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https://www.readbyqxmd.com/read/28421813/hemin-causes-lung-microvascular-endothelial-barrier-dysfunction-by-necroptotic-cell-death
#1
Sunit Singla, Justin R Sysol, Benjamin Dille, Nicole Jones, Jiwang Chen, Roberto F Machado
Hemin, the oxidized prosthetic moiety of hemoglobin, has been implicated in the pathogenesis of acute chest syndrome (ACS) in sickle cell patients by virtue of its endothelial-activating properties. In this study, we examined whether hemin can cause lung microvascular endothelial barrier dysfunction. By assessing transendothelial resistance using electrical cell impedance sensing, and by directly measuring trans-monolayer FITC-dextran flux, we found that hemin does cause endothelial barrier dysfunction in a concentration-dependent manner...
April 19, 2017: American Journal of Respiratory Cell and Molecular Biology
https://www.readbyqxmd.com/read/28382373/antagonists-of-the-system-l-neutral-amino-acid-transporter-lat-promote-endothelial-adhesivity-of-human-red-blood-cells
#2
Laura Beth Mann Dosier, Vikram J Premkumar, Hongmei Zhu, Izzet Akosman, Michael F Wempe, Timothy J McMahon
The system L neutral amino acid transporter (LAT; LAT1, LAT2, LAT3, or LAT4) has multiple functions in human biology, including the cellular import of S-nitrosothiols (SNOs), biologically active derivatives of nitric oxide (NO). SNO formation by haemoglobin within red blood cells (RBC) has been studied, but the conduit whereby a SNO leaves the RBC remains unidentified. Here we hypothesised that SNO export by RBCs may also depend on LAT activity, and investigated the role of RBC LAT in modulating SNO-sensitive RBC-endothelial cell (EC) adhesion...
April 6, 2017: Thrombosis and Haemostasis
https://www.readbyqxmd.com/read/28360450/renal-medullary-carcinoma-a-rare-entity
#3
Selahattin Çalışkan, Ali Murat Gökçe, Gülistan Gümrükçü, Mine Önenerk
Renal medullary carcinoma (RMC) is an uncommon aggressive neoplasm of the kidney. RMC is biologically aggressive with a very poor prognosis, and metastasis is seen in up to 95% of the patients at diagnosis or shortly thereafter. The common sites of metastasis are respectively lymph nodes, lungs, livers, and adrenal glands in order of frequency. The presence of poorly differentiated eosinophilic cells in a characteristic fibro-inflammatory stroma is seen in histological examination. The origin and pathogenesis of RMC are unclear...
March 2017: Iranian Journal of Medical Sciences
https://www.readbyqxmd.com/read/28348773/pleural-empyema-due-to-salmonella-enterica-serovar-enteritidis-in-an-immunocompetent-elderly-patient-a-case-report
#4
Panagiota Xaplanteri, Stelios F Assimakopoulos, Kostis Karachalios, Dimitrios Siagris, Alexandra Lekkou, Evangelos D Anastassiou, Iris Spiliopoulou, Charalambos Gogos, Fevronia Kolonitsiou
INTRODUCTION: Pleural empyema as a focal infection due to Salmonella enterica serovar Enteritidis is rare and most commonly described among immunosuppressed patients or patients who suffer from sickle cell anaemia and lung malignancies. CASE PRESENTATION: Here, we present an 81-year-old immunocompetent Greek woman with bacteraemia and pleural empyema due to Salmonella Enteritidis without any gastrointestinal symptoms. CONCLUSION: In our case, we suggest that patient's pleural effusion secondary to heart failure was complicated by empyema and that focal intravascular infection was the cause of bacteraemia...
August 2016: JMM Case Reports
https://www.readbyqxmd.com/read/28338216/the-diversified-function-and-potential-therapy-of-ectopic-olfactory-receptors-in-non-olfactory-tissues
#5
REVIEW
Zhe Chen, Hong Zhao, Nian Fu, Linxi Chen
Olfactory receptors (ORs) are mainly distributed in olfactory neurons and play a key role in detecting volatile odorants, eventually resulting in the production of smell perception. Recently, it is also reported that ORs are expressed in non-olfactory tissues including heart, lung, sperm, skin and cancerous tissues. Interestingly, ectopic ORs are associated with the development of diseases in non-olfactory tissues. For instance, ectopic ORs initiate the hypoxic ventilatory responses and maintain the oxygen homeostasis of breathing in the carotid body when oxygen levels decline...
