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https://www.readbyqxmd.com/read/28225753/complement-drives-glucosylceramide-accumulation-and-tissue-inflammation-in-gaucher-disease
#1
Manoj K Pandey, Thomas A Burrow, Reena Rani, Lisa J Martin, David Witte, Kenneth D Setchell, Mary A Mckay, Albert F Magnusen, Wujuan Zhang, Benjamin Liou, Jörg Köhl, Gregory A Grabowski
Gaucher disease is caused by mutations in GBA1, which encodes the lysosomal enzyme glucocerebrosidase (GCase). GBA1 mutations drive extensive accumulation of glucosylceramide (GC) in multiple innate and adaptive immune cells in the spleen, liver, lung and bone marrow, often leading to chronic inflammation. The mechanisms that connect excess GC to tissue inflammation remain unknown. Here we show that activation of complement C5a and C5a receptor 1 (C5aR1) controls GC accumulation and the inflammatory response in experimental and clinical Gaucher disease...
February 22, 2017: Nature
https://www.readbyqxmd.com/read/28222077/association-of-smoking-in-the-home-with-lung-cancer-worry-perceived-risk-and-synergistic-risk
#2
Karen M Butler, Mary Kay Rayens, Amanda T Wiggins, Kathy B Rademacher, Ellen J Hahn
PURPOSE/OBJECTIVES: To examine the association of smoking in the home with lung cancer worry, perceived risk, and synergistic risk, controlling for sociodemographics, family history of lung cancer, and health-related self-concept. The hypothesis is that participants with smoking in the home would have higher scores for lung cancer worry, perceived risk, and synergistic risk.
. DESIGN: Cross-sectional baseline survey. 
. SETTING: Participants recruited from an outpatient clinic and pharmacy at University of Kentucky HealthCare, an academic medical center...
March 1, 2017: Oncology Nursing Forum
https://www.readbyqxmd.com/read/28221763/inactivation-clearance-and-functional-effects-of-lung-instilled-short-and-long-silver-nanowires-in-rats
#3
Kian Fan Chung, Joanna Seiffert, Shu Chen, Ioannis G Theodorou, Angela Erin Goode, Bey Fen Leo, Catriona M McGilvery, Farhana Hussain, Coen Wiegman, Christos Rossios, Jie Zhu, Jicheng Gong, Farid Tariq, Vladimir Yufit, Alexander J Monteith, Teruo Hashimoto, Jeremy N Skepper, Mary P Ryan, Junfeng Jim Zhang, Teresa D Tetley, Alexandra E Porter
There is a potential for silver nanowires (AgNWs) to be inhaled but there is little information on their health effects and their chemical transformation inside the lungs in vivo. We studied the effects of short (S-AgNWs; 1.5µm) and long (L-AgNWs; 10 µm) nanowires instilled into the lungs of Sprague-Dawley rats. S- and L-AgNWs were phagocytosed and degraded by macrophages; there was no frustrated phagocytosis. Interestingly, both AgNWs were internalized in alveolar Type II epithelial cells (ATII), with precipitation of Ag2S on their surface as secondary Ag2S nanoparticles...
February 21, 2017: ACS Nano
https://www.readbyqxmd.com/read/28221211/a-rare-case-of-ck20-ck7-double-negative-merkel-cell-carcinoma
#4
Mary D Le, Luke H OʼSteen, David S Cassarino
The authors present a case of Merkel cell carcinoma (MCC) with unique immunohistochemical staining characteristics. A 57-year-old woman presented with a firm 0.3 cm tan papule on her left nasal-labial fold that was reportedly increasing in size and bleeding. She had a history of multiple head and neck actinic keratoses, papillary thyroid carcinoma, and a family history of an uncle with melanoma. The clinical differential diagnosis was "non-melanoma skin cancer." Histological examination showed a markedly atypical-appearing basaloid neoplasm, present mostly in the dermis, with focal pagetoid spread into the epidermis...
