacob, Laura Price, Simon Bax, Elisabetta A Renzoni, Toby M Maher, Peter MacDonald, David M Hansell, Athol U Wells
BACKGROUND AND OBJECTIVE: In interstitial lung disease (ILD), pulmonary hypertension (PH) is a major adverse prognostic determinant. Transthoracic echocardiography (TTE) is the most widely used tool when screening for PH, although discordance between TTE and right heart catheter (RHC) measured pulmonary haemodynamics is increasingly recognized. We evaluated the predictive utility of the updated European Society of Cardiology/European Respiratory Society (ESC/ERS) TTE screening recommendations against RHC testing in a large, well-characterized ILD cohort...
January 12, 2018: Respirology: Official Journal of the Asian Pacific Society of Respirology
https://www.readbyqxmd.com/read/29325682/computer-based-quantitative-computed-tomography-image-analysis-in-idiopathic-pulmonary-fibrosis-a-mini-review
#13
REVIEW
Hirotsugu Ohkubo, Hiroaki Nakagawa, Akio Niimi
Idiopathic pulmonary fibrosis (IPF) is the most common type of progressive idiopathic interstitial pneumonia in adults. Many computer-based image analysis methods of chest computed tomography (CT) used in patients with IPF include the mean CT value of the whole lungs, density histogram analysis, density mask technique, and texture classification methods. Most of these methods offer good assessment of pulmonary functions, disease progression, and mortality. Each method has merits that can be used in clinical practice...
January 2018: Respiratory Investigation
https://www.readbyqxmd.com/read/29325681/is-hypothyroidism-in-idiopathic-pleuroparenchymal-fibroelastosis-a-novel-lung-thyroid-syndrome
#14
Nobuyasu Awano, Takehiro Izumo, Kensuke Fukuda, Mari Tone, Daisuke Yamada, Tamiko Takemura, Soichiro Ikushima, Toshio Kumasaka
BACKGROUND: Idiopathic pleuroparenchymal fibroelastosis (IPPFE) is a rare type of interstitial pneumonia characterized by fibroelastosis. Patients with IPPFE as well as idiopathic interstitial pneumonia often have autoimmune diseases, which sometimes coincide with hypothyroidism (HypoT). However, there have been no reports on the association between IPPFE and HypoT. The purpose of this study was to evaluate the correlation between IPPFE and HypoT. We also examined the pathological features of the thyroid glands from autopsied cases...
January 2018: Respiratory Investigation
https://www.readbyqxmd.com/read/29325680/clinico-radio-pathological-characteristics-of-unclassifiable-idiopathic-interstitial-pneumonias
#15
Yasuhiko Nakamura, Keishi Sugino, Masashi Kitani, Akira Hebisawa, Naobumi Tochigi, Sakae Homma
BACKGROUND: The purpose of this study was to clarify the clinico-radio-pathological characteristics and prognostic factors of unclassifiable-idiopathic interstitial pneumonias (U-IIPs) diagnosed by surgical lung biopsy. METHODS: Among 86 patients with interstitial pneumonia who underwent surgical lung biopsy from January 2005 to September 2013, 33 (38.4%; 16 male patients; mean age, 64.4 ± 8.8 years) were diagnosed with U-IIPs. They were subsequently categorized into rapidly progressive (n = 7), slowly progressive (n = 7), and stable (n = 19) groups based on the decrease of the percent predicted forced vital capacity or percent predicted diffusing capacity of the lung carbon monoxide and the occurrence of acute exacerbation...
January 2018: Respiratory Investigation
https://www.readbyqxmd.com/read/29325675/mycophenolate-mofetil-as-a-therapeutic-agent-for-interstitial-lung-diseases-in-systemic-sclerosis
#16
REVIEW
Takahiro Ueda, Takuro Sakagami, Toshiaki Kikuchi, Toshinori Takada
Systemic sclerosis (SSc) is an intractable disease that causes fibrosis in all organs. Approximately 40% of patients with SSc have some degree of interstitial lung disease (ILD). One third of patients with SSc and ILD, approximately 15% of all patients, have pulmonary lesions, which slowly progress to respiratory failure resistant to corticosteroid and other treatments. A randomized controlled trial conducted in the United States indicated that one year of treatment with oral cyclophosphamide in patients with SSc-ILD had a significant but modest beneficial effect on lung function, dyspnea, thickening of the skin, and health-related quality of life...
