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Zahava Vadasz, Alexandra Balbir Gurman, Pierluigi Meroni, Dominique Farge, Yair Levi, Francesca Ingegnoli, Yolada Braun-Moscovici, Itzhak Rosner, Gleb Slobodin, Michael Rozenbaum, Nizar Jiries, Lisa Kaly, Nina Boulman, Karina Zilber, Shira Ginsberg, Abid Awisat, Yair Goldberg, Michael Lurie, Maria-Rosa Ghigna, Christophe Guignabert, Marc Humbert, Doron Rimar
OBJECTIVE: Lysyl oxidase (LOX) is an extracellular enzyme that cross-links collagen fibrils. LOX was found to be increased in serum of SSc patients and was suggested to be related to skin fibrosis, yet a vascular source of LOX has been demonstrated in idiopathic pulmonary arterial hypertension (iPAH). We aimed to validate elevated LOX serum levels in SSc and to study its correlation with clinical characteristics and investigate its main source at the tissue level. METHODS: A total of 86 established SSc patients were compared with 86 patients with very early diagnosis of systemic sclerosis (VEDOSS), 110 patients with primary RP (PRP) and 80 healthy controls...
February 15, 2019: Rheumatology
Susan K Mathai, David A Schwartz
Pulmonary fibrosis refers to the development of diffuse parenchymal abnormalities in the lung that cause dyspnea, cough, hypoxemia, and impair gas exchange, ultimately leading to respiratory failure. Though pulmonary fibrosis can be caused by a variety of underlying etiologies, ranging from genetic defects to autoimmune diseases to environmental exposures, once fibrosis develops it is irreversible and most often progressive, such that fibrosis of the lung is one of the leading indications for lung transplantation...
February 5, 2019: Translational Research: the Journal of Laboratory and Clinical Medicine
Eduardo Luis Callejas-Moraga, Alfredo Guillén-Del-Castillo, Ana María Marín-Sánchez, María Roca-Herrera, Eva Balada, Carles Tolosa-Vilella, Vicente Fonollosa-Pla, Carmen Pilar Simeón-Aznar
OBJECTIVES: To evaluate the clinical features and survival of patients with positive anti-RNA polymerase III (anti-RNAP III) in a Spanish single centre. METHODS: We analysed 221 patients with SSc according to LeRoy and Medsger criteria. Twenty-six patients with positivity for anti-RNAP III antibodies were compared with 195 negative patients. Epidemiological, clinical, immunological features and survival were analysed. RESULTS: In patients with anti-RNAP III positivity diffuse cutaneous SSc (dcSSc) subset was the most prevalent (20, 76...
February 11, 2019: Clinical and Experimental Rheumatology
Jeong Seok Lee, Eun Young Lee, You-Jung Ha, Eun Ha Kang, Yun Jong Lee, Yeong Wook Song
BACKGROUND: Biomarkers have been actively investigated to supplement functional and imaging modalities to predict the severity, therapeutic responsiveness, and progression of connective tissue disease-associated interstitial lung disease (CTD-ILD). This study aimed to evaluate Krebs von den Lungen 6 (KL-6) as a potential biomarker reflecting the severity of CTD-ILD as assessed through computed tomography (CT) and pulmonary function test (PFT) parameters. METHODS: This retrospective study included 549 Korean patients with rheumatoid arthritis, systemic sclerosis, inflammatory myositis, and other CTDs with or without concurrent ILD...
February 14, 2019: Arthritis Research & Therapy
Vaishali Kapoor, Abhay Kumar Singh, Buck E Rogers, Dinesh Thotala, Dennis E Hallahan
Targeted molecular imaging allows specific visualization and monitoring of tumors. Cancer-specific peptides have been developed for imaging and therapy. Peptides that specifically target cancer have several advantages including, ease of synthesis, low antigenicity, and enhanced diffusion into tissues. We developed the HVGGSSV peptide as a molecular targeting/imaging agent. HVGGSSV targets Tax interacting protein 1 (TIP1) which is a 14 kDa PDZ domain-containing protein that is overexpressed in cancer. We docked HVGGSSV in silico using the three-dimensional structure of TIP1 and found the binding energy was -6...
