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Pulmonary interstitial disease

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https://www.readbyqxmd.com/read/29782426/nailfold-videocapillaroscopy-changes-are-associated-with-the-presence-and-severity-of-systemic-sclerosis-related-interstitial-lung-disease
#1
Joana Caetano, Filipe S Paula, Marta Amaral, Susana Oliveira, José D Alves
OBJECTIVE: The aim of this study was to evaluate the association of nailfold videocapillaroscopy (NVC) changes with the presence and severity of interstitial lung disease (ILD) in systemic sclerosis. METHODS: This was a cross-sectional analysis of 48 systemic sclerosis patients (21 patients with ILD). The NVC characteristics considered were capillary organization, capillary loss (CL), avascular areas, enlarged and giant capillaries, hemorrhages, abnormally shaped capillaries, edema, and intermittent flux...
May 19, 2018: Journal of Clinical Rheumatology: Practical Reports on Rheumatic & Musculoskeletal Diseases
https://www.readbyqxmd.com/read/29781586/treatment-algorithms-for-systemic-sclerosis-according-to-experts
#2
Andreu Fernández-Codina, Kyle M Walker, Janet E Pope
INTRODUCTION: Treatment for many aspects of systemic sclerosis (SSc) lacks agreement. OBJECTIVES: To generate SSc treatment algorithms endorsed by high percentage of SSc experts. METHODS: Experts from the Scleroderma Clinical Trials Consortium and the Canadian Scleroderma Research group (N=170) were asked whether they agreed with SSc algorithms (from 2012). Two consensus rounds refined agreement; 62 (36%), 54 (32%) and 48 (28%) experts completed surveys...
May 21, 2018: Arthritis & Rheumatology
https://www.readbyqxmd.com/read/29780127/a-case-of-transient-pulmonary-interstitial-lesions-in-aquaporin-4-positive-neuromyelitis-optica-spectrum-disorder
#3
Yuko Asato, Toshiaki Kamitani, Kuniyuki Ootsuka, Mizuki Kuramochi, Kozo Nakanishi, Tetsuya Shimada, Toshiyuki Takahashi, Tatsuro Misu, Masashi Aoki, Kazuo Fujihara, Yoshinori Kawabata
We herein report the case of a 76-year old man with aquaporin-4-Immunoglobulin-G (AQP4-IgG)-positive neuromyelitis optica spectrum disorder (NMOSD), in whom transient interstitial pulmonary lesions developed at the early stage of the disease. Chest X-ray showed multiple infiltrative shadows in both upper lung fields, and computed tomography revealed abnormal shadows distributed randomly in the lungs. Surgical lung biopsy showed features of unclassifiable interstitial pneumonia, characterized by various types of air-space organization, which resulted in obscure lung structure...
May 18, 2018: Internal Medicine
https://www.readbyqxmd.com/read/29777409/hemodynamic-heterogeneity-of-connective-tissue-disease-patients-with-borderline-mean-pulmonary-artery-pressure-and-its-distinctive-characters-from-those-with-normal-pulmonary-artery-pressure-a-retrospective-study
#4
Yusa Asari, Yoshioki Yamasaki, Kosei Tsuchida, Kengo Suzuki, Yoshihiro J Akashi, Takahiro Okazaki, Shoichi Ozaki, Hidehiro Yamada, Kimito Kawahata
To clarify whether patients with connective tissue disease (CTD)-associated borderline mean pulmonary artery pressure (mPAP) have distinctive hemodynamic characteristics from those with normal mPAP and whether pathogenesis is as heterogeneous as manifest pulmonary hypertension (PH). Seventy-five CTD patients who underwent right heart catheterization (RHC) from 2008 through 2016 were retrospectively analyzed. We compared between-group differences in clinical and hemodynamic findings: normal mPAP (n = 35), borderline mPAP (n = 15), and PH (n = 25)...
