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Pulmonary interstitial disease

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https://www.readbyqxmd.com/read/28810314/-clinical-and-pathological-characteristics-of-erdheim-chester-disease-involving-the-lungs
#1
T Lu, S Wang, H Huang, T Wang, M Wang, D R Zhong, R E Feng
Objective: To explore the clinical manifestations, pathological features, differential diagnosis and gene mutation status in patients with pulmonary involvement of Erdheim-Chester disease (ECD). Methods: The clinical data of 4 cases of Erdheim-Chester disease admitted to Peking Union Medical College Hospital from October 2014 to August 2016 were examined for imaging, microscopic and immunohistochemitry findings, and BRAFV600E mutation. The related literatures were reviewed. Results: Among the 4 cases, there were 3 males and 1 female, aging from 7 to 47 years, and the average age was 34...
August 12, 2017: Chinese Journal of Tuberculosis and Respiratory Diseases
https://www.readbyqxmd.com/read/28808949/practical-management-of-respiratory-comorbidities-in-patients-with-rheumatoid-arthritis
#2
REVIEW
James Bluett, Meghna Jani, Deborah P M Symmons
Lung disease is one of the most common causes of extra-articular morbidity and mortality in patients with rheumatoid arthritis (RA). Development of pulmonary manifestations may be due to the systemic disease itself; to serious respiratory adverse events such as pneumonitis and infections secondary to therapy; or to lifestyle habits such as smoking. Rheumatologists often need to make important treatment decisions and plan future care in RA patients with respiratory comorbidities, despite the absence of clear evidence or consensus...
August 14, 2017: Rheumatology and Therapy
https://www.readbyqxmd.com/read/28808495/the-most-frequent-abca3-nonsense-mutation-p-tyr1515-y1515x-causing-lethal-neonatal-respiratory-failure-in-a-term-neonate
#3
AlNashmi AlAnazi, Ralph Epaud, Humariya Heena, Alix de Becdelievre, Abeer Mohammad Miqdad, Pascale Fanen
Defects in the surfactant biosynthesis are associated with respiratory distress syndrome, commonly occurring in premature infants due to lung immaturity. However, interstitial lung diseases have also been observed in full-term infants with mutations in the SFTPC, SFTPB, NKX2-1, or ABCA3 genes, involved in the surfactant metabolism. Herein, we report a newborn baby with neonatal respiratory distress and diffuse lung disease caused by ABCA3 mutation. The baby died at 5 weeks of age after developing pulmonary hypertension...
July 2017: Annals of Thoracic Medicine
https://www.readbyqxmd.com/read/28804709/exploring-animal-models-that-resemble-idiopathic-pulmonary-fibrosis
#4
REVIEW
Jun Tashiro, Gustavo A Rubio, Andrew H Limper, Kurt Williams, Sharon J Elliot, Ioanna Ninou, Vassilis Aidinis, Argyrios Tzouvelekis, Marilyn K Glassberg
Large multicenter clinical trials have led to two recently approved drugs for patients with idiopathic pulmonary fibrosis (IPF); yet, both of these therapies only slow disease progression and do not provide a definitive cure. Traditionally, preclinical trials have utilized mouse models of bleomycin (BLM)-induced pulmonary fibrosis-though several limitations prevent direct translation to human IPF. Spontaneous pulmonary fibrosis occurs in other animal species, including dogs, horses, donkeys, and cats. While the fibrotic lungs of these animals share many characteristics with lungs of patients with IPF, current veterinary classifications of fibrotic lung disease are not entirely equivalent...
2017: Frontiers in Medicine
https://www.readbyqxmd.com/read/28800589/prognostic-factors-and-outcomes-in-japanese-lung-transplant-candidates-with-interstitial-lung-disease
#5
Kohei Ikezoe, Tomohiro Handa, Kiminobu Tanizawa, Toyofumi F Chen-Yoshikawa, Takeshi Kubo, Akihiro Aoyama, Hideki Motoyama, Kyoko Hijiya, Shinsaku Tokuda, Yoshinari Nakatsuka, Yuko Yamamoto, Ayako Oshima, Shin-Ichi Harashima, Sonoko Nagai, Toyohiro Hirai, Hiroshi Date, Kazuo Chin
OBJECTIVE: Young patients with advanced interstitial lung disease (ILD) are potential candidates for cadaveric lung transplantation. This study aimed to examine clinical features, outcomes, and prognostic factors in Japanese ILD patients awaiting lung transplantation. METHODS: We investigated the clinical features and outcomes of 77 consecutive candidates with ILD who were referred to Kyoto University Hospital and subsequently actively listed for lung transplant in the Japan Organ Transplant Network between 2010 and 2014...
