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interstitial lung disease lung function

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https://www.readbyqxmd.com/read/29455320/elevated-serum-krebs-von-den-lungen-6-in-systemic-sclerosis-a-marker-of-lung-fibrosis-and-severity-of-the-disease
#1
Audrey Benyamine, Xavier Heim, Noémie Resseguier, Daniel Bertin, Carine Gomez, Mikaël Ebbo, Jean-Robert Harlé, Gilles Kaplanski, Pascal Rossi, Nathalie Bardin, Brigitte Granel
OBJECTIVES: We aimed to assess the clinical significance of Krebs von den Lungen-6 (KL-6) in the diagnosis and severity of interstitial lung disease (ILD) in a French cohort of patients with systemic sclerosis (SSc). METHODS: Serum KL-6 concentrations were measured with chemiluminescent enzyme immunoassay (CLEIA) in 75 SSc patients. Patients were divided into two groups according to the presence of interstitial lung disease (SSc-ILD versus SSc-without ILD) on chest High-Resolution Computed Tomography...
February 17, 2018: Rheumatology International
https://www.readbyqxmd.com/read/29445936/pulmonary-arterial-hemodynamic-assessment-by-a-novel-index-in-systemic-sclerosis-patients-pulmonary-pulse-transit-time
#2
Mehmet Dogan, Tolga Han Efe, Tolga Cimen, Cem Ozisler, Mehmet Ali Felekoglu, Ahmet Goktug Ertem, Mehmet Erat, Omer Yiginer, Murat Tulmac
OBJECTIVES: Systemic sclerosis (SSc) is a chronic, inflammatory, and autoimmune connective tissue disease that is associated with vascular lesions, and fibrosis of the skin and visceral organs. Cardiac complications may occur as a secondary effect of SSc as a result of pulmonary arterial hypertension and interstitial lung disease. The objective of this study was to assess whether the pulmonary pulse transit time (pPTT) could serve as a diagnostic marker for pulmonary arterial alterations in patients with SSc, prior to development of pulmonary hypertension...
February 14, 2018: Lung
https://www.readbyqxmd.com/read/29444329/gastroesophageal-reflux-symptoms-are-not-sufficient-to-guide-esophageal-function-testing-in-lung-transplant-candidates
#3
S Posner, J Zheng, R K Wood, R A Shimpi, M G Hartwig, S-C Chow, D A Leiman
Gastroesophageal reflux disease and esophageal dysmotility are prevalent in patients with advanced lung disease and are associated with graft dysfunction following lung transplantation. As a result, many transplant centers perform esophageal function testing as part of the wait-listing process but guidelines for testing in this population are lacking. The aim of this study is to describe whether symptoms of gastroesophageal reflux correlate with abnormal results on pH-metry and high-resolution manometry and can be used to identify those who require testing...
February 9, 2018: Diseases of the Esophagus: Official Journal of the International Society for Diseases of the Esophagus
https://www.readbyqxmd.com/read/29441299/livedo-racemosa-reticulated-ulcerations-panniculitis-and-violaceous-plaques-in-a-46-year-old-woman
#4
Alfredo Agulló, Brian Hinds, Mónica Larrea, Ignacio Yanguas
Clinically amyopathic dermatomyositis (CADM) is a subset of dermatomyositis (DM) that has conventional cutaneous manifestations of DM, but paradoxically, little or no muscle involvement. In 2005, a novel antibody was described in association with CADM - anti-melanoma differentiation-associated gene 5 (anti-MDA5). Patients with this serologic marker have a characteristic mucocutaneous phenotype consisting of skin ulceration among other signs. We describe the case of a 46-year-old woman with CADM, elevated anti-MDA5 autoantibodies, and unusual clinical features (livedo racemosa, florid acral edema) among the classical phenotype of MDA5 DM (arthralgias, ulcerations, panniculitis) and classical DM lesions (Gottron papules, heliotrope rash)...