May 18, 2018: Clinical Rheumatology
https://www.readbyqxmd.com/read/29777306/respiratory-manifestations-in-lps-responsive-beige-like-anchor-lrba-protein-deficient-patients
#5
Oded Shamriz, Bella Shadur, Adeeb NaserEddin, Irina Zaidman, Natalia Simanovsky, Orly Elpeleg, Eitan Kerem, Joel Reiter, Polina Stepensky
Lipopolysaccharide (LPS)-responsive beige-like anchor (LRBA) protein deficiency is a rare syndrome of primary immune deficiency and immune dysregulation. In this study, we sought to summarize our experience with respiratory manifestations in LRBA-deficient patients. We conducted a retrospective analysis of the medical records of LRBA-deficient patients treated at Hadassah-Hebrew University Medical Center, Jerusalem, Israel. Data retrieved included pulmonary workup, disease course, treatment, and outcome. Ten patients were included...
May 18, 2018: European Journal of Pediatrics
https://www.readbyqxmd.com/read/29776440/radiological-pleuroparenchymal-fibroelastosis-associated-to-limited-cutaneous-systemic-sclerosis-a-case-report
#6
D Hassoun, S Dirou, P P Arrigoni, C Durant, M Hamidou, A Néel, C Agard
BACKGROUND: Pleuroparenchymal fibroelastosis (PPFE) is a very rare interstitial lung disease (ILD) characterized by progressive fibrotic lesions of the visceral pleura and the sub-pleural parenchyma, affecting predominantly the upper lobes. PPFE may occur in different contextes like bone marrow or lung transplantations, but also in the context of telomeropathy with mutations of telomerase reverse transcriptase (TERT), telomerase RNA component (TERC) or regulator of telomere elongation helicase 1 (RTEL1) genes...
May 18, 2018: BMC Pulmonary Medicine
https://www.readbyqxmd.com/read/29775809/transient-asymptomatic-pulmonary-opacities-during-osimertinib-treatment-and-its-clinical-implication
#7
Hansang Lee, Ho Yun Lee, Jong-Mu Sun, Se-Hoon Lee, Youjin Kim, Song Ee Park, Jin Seok Ahn, Keunchil Park, Myung-Ju Ahn
INTRODUCTION: Osimertinib is an oral, potent, irreversible 3rd generation EGFR tyrosine kinase inhibitor (TKI) approved for the treatment of T790M positive non-small cell lung cancer (NSCLC) patients who failed 1st or 2nd generation EGFR TKIs. Interstitial lung disease (ILD) is a rare complication with osimertinib, occurring in 1-3%. Recently, relatively high incidence of transient asymptomatic pulmonary opacities (TAPOs) which are different from ILD has been described. However, its clinical implication has not been fully determined yet...
May 15, 2018: Journal of Thoracic Oncology
https://www.readbyqxmd.com/read/29773465/pulmonary-manifestations-in-systemic-lupus-erythematosus-pleural-involvement-acute-pneumonitis-chronic-interstitial-lung-disease-and-diffuse-alveolar-hemorrhage
#8
Georgina Aguilera-Pickens, Carlos Abud-Mendoza
Systemic lupus erythematosus is the diffuse autoimmune connective tissue disease that most frequently involves pulmonary involvement, affecting 20% of 90% of the patients. The percentage varies depending on the defining criteria (symptoms, pulmonary tests or histopathological studies). At least once during the disease course, 50% of those affected have pleural and/or pulmonary manifestations, which are associated with higher morbidity and mortality. Pulmonary involvement has no correlation with lupus activity biomarkers, and it is necessary to rule out infectious processes in the initial approach...