March 24, 2017: Journal of Cellular Physiology
https://www.readbyqxmd.com/read/28256526/vascular-permeability-drives-susceptibility-to-influenza-infection-in-a-murine-model-of-sickle-cell-disease
#6
Erik A Karlsson, Thomas H Oguin, Victoria Meliopoulos, Amy Iverson, Alexandria Broadnax, Sun-Woo Yoon, Tamara Pestina, Paul Thomas, Richard Webby, Stacey Schultz-Cherry, Jason W Rosch
Sickle cell disease (SCD) is a major global health concern. Patients with SCD experience disproportionately greater morbidity and mortality in response to influenza infection than do others. Viral infection is one contributing factor for the development of Acute Chest Syndrome (ACS), a major cause of morbidity and mortality in SCD patients. We determined whether the heightened sensitivity to influenza infection could be reproduced in the two different SCD murine models to ascertain the underlying mechanisms of increased disease severity...
March 3, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28248586/reply-decline-of-lung-function-in-children-with-sickle-cell-disease-is-not-associated-with-restrictive-defects
#7
Robyn T Cohen, Mark Rodeghier, Michael R DeBaun
No abstract text is available yet for this article.
March 2017: Annals of the American Thoracic Society
https://www.readbyqxmd.com/read/28248574/decline-of-lung-function-in-children-with-sickle-cell-disease-is-not-associated-with-restrictive-defects
#8
Rucheet Patel, Olufolake Adisa, Anne M Fitzpatrick
No abstract text is available yet for this article.
March 2017: Annals of the American Thoracic Society
https://www.readbyqxmd.com/read/28248201/intravascular-hemolysis-and-the-pathophysiology-of-sickle-cell-disease
#9
REVIEW
Gregory J Kato, Martin H Steinberg, Mark T Gladwin
Hemolysis is a fundamental feature of sickle cell anemia that contributes to its pathophysiology and phenotypic variability. Decompartmentalized hemoglobin, arginase 1, asymmetric dimethylarginine, and adenine nucleotides are all products of hemolysis that promote vasomotor dysfunction, proliferative vasculopathy, and a multitude of clinical complications of pulmonary and systemic vasculopathy, including pulmonary hypertension, leg ulcers, priapism, chronic kidney disease, and large-artery ischemic stroke. Nitric oxide (NO) is inactivated by cell-free hemoglobin in a dioxygenation reaction that also oxidizes hemoglobin to methemoglobin, a non-oxygen-binding form of hemoglobin that readily loses heme...
March 1, 2017: Journal of Clinical Investigation
https://www.readbyqxmd.com/read/28183857/coexistent-sickle-cell-disease-has-no-impact-on-the-safety-or-outcome-of-lytic-therapy-in-acute-ischemic-stroke-findings-from-get-with-the-guidelines-stroke
#10
Robert J Adams, Margueritte Cox, Shelly D Ozark, Julie Kanter, Phillip J Schulte, Ying Xian, Gregg C Fonarow, Eric E Smith, Lee H Schwamm
BACKGROUND AND PURPOSE: The recommended treatment for ischemic stroke is tPA (tissue-type plasminogen activator). Although sickle cell disease (SCD) represents no known contraindication to tPA, National Heart Lung and Blood Institute of the National Institutes of Health recommended acute exchange transfusion for stroke in SCD, not tPA. Data on safety and outcomes of tPA in patients are needed to guide tPA use in SCD. METHODS: We matched patients from the American Heart Association and American Stroke Association Get With The Guidelines-Stroke registry with SCD to patients without SCD and compared usage, complications, and discharge outcomes after tPA...
February 9, 2017: Stroke; a Journal of Cerebral Circulation
https://www.readbyqxmd.com/read/28117470/pulmonary-function-in-children-and-adolescents-with-sickle-cell-disease-have-we-paid-proper-attention-to-this-problem
#11
Ana Karine Vieira, Cristina Gonçalves Alvim, Maria Cristina Marquez Carneiro, Cássio da Cunha Ibiapina
Objective: To evaluate pulmonary function and functional capacity in children and adolescents with sickle cell disease. Methods: This was a cross-sectional study involving 70 children and adolescents (8-15 years of age) with sickle cell disease who underwent pulmonary function tests (spirometry) and functional capacity testing (six-minute walk test). The results of the pulmonary function tests were compared with variables related to the severity of sickle cell disease and history of asthma and of acute chest syndrome...