January 2018: Respiratory Investigation
https://www.readbyqxmd.com/read/29325580/the-predictive-prognostic-factors-for-polymyositis-dermatomyositis-associated-interstitial-lung-disease
#17
Yumiko Sugiyama, Ryusuke Yoshimi, Maasa Tamura, Mitsuhiro Takeno, Yosuke Kunishita, Daiga Kishimoto, Yuji Yoshioka, Kouji Kobayashi, Kaoru Takase-Minegishi, Toshiyuki Watanabe, Naoki Hamada, Hideto Nagai, Naomi Tsuchida, Yutaro Soejima, Hiroto Nakano, Reikou Kamiyama, Takeaki Uehara, Yohei Kirino, Akiko Sekiguchi, Atsushi Ihata, Shigeru Ohno, Shouhei Nagaoka, Hideaki Nakajima
BACKGROUND: Interstitial lung disease (ILD) is the principal cause of death in polymyositis/dermatomyositis (PM/DM). Here we investigated prognostic factors for death and serious infection in PM/DM-ILD using the multicenter database. METHODS: We retrospectively reviewed baseline demographic, clinical and laboratory findings, treatment regimens and outcomes in patients with PM/DM-ILD. The distribution of ILD lesions was evaluated in four divided lung zones of high-resolution computed tomography images...
January 11, 2018: Arthritis Research & Therapy
https://www.readbyqxmd.com/read/29325094/functional-rescue-of-misfolding-abca3-mutations-by-small-molecular-correctors
#18
Susanna Kinting, Stefanie Höppner, Ulrike Schindlbeck, Maria E Forstner, Jacqueline Harfst, Thomas Wittmann, Matthias Griese
ABCA3, a phospholipid transporter in lung lamellar bodies (LB), is essential for the assembly of pulmonary surfactant and LB biogenesis. Mutations in the ABCA3 gene are an important genetic cause for respiratory distress syndrome in neonates and interstitial lung disease in children and adults, for which there is currently no cure.The aim of this study was to prove that disease causing misfolding ABCA3 mutations can be corrected in vitro and to investigate available options for correction.We stably expressed HA-tagged wild type ABCA3 or variants p...
January 9, 2018: Human Molecular Genetics
https://www.readbyqxmd.com/read/29324936/the-impact-of-lung-perfusion-scintigraphy-in-the-emergency-management-of-patients-with-suspected-pulmonary-embolism
#19
Cristina Ferrari, Alessandra Cimino, Giacomo Bianco, Francesca Iuele, Alessandra Di Palo, Margherita Fanelli, Artor Niccoli-Asabella, Giuseppe Rubini
OBJECTIVE: Pulmonary Embolism (PE) is an emergency condition that requires immediate treatment. As the symptoms and the risk factors are nonspecific, PE differential diagnosis is often required. Even if angio-CT is considered the gold standard for PE diagnosis, the frequent allergic condition and/or chronic renal failure of patients make, in most cases, not possible the use of contrast enhancement in emergency with even more increasing use of Lung Perfusion Scintigraphy (LPS), as a simple and fast examination with no preparation/contraindication...
September 2017: Hellenic Journal of Nuclear Medicine
https://www.readbyqxmd.com/read/29323413/extracorporeal-membrane-oxygenation-for-refractory-severe-respiratory-failure-in-acute-interstitial-pneumonia
#20
Gabriela Gonçalves-Venade, Nuno Lacerda-Príncipe, Roberto Roncon-Albuquerque, José Artur Paiva
Acute interstitial pneumonia (AIP) is a rare idiopathic interstitial lung disease with rapid progressive respiratory failure and high mortality. In the present report, three cases of AIP complicated by refractory respiratory failure supported with extracorporeal membrane oxygenation (ECMO) are presented. One male and two female patients (ages 27-59) were included. Venovenous ECMO support was provided using miniaturized systems, with two-site femoro-jugular circuit configuration. Despite lung protective ventilation, prone position and neuromuscular blockade, refractory respiratory failure of unknown etiology supervened (ratio of arterial oxygen partial pressure to fractional inspired oxygen 46-130) and ECMO was initiated after 3-7 days of mechanical ventilation...
January 11, 2018: Artificial Organs
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