May 14, 2018: Reumatología Clinica
https://www.readbyqxmd.com/read/29773297/the-clinical-practice-of-high-flow-nasal-cannula-oxygen-therapy-in-adults-a-japanese-cross-sectional-multicenter-survey
#9
Jiro Ito, Kazuma Nagata, Susumu Sato, Akira Shiraki, Naoki Nishimura, Shinyu Izumi, Ryo Tachikawa, Takeshi Morimoto, Keisuke Tomii
BACKGROUND: High-flow nasal cannula oxygen therapy (HFNC) is widely used mainly in the acute care setting, but limited data are available on real-world practice in adults. The objective of this study was to describe HFNC practices in Japanese adults. METHODS: A retrospective cross-sectional multicenter survey of adult patients receiving HFNC from January through March 2015 was conducted in 33 participating hospitals in Japan. RESULTS: We obtained information on 321 patients (median age, 76; 218 men, 103 women; median estimated PaO2 /FI O2, 178 mm Hg) from 22 hospitals...
May 2018: Respiratory Investigation
https://www.readbyqxmd.com/read/29769294/pulmonary-function-tests-as-outcomes-for-systemic-sclerosis-interstitial-lung-disease
#10
REVIEW
Melissa Caron, Sabrina Hoa, Marie Hudson, Kevin Schwartzman, Russell Steele
Interstitial lung disease (ILD) is the leading cause of morbidity and mortality in systemic sclerosis (SSc). We performed a systematic review to characterise the use and validation of pulmonary function tests (PFTs) as surrogate markers for systemic sclerosis-associated interstitial lung disease (SSc-ILD) progression.Five electronic databases were searched to identify all relevant studies. Included studies either used at least one PFT measure as a longitudinal outcome for SSc-ILD progression ( i.e. outcome studies) and/or reported at least one classical measure of validity for the PFTs in SSc-ILD ( i...
June 30, 2018: European Respiratory Review: An Official Journal of the European Respiratory Society
https://www.readbyqxmd.com/read/29768275/predictive-factors-for-the-long-term-deterioration-of-pulmonary-function-in-interstitial-lung-disease-associated-with-anti-aminoacyl-trna-synthetase-antibodies
#11
Hideaki Yamakawa, Eri Hagiwara, Hideya Kitamura, Tae Iwasawa, Ryota Otoshi, Naoto Aiko, Takuma Katano, Ryota Shintani, Satoshi Ikeda, Ryo Okuda, Akimasa Sekine, Tomohisa Baba, Shinichiro Iso, Kazuyoshi Kuwano, Shinji Sato, Takashi Ogura
BACKGROUND: Little has been reported on long-term pulmonary function trends among patients with interstitial lung disease associated with anti-aminoacyl-tRNA synthetase antibodies (ARS-ILD). OBJECTIVES: To clarify the factors predictive of progression in ARS-ILD based on patients' initial clinical and radiological features. METHODS: The clinical courses of 88 patients with > 1 year of follow-up data on pulmonary function tests (PFTs) were retrospectively analyzed...
May 16, 2018: Respiration; International Review of Thoracic Diseases
https://www.readbyqxmd.com/read/29766677/sleeping-child-neuroendocrine-cell-hyperplasia-of-infancy-and-polysomnography
#12
Deborah R Liptzin, Stephen M M Hawkins, Brandie D Wagner, Robin R Deterding
OBJECTIVES: Neuroendocrine cell hyperplasia of infancy (NEHI) is a children's interstitial and diffuse lung disease of unknown etiology that presents in infancy with characteristic findings of tachypnea, retractions, crackles, and hypoxemia. At the present, the mainstay of treatment is oxygen supplementation to normalize oxygen saturations and decrease work of breathing. There are characteristic pulmonary function, radiographic, and histologic findings, but polysomnography (PSG) data has not been reported...
May 15, 2018: Pediatric Pulmonology
https://www.readbyqxmd.com/read/29765788/early-pulmonary-interstitial-emphysema-in-preterm-neonates-respiratory-management-and-case-report-in-nonventilated-very-low-birth-weight-twins
#13
Judith Gronbach, Harald Ehrhardt, Klaus-Peter Zimmer, Markus Waitz
Early pulmonary interstitial emphysema in extreme preterm neonates is closely linked with respiratory distress syndrome and exposure to mechanical ventilation. In severe cases, maintaining adequate gas exchange aiming to avoid further lung damage and other neonatal morbidities associated with systemic/pulmonary hypoperfusion, prolonged hypoxia, and respiratory acidosis can be challenging and requires in-depth knowledge into the pathophysiology of the disease. Herein, we report on very low birth weight twins who developed early pulmonary interstitial emphysema during noninvasive respiratory support...
