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https://www.readbyqxmd.com/read/28905364/a-clinical-risk-score-for-pulmonary-artery-thrombosis-during-acute-chest-syndrome-in-adult-patients-with-sickle-cell-disease
#1
Anaïs Winchenne, Jérôme Cecchini, Jean-François Deux, Nicolas De Prost, Keyvan Razazi, Guillaume Carteaux, Frederic Galacteros, Anoosha Habibi, Pablo Bartolucci, Giovanna Melica, Mehdi Khellaf, Marc Michel, Bernard Maitre, Armand Mekontso Dessap
Pulmonary artery thrombosis (PAT) is involved in lung vascular dysfunction during acute chest syndrome (ACS) complicating sickle cell disease (SCD). No clinical score is available to identify patients eligible for multi-detector computed tomography (MDCT) angiography during ACS. This retrospective study aimed to develop a risk score for PAT during ACS (PAT-ACS risk score). Patients with SCD were investigated by MDCT during ACS. A logistic regression was performed to determine independent risks factors for PAT and to build the PAT-ACS risk score...
September 14, 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/28892533/association-of-guideline-adherent-antibiotic-treatment-with-readmission-of-children-with-sickle-cell-disease-hospitalized-with-acute-chest-syndrome
#2
David G Bundy, Troy E Richardson, Matthew Hall, Jean L Raphael, David C Brousseau, Staci D Arnold, Ram V Kalpatthi, Angela M Ellison, Suzette O Oyeku, Samir S Shah
Importance: Acute chest syndrome (ACS) is a common, serious complication of sickle cell disease (SCD) and a leading cause of hospitalization and death in both children and adults with SCD. Little is known about the effectiveness of guideline-recommended antibiotic regimens for the care of children hospitalized with ACS. Objectives: To use a large, national database to describe patterns of antibiotic use for children with SCD hospitalized for ACS and to determine whether receipt of guideline-adherent antibiotics was associated with lower readmission rates...
September 11, 2017: JAMA Pediatrics
https://www.readbyqxmd.com/read/28819305/cardiorespiratory-pathogenesis-of-sickle-cell-disease-in-a-mouse-model
#3
Jun Ren, Xiuqing Ding, Marie Trudel, John J Greer, Joanna E MacLean
The nature and development of cardiorespiratory impairments associated with sickle cell disease are poorly understood. Given that the mechanisms of these impairments cannot be addressed adequately in clinical studies, we characterized cardiorespiratory pathophysiology from birth to maturity in the sickle cell disease SAD mouse model. We identified two critical phases of respiratory dysfunction in SAD mice; the first prior to weaning and the second in adulthood. At postnatal day 3, 43% of SAD mice showed marked apneas, anemia, and pulmonary vascular congestion typical of acute chest syndrome; none of these mice survived to maturity...
August 17, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28769584/the-effect-of-hypnosis-on-pain-and-peripheral-blood-flow-in-sickle-cell-disease-a-pilot-study
#4
Ravi R Bhatt, Sarah R Martin, Subhadra Evans, Kirsten Lung, Thomas D Coates, Lonnie K Zeltzer, Jennie C Tsao
BACKGROUND: Vaso-occlusive pain crises (VOCs) are the "hallmark" of sickle-cell disease (SCD) and can lead to sympathetic nervous system dysfunction. Increased sympathetic nervous system activation during VOCs and/or pain can result in vasoconstriction, which may increase the risk for subsequent VOCs and pain. Hypnosis is a neuromodulatory intervention that may attenuate vascular and pain responsiveness. Due to the lack of laboratory-controlled pain studies in patients with SCD and healthy controls, the specific effects of hypnosis on acute pain-associated vascular responses are unknown...
2017: Journal of Pain Research
https://www.readbyqxmd.com/read/28689245/imaging-of-renal-medullary-carcinoma-in-children-and-young-adults-a-report-from-the-children-s-oncology-group
#5
Jesse K Sandberg, Elizabeth A Mullen, Mariana M Cajaiba, Ethan A Smith, Sabah Servaes, Elizabeth J Perlman, James I Geller, Peter F Ehrlich, Nicholas G Cost, Jeffrey S Dome, Conrad V Fernandez, Geetika Khanna
BACKGROUND: Renal medullary carcinoma is a rare renal malignancy of childhood. There are no large series describing the imaging appearance of renal medullary carcinoma in children. OBJECTIVE: To characterize the clinical and imaging features of pediatric renal medullary carcinoma at initial presentation. MATERIALS AND METHODS: We retrospectively analyzed images of 25 pediatric patients with renal medullary carcinoma enrolled in the Children's Oncology Group renal tumors classification, biology and banking study (AREN03B2) from March 2006 to August 2016...
July 8, 2017: Pediatric Radiology
https://www.readbyqxmd.com/read/28542880/suicidal-death-of-erythrocytes-in-cancer-and-its-chemotherapy-a-potential-target-in-the-treatment-of-tumor-associated-anemia
#6
REVIEW
Elisabeth Lang, Rosi Bissinger, Syed M Qadri, Florian Lang
In analogy to apoptosis of nucleated cells, erythrocytes may enter eryptosis characterized by cell shrinkage and cell membrane scrambling. Eryptotic erythrocytes are rapidly cleared from circulating blood and may adhere to the vascular wall. Stimulation of eryptosis thus impairs microcirculation and leads to anemia as soon as the loss of erythrocytes cannot be fully compensated by enhanced erythropoiesis. Signaling stimulating eryptosis includes increase of cytosolic Ca(2+) -activity, ceramide, caspases, calpain, p38-kinase, protein-kinase C, Janus-activated kinase 3, casein-kinase 1α, and cyclin-dependent kinase 4...
October 15, 2017: International Journal of Cancer. Journal International du Cancer
https://www.readbyqxmd.com/read/28518049/red-blood-cell-storage-time-and-transfusion-current-practice-concerns-and-future-perspectives
#7
REVIEW
María García-Roa, María Del Carmen Vicente-Ayuso, Alejandro M Bobes, Alexandra C Pedraza, Ataúlfo González-Fernández, María Paz Martín, Isabel Sáez, Jerard Seghatchian, Laura Gutiérrez
Red blood cells (RBCs) units are the most requested transfusion product worldwide. Indications for transfusion include symptomatic anaemia, acute sickle cell crisis, and acute blood loss of more than 30% of the blood volume, with the aim of restoring tissue oxygen delivery. However, stored RBCs from donors are not a qualitative equal product, and, in many ways, this is a matter of concern in the transfusion practice. Besides donor-to-donor variation, the storage time influences the RBC unit at the qualitative level, as RBCs age in the storage bag and are exposed to the so-called storage lesion...
May 2017: Blood Transfusion, Trasfusione del Sangue
https://www.readbyqxmd.com/read/28421813/hemin-causes-lung-microvascular-endothelial-barrier-dysfunction-by-necroptotic-cell-death
#8
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 in patients with sickle cell disease 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 fluorescein isothiocyanate-dextran flux, we found that hemin does cause endothelial barrier dysfunction in a concentration-dependent manner...
September 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
#9
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...
June 28, 2017: Thrombosis and Haemostasis
https://www.readbyqxmd.com/read/28360450/renal-medullary-carcinoma-a-rare-entity
#10
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
#11
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
#12
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
#13
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
#14
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
#15
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
#16
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
#17
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...
March 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
#18
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
#19
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
#20
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
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