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https://www.readbyqxmd.com/read/29782549/sphingosine-1-phosphate-receptor-2-facilitates-pulmonary-fibrosis-through-potentiating-il-13-pathway-in-macrophages
#1
Juanjuan Zhao, Yasuo Okamoto, Yuya Asano, Kazuhiro Ishimaru, Sho Aki, Kazuaki Yoshioka, Noriko Takuwa, Takashi Wada, Yutaka Inagaki, Chiaki Takahashi, Takumi Nishiuchi, Yoh Takuwa
Idiopathic pulmonary fibrosis is a devastating disease with poor prognosis. The pathogenic role of the lysophospholipid mediator sphingosine-1-phosphate and its receptor S1PR2 in lung fibrosis is unknown. We show here that genetic deletion of S1pr2 strikingly attenuated lung fibrosis induced by repeated injections of bleomycin in mice. We observed by using S1pr2LacZ/+ mice that S1PR2 was expressed in alveolar macrophages, vascular endothelial cells and alveolar epithelial cells in the lung and that S1PR2-expressing cells accumulated in the fibrotic legions...
2018: PloS One
https://www.readbyqxmd.com/read/29780123/the-efficacy-and-safety-of-long-term-pirfenidone-therapy-in-patients-with-idiopathic-pulmonary-fibrosis
#2
Kazumasa Ogawa, Atsushi Miyamoto, Shigeo Hanada, Yui Takahashi, Kyoko Murase, Sayaka Mochizuki, Hironori Uruga, Hisashi Takaya, Nasa Morokawa, Kazuma Kishi
Objective Pirfenidone (PFD) is often used for years, but the efficacy and safety of long-term PFD therapy in patients with idiopathic pulmonary fibrosis (IPF) are not fully understood. Methods and Patients We retrospectively evaluated 46 patients with IPF who received PFD between February 2009 and August 2014. The efficacy and safety of PFD therapy were compared between 2 groups: long-term therapy patients who received PFD for over 1 year (group L, n=30, 65%) and short-term therapy patients who could not receive PFD for more than 1 year due to worsening of their condition or side effects (group S, n=16, 35%)...
May 18, 2018: Internal Medicine
https://www.readbyqxmd.com/read/29777878/pulmonary-delivery-of-polyplexes-for-combined-pai-1-gene-silencing-and-cxcr4-inhibition-to-treat-lung-fibrosis
#3
B S Ling Ding, B S Chenfei Zhu, M S Fei Yu, M S Pengkai Wu, M S Gang Chen, B S Aftab Ullah, Kaikai Wang, Minjie Sun, Jing Li, David Oupický
This report describes the development of polyplexes based on CXCR4-inhibiting poly(ethylenimine) derivative (PEI-C) for pulmonary delivery of siRNA to silence plasminogen activator inhibitor-1 (siPAI-1) as a new combination treatment of pulmonary fibrosis (PF). Safety and delivery efficacy of the PEI-C/siPAI-1 polyplexes was investigated in vitro in primary lung fibroblasts isolated from mice with bleomycin-induced PF. Biodistribution analysis following intratracheal administration of fluorescently labeled polyplexes showed prolonged retention in the lungs...
May 16, 2018: Nanomedicine: Nanotechnology, Biology, and Medicine
https://www.readbyqxmd.com/read/29774649/end-of-life-trajectory-of-coping-and-self-care-of-patients-with-idiopathic-pulmonary-fibrosis-a-meta-synthesis-using-meta-ethnography
#4
Yasuko Igai
AIM: This study aimed to extract patient narratives regarding the end-of-life trajectory of their self-care and coping experiences as patients with idiopathic pulmonary fibrosis and to use meta-ethnography to discover common metaphors of their experience in order to inform the development of nursing interventions. METHODS: A comprehensive search of qualitative research using electronic databases, in English and Japanese, regarding patients with IPF, identified four articles...