January 2018: Indian Dermatology Online Journal
https://www.readbyqxmd.com/read/29426066/screening-high-resolution-computed-tomography-of-the-chest-to-detect-interstitial-lung-disease-in-systemic-sclerosis-a-global-survey-of-rheumatologists
#5
Elana J Bernstein, Dinesh Khanna, David J Lederer
Interstitial lung disease (ILD) affects approximately 40-60% of adults with systemic sclerosis (SSc) and is the leading cause of death and hospitalization in this population (1-4). Despite the life-threatening nature of ILD and the availability of existing therapies (5, 6), there are no clinical practice guidelines that recommend screening for ILD in SSc. Although pulmonary function tests (PFTs) are widely used by rheumatologists as a screening test for SSc-ILD, studies have shown that they are neither sensitive nor specific for the detection of ILD in this population (3)...
February 9, 2018: Arthritis & Rheumatology
https://www.readbyqxmd.com/read/29423877/nhlrc2-variants-identified-in-patients-with-fibrosis-neurodegeneration-and-cerebral-angiomatosis-finca-characterisation-of-a-novel-cerebropulmonary-disease
#6
Johanna Uusimaa, Riitta Kaarteenaho, Teija Paakkola, Hannu Tuominen, Minna K Karjalainen, Javad Nadaf, Teppo Varilo, Meri Uusi-Mäkelä, Maria Suo-Palosaari, Ilkka Pietilä, Anniina E Hiltunen, Lloyd Ruddock, Heli Alanen, Ekaterina Biterova, Ilkka Miinalainen, Annamari Salminen, Raija Soininen, Aki Manninen, Raija Sormunen, Mika Kaakinen, Reetta Vuolteenaho, Riitta Herva, Päivi Vieira, Teija Dunder, Hannaleena Kokkonen, Jukka S Moilanen, Heikki Rantala, Lawrence M Nogee, Jacek Majewski, Mika Rämet, Mikko Hallman, Reetta Hinttala
A novel multi-organ disease that is fatal in early childhood was identified in three patients from two non-consanguineous families. These children were born asymptomatic but at the age of 2 months they manifested progressive multi-organ symptoms resembling no previously known disease. The main clinical features included progressive cerebropulmonary symptoms, malabsorption, progressive growth failure, recurrent infections, chronic haemolytic anaemia and transient liver dysfunction. In the affected children, neuropathology revealed increased angiomatosis-like leptomeningeal, cortical and superficial white matter vascularisation and congestion, vacuolar degeneration and myelin loss in white matter, as well as neuronal degeneration...
February 8, 2018: Acta Neuropathologica
https://www.readbyqxmd.com/read/29422289/prevalence-and-effects-of-emphysema-in-never-smokers-with-rheumatoid-arthritis-interstitial-lung-disease
#7
Joseph Jacob, Jin Woo Song, Hee-Young Yoon, Gary Cross, Joseph Barnett, Wen Ling Woo, Fern Adams, Maria Kokosi, Anand Devaraj, Elisabetta Renzoni, Toby M Maher, Dong Soon Kim, Athol U Wells
AIMS: Autoimmune conditions such as rheumatoid arthritis-related interstitial lung disease (RA-ILD) have been linked to the existence of emphysema in never-smokers. We aimed to quantify emphysema prevalence in RA-ILD never-smokers and investigate whether combined pulmonary fibrosis and emphysema (CPFE) results in a worsened prognosis independent of baseline disease extent. METHODS: RA-ILD patients presenting to the Royal Brompton Hospital (n=90) and Asan Medical Center (n=155) had CT's evaluated for a definite usual interstitial pneumonia (UIP) pattern, and visual extents of emphysema and ILD...