March 2017: American Journal of Dermatopathology
https://www.readbyqxmd.com/read/28217316/subacute-right-heart-failure-revealing-three-simultaneous-causes-of-post-embolic-pulmonary-hypertension-in-metastatic-dissemination-of-breast-cancer
#5
Flavien Vincent, Nicolas Lamblin, Marion Classe, Guillaume Schurtz, Antoine Rauch, Marie Fertin, Pascal De Groote
A 72-year-old woman with history of breast cancer only treated surgically was referred to our department for pulmonary hypertension (PH) suspicion. Echocardiogram revealed elevated right ventricular systolic pressure. Computed tomography (CT) angiogram showed no pulmonary embolism (PE), but lung scan revealed two ventilation-perfusion mismatch areas. Right cardiac catheterization established precapillary PH. Despite treatment with PH specific therapy (sildenafil, ambrisentan, and epoprostenol), her condition worsened rapidly with acute right heart failure (RHF)...
February 2017: ESC Heart Failure
https://www.readbyqxmd.com/read/28214087/targeting-the-pd-1-pd-l1-axis-in-non-small-cell-lung-cancer
#6
REVIEW
Rajiv Kumar, Dearbhaile Collins, Saoirse Dolly, Fiona McDonald, Mary E R O'Brien, Timothy A Yap
The last decade has witnessed rapid advances in the discovery and development of immune checkpoint inhibitors in cancer medicine, particularly drugs targeting programmed cell death 1 (PD-1) and programmed cell death ligand 1 (PD-L1) in non-small cell lung cancer (NSCLC). The proven antitumor efficacy coupled with low rates of drug-related toxicities observed, albeit idiosyncratic, with these novel immunotherapeutics have led to the registration of multiple PD-1 and PD-L1 inhibitors, such as nivolumab, pembrolizumab, and atezolizumab, in second-line advanced NSCLC, whereas durvalumab and avelumab are in late-phase clinical testing...
December 23, 2016: Current Problems in Cancer
https://www.readbyqxmd.com/read/28212574/the-combined-action-of-mast-cell-chymase-tryptase-and-carboxypeptidase-a3-protects-against-melanoma-colonization-of-the-lung
#7
Mirjana Grujic, Aida Paivandy, Ann-Marie Gustafson, Allan R Thomsen, Helena Öhrvik, Gunnar Pejler
Mast cell secretory granules are densely packed with various bioactive mediators including proteases of chymase, tryptase and CPA3 type. Previous studies have indicated that mast cells can affect the outcome of melanoma but the contribution of the mast cell granule proteases to such effects has not been clear. Here we addressed this issue by assessing mice lacking either the chymase Mcpt4, the tryptase Mcpt6 or carboxypeptidase A3 (Cpa3), as well as mice simultaneously lacking all three proteases, in a model of melanoma dissemination from blood to the lung...
February 15, 2017: Oncotarget
https://www.readbyqxmd.com/read/28210624/rna-sequencing-analysis-reveals-interactions-between-breast-cancer-or-melanoma-cells-and-the-tissue-microenvironment-during-brain-metastasis
#8
Ryo Sato, Teppei Nakano, Mari Hosonaga, Oltea Sampetrean, Ritsuko Harigai, Takashi Sasaki, Ikuko Koya, Hideyuki Okano, Jun Kudoh, Hideyuki Saya, Yoshimi Arima
Metastasis is the main cause of treatment failure and death in cancer patients. Metastasis of tumor cells to the brain occurs frequently in individuals with breast cancer, non-small cell lung cancer, or melanoma. Despite recent advances in our understanding of the causes and in the treatment of primary tumors, the biological and molecular mechanisms underlying the metastasis of cancer cells to the brain have remained unclear. Metastasizing cancer cells interact with their microenvironment in the brain to establish metastases...
