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Sickle cell diseases

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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/28096988/successful-outcome-of-three-patients-with-sickle-cell-disease-and-fat-embolism-syndrome-treated-with-intensive-exchange-transfusion
#2
Paul Greaves, Vivek Mathew, Catherine Peters, Susan Rowe, Roger J Amos, Dimitris A Tsitsikas
Fat embolism syndrome (FES) is a rare complication of sickle-cell disease (SCD) associated with extremely high mortality rates. It affects predominantly non-SS patients and those with previously mild disease. Rapid institution of exchange transfusion with an aim to reduce HbS to very low levels as soon as FES is suspected can be life-saving.
January 2017: Clinical Case Reports
https://www.readbyqxmd.com/read/28096387/kinetic-assay-shows-that-increasing-red-cell-volume-could-be-a-treatment-for-sickle-cell-disease
#3
Quan Li, Eric R Henry, James Hofrichter, Jeffrey F Smith, Troy Cellmer, Emily B Dunkelberger, Belhu B Metaferia, Stacy Jones-Straehle, Sarah Boutom, Garrott W Christoph, Terri H Wakefield, Mary E Link, Dwayne Staton, Erica R Vass, Jeffery L Miller, Matthew M Hsieh, John F Tisdale, William A Eaton
Although it has been known for more than 60 years that the cause of sickle cell disease is polymerization of a hemoglobin mutant, hydroxyurea is the only drug approved for treatment by the US Food and Drug Administration. This drug, however, is only partially successful, and the discovery of additional drugs that inhibit fiber formation has been hampered by the lack of a sensitive and quantitative cellular assay. Here, we describe such a method in a 96-well plate format that is based on laser-induced polymerization in sickle trait cells and robust, automated image analysis to detect the precise time at which fibers distort ("sickle") the cells...
January 17, 2017: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/28094851/blood-transfusion-for-preventing-primary-and-secondary-stroke-in-people-with-sickle-cell-disease
#4
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/28094443/validity-of-simple-clinical-and-biological-parameters-as-screening-tool-for-sickle-cell-anemia-for-referral-to-tertiary-center-in-highly-resource-constraints
#5
Bertin Tshimanga Kadima, Jean Lambert Gini-Ehungu, Fiston Ikwa Ndol Mbutiwi, John Tunda Bahati, Michel Ntetani Aloni
BACKGROUND: In the Democratic Republic of Congo, the incidence of sickle cell anemia (SCA) is estimated around 40 000 neonates per year. However, it is notoriously difficult to perform conventional electrophoresis in all hospitals and laboratories, especially at peripheral levels and rural area. A panel of multiple clinical and laboratory features that would enhance sickle cell disease were assessed for the detection of the disease in highly resource-scarce settings. METHODS: A prospective study was conducted in Kinshasa...
January 17, 2017: Journal of Clinical Laboratory Analysis
https://www.readbyqxmd.com/read/28093814/management-of-sickle-cell-disease-in-patients-undergoing-cardiac-surgery
#6
REVIEW
Todd C Crawford, Michael V Carter, Rina K Patel, Alejandro Suarez-Pierre, Sophie Z Lin, Jonathan Trent Magruder, Joshua C Grimm, Duke E Cameron, William A Baumgartner, Kaushik Mandal
Sickle cell disease is a life-limiting inherited hemoglobinopathy that poses inherent risk for surgical complications following cardiac operations. In this review, we discuss preoperative considerations, intraoperative decision-making, and postoperative strategies to optimize the care of a patient with sickle cell disease undergoing cardiac surgery.
January 16, 2017: Journal of Cardiac Surgery
https://www.readbyqxmd.com/read/28090285/pulmonary-vascular-and-ventricular-dysfunction-in-the-susceptible-patient-2015-grover-conference-series
#7
REVIEW
Bradley A Maron, Roberto F Machado, Larissa Shimoda
Pulmonary blood vessel structure and tone are maintained by a complex interplay between endogenous vasoactive factors and oxygen-sensing intermediaries. Under physiological conditions, these signaling networks function as an adaptive interface between the pulmonary circulation and environmental or acquired perturbations to preserve oxygenation and maintain systemic delivery of oxygen-rich hemoglobin. Chronic exposure to hypoxia, however, triggers a range of pathogenetic mechanisms that include hypoxia-inducible factor 1α (HIF-1α)-dependent upregulation of the vasoconstrictor peptide endothelin 1 in pulmonary endothelial cells...
