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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/28808495/the-most-frequent-abca3-nonsense-mutation-p-tyr1515-y1515x-causing-lethal-neonatal-respiratory-failure-in-a-term-neonate
#2
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/28805996/what-can-we-learn-from-pulmonary-function-testing-in-heart-failure
#3
REVIEW
Helgo Magnussen, Marco Canepa, Peter Emilio Zambito, Vito Brusasco, Thomas Meinertz, Stephan Rosenkranz
Pulmonary diseases frequently coexist in heart failure (HF), thus posing diagnostic and therapeutic challenges to cardiologists evaluating patients with overlapping symptoms and implementing recommended HF treatments. There is a growing body of evidence suggesting that pulmonary function testing might provide useful information for the best management of these patients. The availability of portable devices, allowing the measurement of spirometry and lung diffusion capacity for carbon monoxide outside of hospital-based pulmonary lung function laboratories, provides an opportunity for a more widespread use of these measures in the cardiology community, but their interpretation can be challenging...
August 14, 2017: European Journal of Heart Failure
https://www.readbyqxmd.com/read/28796717/utility-of-flexible-bronchoscopic-cryobiopsy-for-diagnosis-of-diffuse-parenchymal-lung-diseases
#4
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/28794368/interstitial-lung-disease-induced-by-osimertinib-for-epidermal-growth-factor-receptor-egfr-t790m-positive-non-small-cell-lung-cancer
#5
Yoshiya Matsumoto, Tomoya Kawaguchi, Norio Yamamoto, Kenji Sawa, Naoki Yoshimoto, Tomohiro Suzumura, Tetsuya Watanabe, Shigeki Mitsuoka, Kazuhisa Asai, Tatsuo Kimura, Naruo Yoshimura, Yuko Kuwae, Kazuto Hirata
A 75-year-old man with stage IV lung adenocarcinoma was treated with osimertinib due to disease progression despite having been administered erlotinib. Both an epidermal growth factor receptor (EGFR) L858R mutation on exon 21 and a T790M mutation on exon 20 were detected in a specimen from a recurrent primary tumor. Five weeks after osimertinib initiation, he developed general fatigue and dyspnea. Chest computed tomography scan revealed diffuse ground glass opacities and consolidation on both lungs. An analysis of the bronchoalveolar lavage fluid revealed marked lymphocytosis, and a transbronchial lung biopsy specimen showed a thickened interstitium with fibrosis and prominent lymphocytic infiltration...
August 10, 2017: Internal Medicine
https://www.readbyqxmd.com/read/28774811/the-interaction-of-dendrimer-doxorubicin-conjugates-with-a-model-pulmonary-epithelium-and-their-cosolvent-free-pseudo-solution-formulations-in-pressurized-metered-dose-inhalers
#6
Qian Zhong, Bruno V Humia, Alisha R Punjabi, Francine F Padilha, Sandro R P da Rocha
Oral inhalation (OI) of nano-chemotherapeutics holds great potentials in the treatment of lung cancers as it enables direct targeting of drugs to lung tissues, spatial and temporal control of drug release, and decrease in drug-associated systemic and local lung toxicity. Therefore, the design of chemistry of the nanocarriers and their OI formulations for chemotherapeutics delivery to the peripheral lungs and extrapulmonary tissues of relevance such as lymph nodes, may thus afford new opportunities for treating such relevant diseases...
July 31, 2017: European Journal of Pharmaceutical Sciences
https://www.readbyqxmd.com/read/28766543/-bone-marrow-involvement-in-primary-mediastinal-b-cell-lymphoma
#7
A U Magomedova, E A Fastova, A M Kovrigina, T N Obukhova, N I Skidan, Ya K Mangasarova, A I Vorobyev, S K Kravchenko
Primary mediastinal large B-cell lymphoma (PMBCL) is a distinct type of large B-cell lymphoma. In this type of the disease, the neoplastic process is located in the anterior and superior mediastinum, frequently with compression of the superior vena cava and with tumor invasion into the adjacent organs and tissues: the pericardium, lung, pleura, etc. Despite the fact that in PMBCL progression, there may be involvement of extranodal organs, such as the kidney, adrenal glands, liver, and central nervous system, bone marrow (BM) injury is generally absent...
2017: Terapevticheskiĭ Arkhiv
https://www.readbyqxmd.com/read/28766540/-leukemization-of-follicular-lymphoma-the-features-of-diagnostic-and-clinical-course-of-a-rare-form-of-the-disease
#8
E S Nesterova, S K Kravchenko, Ya K Mangasarova, L V Plastinina, V N Dvirnyk, A M Kovrigina, I A Shchupletsova, T N Obukhova, E G Gemdzhian, I A Vorobyev, A I Vorobyev
AIM: To characterize a group of patients with follicular lymphoma (FL) with leukemization and to evaluate the efficiency of different therapy options (R-CHOP/R-FMC/high-dose chemotherapy (HDCT)). SUBJECTS AND METHODS: 18 (7.2%) out of 250 patients diagnosed with FL, who were examined and treated at the National Research Center for Hematology, Ministry of Health of the Russian Federation, were found to have leukemic FL (tumor cells in the peripheral blood smears were detected by cytology and flow cytofluorometry...
