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Sickle cell anemia

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https://www.readbyqxmd.com/read/28428653/doppler-velocimetry-of-the-orbital-arteries-in-patients-with-sickle-cell-anemia-relationship-with-biomarkers-of-hemolysis
#1
Thiago de Oliveira Ferrão, Paulo Ricardo Martins-Filho, Cleverton Aragão, Marlyson Santana, Allan Nascimento, Thayane Cardoso, Rosana Cipolotti
OBJECTIVE: To investigate orbital vascular resistance by Doppler velocimetry in patients with steady-state sickle cell anemia, as well as to characterize its relationship with biomarkers of hemolysis. MATERIALS AND METHODS: This was a cross-sectional study of two groups: 71 outpatients with sickle cell anemia; and 32 age- and gendermatched, healthy subjects (control group). All participants underwent Doppler velocimetry of the orbital arteries and laboratory tests...
March 2017: Radiologia Brasileira
https://www.readbyqxmd.com/read/28425749/hemolytic-transfusion-reaction-attributable-to-anti-dia
#2
Arthur J Joyce, Kelli M Quantock, Ray Banh, Yew W Liew
In situations when a patient's antibody detection test is negative, many institutions have moved from an indirect antiglobulin test (IAT) crossmatch to an electronic crossmatch system. Here we report a case of an acute hemolytic transfusion reaction attributable to anti-Dia in a patient with a negative antibody detection test. A 22-year-old female patient with a diagnosis of β thalassemia and sickle cell anemia commenced a routine exchange transfusion of 5 units of red blood cells (RBCs) in the apheresis unit as part of her regular treatment...
January 2017: Immunohematology
https://www.readbyqxmd.com/read/28424766/the-need-for-hematology-nurse-education-in-low-and-middle-income-countries-a-community-case-study-in-tanzania
#3
Julie M Buser
Hematology-related diseases, such as anemia, malaria, sickle cell disease (SCD), and blood cancers, have differing rates of survival between high-income and low- and middle-income countries (LMICs). Nurses in LMICs have an unmet need for specialty training and education to address hematology and hemato-oncology disorders. A gap in the literature exists about hematology nurse education and clinical service demands in LMICs. This community case study documents a collaborative hematology and basic hemato-oncology education program to sustainably strengthen nurse capacity at a national referral hospital and university in Tanzania...
2017: Frontiers in Public Health
https://www.readbyqxmd.com/read/28417646/cerebral-hemodynamic-assessment-and-neuroimaging-across-the-lifespan-in-sickle-cell-disease
#4
Lori C Jordan, Michael R DeBaun
Children and adults with sickle cell anemia (SCA) have a higher risk of strokes compared to age- and race-matched peers. Velocity in the middle cerebral or distal internal carotid artery as measured by transcranial Doppler ultrasound is a recognized method to identify children but not adults with SCA at high-risk for first stroke. For both children and adults with SCA that have had a stroke, no methods clearly identify individuals at highest risk of recurrent strokes or an initial silent stroke, the most common neurological injury...
January 1, 2017: Journal of Cerebral Blood Flow and Metabolism
https://www.readbyqxmd.com/read/28412236/are-dental-and-jaw-changes-more-prevalent-in-a-brazilian-population-with-sickle-cell-anemia
#5
Halinna Larissa Cruz Correia de Carvalho, José Yagoh Saraiva Rolim, Érika Bárbara Abreu Fonseca Thomaz, Soraia de Fátima Carvalho Souza
OBJECTIVE: The aim of this study was to analyze the prevalence of dental and jaw changes in individuals with sickle cell anemia (SCA) compared with a control group from a Brazilian population. STUDY DESIGN: This was a retrospective cross-sectional study. Participants were divided into 2 groups: SCA and control (123 patients per group). Intraoral periapical radiographs were taken. Changes in bone pattern and dental changes were investigated by using the following radiographic parameters: (1) trabecular bone (step-ladder or spider web); (2) integrity of the lamina dura (loss of sharpness and continuity, partial or complete absence, and increased thickness); and (3) dental changes (pulp chamber, root surface, periapex, dental form, number, and position)...
