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Acute chest syndrome in sickle cell disease

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https://www.readbyqxmd.com/read/28097236/lung-vaso-occlusion-in-sickle-cell-disease-mediated-by-arteriolar-neutrophil-platelet-microemboli
#1
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/28094851/blood-transfusion-for-preventing-primary-and-secondary-stroke-in-people-with-sickle-cell-disease
#2
REVIEW
Lise J Estcourt, Patricia M Fortin, Sally Hopewell, Marialena Trivella, Winfred C Wang
BACKGROUND: Sickle cell disease is one of the commonest severe monogenic disorders in the world, due to the inheritance of two abnormal haemoglobin (beta globin) genes. Sickle cell disease can cause severe pain, significant end-organ damage, pulmonary complications, and premature death. Stroke affects around 10% of children with sickle cell anaemia (HbSS). Chronic blood transfusions may reduce the risk of vaso-occlusion and stroke by diluting the proportion of sickled cells in the circulation...
January 17, 2017: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/28060121/the-sickle-%C3%AE-thalassemia-phenotype
#3
Adekunle D Adekile, Nagihan Akbulut, Asmaa F Azab, Sundus Al-Sharida, Diana Thomas
Sβ-thalassemia (Sβ-thal) is common among Gulf Arab patients with sickle cell disease, but the phenotype of this group had not been well-documented. We have studied a group of Kuwaiti patients and compared the phenotype in the homozygotes (SS) and Sβ-thal patients. Complete blood count, hemoglobin quantitation, serum bilirubin, and lactate dehydrogenase were determined with standard techniques. The patients were screened for α-globin genotype. The Sβ-thal patients were also screened for the HBG2 Xmn-1 polymorphism...
January 5, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28008687/hot-off-the-press-which-febrile-children-with-sickle-cell-disease-need-a-chest-x-ray
#4
Justin Morgenstern, Corey Heitz, William K Milne
This retrospective chart review examined the rate of acute chest syndrome (ACS) in febrile children (aged 3 months to 21 years) with sickle cell disease and used recursive partitioning to determine which clinical factors were predictive of a diagnosis of ACS. Over the course of 2 years, 697 children made 1837 visits to one of two pediatric emergency departments. ACS was diagnosed in 185 (10%) of the visits. This article is protected by copyright. All rights reserved.
December 23, 2016: Academic Emergency Medicine: Official Journal of the Society for Academic Emergency Medicine
https://www.readbyqxmd.com/read/28005272/prophylactic-versus-selective-blood-transfusion-for-sickle-cell-disease-in-pregnancy
#5
REVIEW
Babasola O Okusanya, Olufemi T Oladapo
BACKGROUND: Pregnant women with sickle cell disease (HbSS, HbSC and HbSβThal) may require blood transfusion to prevent severe anaemia or to manage potential medical complications. Preventive blood transfusion in the absence of complications starting from the early weeks of pregnancy or blood transfusion only for medical or obstetric indications have been used as management policies. There is currently no consensus on the blood transfusion policy that guarantees optimal clinical benefits with minimal risks for such women and their babies...
22, 2016: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/27994488/an-unfortunate-case-of-acquired-hemochromatosis-a-case-report-review-of-the-clinical-presentation-diagnosis-management-and-prognosis
#6
Anam Tariq, Kevin Westra, Arben Santo
BACKGROUND: While blood transfusions are commonly used for prophylaxis and treatment for acute chest syndromes and strokes in sickle cell patients, accumulation of excess iron resulting in secondary hemochromatosis remains a rare disease. Chelation is the mainstay for preventing and treating iron overload to deter potential end-organ damages; it is rare when therapy fails. CASE REPORT: A 52-year-old African American woman with chronic anemia secondary to sickle cell anemia and history of multiple blood transfusions presented with elevated serum ferritin (8000 ng/mL) and bilirubin (16...
2016: International Medical Case Reports Journal
https://www.readbyqxmd.com/read/27981643/ugt1a1-ta-n-genotype-is-not-the-major-risk-factor-of-cholelithiasis-in-sickle-cell-disease-children
#7
Philippe Joly, Céline Renoux, Philippe Lacan, Yves Bertrand, Giovanna Cannas, Nathalie Garnier, Daniella Cuzzubbo, Kamila Kebaïli, Cécile Renard, Alexandra Gauthier, Vincent Pialoux, Cyril Martin, Marc Romana, Philippe Connes
Because of the increased hemolytic rate, a significant proportion of patients with sickle cell disease (SCD) are prone to develop cholelithiasis. The present study investigated the role of several genetic factors (UGT1A1 promoter (TA)n repeat polymorphism, alpha-globin status), hematological parameters, clinical severity and hydroxyurea (HU) therapy on the occurrence of cholelithiasis in SCD METHODS: One hundred and fifty eight children (2-18 years old) and regularly followed at the university hospital of Lyon (France) were included...