2017: Terapevticheskiĭ Arkhiv
https://www.readbyqxmd.com/read/28763468/predictors-of-lung-function-test-severity-and-outcome-in-systemic-sclerosis-associated-interstitial-lung-disease
#9
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/28760854/oncogenic-kras-and-p53-loss-drive-gastric-tumorigenesis-in-mice-that-can-be-attenuated-by-e-cadherin-expression
#10
Jacob Till, Changhwan Yoon, Bang-Jin Kim, Kerry C Roby, Prince Addai, Evan Jonokuchi, Laura H Tang, Sam S Yoon, Sandra Ryeom
Gastric adenocarcinoma (GA) is the third leading cause of cancer-related death worldwide, but no models exist to readily investigate distant metastases which are mainly responsible for mortality in this disease. Here we report the development of a genetically engineered mouse model of GA tumorigenesis based on Kras(G12D) expression plus inactivation of E-cadherin (Cdh1) and p53 in the gastric parietal cell lineage. Intestinal and diffuse gastric tumors arise rapidly in this model, which displays a median survival of 76 days...
July 31, 2017: Cancer Research
https://www.readbyqxmd.com/read/28760669/an-immunoproteomic-approach-revealed-antigenic-proteins-enhancing-serodiagnosis-performance-of-bird-fancier-s-lung
#11
Adeline Rouzet, Gabriel Reboux, Jean-Charles Dalphin, Anne Gondouin, Paul De Vuyst, Thierry Balliau, Laurence Millon, Benoit Valot, Sandrine Roussel
BACKGROUND: Bird fancier's lung (BFL) caused by repeated inhalation of avian proteins is the most common form of hypersensitivity pneumonitis. However, the exact identification of proteins involved is unknown, and serological test use for diagnosis need to be standardized. The objectives of this study were (i) to identify antigenic proteins from pigeon droppings (ii) to provide information about their location in avian matrices and (iii) to produce them in recombinant proteins to evaluate their diagnostic performances...
July 28, 2017: Journal of Immunological Methods
https://www.readbyqxmd.com/read/28758849/clinical-profile-of-unclassifiable-interstitial-lung-disease-comparison-with-chronic-fibrosing-idiopathic-interstitial-pneumonias
#12
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
https://www.readbyqxmd.com/read/28758573/shrinking-lung-syndrome-in-systemic-lupus-erythematosus-a-single-centre-experience
#13
M Deeb, K Tselios, D D Gladman, J Su, M B Urowitz
Introduction Shrinking lung syndrome (SLS) is a rare manifestation of systemic lupus erythematosus (SLE), characterized by decreased lung volumes and extra-pulmonary restriction. The aim of this study was to describe the characteristics of SLS in our lupus cohort with emphasis on prevalence, presentation, treatment and outcomes. Patients and methods Patients attending the Toronto Lupus Clinic since 1980 ( n = 1439) and who had pulmonary function tests (PFTs) performed during follow-up were enrolled ( n = 278)...
January 1, 2017: Lupus
https://www.readbyqxmd.com/read/28754682/autoimmunity-to-vimentin-is-associated-with-outcomes-of-patients-with-idiopathic-pulmonary-fibrosis
#14
Fu Jun Li, Ranu Surolia, Huashi Li, Zheng Wang, Tejaswini Kulkarni, Gang Liu, Joao A de Andrade, Daniel J Kass, Victor J Thannickal, Steven R Duncan, Veena B Antony
Autoimmunity has been implicated in the pathogenesis of idiopathic pulmonary fibrosis (IPF); however, the repertoire of autoantigens involved in this disease and the clinical relevance of these autoimmune responses are still being explored. Our initial discovery assays demonstrated that circulating and intrapulmonary vimentin levels are increased in IPF patients. Subsequent studies showed native vimentin induced HLA-DR-dependent in vitro proliferation of CD4 T cells from IPF patients and enhanced the production of IL-4, IL-17, and TGF-β1 by these lymphocytes in contrast to normal control specimens...
July 28, 2017: Journal of Immunology: Official Journal of the American Association of Immunologists
https://www.readbyqxmd.com/read/28752492/pulmonary-involvement-in-systemic-vasculitis
#15
REVIEW
Luis Felipe Flores-Suárez, Marco A Alba, Heidegger Mateos-Toledo, Natllely Ruiz
PURPOSE OF REVIEW: The purpose of this study is to describe the most relevant advances concerning lung involvement in the ANCA-associated vasculitides (excluding eosinophilic granulomatosis with polyangiitis which may have different disease mechanisms). Focus is on pathophysiology, recent important imagenological procedures, treatment, and outcome. RECENT FINDINGS: Emerging information exists on potential newly investigated diagnostic procedures (v.g. transbronchial cryobiopsies), detailed tomographic abnormalities, the potential favorable role of rituximab and the still uncertain one of plasma exchange in the treatment, and the increasing description of interstitial lung disease...
