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Acute chest syndrome in sickle cell disease

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https://www.readbyqxmd.com/read/28512745/implementation-of-multi-disciplinary-care-reduces-maternal-mortality-in-women-with-sickle-cell-disease-living-in-low-resource-setting
#1
Eugenia Vicky Asare, Edeghonghon Olayemi, Theodore Boafor, Yvonne Dei-Adomakoh, Enoch Mensah, Harriet Ghansah, Yvonne Osei-Bonsu, Selina Crabbe, Latif Musah, Charles Hayfron-Benjamin, Brittany Covert, Adetola A Kassim, Andra James, Mark Rodeghier, Michael R DeBaun, Samuel A Oppong
Sickle cell disease (SCD) is associated with adverse pregnancy outcome. In women with SCD living in low-resource settings, pregnancy is associated with significantly increased maternal and perinatal mortality rates. We tested the hypothesis that implementing a multidisciplinary obstetric and hematology care team in a low-resource setting would significantly reduce maternal and perinatal mortality rates. We conducted a before-and-after study, at the Korle-Bu Teaching Hospital in Accra, Ghana, to evaluate the effect of a multidisciplinary obstetric-hematology care team for women with SCD in a combined SCD-Obstetric Clinic...
May 16, 2017: American Journal of Hematology
https://www.readbyqxmd.com/read/28500860/interventions-for-preventing-silent-cerebral-infarcts-in-people-with-sickle-cell-disease
#2
REVIEW
Lise J Estcourt, Patricia M Fortin, Sally Hopewell, Marialena Trivella, Carolyn Doree, Miguel R Abboud
BACKGROUND: Sickle cell disease (SCD) is one of the commonest severe monogenic disorders in the world, due to the inheritance of two abnormal haemoglobin (beta globin) genes. SCD can cause severe pain, significant end-organ damage, pulmonary complications, and premature death. Silent cerebral infarcts are the commonest neurological complication in children and probably adults with SCD. Silent cerebral infarcts also affect academic performance, increase cognitive deficits and may lower intelligence quotient...
May 13, 2017: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/28500682/red-blood-cell-transfusions-during-sickle-cell-anemia-vaso-occlusive-crises-a-report-from-the-magnesium-in-crisis-magic-study
#3
Monica L Hulbert, Julie A Panepinto, J Paul Scott, Robert I Liem, Lawrence J Cook, Timothy Simmons, David C Brousseau
BACKGROUND: Little is known about red blood cell (RBC) transfusion practices for children hospitalized for a sickle cell vaso-occlusive pain crisis (VOC). We hypothesized that transfusion would be associated with the development of acute chest syndrome (ACS), lower hemoglobin (Hb) concentration, and lack of hydroxyurea therapy. STUDY DESIGN AND METHODS: This is a secondary analysis of all children admitted for a sickle cell pain crisis enrolled in the Magnesium in Crisis (MAGiC) randomized trial; all had HbSS or S-β(0) thalassemia...
May 12, 2017: Transfusion
https://www.readbyqxmd.com/read/28489923/differences-of-microparticle-patterns-between-sickle-cell-anemia-and-hemoglobin-sc-patients
#4
Yohann Garnier, Séverine Ferdinand, Maryse Etienne-Julan, Gisèle Elana, Marie Petras, Lydia Doumdo, Benoit Tressières, Marie-Laure Lalanne-Mistrih, Marie-Dominique Hardy-Dessources, Philippe Connes, Marc Romana
Sickle cell anemia (SCA) and hemoglobin SC (HbSC) disease are the two most common forms of sickle cell disease (SCD), a frequent hemoglobinopathy which exhibits a highly variable clinical course. Although high levels of microparticles (MPs) have been consistently reported in SCA and evidence of their harmful impact on the SCA complication occurrences have been provided, no data on MP pattern in HbSC patients has been reported so far. In this study, we determined and compared the MP patterns of 84 HbSC and 96 SCA children, all at steady-state, using flow cytometry...
2017: PloS One
https://www.readbyqxmd.com/read/28486752/exosomes-contribute-to-endothelial-integrity-and-acute-chest-syndrome-risk-preliminary-findings
#5
Gabrielle Lapping-Carr, Abdelnaby Khalyfa, Stephanie Rangel, Wendy Darlington, Eric C Beyer, Radhika Peddinti, John M Cunningham, David Gozal
BACKGROUND: Acute Chest Syndrome (ACS) is one of the leading causes of death among children with Sickle Cell Disease (SCD). Disruption of microvascular integrity is critical to the pathophysiology of ACS, but the factors governing its phenotypic variability are incompletely understood. Because circulating exosomes have been implicated in vascular dysfunction in various diseases, we hypothesized that exosomes induce endothelial dysfunction in patients who experience ACS. PROCEDURE: Cross-sectional cohort study including 33 outpatients with SCD (without new health-related complaints or recent transfusions) and a cohort of control patients...