December 16, 2016: European Journal of Haematology
https://www.readbyqxmd.com/read/27959701/crizanlizumab-for-the-prevention-of-pain-crises-in-sickle-cell-disease
#8
Kenneth I Ataga, Abdullah Kutlar, Julie Kanter, Darla Liles, Rodolfo Cancado, João Friedrisch, Troy H Guthrie, Jennifer Knight-Madden, Ofelia A Alvarez, Victor R Gordeuk, Sandra Gualandro, Marina P Colella, Wally R Smith, Scott A Rollins, Jonathan W Stocker, Russell P Rother
Background The up-regulation of P-selectin in endothelial cells and platelets contributes to the cell-cell interactions that are involved in the pathogenesis of vaso-occlusion and sickle cell-related pain crises. The safety and efficacy of crizanlizumab, an antibody against the adhesion molecule P-selectin, were evaluated in patients with sickle cell disease. Methods In this double-blind, randomized, placebo-controlled, phase 2 trial, we assigned patients to receive low-dose crizanlizumab (2.5 mg per kilogram of body weight), high-dose crizanlizumab (5...
December 3, 2016: New England Journal of Medicine
https://www.readbyqxmd.com/read/27957377/rare-case-of-posterior-reversible-leukoencephalopathy-syndrome-secondary-to-acute-chest-syndrome
#9
Rohit Aiyer, Daniel Klein, Yasir El-Sherif
We present a case of 29/m with a history of sickle cell disease who presented to the emergency department with sudden onset of chest, trunk, extremity, and back pain, consistent in quality and severity with the patient's usual pain crises. Soon after admission to the medical unit for acute chest syndrome (ACS), the patient developed sudden onset of hypertension associated with left sided hemiplegia, lethargy, dysarthria, aphasia, and left sided facial droop. Neuroimaging revealed that on MRI Brain there was multifocal extensive signal abnormality and a small focal areas of hemorrhage compatible with posterior reversible leukoencephalopathy syndrome (PRES)...
2016: Case Reports in Radiology
https://www.readbyqxmd.com/read/27913538/sickle-cell-disease-when-and-how-to-transfuse
#10
Jo Howard
Blood transfusion remains an important therapeutic intervention in patients with sickle cell disease (SCD), aiming to both increase the oxygen carrying capacity of blood and to reduce the complications of vaso-occlusion. Simple, manual exchange and automated exchange can be effective in reducing the acute and chronic complications of SCD, and the advantages and disadvantages of each methodology mean they all have a role in different situations. Evidence for the role of emergency transfusion in the management of the acute complications of SCD, including acute pain and acute chest syndrome, comes from observational data...
December 2, 2016: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/27845754/secondhand-smoke-is-an-important-modifiable-risk-factor-in-sickle-cell-disease-a-review-of-the-current-literature-and-areas-for-future-research
#11
REVIEW
S Christy Sadreameli, Benjamin T Kopp, Susan E Creary, Michelle N Eakin, Sharon McGrath-Morrow, John J Strouse
Sickle cell disease (SCD) is an autosomal recessive hemoglobinopathy that causes significant morbidity and mortality related to chronic hemolytic anemia, vaso-occlusion, and resultant end-organ damage. Tobacco smoke exposure (TSE) through secondhand smoke exposure in people with SCD of all ages and through primary smoking in adolescents and adults is associated with significantly increased morbidity, with increased rates of emergency department visits and hospitalizations for painful vaso-occlusive crises and acute chest syndrome (ACS)...
November 12, 2016: International Journal of Environmental Research and Public Health
https://www.readbyqxmd.com/read/27775898/managing-acute-complications-of-sickle-cell-disease-in-pediatric-patients
#12
REVIEW
Sathyaseelan Subramaniam, Jennifer H Chao
Sickle cell disease is a chronic hematologic disease with a variety of acute, and often recurring, complications. Vaso-occlusive crisis, a unique but common presentation in sickle cell disease, can be challenging to manage. Acute chest syndrome is the leading cause of death in patients with sickle cell disease, occurring in more than half of patients who are hospitalized with a vaso-occlusive crisis. Uncommon diagnoses in children, such as stroke, priapism, and transient red cell aplasia, occur more frequently in patients with sickle cell disease and necessitate a degree of familiarity with the disease process and its management...
November 2016: Pediatric Emergency Medicine Practice
https://www.readbyqxmd.com/read/27673392/inhaled-bronchodilators-for-acute-chest-syndrome-in-people-with-sickle-cell-disease
#13
REVIEW
Jennifer M Knight-Madden, Ian R Hambleton
BACKGROUND: Bronchodilators are used to treat bronchial hyper-responsiveness in asthma. Bronchial hyper-responsiveness may be a component of acute chest syndrome in people with sickle cell disease. Therefore, bronchodilators may be useful in the treatment of acute chest syndrome. This is an update of a previously published Cochrane Review. OBJECTIVES: To assess the benefits and risks associated with the use of bronchodilators in people with acute chest syndrome...