2017: BioMed Research International
https://www.readbyqxmd.com/read/28209722/long-term-outcome-of-hematopoietic-stem-cell-transplantation-for-il2rg-jak3-scid-a-cohort-report
#9
Intan Juliana Abd Hamid, Mary A Slatter, Fiona McKendrick, Mark S Pearce, Andrew R Gennery
Hematopoietic stem cell transplantation (HSCT) cures the T-lymphocyte, B-lymphocyte and Natural Killer (NK) cell differentiation defect in IL2RG/JAK3 SCID. We evaluated long-term clinical features, longitudinal immunoreconstitution, donor chimerism and quality of life (QoL) of IL2RG/JAK3 SCID patients >2 years post-HSCT at our center. Clinical data were collated and patients/families answered PedsQL Generic Core Scale v4.0 questionnaires. We performed longitudinal analyses of CD3+, CD4+ naïve T-lymphocyte, CD19+ and NK cell numbers from pre-transplant until 15 years post-transplant...
February 16, 2017: Blood
https://www.readbyqxmd.com/read/28203372/diagnostic-challenges-and-management-of-a-patient-with-acromegaly-due-to-ectopic-growth-hormone-releasing-hormone-secretion-from-a-bronchial-carcinoid-tumour
#10
Nikolaos Kyriakakis, Jacqueline Trouillas, Mary N Dang, Julie Lynch, Paul Belchetz, Márta Korbonits, Robert D Murray
: A male patient presented at the age of 30 with classic clinical features of acromegaly and was found to have elevated growth hormone levels, not suppressing during an oral glucose tolerance test. His acromegaly was originally considered to be of pituitary origin, based on a CT scan, which was interpreted as showing a pituitary macroadenoma. Despite two trans-sphenoidal surgeries, cranial radiotherapy and periods of treatment with bromocriptine and octreotide, his acromegaly remained active clinically and biochemically...
2017: Endocrinology, Diabetes & Metabolism Case Reports
https://www.readbyqxmd.com/read/28203069/the-pharmacological-cost-of-copd-during-greek-economic-crisis
#11
Eirini Stafyla, Theodora Kerenidi, Irini Gerogianni, Mary Geitona, Zoe Daniil, Konstantinos I Gourgoulianis
INTRODUCTION: The economic crisis in Greece has substantially affected patients with COPD. The reduction of disposable income has its consequences on patients' ability to afford their medication. The aim of the study is to evaluate the cost of treatment for patients with COPD and the influence of the financial crisis to the patients. METHODS: Data were collected from 189 patients (male: 178, mean age: 70.1±8.4) who visited the outpatient department of University Hospital of Larissa in 2014 and 2015...
2017: International Journal of Chronic Obstructive Pulmonary Disease
https://www.readbyqxmd.com/read/28202768/respiratory-morbidity-in-infants-born-with-a-congenital-lung-malformation
#12
Celine Delestrain, Naziha Khen-Dunlop, Alice Hadchouel, Pierrick Cros, Héloïse Ducoin, Michael Fayon, Isabelle Gibertini, André Labbé, Géraldine Labouret, Marie-Noëlle Lebras, Guillaume Lezmi, Fouad Madhi, Guillaume Thouvenin, Caroline Thumerelle, Christophe Delacourt
BACKGROUND AND OBJECTIVES: The actual frequency of respiratory symptoms related to congenital pulmonary malformations (CPMs) remains undetermined. The goal of this study was to prospectively evaluate the respiratory symptoms occurring in infants with prenatally diagnosed CPMs, identify factors associated with the occurrence of these symptoms, and evaluate their resolution after surgery. METHODS: Infectious and noninfectious respiratory symptoms were prospectively collected in a French multicenter cohort of children with CPMs...
February 15, 2017: Pediatrics
https://www.readbyqxmd.com/read/28202251/high-incidence-of-non-tuberculous-mycobacteria-positive-cultures-among-adolescent-with-cystic-fibrosis
#13
Zoé Cavalli, Quitterie Reynaud, Romain Bricca, Raphaële Nove-Josserand, Stéphane Durupt, Philippe Reix, Marie Perceval, Michèle Pérouse de Montclos, Gérard Lina, Isabelle Durieu
BACKGROUND: We evaluated the prevalence of non-tuberculous mycobacteria (NTM)-positive cultures among our cystic fibrosis (CF) center patients, reviewed risk factors for NTM positivity, and determined its impact on lung function evolution. METHODS: From 2009 to 2014, CF adults and children attending the CF center of Lyon (France) and having at least one positive NTM isolate were included. Each case was matched by age and gender with two CF patients with no NTM isolate (controls)...