September 2017: Current Rheumatology Reports
https://www.readbyqxmd.com/read/28749191/disease-related-autoantibody-profile-in-patients-with-systemic-sclerosis
#16
Christos Liaskos, Emmanouela Marou, Theodora Simopoulou, Maria Barmakoudi, Georgios Efthymiou, Thomas Scheper, Wolfgang Meyer, Dimitrios P Bogdanos, Lazaros I Sakkas
BACKGROUND: Autoantibodies (autoAbs) help in diagnosis and predicting clinical phenotypes in systemic sclerosis (SSc). AIM OF THE STUDY: To determine the clinical utility of 13 SSc-related autoAbs in SSc patients. MATERIAL AND METHODS: A total of 131 consecutive patients with SSc (111 female, mean age 58.1 ± 14 years; 49 with diffused cutaneous SSc [dcSSc] and 82 with limited cutaneous SSc [lcSSc]) were analysed by a multiplex line immunoassay (Euroimmun) for autoantibodies (autoAbs) against 13 SSc-related antigens...
July 27, 2017: Autoimmunity
https://www.readbyqxmd.com/read/28744781/lung-function-in-pregnancy-in-langerhans-cell-histiocytosis
#17
Elżbieta Radzikowska, Elżbieta Wiatr, Monika Franczuk, Iwona Bestry, Kazimierz Roszkowski-Śliż
Pulmonary Langerhans cell histiocytosis (LCH) is a rare disease, affecting usually young people. The course of the disease is variable. In some pulmonary LCH patients a severe lung destruction and progression in spite of chemotherapy is observed, but in others just a cessation of smoking induces a regression of the disease. In the present study we seek to determine the influence of pregnancy on pulmonary function in LCH patients, an unchartered area of research. We addressed the issue by investigating eight pregnant women out of the 45 women hospitalized with the diagnosis of pulmonary LCH in the period from 2000 to 2015...
July 26, 2017: Advances in Experimental Medicine and Biology
https://www.readbyqxmd.com/read/28743348/-advances-in-biomarkers-of-ards
#18
Xuan Fu, Jinle Lin, Wenwu Zhang, Lijun Wang
Acute respiratory distress syndrome (ARDS) is a common diseases in critical ill patients, which is an acute-onset hypoxic condition with radiographic bilateral lung infiltration. And it can be divided into two phases in pathophysiology: an acute exudative phase combining diffuse alveolar damage and lung edema followed by a later fibro proliferative phase. The early prediction and risk assessment of patients with ARDS is still very poor in the clinical. In recent years, researchers gradually focus their research on exploration of biomarkers of ARDS...
July 2017: Zhonghua Wei Zhong Bing Ji Jiu Yi Xue
https://www.readbyqxmd.com/read/28742087/type-1-skewed-neuroinflammation-and-vascular-damage-associated-with-orientia-tsutsugamushi-infection-in-mice
#19
Lynn Soong, Thomas R Shelite, Yan Xing, Harica Kodakandla, Yuejin Liang, Brandon J Trent, Paulina Horton, Kathryn C Smith, Zhenyang Zhao, Jiaren Sun, Donald H Bouyer, Jiyang Cai
BACKGROUND: Scrub typhus is a life-threatening disease, due to infection with O. tsutsugamushi, a Gram-negative bacterium that preferentially replicates in endothelial cells and professional phagocytes. Meningoencephalitis has been reported in scrub typhus patients and experimentally-infected animals; however, the neurological manifestation and its underlying mechanisms remain poorly understood. To address this issue, we focused on Orientia tsutsugamushi Karp strain (OtK), and examined host responses in the brain during lethal versus self-healing scrub typhus disease in our newly established murine models...
July 2017: PLoS Neglected Tropical Diseases
https://www.readbyqxmd.com/read/28740835/severe-pulmonary-hypertension-due-to-combined-pulmonary-fibrosis-and-emphysema-another-cause-of-death-among-smokers
#20
REVIEW
André Carramenha de Góes Hirano, Eduardo Pelegrineti Targueta, Fernando Peixoto Ferraz de Campos, João Augusto Dos Santos Martines, Dafne Andrade, Silvana Maria Lovisolo, Aloisio Felipe-Silva
In 2005, the combined pulmonary fibrosis and emphysema (CPFE) was first defined as a distinct entity, which comprised centrilobular or paraseptal emphysema in the upper pulmonary lobes, and fibrosis in the lower lobes accompanied by reduced diffused capacity of the lungs for carbon monoxide (DLCO). Recently, the fibrosis associated with the connective tissue disease was also included in the diagnosis of CPFE, although the exposure to tobacco, coal, welding, agrochemical compounds, and tire manufacturing are the most frequent causative agents...
April 2017: Autopsy & case reports
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