November 2016: Jornal Brasileiro de Pneumologia: Publicaça̋o Oficial da Sociedade Brasileira de Pneumologia e Tisilogia
https://www.readbyqxmd.com/read/28105733/vitamin-d-supplementation-for-sickle-cell-disease
#12
REVIEW
Htoo Htoo Kyaw Soe, Adinegara Bl Abas, Nan Nitra Than, Han Ni, Jaspal Singh, Abdul Razzak Bin Mohd Said, Ifeyinwa Osunkwo
BACKGROUND: Sickle cell disease is a genetic chronic haemolytic and pro-inflammatory disorder. The clinical manifestations of sickle cell disease result from the presence of mutations on the beta globin genes that generate an abnormal haemoglobin product (called haemoglobin S) within the red blood cell. Sickle cell disease can lead to many complications such as acute chest syndrome, stroke, acute and chronic bone complications (including painful vaso-occlusive crisis, osteomyelitis, osteonecrosis and osteoporosis)...
January 20, 2017: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/28097236/lung-vaso-occlusion-in-sickle-cell-disease-mediated-by-arteriolar-neutrophil-platelet-microemboli
#13
Margaret F Bennewitz, Maritza A Jimenez, Ravi Vats, Egemen Tutuncuoglu, Jude Jonassaint, Gregory J Kato, Mark T Gladwin, Prithu Sundd
In patients with sickle cell disease (SCD), the polymerization of intraerythrocytic hemoglobin S promotes downstream vaso-occlusive events in the microvasculature. While vaso-occlusion is known to occur in the lung, often in the context of systemic vaso-occlusive crisis and the acute chest syndrome, the pathophysiological mechanisms that incite lung injury are unknown. We used intravital microscopy of the lung in transgenic humanized SCD mice to monitor acute vaso-occlusive events following an acute dose of systemic lipopolysaccharide sufficient to trigger events in SCD but not control mice...
January 12, 2017: JCI Insight
https://www.readbyqxmd.com/read/27887664/translating-sickle-cell-guidelines-into-practice-for-primary-care-providers-with-project-echo
#14
Lisa M Shook, Christina B Farrell, Karen A Kalinyak, Stephen C Nelson, Brandon M Hardesty, Angeli G Rampersad, Kay L Saving, Wanda J Whitten-Shurney, Julie A Panepinto, Russell E Ware, Lori E Crosby
BACKGROUND: Approximately 100,000 persons with sickle cell disease (SCD) live in the United States, including 15,000 in the Midwest. Unfortunately, many patients experience poor health outcomes due to limited access to primary care providers (PCPs) who are prepared to deliver evidence-based SCD care. Sickle Treatment and Outcomes Research in the Midwest (STORM) is a regional network established to improve care and outcomes for individuals with SCD living in Indiana, Illinois, Michigan, Minnesota, Ohio, and Wisconsin...
2016: Medical Education Online
https://www.readbyqxmd.com/read/27835777/inflammatory-and-oxidative-stress-phenotypes-in-transgenic-sickle-cell-mice
#15
Emmanuelle Charrin, Solomon Fiifi Ofori-Acquah, Elie Nader, Sarah Skinner, Philippe Connes, Vincent Pialoux, Philippe Joly, Cyril Martin
The Townes mouse model of homozygous sickle cell disease (SS) has emerged as the major experimental model for studying pathophysiological mechanisms of human sickle cell disease (SCD). We therefore investigated hematological and hemorheological parameters as well as organ-specific inflammatory and oxidative stress molecular profiles in these animals in steady state conditions. Evidences of SCD-related intravascular hemolysis, impaired red blood cell (RBC) deformability, leukocytosis and altered plasma nitric oxide byproducts (NOx) level were found in the SS mice...