January 31, 2018: EBioMedicine
https://www.readbyqxmd.com/read/29413504/several-high-resolution-computed-tomography-findings-associate-with-survival-and-clinical-features-in-rheumatoid-arthritis-associated-interstitial-lung-disease
#8
Hanna M Nurmi, Hannu-Pekka Kettunen, Sanna-Katja Suoranta, Minna K Purokivi, Miia S Kärkkäinen, Tuomas A Selander, Riitta L Kaarteenaho
OBJECTIVE: To compare the presence and extent of several high-resolution computed tomography (HRCT) observations in different subtypes of rheumatoid arthritis-related interstitial lung disease (RA-ILD) and to examine associations between radiological findings, hospitalization, age, RA duration, pulmonary function tests (PFT) and survival. MATERIALS AND METHODS: HRCTs from 60 RA-ILD patients were independently evaluated and re-categorized into usual interstitial pneumonia (UIP), nonspecific interstitial pneumonia (NSIP), organizing pneumonia (OP), diffuse alveolar damage (DAD) and unclassified subtypes by two radiologists...
January 2018: Respiratory Medicine
https://www.readbyqxmd.com/read/29411336/central-sleep-apnea-with-cheyne-stokes-breathing-in-heart-failure-from-research-to-clinical-practice-and-beyond
#9
K Terziyski, A Draganova
Characterized by periodic crescendo-decrescendo pattern of breathing alternating with central apneas, Central sleep apnea (CSA) with Cheyne-Stokes Breathing represents a highly prevalent, yet underdiagnosed comorbidity in chronic heart failure (CHF). A diverse body of evidence demonstrates increased morbidity and mortality in the presence of CSB. CSB has been described in both CHF patients with preserved and reduced ejection fraction, regardless of drug treatment. Risk factors for CSB are older age, male gender, high BMI, atrial fibrillation and hypocapnia...
February 7, 2018: Advances in Experimental Medicine and Biology
https://www.readbyqxmd.com/read/29408012/biomarkers-in-idiopathic-pulmonary-fibrosis
#10
REVIEW
F Drakopanagiotakis, Lukasz Wujak, Malgorzata Wygrecka, P Markart
Idiopathic pulmonary fibrosis (IPF) is a chronic, debilitating, fibrotic lung disease leading to respiratory failure and ultimately to death. Being the prototype of interstitial lung diseases, IPF is characterized by marked heterogeneity regarding its clinical course. Despite significant progress in the understanding of its pathogenesis, we still cannot reliably predict the course of the disease and the response to treatment of an individual patient. Non-invasive biomarkers, in particular serum biomarkers, for the (early) diagnosis, differential diagnosis, prognosis and prediction of therapeutic response are urgently needed...
January 30, 2018: Matrix Biology: Journal of the International Society for Matrix Biology
https://www.readbyqxmd.com/read/29397275/rotation-covariant-tissue-analysis-for-interstitial-lung-diseases-using-learned-steerable-filters-performance-evaluation-and-relevance-for-diagnostic-aid
#11
Ranveer Joyseeree, Henning Müller, Adrien Depeursinge
A novel method to detect and classify several classes of diseased and healthy lung tissue of interstitial lung diseases is presented, as these diseases are hard to diagnose and differentiate. Local organizations of image directions at several scales drive the process of creating discriminative lung tissue texture signatures using spatial and Fourier domain information extracted from the images. The signatures are generated for four diseased tissue classes and healthy tissue, all of which appear in the Interstitial Lung Disease (ILD) database, using a novel one-versus-one approach for learning discriminative filter signatures...