December 2016: Pulmonary Circulation
https://www.readbyqxmd.com/read/28089760/enhanced-long-term-brain-mri-evaluation-of-children-with-sickle-cell-disease-following-hematopoietic-cell-transplantation
#8
Nancy S Green, Monica Bhatia, Erica Y Griffith, Mahvish Qureshi, Courtney Briamonte, Mirko Savone, Stephen Sands, Margaret T Lee, Angela Lignelli, Adam M Brickman
Progressive neurovasculopathy in children with sickle cell disease (SCD) results in decreased cognitive function and quality of life (QoL). Hematopoietic cell transplant (HCT) is believed to halt progression of neurovasculopathy. Quantitative analysis of T2-weighted fluid attenuated inversion recovery (FLAIR) MRI for white matter hyperintensity (WMH) burden provides a meaningful estimate of small vessel cerebrovascular disease. We asked if quantitative analysis of WMH could complement standardized clinical assessment of MRI/MRA for assessing SCD CNS vasculopathy pre- and post-HCT...
January 9, 2017: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/28088643/molecular-mechanisms-of-bio-catalysis-of-heme-extraction-from-hemoglobin
#9
Serzhan Sakipov, Olga Rafikova, Maria G Kurnikova, Ruslan Rafikov
Red blood cell hemolysis in sickle cell disease (SCD) releases free hemoglobin. Extracellular hemoglobin and its degradation products, free heme and iron, are highly toxic due to oxidative stress induction and decrease in nitric oxide availability. We propose an approach that helps to eliminate extracellular hemoglobin toxicity in SCD by employing a bacterial protein system that evolved to extract heme from extracellular hemoglobin. NEAr heme Transporter (NEAT) domains from iron-regulated surface determinant proteins from Staphylococcus aureus specifically bind free heme as well as facilitate its extraction from hemoglobin...
January 7, 2017: Redox Biology
https://www.readbyqxmd.com/read/28078488/hydroxyurea-treatment-effect-on-children-with-sickle-cell-disease-and-obstructive-sleep-apnea
#10
Anthony J Grady, Jane S Hankins, Brent Haberman, Robert Schoumacher, Rose Mary Stocks
BACKGROUND: While hydroxyurea is the mainstay of treatment for many of the comorbidities associated with sickle cell disease, its effect on obstructive sleep apnea has not been fully investigated. The purpose of this project is to help characterize the effects of hydroxyurea on obstructive sleep apnea in children with sickle cell disease and determine its therapeutic role in the condition. METHODS: Chart review was conducted on two pediatric patients with sickle cell disease who experienced resolution of obstructive sleep apnea following hydroxyurea administration...
January 11, 2017: Sleep & Breathing, Schlaf & Atmung
https://www.readbyqxmd.com/read/28076805/regulation-of-active-icam-4-on-normal-and-sickle-cell-disease-rbcs-via-akaps-is-revealed-by-afm
#11
Jing Zhang, Krithika Abiraman, Sasia-Marie Jones, George Lykotrafitis, Biree Andemariam
Human healthy (wild-type (WT)) and homozygous sickle (SS) red blood cells (RBCs) express a large number of surface receptors that mediate cell adhesion between RBCs, and between RBCs and white blood cells, platelets, and the endothelium. In sickle cell disease (SCD), abnormal adhesion of RBCs to endothelial cells is mediated by the intercellular adhesion molecule-4 (ICAM-4), which appears on the RBC membrane and binds to the endothelial αvβ3 integrin. This is a key factor in the initiation of vaso-occlusive episodes, the hallmark of SCD...
January 10, 2017: Biophysical Journal
https://www.readbyqxmd.com/read/28070238/hematological-disorders-and-pulmonary-hypertension
#12
REVIEW
Rajamma Mathew, Jing Huang, Joseph M Wu, John T Fallon, Michael H Gewitz
Pulmonary hypertension (PH), a serious disorder with a high morbidity and mortality rate, is known to occur in a number of unrelated systemic diseases. Several hematological disorders such as sickle cell disease, thalassemia and myeloproliferative diseases develop PH which worsens the prognosis. Associated oxidant injury and vascular inflammation cause endothelial damage and dysfunction. Pulmonary vascular endothelial damage/dysfunction is an early event in PH resulting in the loss of vascular reactivity, activation of proliferative and antiapoptotic pathways leading to vascular remodeling, elevated pulmonary artery pressure, right ventricular hypertrophy and premature death...
December 26, 2016: World Journal of Cardiology
https://www.readbyqxmd.com/read/28065838/current-results-and-future-research-priorities-in-late-effects-after-hematopoietic-stem-cell-transplantation-hct-for-children-with-sickle-cell-disease-and-thalassemia-a-consensus-statement-from-the-second-pediatric-blood-and-marrow-transplant-consortium-international
#13
Shalini Shenoy, Emanuele Angelucci, Staci D Arnold, K Scott Baker, Monica Bhatia, Dorine Bresters, Andrew C Dietz, Josu De La Fuente, Christine Duncan, Javid Gaziev, Allison A King, Michael A Pulsipher, Angela Smith, Mark C Walters
Sustained donor engraftment after allogeneic hematopoietic cell transplantation (HCT) converts to healthy donor hemoglobin synthesis and halts disease symptoms in patients with sickle cell disease (SCD) and thalassemia major. A disease free survival probability that exceeds 90% has been reported when HCT using an HLA-matched sibling donor is performed in young patients with low risk disease or treatment related risk factors. Alternate donor HCT and HCT in adults is performed infrequently due to a higher risk profile...