May 17, 2018: Japan Journal of Nursing Science: JJNS
https://www.readbyqxmd.com/read/29774214/the-expression-of-aqp1-is-modified-in-lung-of-patients-with-idiopathic-pulmonary-fibrosis-addressing-a-possible-new-target
#5
Ana Galán-Cobo, Elena Arellano-Orden, Rocío Sánchez Silva, José Luis López-Campos, César Gutiérrez Rivera, Lourdes Gómez Izquierdo, Nela Suárez-Luna, María Molina-Molina, José A Rodríguez Portal, Miriam Echevarría
Activation of the epithelial-mesenchymal transition process (EMT) by which alveolar cells in human lung tissue undergo differentiation giving rise to a mesenchymal phenotype (fibroblast/miofibroblasts) has been well recognized as a key element in the origin of idiopathic pulmonary fibrosis (IPF). Here we analyzed expression of AQP1 in lung biopsies of patients diagnosed with IPF, and compared it to biopsies derived from patients with diverse lung pneumonies, such as hypersensitivity pneumonitis, sarcoidosis or normal lungs...
2018: Frontiers in Molecular Biosciences
https://www.readbyqxmd.com/read/29773608/what-s-in-a-name-that-which-we-call-ipf-by-any-other-name-would-act-the-same
#6
Athol U Wells, Kevin K Brown, Kevin R Flaherty, Martin Kolb, Victor J Thannickal
Idiopathic pulmonary fibrosis (IPF) remains a truly idiopathic fibrotic disease, with a modest genetic predilection and candidate triggers but no overall explanation for the development of disease in non-familial cases. Agreement on terminology has contributed to major clinical and translational advances since the millennium. It is likely that the entity currently captured by the term "IPF" will be radically reclassified over the next decade, either through "splitting" (into IPF subgroups responding selectively to individual disease-modifying agents) or through "lumping" of IPF with other forms of progressive fibrotic lung disease (with shared pathogenetic mechanisms and IPF-like disease behaviour)...
May 2018: European Respiratory Journal: Official Journal of the European Society for Clinical Respiratory Physiology
https://www.readbyqxmd.com/read/29773606/optimising-experimental-research-in-respiratory-diseases-an-ers-statement
#7
Philippe Bonniaud, Aurélie Fabre, Nelly Frossard, Christophe Guignabert, Mark Inman, Wolfgang M Kuebler, Tania Maes, Wei Shi, Martin Stampfli, Stefan Uhlig, Eric White, Martin Witzenrath, Pierre-Simon Bellaye, Bruno Crestani, Oliver Eickelberg, Heinz Fehrenbach, Andreas Guenther, Gisli Jenkins, Guy Joos, Antoine Magnan, Bernard Maitre, Ulrich A Maus, Petra Reinhold, Juanita H J Vernooy, Luca Richeldi, Martin Kolb
Experimental models are critical for the understanding of lung health and disease and are indispensable for drug development. However, the pathogenetic and clinical relevance of the models is often unclear. Further, the use of animals in biomedical research is controversial from an ethical perspective.The objective of this task force was to issue a statement with research recommendations about lung disease models by facilitating in-depth discussions between respiratory scientists, and to provide an overview of the literature on the available models...
May 2018: European Respiratory Journal: Official Journal of the European Society for Clinical Respiratory Physiology
https://www.readbyqxmd.com/read/29772026/correction-bronchoalveolar-lavage-bal-cells-in-idiopathic-pulmonary-fibrosis-express-a-complex-pro-inflammatory-pro-repair-angiogenic-activation-pattern-likely-associated-with-macrophage-iron-accumulation
#8
https://www.readbyqxmd.com/read/29772024/use-of-human-amniotic-epithelial-cells-in-mouse-models-of-bleomycin-induced-lung-fibrosis-a-systematic-review-and-meta-analysis
#9
Fang He, Aiting Zhou, Shuo Feng
BACKGROUND: Idiopathic pulmonary fibrosis (IPF) urgently requires effective treatment. Bleomycin-induced lung injury models are characterized by initial inflammation and secondary fibrosis, consistent with the pathological features of IPF. Human amniotic epithelial cells (hAECs) exhibit good differentiation potential and paracrine activity and are thus ideal for cell-based clinical therapies. The therapeutic effects of hAECs on lung fibrosis are attributed to many factors. We performed a systematic review of preclinical studies investigating the treatment of pulmonary fibrosis with hAECs to provide suggestions for their clinical use...
