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Sickle cell anemia

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https://www.readbyqxmd.com/read/28338216/the-diversified-function-and-potential-therapy-of-ectopic-olfactory-receptors-in-non-olfactory-tissues
#1
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
https://www.readbyqxmd.com/read/28332727/protective-bcl11a-and-hbs1l-myb-polymorphisms-in-a-cohort-of-102-congolese-patients-suffering-from-sickle-cell-anemia
#2
Tite Minga Mikobi, Prosper Tshilobo Lukusa, Michel Ntetani Aloni, Aimé Zola Lumaka, Didine Kinkodi Kaba, Koenraad Devriendt, Gert Matthijs, Jean Marie Mbuyi Muamba, Valérie Race
BACKGROUND: We aimed to investigate the distribution of selected BCL11A and HMIP polymorphisms (SNP's), and to assess the correlation with HPFH in a cohort of sickle cell patients. METHODS: A preliminary cross-sectional study was conducted in 102 patients. Group 1 was composed of patients with HPFH and Group 2 consisted of patients without HbF. We assessed 8 SNPs previously associated with HPFH in cohorts genetically close to the Congolese population. Observed frequencies were compared to expected frequencies...
March 23, 2017: Journal of Clinical Laboratory Analysis
https://www.readbyqxmd.com/read/28331646/conversion-of-low-flow-priapism-to-high-flow-state-using-t-shunt-with-tunneling
#3
Neil A Mistry, Nicholas N Tadros, Jason C Hedges
Introduction. The three types of priapism are stuttering, arterial (high-flow, nonischemic), and venoocclusive (low-flow, ischemic). These are usually distinct entities and rarely occur in the same patient. T-shunts and other distal shunts are frequently combined with tunneling, but a seldom recognized potential complication is conversion to a high-flow state. Case Presentation. We describe 2 cases of men who presented with low-flow priapism episodes that were treated using T-shunts with tunneling that resulted with both men having recurrent erections shortly after surgery that were found to be consistent with high-flow states...
2017: Case Reports in Urology
https://www.readbyqxmd.com/read/28331623/gum-arabic-as-novel-anti-oxidant-agent-in-sickle-cell-anemia-phase-ii-trial
#4
Lamis Kaddam, Imad Fadl-Elmula, Omer Ali Eisawi, Haydar Awad Abdelrazig, Mohammed Abdelraman Salih, Florian Lang, Amal M Saeed
BACKGROUND: Sickle cell anemia patients suffer from oxidative stress due to chronic inflammation and self-oxidation of sickle hemoglobin (Hb S). Chronic oxidative stress contributes to endothelial dysfunction, inflammation and multiple organ damage in sickle cell disease (SCD). Thus, antioxidant medication may favorably influence the disease. Gum Arabic (GA), edible, dried, gummy exudates from Acacia Senegal tree, has been claimed to act as an anti-oxidant and cytoprotective agent, protecting against experimental hepatic, renal and cardiac toxicities in rats...
2017: BMC Hematology
https://www.readbyqxmd.com/read/28323673/clinical-pearls-of-maternal-critical-care-part-2-sickle-cell-disease-in-pregnancy
#5
Vinod Patil, Gamunu Ratnayake, Galina Fastovets
PURPOSE OF REVIEW: The current review outlines the challenges in managing pregnant women with sickle-cell anemia, who are at risk of becoming critically ill during pregnancy. RECENT FINDINGS: Sickle obstetric patients pose unique challenges to the anesthetist and intensivist. We discuss the role of prophylactic transfusions for specific indications like acute anemia and twin pregnancies. The management and prevention of vaso-occlusive crises and chest crisis are also outlined...
March 18, 2017: Current Opinion in Anaesthesiology
https://www.readbyqxmd.com/read/28298423/rounding-up-sickle-cells-with-gene-therapy
#6
Leah Byrne
A report of a patient treated with ex vivo lentiviral gene transfer to hematopoietic stem cells shows the promise of gene therapy for sickle cell anemia.
March 15, 2017: Science Translational Medicine
https://www.readbyqxmd.com/read/28293403/hydroxycarbamine-from-an-old-drug-used-in-malignant-hemopathies-to-a-current-standard-in-sickle-cell-disease
#7
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/28286630/a-fatal-case-of-immune-hyperhemolysis-with-bone-marrow-necrosis-in-a-patient-with-sickle-cell-disease
#8
Matthew S Karafin, Arun Singavi, Susan T Johnson, Joshua J Field
In patients with sickle cell disease, hyperhemolysis is a rare but life-threatening complication of transfusion. In this case report, we describe a 61 year-old woman with hemoglobin sickle cell (SC) disease and history of alloimmunization who developed hyperhemolysis associated with a transfusion. She was found to have a warm and a clinically-significant cold autoantibody. Severe anemia (Hb 2.7 g/dL) with reticulocytopenia and thrombocytopenia prompted a bone marrow biopsy, which demonstrated extensive bone marrow necrosis...
February 23, 2017: Hematology Reports
https://www.readbyqxmd.com/read/28281830/imaging-of-musculoskeletal-manifestations-in-sickle-cell-disease-patients
#9
Vijaya Kosaraju, Alok Harwani, Sasan Partovi, Nicholas Bhojwani, Vasant Garg, Sabarish Ayyappan, Christos Kosmas, Mark Robbin
Sickle cell disease (SCD) is a hereditary red cell disorder with clinical manifestations secondary to sickling or crescent shaped distortion of the red blood cells. Major clinical manifestations of sickle cell disease include hemolytic anemia and vaso-occlusive phenomena resulting in ischemic tissue injury and organ damage. Chronic sequelae of the anemia and vaso-occlusive processes involving the musculoskeletal system include complications related to extramedullary hematopoiesis, osteonecrosis, myonecrosis, and osteomyelitis...