September 27, 2016: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/27642150/-pulmonary-complications-of-sickle-cell-disease-in-children
#14
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/27625358/a-trial-of-unrelated-donor-marrow-transplantation-for-children-with-severe-sickle-cell-disease
#15
Shalini Shenoy, Mary Eapen, Julie A Panepinto, Brent R Logan, Juan Wu, Allistair Abraham, Joel Brochstein, Sonali Chaudhury, Kamar Godder, Ann E Haight, Kimberly A Kasow, Kathryn Leung, Martin Andreansky, Monica Bhatia, Jignesh Dalal, Hilary Haines, Jennifer Jaroscak, Hillard M Lazarus, John E Levine, Lakshmanan Krishnamurti, David Margolis, Gail C Megason, Lolie C Yu, Michael A Pulsipher, Iris Gersten, Nancy DiFronzo, Mary M Horowitz, Mark C Walters, Naynesh Kamani
Children with sickle cell disease experience organ damage, impaired quality of life, and premature mortality. Allogeneic bone marrow transplant from an HLA-matched sibling can halt disease progression but is limited by donor availability. A Blood and Marrow Transplant Clinical Trials Network (BMT CTN) phase 2 trial conducted from 2008 to 2014 enrolled 30 children aged 4 to 19 years; 29 were eligible for evaluation. The primary objective was 1-year event-free survival (EFS) after HLA allele-matched (at HLA-A, -B, -C, and -DRB1 loci) unrelated donor transplant...
November 24, 2016: Blood
https://www.readbyqxmd.com/read/27598353/symptomatic-avascular-necrosis-an-understudied-risk-factor-for-acute-care-utilization-by-patients-with-scd
#16
Tiffany Yu, Timothy Campbell, Isabella Ciuffetelli, Carlton Haywood, Christopher Patrick Carroll, Linda Resar, John J Strouse, Sophie Lanzkron
OBJECTIVES: Sickle cell disease (SCD) is associated with high healthcare utilization rates and poor outcomes in a subset of patients, although the underlying factors that predict this phenotype are poorly understood. Prior studies suggest that comorbid avascular necrosis (AVN) contributes to high healthcare utilization. We sought to clarify whether AVN independently predicts acute care utilization in adults with SCD and to identify characteristics of those with AVN that predict higher utilization...
September 2016: Southern Medical Journal
https://www.readbyqxmd.com/read/27574910/blood-transfusions-for-treating-acute-chest-syndrome-in-people-with-sickle-cell-disease
#17
REVIEW
Saeed Dastgiri, Roya Dolatkhah
BACKGROUND: Sickle cell disease is an inherited autosomal recessive blood condition and is one of the most prevalent genetic blood diseases worldwide. Acute chest syndrome is a frequent complication of sickle cell disease, as well as a major cause of morbidity and the greatest single cause of mortality in children with sickle cell disease. Standard treatment may include intravenous hydration, oxygen as treatment for hypoxia, antibiotics to treat the infectious cause and blood transfusions may be given...
August 30, 2016: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/27553919/emergency-medicine-management-of-sickle-cell-disease-complications-an-evidence-based-update
#18
Erica Simon, Brit Long, Alex Koyfman
BACKGROUND: Sickle cell disease (SCD) affects approximately 100,000 individuals in the United States. Due to alterations in the structural conformation of hemoglobin molecules under deoxygenated conditions, patients with SCD are predisposed to numerous sequelae, many of which require acute intervention. OBJECTIVE: Our aim was to provide emergency physicians with an evidence-based update regarding the diagnosis and management of SCD complications. DISCUSSION: SCD patients experience significant morbidity and mortality secondary to cerebrovascular accident, acute chest syndrome, acute vaso-occlusive pain crises, SCD-related multi-organ failure, cholecystitis, acute intrahepatic cholestasis, acute sickle hepatic crisis, acute hepatic sequestration, priapism, and renal disease...
October 2016: Journal of Emergency Medicine
https://www.readbyqxmd.com/read/27535451/phenotypic-diversity-of-sickle-cell-disease-in-patients-with-a-double-heterozygosity-for-hb-s-and-hb-d-punjab
#19
Lidiane S Torres, Jéssika V Okumura, Édis Belini-Júnior, Renan G Oliveira, Patrícia P Nascimento, Danilo G H Silva, Clarisse L C Lobo, Sonia M Oliani, Claudia R Bonini-Domingos
Phenotypic heterogeneity for sickle cell disease is associated to several genetic factors such as genotype for sickle cell disease, β-globin gene cluster haplotypes and Hb F levels. The coinheritance of Hb S (HBB: c.20A > T) and Hb D-Punjab (HBB: c.364G > C) results in a double heterozygosity, which constitutes one of the genotypic causes of sickle cell disease. This study aimed to assess the phenotypic diversity of sickle cell disease presented by carriers of the Hb S/Hb D-Punjab genotype and the Bantu [- + - - - -] haplotype...
September 2016: Hemoglobin
https://www.readbyqxmd.com/read/27509149/acute-chest-syndrome-asthma-and-lung-function-in-sickle-cell-disease-which-is-the-chicken-and-which-is-the-egg
#20
Anastassios C Koumbourlis
No abstract text is available yet for this article.
August 2016: Annals of the American Thoracic Society
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