2017: PloS One
https://www.readbyqxmd.com/read/28796717/utility-of-flexible-bronchoscopic-cryobiopsy-for-diagnosis-of-diffuse-parenchymal-lung-diseases
#6
Robert J Lentz, Trevor M Taylor, Jonathan A Kropski, Kim L Sandler, Joyce E Johnson, Timothy S Blackwell, Fabien Maldonado, Otis B Rickman
BACKGROUND: Initial reports of transbronchial cryobiopsy for diffuse parenchymal lung disease (DPLD) suggest the diagnostic yield approaches that of surgical lung biopsy (SLB) with an excellent safety profile. Centers performing cryobiopsy differ significantly in procedure technique; an optimal technique minimizing complications but still capable of diagnosing a wide range of DPLDs has not been established. We evaluated our practice of flexible bronchoscopic cryobiopsy in a primarily outpatient setting for patients who required a tissue diagnosis for DPLD of uncertain etiology...
August 7, 2017: Journal of Bronchology & Interventional Pulmonology
https://www.readbyqxmd.com/read/28793886/cathepsin-s-degraded-decorin-are-elevated-in-fibrotic-lung-disorders-development-and-biological-validation-of-a-new-serum-biomarker
#7
S N Kehlet, C L Bager, N Willumsen, B Dasgupta, C Brodmerkel, M Curran, S Brix, D J Leeming, M A Karsdal
BACKGROUND: Decorin is one of the most abundant proteoglycans of the extracellular matrix and is mainly secreted and deposited in the interstitial matrix by fibroblasts where it plays an important role in collagen turnover and tissue homeostasis. Degradation of decorin might disturb normal tissue homeostasis contributing to extracellular matrix remodeling diseases. Here, we present the development and validation of a competitive enzyme-linked immunosorbent assay (ELISA) quantifying a specific fragment of degraded decorin, which has potential as a novel non-invasive serum biomarker for fibrotic lung disorders...
August 9, 2017: BMC Pulmonary Medicine
https://www.readbyqxmd.com/read/28790293/-critical-care-managements-after-lung-transplantation
#8
Masaki Anraku
Lung transplantation is a viable treatment option for patients with end-stage lung diseases such as interstitial pneumonia/pulmonary fibrosis, emphysema, pulmonary hypertension, and so on. Collecting available clinical, physiological, serological, and surgical information of both donor and recipient is vital when planning relevant postoperative managements. The goal of the managements is to keep the transplanted lung (s) functional while preventing/treating infection, rejection, and ischemiareperfusion lung injury...
July 2017: Kyobu Geka. the Japanese Journal of Thoracic Surgery
https://www.readbyqxmd.com/read/28790280/-perioperative-management-of-patients-with-pulmonary-comorbidities-undergoing-lung-resection
#9
Sumiko Maeda
Chronic obstructive pulmonary disease (COPD) and interstitial lung disease (ILD) are 2 major pulmonary comorbidities of primary lung cancer patients. The COPD patients are at risk for respiratory failure after a major lung resection when lung resection exceeds the patient's pulmonary reserve. It is important to assess a postoperative risk based on the patient's cardiopulmonary function. Cardiac risks are initially evaluated for all the candidates for lung resection, then, ppoFEV1% and ppoDLco% are calculated...
July 2017: Kyobu Geka. the Japanese Journal of Thoracic Surgery
https://www.readbyqxmd.com/read/28783251/safety-and-efficacy-of-allogeneic-lung-spheroid-cells-in-a-mismatched-rat-model-of-pulmonary-fibrosis
#10
Jhon Cores, M Taylor Hensley, Kathryn Kinlaw, S Michaela Rikard, Phuong-Uyen Dinh, Dipti Paudel, Junnan Tang, Adam C Vandergriff, Tyler A Allen, Yazhou Li, Jianhua Liu, Bo Niu, Yuepeng Chi, Thomas Caranasos, Leonard J Lobo, Ke Cheng
Idiopathic pulmonary fibrosis is a devastating interstitial lung disease characterized by the relentless deposition of extracellular matrix causing lung distortions and dysfunctions. The prognosis after detection is merely 3-5 years and the only two Food and Drug Administration-approved drugs treat the symptoms, not the disease, and have numerous side effects. Stem cell therapy is a promising treatment strategy for pulmonary fibrosis. Current animal and clinical studies focus on the use of adipose or bone marrow-derived mesenchymal stem cells...
August 7, 2017: Stem Cells Translational Medicine
https://www.readbyqxmd.com/read/28777013/investigational-drugs-for-idiopathic-pulmonary-fibrosis
#11
Francesco Varone, Giuliano Montemurro, Francesco Macagno, Mariarosaria Calvello, Emanuele Conte, Enrica Intini, Bruno Iovene, Paolo Maria Leone, Pier-Valerio Mari, Luca Richeldi
IPF is a specific form of chronic fibrosing interstitial pneumonia of unknown cause, characterized by progressive worsening in lung function and an unfavorable prognosis. Current concepts on IPF pathogenesis are based on a dysregulated wound healing response, leading to an over production of extracellular matrix. Based on recent research however, several other mechanisms are now proposed as potential targets for novel therapeutic strategies. Areas covered: This review analyzes the current investigational strategies targeting extracellular matrix deposition, tyrosine-kinase antagonism, immune and autoimmune response, and cell-based therapy...