March 9, 2017: Oral Surgery, Oral Medicine, Oral Pathology and Oral Radiology
https://www.readbyqxmd.com/read/28411485/cerebral-and-muscle-microvascular-oxygenation-in-children-with-sickle-cell-disease-influence-of-hematology-hemorheology-and-vasomotion
#6
Keyne Charlot, Sophie Antoine-Jonville, Berenike Moeckesch, Stéphane Jumet, Marc Romana, Xavier Waltz, Lydia Divialle-Doumdo, Marie-Dominique Hardy-Dessources, Marie Petras, Benoît Tressières, Vanessa Tarer, Olivier Hue, Maryse Etienne-Julan, Philippe Connes
The present study investigated cerebral and muscle hemoglobin oxygen saturation (tissue oxygen index, TOI) in children with sickle cell anemia (SS), sickle cell hemoglobin C disease (SC) and healthy children (AA). TOI was measured by near-infrared spectroscopy (NIRS) and spectral analysis of the TOI variability was used to assess flowmotion and vasomotion. Arterial oxyhemoglobin saturation (SpO2), hemorheological and hematological parameters were also measured in SS and SC children. Both TOI were lower in SS compared to both AA and SC children, with SC exhibiting lower values than AA children...
April 4, 2017: Blood Cells, Molecules & Diseases
https://www.readbyqxmd.com/read/28402148/should-modulation-of-p50-be-a-therapeutic-target-in-the-critically-ill
#7
Amudan J Srinivasan, Clare Morkane, Daniel S Martin, Ian J Welsby
A defining feature of human hemoglobin is its oxygen binding affinity, quantified by the partial pressure of oxygen at which hemoglobin is 50% saturated (p50), and the variability of this parameter over a range of physiological and environmental states. Modulation of this property of hemoglobin can directly affect the degree of peripheral oxygen offloading and tissue oxygenation. Areas covered: This review summarizes the role of hemoglobin oxygen affinity in normal and abnormal physiology and discusses the current state of the literature regarding artificial modulation of p50...
April 12, 2017: Expert Review of Hematology
https://www.readbyqxmd.com/read/28401280/-fish-mouth-vertebrae-in-sickle-cell-anemia
#8
REVIEW
Eduardo Kaiser Ururahy Nunes Fonseca, Adham do Amaral E Castro, Durval do Carmo Barros Santos, Laercio Alberto Rosemberg
No abstract text is available yet for this article.
April 11, 2017: Abdominal Radiology
https://www.readbyqxmd.com/read/28399852/association-of-classical-markers-and-establishment-of-the-dyslipidemic-sub-phenotype-of-sickle-cell-anemia
#9
Milena Magalhães Aleluia, Caroline Conceição da Guarda, Rayra Pereira Santiago, Teresa Cristina Cardoso Fonseca, Fábia Idalina Neves, Regiana Quinto de Souza, Larissa Alves Farias, Felipe Araújo Pimenta, Luciana Magalhães Fiuza, Thassila Nogueira Pitanga, Júnia Raquel Dutra Ferreira, Elisângela Vitória Adorno, Bruno Antônio Veloso Cerqueira, Marilda de Souza Gonçalves
BACKGROUND: Sickle cell anemia (SCA) patients exhibit sub-phenotypes associated to hemolysis and vaso-occlusion. The disease has a chronic inflammatory nature that has been also associated to alterations in the lipid profile. This study aims to analyze hematological and biochemical parameters to provide knowledge about the SCA sub-phenotypes previously described and suggest a dyslipidemic sub-phenotype. METHODS: A cross-sectional study was conducted from 2013 to 2014, and 99 SCA patients in steady state were enrolled...
April 11, 2017: Lipids in Health and Disease
https://www.readbyqxmd.com/read/28397407/sanguinate-pegylated-carboxyhemoglobin-bovine-mechanism-of-action-and-clinical-update
#10
Abraham Abuchowski
Historically, blood substitutes were under development that would provide oxygen carrying capacity as well as fluid replacement for both trauma and surgical indications. Their development was halted by the inability of the products to deliver therapeutic amounts of oxygen targeted to hypoxic tissue as well as from the inherent toxicity of the molecules. This led to the concept of an oxygen therapeutic that would be targeted for indications caused by anemia/ischemia/hypoxia but would not exhibit the toxicity that plagued earlier products...