April 2018: American Journal of Perinatology Reports
https://www.readbyqxmd.com/read/29764749/prognosis-of-chronic-pulmonary-aspergillosis-in-patients-with-pulmonary-non-tuberculous-mycobacterial-disease
#14
Maiko Naito, Yu Kurahara, Shiomi Yoshida, Naoya Ikegami, Takehiko Kobayashi, Shojiro Minomo, Kazunobu Tachibana, Kazunari Tsuyuguchi, Seiji Hayashi, Katsuhiro Suzuki
BACKGROUND: Pulmonary non-tuberculous mycobacterial disease (PNTM) is a known risk factor for chronic pulmonary aspergillosis (CPA). However, few studies have focused on the prognosis of PNTM-associated CPA. In the present investigation, we aimed to elucidate the clinical course and prognostic factors of CPA in patients with PNTM. METHODS: We retrospectively investigated the medical records of 62 patients with CPA and a history of PNTM who were admitted to Kinki-chuo Chest Medical Center between 2010 and 2015...
May 11, 2018: Respiratory Investigation
https://www.readbyqxmd.com/read/29764401/management-of-acute-respiratory-failure-in-interstitial-lung-diseases-overview-and-clinical-insights
#15
REVIEW
Paola Faverio, Federica De Giacomi, Luca Sardella, Giuseppe Fiorentino, Mauro Carone, Francesco Salerno, Jousel Ora, Paola Rogliani, Giulia Pellegrino, Giuseppe Francesco Sferrazza Papa, Francesco Bini, Bruno Dino Bodini, Grazia Messinesi, Alberto Pesci, Antonio Esquinas
BACKGROUND: Interstitial lung diseases (ILDs) are a heterogeneous group of diseases characterized by widespread fibrotic and inflammatory abnormalities of the lung. Respiratory failure is a common complication in advanced stages or following acute worsening of the underlying disease. Aim of this review is to evaluate the current evidence in determining the best management of acute respiratory failure (ARF) in ILDs. METHODS: A literature search was performed in the Medline/PubMed and EMBASE databases to identify studies that investigated the management of ARF in ILDs (the last search was conducted on November 2017)...
May 15, 2018: BMC Pulmonary Medicine
https://www.readbyqxmd.com/read/29764379/identification-of-usual-interstitial-pneumonia-pattern-using-rna-seq-and-machine-learning-challenges-and-solutions
#16
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
#17
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/29763468/clinical-phenotypes-and-survival-of-pre-capillary-pulmonary-hypertension-in-systemic-sclerosis
#18
David Launay, David Montani, Paul M Hassoun, Vincent Cottin, Jérôme Le Pavec, Pierre Clerson, Olivier Sitbon, Xavier Jaïs, Laurent Savale, Jason Weatherald, Vincent Sobanski, Stephen C Mathai, Majid Shafiq, Jean-François Cordier, Eric Hachulla, Gérald Simonneau, Marc Humbert
Pre-capillary pulmonary hypertension (PH) in systemic sclerosis (SSc) is a heterogeneous condition with an overall bad prognosis. The objective of this study was to identify and characterize homogeneous phenotypes by a cluster analysis in SSc patients with PH. Patients were identified from two prospective cohorts from the US and France. Clinical, pulmonary function, high-resolution chest tomography, hemodynamic and survival data were extracted. We performed cluster analysis using the k-means method and compared survival between clusters using Cox regression analysis...
2018: PloS One
https://www.readbyqxmd.com/read/29761229/idiopathic-pulmonary-fibrosis-among-young-patients-challenges-in-diagnosis-and-management
#19
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
#20
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
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