January 5, 2017: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/28065813/aapt-diagnostic-criteria-for-chronic-sickle-cell-disease-pain
#14
REVIEW
Carlton Dampier, Tonya M Palermo, Deepika S Darbari, Kathryn Hassell, Wally Smith, William Zempsky
: Pain in sickle cell disease (SCD) is associated with increased morbidity, mortality, and high healthcare costs. While episodic acute pain is the hallmark of this disorder, there is an increasing awareness that chronic pain is part of the pain experience of many older adolescents and adults. A common set of criteria for classifying chronic pain associated with SCD would enhance SCD pain research efforts in epidemiology, pain mechanisms, and clinical trials of pain management interventions, and ultimately improve clinical assessment and management...
January 5, 2017: Journal of Pain: Official Journal of the American Pain Society
https://www.readbyqxmd.com/read/28065771/risk-factors-for-30-day-readmission-in-adults-with-sickle-cell-disease
#15
Max A Brodsky, Mark Rodeghier, Maureen Sanger, Jeannie Byrd, Brandi McClain, Brittany Covert, Dionna O Roberts, Karina Wilkerson, Michael R DeBaun, Adetola A Kassim
BACKGROUND: Readmission to the hospital within 30-days is a measure of quality care; however, only few modifiable risk factors for 30-day readmission in adults with sickle cell disease are known. METHODS: We performed a retrospective review of the medical records of adults with sickle cell disease at a tertiary care center, to identify potentially modifiable risk factors for 30-day re-admission due to vaso-occulsive pain episodes. A total of 88 patients > 18 years of age were followed for 3...
January 5, 2017: American Journal of Medicine
https://www.readbyqxmd.com/read/28060121/the-sickle-%C3%AE-thalassemia-phenotype
#16
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/28059954/perceived-racial-bias-and-health-related-stigma-among-youth-with-sickle-cell-disease
#17
Emily O Wakefield, Jill M Popp, Lourdes P Dale, James P Santanelli, Ashley Pantaleao, William T Zempsky
OBJECTIVE: Little is known about the role of perceived racial bias and health-related stigma on the health of youth with sickle cell disease (SCD). The purpose of this study was to investigate the occurrence of perceived racial bias and health-related stigma among youth with SCD and its relationship with psychological and physical well-being. METHODS: Twenty-eight youth with SCD, ages 13 to 21, were recruited from outpatient and inpatient settings at an urban children's medical center...
January 4, 2017: Journal of Developmental and Behavioral Pediatrics: JDBP
https://www.readbyqxmd.com/read/28054978/hemoglobin-based-blood-substitutes-and-the-treatment-of-sickle-cell-disease-more-harm-than-help
#18
REVIEW
Abdu I Alayash
Intense efforts have been made by both industry and academia over the last three decades to produce viable hemoglobin (Hb)-based oxygen carriers (HBOCs), also known as "blood substitutes". Human trials conducted so far by several manufactures in a variety of clinical indications, including trauma, and elective surgeries have failed and no product has gained the Food and Drug Administration approval for human use. Safety concerns due to frequent incidences of hemodynamic, cardiac events, and even death led to the termination of some of these trials...
January 4, 2017: Biomolecules
https://www.readbyqxmd.com/read/28050373/histopathologic-findings-in-autopsies-with-emphasis-on-interesting-and-incidental-findings-a-pathologist-s-perspective
#19
Sapna Patel, B R Rajalakshmi, G V Manjunath
INTRODUCTION: Autopsy aids to the knowledge of pathology by unveiling the rare lesions which are a source of learning from a pathologist's perspective Some of them are only diagnosed at autopsy as they do not cause any functional derangement. This study emphasizes the various incidental lesions which otherwise would have been unnoticed during a person's life. AIM: The aim of this study was to determine the spectrum of histopathological findings including neoplastic lesions related or unrelated to the cause of death...
November 2016: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28050370/an-unusual-case-of-septic-arthritis-of-the-hip
#20
Ujjwayini Ray, Soma Dutta, Arpita Sutradhar
Non-typhoidal Salmonella, particularly Salmonella enterica serovar typhimurium is food borne pathogen causing mild self-limiting diarrhoea in healthy adults. It can occasionally cause extraintestinal focal infection in susceptible patients. Salmonella, as the aetiological agent of osteomyelitis and septic arthritis is rare and has been mostly reported in patients with sickle cell disease or thalassaemia. We report a case of septic arthritis by Salmonellatyphimurium in an immunocompromised patient who was successfully treated following timely isolation and identification of the aetiological agent...
November 2016: Journal of Clinical and Diagnostic Research: JCDR
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