November 2016: Blood Cells, Molecules & Diseases
https://www.readbyqxmd.com/read/27814863/predictive-models-of-six-minute-walking-distance-in-adults-with-sickle-cell-anemia-implications-for-rehabilitation
#16
Cirlene de Lima Marinho, Maria Christina Paixão Maioli, Andrea Ribeiro Soares, Ricardo Bedirian, Pedro Lopes de Melo, Fernando Silva Guimarães, Arthur de Sá Ferreira, Agnaldo José Lopes
BACKGROUND: Sickle cell anemia (SCA) is characterized by a broad spectrum of abnormalities that affect most body organs and systems. To date, there is few data on the influence of these patients' clinical characteristics on the functional exercise capacity. AIM: To investigate the effect of the clinical complications on the functional exercise capacity of adult SCA patients. METHOD: Cross-sectional study, where 45 SCA patients underwent clinical evaluations, echocardiography, pulmonary function testing, and determination of six-minute walking distance (6MWD)...
October 2016: Journal of Bodywork and Movement Therapies
https://www.readbyqxmd.com/read/27777473/changes-seen-on-computed-tomography-of-the-chest-in-mildly-symptomatic-adult-patients-with-sickle-cell-disease
#17
Ursula David Alves, Agnaldo José Lopes, Maria Christina Paixão Maioli, Andrea Ribeiro Soares, Pedro Lopes de Melo, Roberto Mogami
OBJECTIVE: To describe and quantify the main changes seen on computed tomography of the chest in mildly symptomatic adult patients with sickle cell disease, as well as to evaluate the radiologist accuracy in determining the type of hemoglobinopathy. MATERIALS AND METHODS: A prospective study involving 44 adult patients with sickle cell disease who underwent inspiration and expiration computed tomography of the chest. The frequency of tomography findings and the extent of involvement are reported...
July 2016: Radiologia Brasileira
https://www.readbyqxmd.com/read/27692991/chronic-brain-damage-in-sickle-cell-disease-and-its-relation-with-quality-of-life
#18
Elena Cela, Ana G Vélez, Alejandra Aguado, Gabriela Medín, José M Bellón, Cristina Beléndez
BACKGROUND AND OBJECTIVE: Sickle cell anaemia causes progressive organ damage. The objective is to describe school performance of patients with sickle cell anaemia and their clinical parameters and quality of life that may have an influence. The hypothesis is that if school alterations occur without other objective data, additional factors must be present besides the disease itself. PATIENTS AND METHODS: Transversal study performed in November 2015 considering analytical variables, complications and neuroradiological images of children with sickle cell anaemia, and family survey on school performance and quality of life...
December 16, 2016: Medicina Clínica
https://www.readbyqxmd.com/read/27642150/-pulmonary-complications-of-sickle-cell-disease-in-children
#19
T Pincez, L Calamy, Z Germont, A Lemoine, A-A Lopes, A Massiot, J Tencer, C Thivent, A Hadchouel
Acute and chronic pulmonary complications are frequent in sickle cell disease (SCD), with different spectrum and characteristics in children and adults. Chronic hypoxia is frequent and plays a role in several respiratory complications in SCD. Furthermore, hypoxia has been associated with a higher risk of cerebral ischemia. Despite differing oxygen affinity between hemoglobin A and S, standard pulse oximetry was shown to be accurate in diagnosing hypoxia in SCD patients. Whereas acute hypoxia management is similar to non-SCD patients, chronic hypoxia treatment is mainly based on a transfusion program rather than long-term oxygen therapy...
October 2016: Archives de Pédiatrie: Organe Officiel de la Sociéte Française de Pédiatrie
https://www.readbyqxmd.com/read/27612006/peroxisome-proliferator-activated-receptor-%C3%AE-regulates-the-v-ets-avian-erythroblastosis-virus-e26-oncogene-homolog-1-microrna-27a-axis-to-reduce-endothelin-1-and-endothelial-dysfunction-in-the-sickle-cell-mouse-lung
#20
Bum-Yong Kang, Kathy Park, Jennifer M Kleinhenz, Tamara C Murphy, Roy L Sutliff, David Archer, C Michael Hart
Pulmonary hypertension (PH), a serious complication of sickle cell disease (SCD), causes significant morbidity and mortality. Although a recent study determined that hemin release during hemolysis triggers endothelial dysfunction in SCD, the pathogenesis of SCD-PH remains incompletely defined. This study examines peroxisome proliferator-activated receptor γ (PPARγ) regulation in SCD-PH and endothelial dysfunction. PH and right ventricular hypertrophy were studied in Townes humanized sickle cell (SS) and littermate control (AA) mice...
January 2017: American Journal of Respiratory Cell and Molecular Biology
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