2018: PloS One
https://www.readbyqxmd.com/read/29770205/beginning-at-the-ends-telomeres-and-human-disease
#10
REVIEW
Sharon A Savage
Studies of rare and common illnesses have led to remarkable progress in the understanding of the role of telomeres (nucleoprotein complexes at chromosome ends essential for chromosomal integrity) in human disease. Telomere biology disorders encompass a growing spectrum of conditions caused by rare pathogenic germline variants in genes encoding essential aspects of telomere function. Dyskeratosis congenita, a disorder at the severe end of this spectrum, typically presents in childhood with the classic triad of abnormal skin pigmentation, nail dystrophy, and oral leukoplakia, accompanied by a very high risk of bone marrow failure, cancer, pulmonary fibrosis, and other medical problems...
2018: F1000Research
https://www.readbyqxmd.com/read/29770156/probing-into-the-mechanism-of-alkaline-citrus-extract-promoted-apoptosis-in-pulmonary-fibroblasts-of-bleomycin-induced-pulmonary-fibrosis-mice
#11
Qi Wu, Yao Zhou, Fan-Chao Feng, Yi-Han Jin, Zhi-Chao Wang, Xian-Mei Zhou
We extracted the primary pulmonary fibroblasts of the normal and bleomycin-induced pulmonary fibrosis mice and investigated the functioning mechanism of citrus alkaline extract (CAE) in the induction of pulmonary fibroblast apoptosis. The expression intensity of vimentin of the pulmonary fibroblasts in the model mice was higher than that in the normal mice. Meanwhile, the positive expression rate and expression intensity of alpha smooth muscle actin ( α -SMA) of the pulmonary fibroblasts in the model mice were higher than those in the normal mice...
2018: Evidence-based Complementary and Alternative Medicine: ECAM
https://www.readbyqxmd.com/read/29769446/ubiquitination-and-deubiquitination-emerge-as-players-in-idiopathic-pulmonary-fibrosis-pathogenesis-and-treatment
#12
REVIEW
Shuang Li, Jing Zhao, Dong Shang, Daniel J Kass, Yutong Zhao
Idiopathic pulmonary fibrosis (IPF) is a fatal fibrotic lung disease that is associated with aberrant activation of TGF-β, myofibroblast differentiation, and abnormal extracellular matrix (ECM) production. Proper regulation of protein stability is important for maintenance of intracellular protein homeostasis and signaling. Ubiquitin E3 ligases mediate protein ubiquitination, and deubiquitinating enzymes (DUBs) reverse the process. The role of ubiquitin E3 ligases and DUBs in the pathogenesis of IPF is relatively unexplored...
May 17, 2018: JCI Insight
https://www.readbyqxmd.com/read/29768342/mucosa-associated-lymphoid-tissue-lymphoma-of-the-trachea-associated-with-idiopathic-pulmonary-fibrosis-a-case-report-and-literature-review
#13
June Hong Ahn, Jin Hong Chung, Kyeong-Cheol Shin, Eun Young Choi, Hyun Jung Jin, Joon Hyuk Choi, Kwan Ho Lee
RATIONALE: Mucosa-associated lymphoid tissue (MALT) lymphoma of the trachea is a rare disease that has been shown to be associated with chronic antigenic stimulation. There have been few reports of MALT lymphoma of the trachea in association with idiopathic pulmonary fibrosis (IPF). PATIENT CONCERNS: A 73-year-old patient visited with a 2-year history of dyspnea on exertion and productive cough, which had worsened 1 month ago. DIAGNOSES: MALT lymphoma of the trachea associated with IPF...