February 12, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28196607/development-of-a-model-web-based-system-to-support-a-statewide-quality-consortium-in-radiation-oncology
#14
Jean M Moran, Mary Feng, Lisa A Benedetti, Robin Marsh, Kent A Griffith, Martha M Matuszak, Michael Hess, Matthew McMullen, Jennifer H Fisher, Teamour Nurushev, Margaret Grubb, Stephen Gardner, Daniel Nielsen, Reshma Jagsi, James A Hayman, Lori J Pierce
PURPOSE: A database in which patient data are compiled allows analytic opportunities for continuous improvements in treatment quality and comparative effectiveness research. We describe the development of a novel, web-based system that supports the collection of complex radiation treatment planning information from centers that use diverse techniques, software, and hardware for radiation oncology care in a statewide quality collaborative, the Michigan Radiation Oncology Quality Consortium (MROQC)...
October 20, 2016: Practical Radiation Oncology
https://www.readbyqxmd.com/read/28195673/ultrasound-enhanced-sirna-delivery-using-magnetic-nanoparticle-loaded-chitosan-deoxycholic-acid-nanodroplets
#15
Jeong Yu Lee, Calum Crake, Boon Teo, Dario Carugo, Marie de Saint Victor, Anjali Seth, Eleanor Stride
Small interfering RNA (siRNA) has significant therapeutic potential but its clinical translation has been severely inhibited by a lack of effective delivery strategies. Previous work has demonstrated that perfluorocarbon nanodroplets loaded with magnetic nanoparticles can facilitate the intracellular delivery of a conventional chemotherapeutic drug. The aim of this study is to determine whether a similar agent can provide a means of delivering siRNA, enabling efficient transfection without degradation of the molecule...
February 13, 2017: Advanced Healthcare Materials
https://www.readbyqxmd.com/read/28192114/sirolimus-and-autophagy-inhibition-in-lam-results-of-a-phase-i-clinical-trial
#16
Souheil El-Chemaly, Angelo Taveira-Dasilva, Hilary J Goldberg, Elizabeth Peters, Mary Haughey, Don Bienfang, Amanda M Jones, Patricia Julien-Williams, Ye Cui, Julian A Villalba, Shefali Bagwe, Rie Maurer, Ivan O Rosas, Joel Moss, Elizabeth P Henske
BACKGROUND: Animal and cellular studies support the importance of autophagy inhibition in lymphangioleiomyomatosis (LAM). In a cohort of subjects with LAM, we tested the hypothesis that treatment with sirolimus and hydroxycholoroquine (an autophagy inhibitor) at 2 different dose levels is safe and well tolerated. Secondary endpoints included changes in lung function. METHODS: This 48-week, two-center Phase I trial evaluated the safety of escalating oral hydroxychloroquine doses (100-200 mg) given twice a day in combination with sirolimus to eligible patients ≥18 years old with LAM...
February 10, 2017: Chest
https://www.readbyqxmd.com/read/28187909/nonadherence-to-the-medical-regimen-after-lung-transplantation-a-systematic-review
#17
REVIEW
Lu Hu, Jennifer H Lingler, Susan M Sereika, Lora E Burke, Donna Kaltenbaugh Malchano, Annette DeVito Dabbs, Mary Amanda Dew
This study reports on the first systematic review focused on lung transplant recipients (LTRs) and provides evidence regarding 1) prevalence of nonadherence to the post-transplant medical regimen; 2) risk factors for nonadherence; 3) impact of adherence-promoting interventions; and 4) transplant-related clinical outcomes of nonadherence in LTRs. Following the PRISMA guidelines, a literature search of 5 databases was conducted, yielding 30 relevant articles. Findings suggested that nonadherence rates varied greatly across regimen components and were not consistently associated with any single risk factor...