May 9, 2017: Pediatric Pulmonology
https://www.readbyqxmd.com/read/28470719/from-total-blood-exchange-to-erythrocytapheresis-and-back-to-treat-complications-of-sickle-cell-disease
#6
Samir K Ballas
Erythrocytapheresis is an important procedure in the management of certain complications of sickle cell disease, including acute stroke, stroke prevention, acute chest syndrome, and multiorgan failure. Erythrocytapheresis in sickle cell disease simply entails the removal of the patient's red blood cells containing the abnormal sickle hemoglobin and replacing them with normal red blood cells carrying normal hemoglobin. In these procedures, the patient's plasma is not exchanged but is returned to the patient...
May 3, 2017: Transfusion
https://www.readbyqxmd.com/read/28457019/dynamics-of-von-willebrand-factor-reactivity-in-sickle-cell-disease-during-vaso-occlusive-crisis-and-steady-state
#7
Joep W R Sins, Marein Schimmel, Brenda M Luken, Erfan Nur, Sacha S Zeerleder, Charlotte F J van Tuijn, Dees P M Brandjes, Wil F Kopatz, Rolf T Urbanus, Joost C M Meijers, Bart J Biemond, Karin Fijnvandraat
BACKGROUND: Endothelial activation plays a central role in the pathophysiology of vaso-occlusion in sickle cell disease (SCD), facilitating adhesive interactions with circulating blood cells. Upon activation various adhesive molecules are expressed, including von Willebrand factor (VWF). Increased VWF levels have been observed in patients with SCD during steady state. However, the role of VWF in the pathogenesis of SCD vaso-occlusion is unclear. OBJECTIVES: To longitudinally assess quantity and reactivity of VWF and its regulating protease ADAMTS-13 during vaso-occlusive crisis (VOC)...
April 29, 2017: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/28455727/perioperative-considerations-for-patients-with-sickle-cell-disease-a-narrative-review
#8
Narjeet Khurmi, Andrew Gorlin, Lopa Misra
PURPOSE: Approximately 200,000 individuals worldwide are born annually with sickle cell disease (SCD). Regions with the highest rates of SCD include Africa, the Mediterranean, and Asia, where its prevalence is estimated to be 2-6% of the population. An estimated 70,000-100,000 people in the United States have SCD. Due to enhanced newborn screening, a better understanding of this disease, and more aggressive therapy, many sickle cell patients survive into their adult years and present more frequently for surgery...
April 28, 2017: Canadian Journal of Anaesthesia, Journal Canadien D'anesthésie
https://www.readbyqxmd.com/read/28453928/the-clinical-severity-of-hemoglobin-s-black-a-%C3%AE-%C3%AE-%C3%AE-0-thalassemia
#9
Maria I Cancio, Banu Aygun, David H K Chui, Jennifer A Rothman, J Paul Scott, Jeremie H Estepp, Jane S Hankins
Hemoglobin S/Black ((A) γδβ)(0) -thalassemia is a rare sickle cell disease (SCD) variant. On the basis of limited descriptions in the literature, the disease is reported as a mild microcytic anemia with an uncomplicated course. We report the clinical and laboratory data of nine patients whose diagnoses were confirmed by DNA-based techniques. Despite having mild anemia and high fetal hemoglobin level postinfancy, these patients developed many of the classic complications of SCD, including vaso-occlusive crisis, acute chest syndrome, avascular necrosis, and cholelithiasis...
April 28, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28443234/plastic-bronchitis-an-unusual-complication-of-acute-chest-syndrome-in-adult
#10
Sarah Feray, Pierre Mora, Maxens Decavele, Tài Pham, El Mahdi Hafiani, Muriel Fartoukh
Plastic bronchitis is used to designate endobronchial plugs of rubber-like consistency that form into bronchial trees. It has been described in several diseases like asthma, cystic fibrosis, pulmonary infection, cyanotic congenital heart disease and in few young children with homozygous sickle cell disease. We report the first sickle cell adult case of plastic bronchitis during acute chest syndrome. He developed severe acute respiratory distress syndrome. This unusual presentation related to obstruction by voluminous casts may alert physicians to focus more on the bronchi in sickle cell patients...
