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https://www.readbyqxmd.com/read/29331337/elastin-in-lung-development-and-disease-pathogenesis
#1
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/29330374/predicting-fibrosis-progression-in-renal-transplant-recipients-using-laser-based-infrared-spectroscopic-imaging
#2
Vishal K Varma, Andre Kajdacsy-Balla, Sanjeev Akkina, Suman Setty, Michael J Walsh
Renal transplants have not seen a significant improvement in their 10-year graft life. Chronic damage accumulation often leads to interstitial fibrosis and tubular atrophy (IF/TA) and thus graft function loss over time. For this reason, IF/TA has been the chief suspect for a potential prognostic marker for long term outcomes. In this study, we have used infrared spectroscopic (IR) imaging to interrogate the biochemistry of regions of fibrosis from renal transplant biopsies to identify a biochemical signature that can predict rapid progression of fibrosis...
January 12, 2018: Scientific Reports
https://www.readbyqxmd.com/read/29330249/is-it-idiopathic-pulmonary-fibrosis-or-not
#3
REVIEW
Mary Salvatore, Genta Ishikawa, Maria Padilla
Pulmonary fibrosis is not uncommon. Usual interstitial pneumonitis (UIP)/idiopathic pulmonary fibrosis (IPF) is the most common of the idiopathic pulmonary fibrotic diseases and has the worst prognosis with a mean life expectancy of 3.8 years. The American Thoracic Society has provided guidelines for the accurate diagnosis of IPF.In 2014, 2 antifibrotic medications were approved in the United States that target the multiple fibrotic pathways of UIP, which increased the need for early and accurate diagnosis of IPF...
January 2018: Journal of the American Board of Family Medicine: JABFM
https://www.readbyqxmd.com/read/29329683/respiratory-disease-in-ball-pythons-python-regius-experimentally-infected-with-ball-python-nidovirus
#4
Laura L Hoon-Hanks, Marylee L Layton, Robert J Ossiboff, John S L Parker, Edward J Dubovi, Mark D Stenglein
Circumstantial evidence has linked a new group of nidoviruses with respiratory disease in pythons, lizards, and cattle. We conducted experimental infections in ball pythons (Python regius) to test the hypothesis that ball python nidovirus (BPNV) infection results in respiratory disease. Three ball pythons were inoculated orally and intratracheally with cell culture isolated BPNV and two were sham inoculated. Antemortem choanal, oroesophageal, and cloacal swabs and postmortem tissues of infected snakes were positive for viral RNA, protein, and infectious virus by qRT-PCR, immunohistochemistry, western blot and virus isolation...
January 9, 2018: Virology
https://www.readbyqxmd.com/read/29329133/cutaneous-angiosarcoma-of-the-eyelid-mimicking-morbihan-disease
#5
Donna C Ferguson, Louise A Mawn, Rami N Al-Rohil
BACKGROUND: Cutaneous angiosarcoma presents clinically in numerous ways, and can be mistaken for a different clinical entity, particularly when arising at unusual anatomic locations such as the eyelid. CASE PRESENTATION: A 57-year-old woman presented with a 1-year history of eyelid swelling. Concurrent imaging was also suggestive of an edematous process. Multiple superficial biopsies showed nonspecific dermal inflammation and interstitial edema. A diagnosis of Morbihan disease (chronic and idiopathic lymphedema of the eyelid) was rendered, and the patient was treated with compression and local therapy without clinical improvement...
January 11, 2018: American Journal of Dermatopathology
https://www.readbyqxmd.com/read/29327719/the-histomorphological-spectrum-of-restrictive-chronic-lung-allograft-dysfunction-and-implications-for-prognosis
#6
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
#7
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
#8
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/29327205/nociceptive-roles-of-trpm2-ion-channel-in-pathologic-pain
#9
REVIEW
Yongwoo Jang, Pyung Sun Cho, Young Duk Yang, Sun Wook Hwang
Pain is a protective mechanism that enables us to avoid potentially harmful environments. However, when pathologically persisted and aggravated under severely injured or inflamed conditions, pain often reduces the quality of life and thus is considered as a disease to eliminate. Inflammatory and/or neuropathic mechanisms may exaggerate interactions between damaged tissues and neural pathways for pain mediation. Similar mechanisms also promote the communication among cellular participants in synapses at spinal or higher levels, which may amplify nociceptive firing and subsequent signal transmission, deteriorating the pain sensation...
January 11, 2018: Molecular Neurobiology
https://www.readbyqxmd.com/read/29327122/understanding-qi-running-in-the-meridians-as-interstitial-fluid-flowing-via-interstitial-space-of-low-hydraulic-resistance
#10
Wei-Bo Zhang, De-Xian Jia, Hong-Yan Li, Yu-Long Wei, Huang Yan, Peng-Na Zhao, Fei-Fei Gu, Guang-Jun Wang, Yan-Ping Wang
Qi, blood and the meridians are fundamental concepts in Chinese medicine (CM), which are components of the human body and maintain physiological function. Pathological changes of qi, blood and meridians may lead to discomfort and disease. Treatment with acupuncture or herbal medicine aims to regulate qi and blood so as to recover normal function of the meridians. This paper explores the nature of qi as well as compares and correlates them with the structures of the human body. We propose a conceptualization of qi as being similar to the interstitial flfl uid, and the meridians as being similar to interstitial space of low hydraulic resistance in the body...
January 9, 2018: Chinese Journal of Integrative Medicine
https://www.readbyqxmd.com/read/29325682/computer-based-quantitative-computed-tomography-image-analysis-in-idiopathic-pulmonary-fibrosis-a-mini-review
#11
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
#12
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
#13
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
#14
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
#15
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
#16
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
#17
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
#18
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
https://www.readbyqxmd.com/read/29323079/immunoglobulin-g4-related-tubulointerstitial-nephritis-a-not-to-be-missed-diagnosis
#19
Smita Mary Matthai, Anjali Mohapatra, Raiyani Palak, Gopal Basu
Immunoglobulin G4-related tubulointerstitial nephritis (IgG4-TIN) is a newly recognized clinicopathological entity characterized by a dense interstitial infiltrate of IgG4-positive plasma cells accompanied by fibrosis and obliterative phlebitis causing acute or chronic renal dysfunction amenable to corticosteroid therapy. IgG4-TIN is the dominant manifestation of renal involvement in IgG4-related disease (IgG4-RD) which is a novel, immune-mediated, fibroinflammatory and multiorgan disorder. We describe a case of IgG4-TIN with isolated renal involvement in an elderly male patient with poor response to corticosteroid therapy...
October 2017: Indian Journal of Pathology & Microbiology
https://www.readbyqxmd.com/read/29323059/histological-and-morphometric-analysis-of-dilated-cardiomyopathy-with-special-reference-to-collagen-iv-expression
#20
Parul Jain, Sudheer Arava, Sandeep Seth, Sanjeev Lalwani, Ruma Ray
INTRODUCTION: Collagen distribution alterations are well known in dilated cardiomyopathy. There are also changes in microvasculature along with other histomorphorphological features. AIMS AND OBJECTIVES: To study the histomorphological features of DCM along with their quantitative correlation with LVEF. Alterations in collagen IV distribution pattern and microvasculature in DCM were also evaluated. MATERIALS AND METHODS: The present study includes 34 right ventricular endomyocardial biopsies, 7 explanted native hearts and 41 autopsy control hearts...
October 2017: Indian Journal of Pathology & Microbiology
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