May 2018: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29765791/cutaneous-leukocytoclastic-vasculitis-following-influenza-vaccination-in-older-adults-report-of-bullous-purpura-in-an-octogenarian-after-influenza-vaccine-administration
#14
Stella X Chen, Philip R Cohen
The influenza vaccination is recommended annually for protection against influenza infection. Adults over 65 years of age are especially vulnerable to complications from influenza infection; in addition, they constitute the largest group of influenza vaccination recipients each year. Cutaneous leukocytoclastic vasculitis involves inflammation of small vessel walls by neutrophils. An 88-year-old man with a history of idiopathic pulmonary fibrosis who developed bullous cutaneous leukocytoclastic vasculitis 14 days after receiving the influenza vaccine is described and the characteristics of influenza-associated cutaneous leukocytoclastic vasculitis in older individuals are reviewed...
March 14, 2018: Curēus
https://www.readbyqxmd.com/read/29764379/identification-of-usual-interstitial-pneumonia-pattern-using-rna-seq-and-machine-learning-challenges-and-solutions
#15
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
#16
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/29761573/using-fat-to-fight-disease-a-systematic-review-of-non-homologous-adipose-derived-stromal-stem-cell-therapies
#17
REVIEW
Marjorie E Bateman, Amy L Strong, Jeffrey M Gimble, Bruce A Bunnell
OBJECTIVE: The objective of this review is to describe the safety and efficacy of ASC and SVF in treating common diseases and the next steps in research that must occur prior to clinical use. EVIDENCE REVIEW: Pubmed, Ovid Medline, Embase, Web of Science, and the Cochrane Library were searched for articles about use of SVF or ASC for disease therapy published between 2012 and 2017. One meta-analysis, 2 randomized controlled trials, and 16 case series were included, representing 844 human patients...
May 15, 2018: Stem Cells
https://www.readbyqxmd.com/read/29761229/idiopathic-pulmonary-fibrosis-among-young-patients-challenges-in-diagnosis-and-management
#18
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/29758578/-cf-lung-disease-a-german-s3-guideline-module-2-diagnostics-and-treatment-in-chronic-infection-with-pseudomonas-aeruginosa
#19
C Schwarz, B Schulte-Hubbert, J Bend, M Abele-Horn, I Baumann, W Bremer, F Brunsmann, D Dieninghoff, O Eickmeier, H Ellemunter, R Fischer, J Grosse-Onnebrink, J Hammermann, H Hebestreit, M Hogardt, C Hügel, M Hug, S Illing, A Jung, B Kahl, A Koitschev, R Mahlberg, J G Mainz, F Mattner, A Mehl, A Möller, C Muche-Borowski, T Nüßlein, M Puderbach, S Renner, E Rietschel, F C Ringshausen, S Schmidt, L Sedlacek, H Sitter, C Smaczny, B Tümmler, R Vonberg, M O Wielpütz, H Wilkens, B Wollschläger, J Zerlik, U Düesberg, S van Koningsbruggen-Rietschel
Cystic Fibrosis (CF) is the most common autosomal-recessive genetic disease affecting approximately 8000 people in Germany. The disease is caused by mutations in the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) gene leading to dysfunction of CFTR, a transmembrane chloride channel. This defect causes insufficient hydration of the epithelial lining fluid which leads to chronic inflammation of the airways. Recurrent infections of the airways as well as pulmonary exacerbations aggravate chronic inflammation, lead to pulmonary fibrosis and tissue destruction up to global respiratory insufficiency, which is responsible for the mortality in over 90 % of patients...
May 2018: Pneumologie
https://www.readbyqxmd.com/read/29758336/grand-rounds-hepatic-manifestations-of-telomere-biology-disorders
#20
REVIEW
Mrinal M Patnaik, Patrick S Kamath, Douglas A Simonetto
Clinical Case A 51 year old Caucasian male was referred for evaluation of variceal bleeding. Laboratory tests were remarkable for mild thrombocytopenia and moderate alkaline phosphatase elevation. Synthetic liver function was well preserved. Abdominal computed tomography scan revealed moderate splenomegaly, gastric varices, and normal hepatic contour. A transjugular liver biopsy was performed revealing findings of nodular regenerative hyperplasia (NRH) with no significant fibrosis or necroinflammatory activity...
May 11, 2018: Journal of Hepatology
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