2017: Respiratory Medicine Case Reports
https://www.readbyqxmd.com/read/28426137/hydroxyurea-hydroxycarbamide-for-sickle-cell-disease
#11
REVIEW
Sarah J Nevitt, Ashley P Jones, Jo Howard
BACKGROUND: Sickle cell disease (SCD) is one of the most common inherited diseases worldwide. It is associated with lifelong morbidity and a reduced life expectancy. Hydroxyurea (hydroxycarbamide), an oral chemotherapeutic drug, ameliorates some of the clinical problems of SCD, in particular that of pain, by raising fetal haemoglobin. This is an update of a previously published Cochrane Review. OBJECTIVES: To assess the effects of hydroxyurea therapy in people with SCD (all genotypes), of any age, regardless of setting...
April 20, 2017: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/28421813/hemin-causes-lung-microvascular-endothelial-barrier-dysfunction-by-necroptotic-cell-death
#12
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/28373264/association-of-circulating-transcriptomic-profiles-with-mortality-in-sickle-cell-disease
#13
Ankit A Desai, Zhengdeng Lei, Neil Bahroos, Mark Maienschein-Cline, Santosh L Saraf, Xu Zhang, Binal N Shah, Seyed M Nouraie, Taimur Abbasi, Amit R Patel, Roberto M Lang, Yves Lussier, Joe G N Garcia, Victor R Gordeuk, Roberto F Machado
Sickle cell disease (SCD) complications are associated with increased morbidity and risk of mortality. We sought to identify a circulating transcriptomic profile predictive of these poor outcomes in SCD. Training and Testing cohorts consisting of adult patients with SCD were recruited and prospectively followed. A pathway-based signature derived from grouping peripheral blood mononuclear cell transcriptomes distinguished two patient clusters with differences in survival in the Training cohort. These findings were validated in a Testing cohort where the association between Cluster 1 molecular profiling and mortality remained significant in a fully adjusted model...
April 3, 2017: Blood
https://www.readbyqxmd.com/read/28293403/hydroxycarbamine-from-an-old-drug-used-in-malignant-hemopathies-to-a-current-standard-in-sickle-cell-disease
#14
REVIEW
Giovanna Cannas, Solène Poutrel, Xavier Thomas
While hydroxycarbamide (hydroxyurea, HU) has less and fewer indications in malignant hemopathies, it represents the only widely used drug which modifies sickle cell disease pathogenesis. Clinical experience with HU for patients with sickle cell disease has been accumulated over the past 25 years in Western countries. The review of the literature provides increasing support for safety and efficacy in both children and adults for reducing acute vaso-occlusive events including pain episodes and acute chest syndrome...
2017: Mediterranean Journal of Hematology and Infectious Diseases
https://www.readbyqxmd.com/read/28267033/factors-influencing-utilization-of-hospital-services-by-adult-sickle-cell-disease-patients-a-systematic-review
#15
Irina Benenson, Yuri Jadotte, Mercedes Echevarria
BACKGROUND: Painful vaso-occlusive crisis is a hallmark of sickle cell disease (SCD) that commonly results in utilization of hospital services. Recurrent use of hospital services by SCD patients is associated with high healthcare costs and adverse clinical outcomes. Understanding the factors influencing the pattern of utilization is a first step in improving medical care of this patient population while reducing healthcare expenditures. OBJECTIVES: The primary objective of this systematic review was to determine what modifiable and non-modifiable factors influence utilization of hospital services by adult SCD patients...
March 2017: JBI Database of Systematic Reviews and Implementation Reports
https://www.readbyqxmd.com/read/28256526/vascular-permeability-drives-susceptibility-to-influenza-infection-in-a-murine-model-of-sickle-cell-disease
#16
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/28228943/a-case-series-of-cholecystectomy-in-jamaican-sickle-cell-disease-patients-the-need-for-a-new-strategy
#17
Pierre-Anthony Leake, Marvin Reid, Joseph Plummer
High morbidity rates related to cholecystectomy in sickle cell disease (SCD) patients have been previously reported in the region. This study serves to assess the current outcomes related to cholecystectomy in a Jamaican SCD population. METHODS: A retrospective chart review of SCD patients undergoing elective cholecystectomy at the University Hospital of the West Indies over a 6-year period was performed providing relevant information for analysis. Patients were grouped on an intention-to-treat basis into an open and laparoscopic group...
March 2017: Annals of Medicine and Surgery
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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