February 11, 2019: Journal of Controlled Release: Official Journal of the Controlled Release Society
Amber Young, Dharshan Vummidi, Scott Visovatti, Kate Homer, Holly Wilhalme, Eric S White, Kevin Flaherty, Vallerie McLaughlin, Dinesh Khanna
OBJECTIVE: Systemic sclerosis (SSc) is associated with interstitial lung disease (ILD) and pulmonary hypertension (PH). We sought to determine prevalence, characteristics, treatment, and outcomes for subjects with PH in a SSc-associated ILD (SSc-ILD) cohort. METHODS: Subjects with SSc-ILD on high resolution computed tomography (HRCT) were included in a prospective observational cohort. Subjects had been screened for PH based on a standardized screening algorithm and underwent right heart catheterization (RHC), if indicated...
February 14, 2019: Arthritis & Rheumatology
Ahmed Abdel Khalek Abdel Razek, Mahmoud Thabet, Eman Abdel Salam
PURPOSE: This study aimed to assess apparent diffusion coefficient (ADC) of the placenta and fetal organs in intrauterine growth restriction (IUGR). MATERIALS AND METHODS: A prospective study of 30 consecutive pregnant women (aged 21-38 years with mean age of 31.5 years and a mean gestational week of 35 ± 2.3) with IUGR and 15 age-matched pregnant women was conducted. All patients and controls underwent diffusion-weighted magnetic resonance imaging. The ADCs of the placenta and fetal brain, kidney, and lung were calculated and correlated with neonates needing intensive care unit (ICU) admission...
February 11, 2019: Journal of Computer Assisted Tomography
Daniela A B Cervilha, Juliana T Ito, Juliana D Lourenço, Clarice R Olivo, Beatriz M Saraiva-Romanholo, Rildo A Volpini, Manoel C Oliveira-Junior, Thais Mauad, Milton A Martins, Iolanda F L C Tibério, Rodolfo P Vieira, Fernanda D T Q S Lopes
We proposed an experimental model to verify the Th17/Treg cytokine imbalance in COPD exacerbation. Forty C57BL/6 mice were exposed to room air or cigarette smoke (CS) (12 ± 1 cigarettes, twice a day, 30 min/exposure and 5 days/week) and received saline (50 µl) or lipopolysaccharide (LPS) (1 mg/kg in 50 µl of saline) intratracheal instillations. We analyzed the mean linear intercept, epithelial thickness and inflammatory profiles of the bronchoalveolar lavage fluid and lungs. We evaluated macrophages, neutrophils, CD4+ and CD8+ T cells, Treg cells, and IL-10+ and IL-17+ cells, as well as STAT-3, STAT-5, phospho-STAT3 and phospho-STAT5 levels using immunohistochemistry and IL-17, IL-6, IL-10, INF-γ, CXCL1 and CXCL2 levels using ELISA...
February 13, 2019: Scientific Reports
Oksana Sokhatska, Eva Padrão, Bernardo Sousa-Pinto, Marília Beltrão, Patrícia Caetano Mota, Natália Melo, Luís Delgado, António Morais
BACKGROUND: Diffuse lung diseases (DLD) are characterized by different immunophenotypes in the bronchoalveolar lavage fluid (BALF). We aimed to evaluate the diagnostic value of BALF NK and NKT cell counts of patients with DLD and lymphocytic alveolitis. METHODS: We assessed 202 patients with DLD, who underwent BALF immunophenotyping. Samples were routinely processed by flow cytometry and lymphocyte subsets were compared between patients with sarcoidosis (n = 106), hypersensitivity pneumonitis (HP; n = 53), and other DLDs (n = 43)...