August 10, 2017: Expert Opinion on Investigational Drugs
https://www.readbyqxmd.com/read/28775045/prevalence-and-incidence-of-interstitial-lung-diseases-in-a-multi-ethnic-county-of-greater-paris
#12
Boris Duchemann, Isabella Annesi-Maesano, Camille Jacobe de Naurois, Shreosi Sanyal, Pierre-Yves Brillet, Michel Brauner, Marianne Kambouchner, Sophie Huynh, Jean Marc Naccache, Raphael Borie, Jacques Piquet, Arsène Mekinian, Jerôme Virally, Yurdagul Uzunhan, Jacques Cadranel, Bruno Crestani, Olivier Fain, Francois Lhote, Robin Dhote, Nathalie Saidenberg-Kermanac'h, Paul-André Rosental, Dominique Valeyre, Hilario Nunes
The objective of the study was to estimate the prevalence and incidence of interstitial lung diseases (ILDs) in Seine-Saint-Denis, a multi-ethnic county of Greater Paris, France.Patients with ILDs were identified between January and December 2012 by using several sources; all potentially involved medical specialists from public and private hospitals, community-based pulmonologists and general practitioners, and the Social Security system. Diagnoses were validated centrally by an expert multidisciplinary discussion...
August 2017: European Respiratory Journal: Official Journal of the European Society for Clinical Respiratory Physiology
https://www.readbyqxmd.com/read/28774636/sleep-as-a-new-target-for-improving-outcomes-in-idiopathic-pulmonary-fibrosis-ipf
#13
REVIEW
Charalampos Mermigkis, Izolde Bouloukaki, Sophia E Schiza
Idiopathic pulmonary fibrosis (IPF) represents the most common type of interstitial pneumonias but remains a disease with a poor outcome. In recent years, two drugs (pirfenidone and nintedanib) have shown promising results at stalling disease progression, however, the interplay of sleep disruption or sleep disorders overall and in relation to medication effectiveness remains understudied. In the past, there was limited interest related to the role of sleep in IPF. Treating physicians tended to point only on the daily disabling symptoms disregarding the possible significant role of sleep alterations or co-existing sleep disorders...
July 31, 2017: Chest
https://www.readbyqxmd.com/read/28770888/spectrum-of-interstitial-lung-disease-from-a-tertiary-care-hospital-in-karachi
#14
Ali Bin Sarwar Zubairi, Maryam Hassan, Talha Shahzad, Sajjad Sarwar, Aamir Abbas, Huzaifa Ahmad, Muhammad Irfan
OBJECTIVE: To determine the clinical features and patterns of interstitial lung disease. METHODS: This retrospective study was conducted at the Aga Khan University Hospital, Karachi, and comprised record of patients diagnosed with interstitial lung disease from January 2005 to December 2015. All patients aged 16 years and above diagnosed with interstitial lung disease on the basis of clinical features, radiological features on high-resolution computed tomography of the chest, and lung biopsies were included...
July 2017: JPMA. the Journal of the Pakistan Medical Association
https://www.readbyqxmd.com/read/28770204/development-of-a-multivariate-prediction-model-for-early-onset-bronchiolitis-obliterans-syndrome-and-restrictive-allograft-syndrome-in-lung-transplantation
#15
Angela Koutsokera, Pierre J Royer, Jean P Antonietti, Andreas Fritz, Christian Benden, John D Aubert, Adrien Tissot, Karine Botturi, Antoine Roux, Martine L Reynaud-Gaubert, Romain Kessler, Claire Dromer, Sacha Mussot, Hervé Mal, Jean-François Mornex, Romain Guillemain, Christiane Knoop, Marcel Dahan, Paola M Soccal, Johanna Claustre, Edouard Sage, Carine Gomez, Antoine Magnan, Christophe Pison, Laurent P Nicod
BACKGROUND: Chronic lung allograft dysfunction and its main phenotypes, bronchiolitis obliterans syndrome (BOS) and restrictive allograft syndrome (RAS), are major causes of mortality after lung transplantation (LT). RAS and early-onset BOS, developing within 3 years after LT, are associated with particularly inferior clinical outcomes. Prediction models for early-onset BOS and RAS have not been previously described. METHODS: LT recipients of the French and Swiss transplant cohorts were eligible for inclusion in the SysCLAD cohort if they were alive with at least 2 years of follow-up but less than 3 years, or if they died or were retransplanted at any time less than 3 years...