February 7, 2017: Heart & Lung: the Journal of Critical Care
https://www.readbyqxmd.com/read/28183714/dichotomous-alk-ihc-is-a-better-predictor-for-alk-inhibition-outcome-than-traditional-alk-fish-in-advanced-non-small-cell-lung-cancer
#18
Anthonie van der Wekken, Rianne Pelgrim, Nils 't Hart, Naomi Werner, Mirjam Mastik, Lizza Hendriks, Erik Hfm van der Heijden, Monika Looijen-Salamon, A Joop de Langen, Jeske Staal-van den Brekel, Sietske Riemersma, Ben E van den Borne, Ernst-Jan M Speel, Anne-Marie C Dingemans, T Jeroen N Hiltermann, Anke van den Berg, Wim Timens, Ed Schuuring, Harry Jm Groen
ALK rearrangement detection using fluorescence in situ hybridization (FISH) is the standard test to identify non-small cell lung carcinoma (NSCLC) patients eligible for treatment with ALK inhibitors. Recently ALK protein expression in resectable NSCLC showed predictive value. We evaluated tumor response rate and survival after crizotinib treatment of advanced NSCLC patients with ALK activation using both dichotomous immunohistochemical staining (IHC) and FISH. Design Stage IV NSCLC patients treated with crizotinib were selected...
February 9, 2017: Clinical Cancer Research: An Official Journal of the American Association for Cancer Research
https://www.readbyqxmd.com/read/28183533/chronic-embolic-pulmonary-hypertension-caused-by-pulmonary-embolism-and-vascular-endothelial-growth-factor-inhibition
#19
Evandro M Neto-Neves, Mary B Brown, Maria V Zaretskaia, Samin Rezania, Adam G Goodwill, Brian P McCarthy, Scott A Persohn, Paul R Territo, Jeffrey A Kline
Our understanding of the pathophysiological basis of chronic thromboembolic pulmonary hypertension (CTEPH) will be accelerated by an animal model that replicates the phenotype of human CTEPH. Sprague-Dawley rats were administered a combination of a single dose each of plastic microspheres and vascular endothelial growth factor receptor antagonist in polystyrene microspheres (PE) + tyrosine kinase inhibitor SU5416 (SU) group. Shams received volume-matched saline; PE and SU groups received only microspheres or SU5416, respectively...
February 7, 2017: American Journal of Pathology
https://www.readbyqxmd.com/read/28182854/anti-citrullinated-protein-antibodies-are-associated-with-neutrophil-extracellular-traps-in-the-sputum-in-relatives-of-rheumatoid-arthritis-patients
#20
M Kristen Demoruelle, Kylie K Harrall, Linh Ho, Monica M Purmalek, Nickie L Seto, Heather M Rothfuss, Michael H Weisman, Joshua J Solomon, Aryeh Fischer, Yuko Okamoto, Lindsay B Kelmenson, Mark C Parish, Marie Feser, Chelsie Fleischer, Courtney Anderson, Michael Mahler, Jill M Norris, Mariana J Kaplan, Brian D Cherrington, V Michael Holers, Kevin D Deane
OBJECTIVES: Studies suggest that rheumatoid arthritis (RA)-related autoimmunity is initiated at a mucosal site. However, the factors associated with the mucosal generation of this autoimmunity are unknown, especially in individuals who are at-risk for future RA. Therefore, we tested anti-cyclic citrullinated peptide (anti-CCP) antibodies in the sputum of RA-free first-degree relatives (FDRs) of RA patients and patients with classifiable RA. METHODS: We evaluated induced sputum and serum from 67 FDRs and 20 RA subjects for anti-CCP-IgA and anti-CCP-IgG, with cut-off levels for positivity determined in a control population...
February 9, 2017: Arthritis & Rheumatology
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