February 13, 2019: BMC Pulmonary Medicine
David Bennett, Rosa Metella Refini, Maria Lucia Valentini, Annalisa Fui, Antonella Fossi, Maria Pieroni, Maria Antonietta Mazzei, Paola Rottoli
INTRODUCTION: Familial pulmonary fibrosis (FPF) is defined as an idiopathic diffuse parenchymal lung disease affecting two or more members of the same primary biological family. The aim of this study was to compare disease progression and tolerance to pirfenidone in a population of FPF patients who presented with radiological and/or histological evidence of UIP, and a group of idiopathic pulmonary fibrosis (IPF) patients. METHODS: Seventy-three patients (19 with FPF and 54 with IPF) were enrolled and data were collected retrospectively at 6, 12 and 24 months follow-up...
February 13, 2019: Lung
Na Zhang, Mengtao Li, Junyan Qian, Qian Wang, Jiuliang Zhao, Zhenwen Yang, Zhuang Tian, Xiao Zhang, Xiaoxia Zuo, Miaojia Zhang, Ping Zhu, Shuang Ye, Wei Zhang, Yi Zheng, Wufang Qi, Yang Li, Zhuoli Zhang, Feng Ding, Jieruo Gu, Yi Liu, Wei Wei, Xiaofeng Zeng
AIM: Pulmonary arterial hypertension (PAH) is a complex and devastating complication of systemic lupus erythematosus (SLE). We sought to describe the baseline characteristics of right heart catheterization (RHC)-confirmed SLE-associated PAH and identify risk factors for PAH in SLE patients. METHODS: A multicenter, cross-sectional study was conducted using the Chinese SLE Treatment and Research group (CSTAR) registry. Baseline data for patients with SLE-associated PAH and SLE patients without PAH were collected and compared...
February 11, 2019: International Journal of Rheumatic Diseases
Vasin Vasikasin, Worapong Nasomsong, Chutika Srisuttiyakorn, Wat Mitthamsiri, Nittha Oer-Areemitr, Dhitiwat Changpradub
Disseminated phaeohyphomycosis is an extremely rare clinical syndrome, especially in a host without apparent immunological defect. Here, we report a case of disseminated phaeohyphomycosis in a 22-year-old previously healthy man who showed nonmassive hemoptysis from diffuse lung nodules and cavities, together with a hard palate ulcer and generalized subcutaneous nodules. Histopathology, cultures and subsequent molecular assay from two different sites confirmed Curvularia tuberculata infection. The patient was successfully treated with amphotericin B and itraconazole...
February 11, 2019: Mycopathologia
Masanori Yokoba, Tsuyoshi Ichikawa, Shinya Harada, Masahito Naito, Yukitoshi Sato, Masato Katagiri
Background: Pulmonary function and patient complaints appear to improve up to 12 months after lobectomy but long-term prospective studies based on clinical data are scarce. Improvement in pulmonary function may depend on the area and extent of the resection and the time from the operation. This prospective study aimed to determine pulmonary function changes according to the resected lobe. Methods: This prospective study included 59 patients requiring single lobectomy...
December 2018: Journal of Thoracic Disease
Vu Nguyet Minh, Khang Tran Hau, Matsushita Takashi, Vinh Nguyen Ha, Long Hoang Bao, My Le Huyen, Doanh Le Huu, Thuong Nguyen Van, Marco Gandolfi, Francesca Satolli, Claudio Feliciani, Michael Tirant, Aleksandra Vojvodic, Torello Lotti
BACKGROUND: B-cell activating factor (BAFF) is considered to have a role in the pathogenesis of systemic sclerosis (SSc). AIM: We conducted a longitudinal study on early SSc patients to determine the change in BAFF serum level after treatment and its association with organ involvements. METHODS: A total of 46 patients (32 diffuse, 14 limited) were recruited, among which 35 patients (24 diffuse, 11 limited) completed 12-month follow-up. RESULTS: Higher pretreatment BAFF levels were observed in patients with positive anti-topoisomerase antibody (ATA) (2252...