2017: Frontiers in Medicine
https://www.readbyqxmd.com/read/28767680/thoracoabdominal-asynchrony-two-methods-in-healthy-copd-and-interstitial-lung-disease-patients
#16
Mayra Caleffi Pereira, Desiderio Cano Porras, Adriana Claudia Lunardi, Cibele Cristine Berto Marques da Silva, Renata Cléia Claudino Barbosa, Letícia Zumpano Cardenas, Renata Pletsch, Jeferson George Ferreira, Isac de Castro, Celso Ricardo Fernandes de Carvalho, Pedro Caruso, Carlos Roberto Ribeiro de Carvalho, André Luis Pereira de Albuquerque
BACKGROUND: Thoracoabdominal asynchrony is the nonparallel motion of the ribcage and abdomen. It is estimated by using respiratory inductive plethysmography and, recently, using optoelectronic plethysmography; however the agreement of measurements between these 2 techniques is unknown. Therefore, the present study compared respiratory inductive plethysmography with optoelectronic plethysmography for measuring thoracoabdominal asynchrony to see if the measurements were similar or different...
2017: PloS One
https://www.readbyqxmd.com/read/28765242/pulmonary-involvement-in-antineutrophil-cytoplasmic-antibodies-anca-associated-vasculitis-the-influence-of-anca-subtype
#17
Aladdin J Mohammad, Kristian H Mortensen, Judith Babar, Rona Smith, Rachel B Jones, Daiki Nakagomi, Pasupathy Sivasothy, David R W Jayne
OBJECTIVE: To describe pulmonary involvement at time of diagnosis in antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV), as defined by computed tomography (CT). METHODS: Patients with thoracic CT performed on or after the onset of AAV (n = 140; 75 women; granulomatosis with polyangiitis, n = 79; microscopic polyangiitis MPA, n = 61) followed at a tertiary referral center vasculitis clinic were studied. Radiological patterns of pulmonary involvement were evaluated from the CT studies using a predefined protocol, and compared to proteinase 3 (PR3)-ANCA and myeloperoxidase (MPO)-ANCA specificity...
August 1, 2017: Journal of Rheumatology
https://www.readbyqxmd.com/read/28763468/predictors-of-lung-function-test-severity-and-outcome-in-systemic-sclerosis-associated-interstitial-lung-disease
#18
Noémie Le Gouellec, Alain Duhamel, Thierry Perez, Anne-Lise Hachulla, Vincent Sobanski, Jean-Baptiste Faivre, Sandrine Morell-Dubois, Marc Lambert, Pierre-Yves Hatron, Eric Hachulla, Hélène Béhal, Regis Matran, David Launay, Martine Remy-Jardin
Systemic sclerosis-related interstitial lung disease (SSc-ILD) is the leading cause of death in SSc. In this study, we aimed to describe the baseline severity and evolution of forced vital capacity (FVC) and diffusing capacity for carbon monoxide (DLCO) in patients with SSc-ILD and to assess the baseline clinical, biological and high-resolution CT scan (HRCT) predictors of this evolution. Baseline and serial FVC and DLCO were collected in 75 SSc-ILD patients followed during 6.4±4.2 years (n = 557 individual data)...
2017: PloS One
https://www.readbyqxmd.com/read/28761806/scleroderma-related-interstitial-lung-disease
#19
Sally Suliman, Abdalhamid Al Harash, William Neil Roberts, Rafael L Perez, Jesse Roman
Scleroderma-related interstitial lung disease (SSc-ILD) is a pulmonary fibrosing disorder characterized by systemic inflammation and progressive scarring of the lungs that leads to respiratory failure. Although certain immunosuppressive therapies may slow disease progression, current treatment strategies are not curative; consequently, SSc-ILD continues to be a major cause of morbidity and mortality. We present four cases of SSc-ILD that emphasize the importance of early screening and detection, close follow-up, and aggressive management...
2017: Respiratory Medicine Case Reports
https://www.readbyqxmd.com/read/28758849/clinical-profile-of-unclassifiable-interstitial-lung-disease-comparison-with-chronic-fibrosing-idiopathic-interstitial-pneumonias
#20
Daniel Traila, Cristian Oancea, Emanuela Tudorache, Ovidiu Fira Mladinescu, Bogdan Timar, Voicu Tudorache
Objective Unclassifiable interstitial lung disease (ILD) is a common problem in clinical practice. These patients pose a distinct challenge with regard to appropriate evaluation and management. We investigated the clinical features and prognosis of unclassifiable ILD and compared its clinical profile with that of idiopathic pulmonary fibrosis (IPF) and idiopathic nonspecific interstitial pneumonia (NSIP). Methods Patients with IPF (n = 40), NSIP (n = 14), and unclassifiable ILD (n = 27) were selected from an ongoing database...
January 1, 2017: Journal of International Medical Research
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