April 2017: Artificial Organs
https://www.readbyqxmd.com/read/28382567/evaluating-risk-factors-for-chronic-kidney-disease-in-pediatric-patients-with-sickle-cell-anemia
#11
Jeffrey D Lebensburger, Gary R Cutter, Thomas H Howard, Paul Muntner, Daniel I Feig
BACKGROUND: Patients with sickle cell anemia (SCA) have an increased prevalence of nephropathy and mortality from chronic kidney disease (CKD). METHODS: We evaluated the association of hyperuricemia and nocturnal hypertension with lower estimated glomerular filtration rate (eGFR) using cystatin-C in patients aged 10-21 years with the HbSS or HbSB0 form of the disease during a non-acute clinic visit. eGFR and uric acid measurements were obtained in 83 and 81 participants, respectively, and 24-h ambulatory blood pressure monitoring (ABPM) was performed in 44 participants...
April 5, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28379995/investigation-of-mutations-in-the-hbb-gene-using-the-1-000-genomes-database
#12
Tânia Carlice-Dos-Reis, Jaime Viana, Fabiano Cordeiro Moreira, Greice de Lemos Cardoso, João Guerreiro, Sidney Santos, Ândrea Ribeiro-Dos-Santos
Mutations in the HBB gene are responsible for several serious hemoglobinopathies, such as sickle cell anemia and β-thalassemia. Sickle cell anemia is one of the most common monogenic diseases worldwide. Due to its prevalence, diverse strategies have been developed for a better understanding of its molecular mechanisms. In silico analysis has been increasingly used to investigate the genotype-phenotype relationship of many diseases, and the sequences of healthy individuals deposited in the 1,000 Genomes database appear to be an excellent tool for such analysis...
2017: PloS One
https://www.readbyqxmd.com/read/28372488/insulin-secretion-and-resistance-in-normoglycemic-patients-with-sickle-cell-disease
#13
Maria P Yavropoulou, Maria Pikilidou, Despoina Pantelidou, Dimitrios G Tsalikakis, Athanasios Mousiolis, Panagiota Chalkia, John G Yovos, Pantelis Zebekakis
Diabetes mellitus has been described in chronic hemolytic anemias, but data are scarce regarding glucose metabolism in normoglycemic patients. To address this issue, we evaluated insulin sensitivity and secretion in patients with sickle cell disease (SCD) and normal oral glucose tolerance test (OGTT). Forty-five adult patients with homozygous sickle cell disease and Hb S/β-thalassemia (β-thal) (mean age 42.5 ± 9.5 years) and 45 healthy individuals matched for age and body mass index (BMI) were included in the study...
April 3, 2017: Hemoglobin
https://www.readbyqxmd.com/read/28362936/high-frequency-of-blackwater-fever-among-children-presenting-to-hospital-with-severe-febrile-illnesses-in-eastern-uganda
#14
Peter Olupot-Olupot, Charles Engoru, Sophie Uyoga, Rita Muhindo, Alex Macharia, Sarah Kiguli, Robert O Opoka, Samuel Akech, Carolyne Ndila, Richard Nyeko, George Mtove, Julius Nteziyaremye, Martin Chebet, Elizabeth C George, Abdel G Babiker, Diana M Gibb, Thomas N Williams, Kathryn Maitland
Background: In the Fluid Expansion as a Supportive Treatment (FEAST) trial, an unexpectedly high proportion of participants from eastern Uganda presented with blackwater fever (BWF). Methods: We describe the prevalence and outcome of BWF among trial participants and compare the prevalence of 3 malaria-protective red blood cell polymorphisms in BWF cases vs both trial (non-BWF) and population controls. Results: Of 3170 trial participants, 394 (12...
April 1, 2017: Clinical Infectious Diseases: An Official Publication of the Infectious Diseases Society of America
https://www.readbyqxmd.com/read/28362411/continuous-manual-exchange-transfusion-for-patients-with-sickle-cell-disease-an-efficient-method-to-avoid-iron-overload
#15
Bérengère Koehl, Florence Missud, Laurent Holvoet, Ghislaine Ithier, Oliver Sakalian-Black, Zinedine Haouari, Emmanuelle Lesprit, André Baruchel, Malika Benkerrou
Children with sickle cell anemia (SCA) may be at risk of cerebral vasculopathy and strokes, which can be prevented by chronic transfusion programs. Repeated transfusions of packed red blood cells (PRBCs) is currently the simplest and most used technique for chronic transfusion programs. However, iron overload is one of the major side effects of this therapy. More developed methods exist, notably the apheresis of RBC (erythrapheresis), which is currently the safest and most efficient method. However, it is costly, complicated, and cannot be implemented everywhere, nor is it suitable for all patients...