January 30, 2019: Open Access Macedonian Journal of Medical Sciences
Naoyuki Kuse, Minoru Inomata, Nobuyasu Awano, Hanako Yoshimura, Tatsunori Jo, Mari Tone, Atsuko Moriya, Yuan Bae, Toshio Kumasaka, Tamiko Takemura, Takehiro Izumo
BACKGROUND: Transbronchial lung cryobiopsy (TBLC), which is transbronchial lung biopsy performed using a cryoprobe, has emerged as a new method for obtaining lung tissue specimens for diagnosing pulmonary diseases. TBLC has been widely performed, and many reports have documented its safety and feasibility in diagnosing pulmonary diseases such as lung cancer and diffuse parenchymal lung disease. However, to date, no reports have yet been published from Japan. Therefore, the purpose of this study was to describe our experience with TBLC in daily practice in Japan and compare it with those reported in previous studies from other countries...
February 8, 2019: Respiratory Investigation
Lijian Xie, Yun Li, Xunwei Jiang, Jian Zhao, Tingting Xiao
BACKGROUND: Abernethy malformation is an extremely rare congenital malformation characterised by an extrahepatic portosystemic shunt. Children with Abernathy malformation can develop hepatopulmonary syndrome (HPS) with pulmonary arteriovenous fistulas (PAVF) or pulmonary hypertension. PAVF manifests as central cyanosis with effort intolerance. We report a case of PAVF in a Ten-year-old Boy. Persistent symptoms identified Abernathy malformation as the cause of progressive symptoms and current understanding of this rare malformation is reviewed...
February 11, 2019: BMC Pediatrics
Marike Lijkendijk, Peter B Licht, Kirsten Neckelmann
BACKGROUND: Management of chest drains following thoracic surgery remains an area with little consensus. To optimize our chest drainage algorithms with electronic chest drainage systems we conducted a randomized controlled trial comparing low variable suction (-5 cm H2 O) versus high variable suction (-20 cm H2 O). METHODS: We did a prospective open label randomized trial in patients undergoing lobectomy. Sample size was calculated from a clinical relevant difference in chest drain duration as one full day...
February 8, 2019: Annals of Thoracic Surgery
Xingan Wang, Michael Emmet O'Brien, Junyi Yu, Liangfang Fu, Qiang Zhang, Songjian Lu, Xiaojing An, John F McDyer, Rama K Mallampalli
Primary graft dysfunction (PGD), a major cause of morbidity after lung transplantation, is linked to ischemia-reperfusion injury (IRI). Other factors such as preceding brain death (BD), hemorrhagic shock (HS), and pre-engraftment lung management are also important. We hypothesized that a multi-hit isogenic mouse model of lung transplantation is more closely linked to PGD than IRI alone. Left lung transplants were performed between inbred mice. A one-hit model of IRI was established by inducing cold ischemia (CI) to the donor lung at 0°C for 1h, 72h or 96h...
February 11, 2019: American Journal of Respiratory Cell and Molecular Biology
Shun-Hua Chen, Angel Chao, Chia-Lung Tsai, Shih-Che Sue, Chiao-Yun Lin, Yi-Zong Lee, Yi-Lin Hung, An-Shine Chao, Ann-Joy Cheng, Hsin-Shih Wang, Tzu-Hao Wang
The delivery of active proteins into cells (protein transfection) for biological purposes offers considerable potential for clinical applications. Herein we demonstrate that, with a readily available, inexpensive organic agent, the 4-(2-hydroxyethyl)-1-piperazineethanesulfonic acid (HEPES) method can be used for simple and efficient protein transfection. By mixing proteins with a pure HEPES solution before they are applied to live cells, proteins with various molecular weights (including antibodies, recombinant proteins, and peptides) were successfully delivered into the cytoplasm of different cell types...
June 14, 2019: Molecular Therapy. Methods & Clinical Development
Panpan Qian, Chun Hong Peng, Xianwei Ye
Introduction: Recently, interstitial lung disease significantly increases and it is difficult to treat. Cyclophosphamide(CP) is one drug administrated in interstitial lung disease, which can also cause pulmonary fibrosis and lung function lesion. This article present a case which exacerbated interstitial pneumonia after treatment by CP, aiming to enhance the understanding of the side effects of CP and standardize usage of the CP. Case presentation: A patient of nephrotic syndrome administrated with CP suffered respiratory insufficiency requiring mechanical ventilation...
2019: Respiratory Medicine Case Reports
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