January 11, 2018: Computerized Medical Imaging and Graphics: the Official Journal of the Computerized Medical Imaging Society
https://www.readbyqxmd.com/read/29382801/acute-graft-versus-host-disease-following-lung-transplantation-in-a-patient-with-a-novel-tert-mutation
#12
Jonathan R Brestoff, Alexandre T Vessoni, Kirsten A Brenner, Geoffrey L Uy, John F DiPersio, Morey Blinder, Chad A Witt, Derek E Byers, Ramsey R Hachem, Elbert P Truclock, Dayna S Early, Milan J Anadkat, Amy Musiek, Cylen Javidan-Nejad, Dennis M Balfe, Ilana S Rosman, Chang Liu, Lingxin Zhang, George J Despotis, Marianna B Ruzinova, Jennifer K Sehn, Ina Amarillo, Jonathan W Heusel, Wojcieh Swat, Brian S Kim, Lukas D Wartman, Roger D Yusen, Luis F Z Batista
Familial pulmonary fibrosis is associated with loss-of-function mutations in telomerase reverse transcriptase (TERT) and short telomeres. Interstitial lung diseases have become the leading indication for lung transplantation in the USA, and recent data indicate that pathogenic mutations in telomerase may cause unfavourable outcomes following lung transplantation. Although a rare occurrence, solid organ transplant recipients who develop acute graft-versus-host disease (GVHD) have very poor survival. This case report describes the detection of a novel mutation in TERT in a patient who had lung transplantation for familial pulmonary fibrosis and died from complications of acute GVHD...
January 30, 2018: Thorax
https://www.readbyqxmd.com/read/29382380/systemic-sclerosis-associated-interstitial-lung-disease-individualized-immunosuppressive-therapy-and-course-of-lung-function-results-of-the-eustar-group
#13
Sabine Adler, Dörte Huscher, Elise Siegert, Yannick Allanore, László Czirják, Francesco DelGaldo, Christopher P Denton, Oliver Distler, Marc Frerix, Marco Matucci-Cerinic, Ulf Mueller-Ladner, Ingo-Helmut Tarner, Gabriele Valentini, Ulrich A Walker, Peter M Villiger, Gabriela Riemekasten
BACKGROUND: Interstitial lung disease in systemic sclerosis (SSc-ILD) is a major cause of SSc-related death. Imunosuppressive treatment (IS) is used in patients with SSc for various organ manifestations mainly to ameliorate progression of SSc-ILD. Data on everyday IS prescription patterns and clinical courses of lung function during and after therapy are scarce. METHODS: We analysed patients fulfilling American College of Rheumatology (ACR)/European League against Rheumatism (EULAR) 2013 criteria for SSc-ILD and at least one report of IS...
January 30, 2018: Arthritis Research & Therapy
https://www.readbyqxmd.com/read/29344746/desvenlafaxine-induced-interstitial-pneumonitis-a-case-report
#14
Arjan Flora, Daniel Pipoly
A 52-year-old man developed interstitial pneumonitis during treatment with desvenlafaxine for major depressive disorder. The man received desvenlafaxine at 50 mg for symptoms of depression 4 years earlier. Six months after a dose increase to 100 mg, he developed bronchitic symptoms with mild, persistent dyspnea. Investigations revealed a restrictive pattern on pulmonary function testing, bilateral upper lobe reticular opacities with traction bronchiectasis on radiology imaging, and end-stage interstitial fibrosis with honeycomb changes consistent with chronic hypersensitivity pneumonitis on open lung biopsy...
January 17, 2018: Drug Safety—Case Reports
https://www.readbyqxmd.com/read/29341228/bronchoalveolar-lavage-fluid-neutrophilia-is-associated-with-the-severity-of-pulmonary-lesions-during-equine-asthma-exacerbations
#15
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/29331337/elastin-in-lung-development-and-disease-pathogenesis
#16
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/29327616/nintedanib-for-the-treatment-of-idiopathic-pulmonary-fibrosis
#17
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...
February 2018: Expert Opinion on Pharmacotherapy
https://www.readbyqxmd.com/read/29325682/computer-based-quantitative-computed-tomography-image-analysis-in-idiopathic-pulmonary-fibrosis-a-mini-review
#18
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/29325675/mycophenolate-mofetil-as-a-therapeutic-agent-for-interstitial-lung-diseases-in-systemic-sclerosis
#19
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/29325094/functional-rescue-of-misfolding-abca3-mutations-by-small-molecular-correctors
#20
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
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