March 14, 2017: Journal of Visualized Experiments: JoVE
https://www.readbyqxmd.com/read/28355050/release-of-va-records-relating-to-hiv-final-rule
#16
(no author information available yet)
The Department of Veterans Affairs (VA) is amending its medical regulations governing the release of VA medical records. Specifically, VA is eliminating the restriction on sharing a negative test result for the human immunodeficiency virus (HIV) with veterans' outside providers. HIV testing is a common practice today in healthcare and the stigma of testing that may have been seen in the 1980s when HIV was first discovered is no longer prevalent. Continuing to protect negative HIV tests causes delays and an unnecessary burden on veterans when VA tries to share electronic medical information with the veterans' outside providers through electronic health information exchanges...
March 23, 2017: Federal Register
https://www.readbyqxmd.com/read/28346212/stability-of-extemporaneously-prepared-hydroxycarbamide-oral-suspensions
#17
Djamila Kabiche, Issa-Bella Balde, Elyes Majoul, Sofiane Kabiche, Elodie Bourguignon, Jean-Eudes Fontan, Salvatore Cisternino, Jöel Schlatter
Hydroxycarbamide, available as tablets, is a pharmacological agent for fetal hemoglobin induction such as sickle cell anemia. The need for alternative dosage form options for patients unable to take tablets led hospital pharmacies to prepare solutions and suspensions. The objective of this study was to determine the stability of hydroxycarbamide in Ora-Plus in combination with either Ora-Sweet or Ora-Sweet SF, Ora-Blend, or Ora-Blend SF suspending agents. The studied samples were compounded into 100-mg/mL suspensions and stored in 60-mL amber glass bottles at room (22°C to 25°C) or refrigerated (4°C to 8°C) temperature...
March 2017: International Journal of Pharmaceutical Compounding
https://www.readbyqxmd.com/read/28344126/regulation-of-the-nitric-oxide-oxidase-activity-of-myeloperoxidase-by-pharmacological-agents
#18
Sophie L Maiocchi, Jonathan C Morris, Martin D Rees, Shane R Thomas
The leukocyte-derived heme enzyme myeloperoxidase (MPO) is released extracellularly during inflammation and impairs nitric oxide (NO) bioavailability by directly oxidizing NO or producing NO-consuming substrate radicals. Here, structurally diverse pharmacological agents with activities as MPO substrates/inhibitors or antioxidants were screened for their effects on MPO NO oxidase activity in human plasma and physiological model systems containing endogenous MPO substrates/antioxidants (tyrosine, urate, ascorbate)...
March 23, 2017: Biochemical Pharmacology
https://www.readbyqxmd.com/read/28340843/acquired-acrodermatitis-enteropathica-syndrome-in-a-kidney-transplant-receipt-a-case-report
#19
A Bayraktar, H Bakkaloglu, E Demir, A Turkmen, I F Azamat, Y Caliskan, Ş O Sari, N Buyukbabani, C Baykal, A E Aydin
Acrodermatitis enteropathica syndrome (AE) is a clinical entity that results in severe zinc deficiency. It can be genetic or acquired. Acquired AE has been reported in patients with chronic liver disease, malabsorption syndrome, sickle cell anemia, and chronic renal failure. We present a kidney transplant recipient with skin rash and watery diarrhea. The patient had low serum zinc levels, which quickly resolved after zinc supplementation. Skin biopsy showed cytoplasmic pallor and vacuolization and ballooning degeneration of keratinocytes within the superficial epidermis, which may have led to confluent necrosis of keratinocytes...
April 2017: Transplantation Proceedings
https://www.readbyqxmd.com/read/28338216/the-diversified-function-and-potential-therapy-of-ectopic-olfactory-receptors-in-non-olfactory-tissues
#20
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
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