March 10, 2017: British Journal of Radiology
https://www.readbyqxmd.com/read/28276593/association-between-sickle-cell-anemia-and-alpha-thalassemia-reveals-a-high-prevalence-of-the-%C3%AE-3-7-triplication-in-congolese-patients-than-in-worldwide-series
#10
Tite Minga Mikobi, Prosper Tshilobo Lukusa, Michel Ntetani Aloni, Aimé Lumaka, Pierre Zalagile Akilimali, Koenraad Devriendt, Gert Matthijs, Jean-Marie Mbuyi Muamba, Valerie Race
BACKGROUND: Information about the association with alpha thalassemia in sickle cell patients is unknown in the Democratic Republic of Congo. There is very little data on the alpha thalassemia in patients suffering from sickle cell anemia in Central Africa, and their consequences on the clinical expression of the disease. METHODS: A cross-sectional study was conducted in 106 sickle cell patients living in the country's capital Kinshasa. The diagnosis of sickle cell anemia was confirmed with a molecular test using PCR-RFLP (restriction fragment length polymorphism) technique...
March 9, 2017: Journal of Clinical Laboratory Analysis
https://www.readbyqxmd.com/read/28270347/mortality-by-sickle-cell-disease-in-brazil
#11
Giovanna Abadia Oliveira Arduini, Letícia Pinto Rodrigues, Alessandra Bernadete Trovó de Marqui
This work aimed to characterize mortality by sickle cell disease in Brazil. The MEDLINE electronic database was searched using the terms 'mortality' and 'sickle cell disease' and 'Brazil' for articles published in the last five years aiming to provide a current analysis of the subject in question. Eight studies on mortality by sickle cell disease were carried out in the Brazilian states of Maranhão, Bahia, Minas Gerais, Rio de Janeiro and Mato Grosso do Sul. The majority of the deaths occurred in patients with sickle cell anemia, which is the most common genotype and causes the most severe clinical manifestation of the disease...
January 2017: Revista Brasileira de Hematologia e Hemoterapia
https://www.readbyqxmd.com/read/28270345/clinical-and-laboratory-profile-of-patients-with-sickle-cell-anemia
#12
Phelipe Gabriel Dos Santos Sant'Ana, Ariane Moreira Araujo, Cynthia Teixeira Pimenta, Mário Lúcio Pacheco Ker Bezerra, Sílvio Pereira Borges Junior, Viviana Martins Neto, Janaina Sousa Dias, Aline de Freitas Lopes, Danyelle Romana Alves Rios, Melina de Barros Pinheiro
OBJECTIVE: This study aimed to describe and analyze clinical and laboratory characteristics of patients with sickle cell anemia treated at the Hemominas Foundation, in Divinópolis, Brazil. Furthermore, this study aimed to compare the clinical and laboratory outcomes of the group of patients treated with hydroxyurea with those patients that were not treated with hydroxyurea. METHODS: Clinical and laboratorial data were obtained by analyzing medical records of patients with sickle cell anemia...
January 2017: Revista Brasileira de Hematologia e Hemoterapia
https://www.readbyqxmd.com/read/28270343/comprehensive-neuropsychological-evaluation-of-children-and-adolescents-with-sickle-cell-anemia-a-hospital-based-sample
#13
Samantha Nunes, Nayara Argollo, Marivania Mota, Camilo Vieira, Eduardo Pondé de Sena
BACKGROUND: Individuals with sickle cell anemia may suffer symptomatic or silent cerebral infarcts leading to neurocognitive complications. This study investigated the cognitive and intellectual performance of children and adolescents with sickle cell anemia. METHODS: The socioeconomic status, clinical aspects and behavioral profile of 15 young individuals with sickle cell anemia were evaluated. The Wechsler Intelligence Scale for Children, the Developmental Neuropsychological Assessment Test, and the Child Behavior Checklist were applied...
January 2017: Revista Brasileira de Hematologia e Hemoterapia
https://www.readbyqxmd.com/read/28270342/cholelithiasis-and-its-complications-in-sickle-cell-disease-in-a-university-hospital
#14
Raquel Alves Martins, Renato Santos Soares, Fernanda Bernadelli De Vito, Valdirene de Fátima Barbosa, Sheila Soares Silva, Helio Moraes-Souza, Paulo Roberto Juliano Martins
INTRODUCTION: The clinical manifestations of sickle cell disease are related to the polymerization of hemoglobin S. The chronic hemolysis caused by this condition often causes the formation of gallstones that can migrate and block the common bile duct leading to acute abdomen. OBJECTIVE: This study aimed to evaluate the profile of patients with sickle cell disease and cholelithiasis. METHODS: Patients with sickle cell disease were separated into groups according to the presence or absence of cholelithiasis...
January 2017: Revista Brasileira de Hematologia e Hemoterapia
https://www.readbyqxmd.com/read/28270341/a-phase-ib-open-label-randomized-safety-study-of-sanguinate%C3%A2-in-patients-with-sickle-cell-anemia
#15
Hemant Misra, James Bainbridge, John Berryman, Abraham Abuchowski, Kenneth Mauricio Galvez, Luis Fernando Uribe, Angel Luis Hernandez, Nestor Rodolfo Sosa
BACKGROUND: Treatment of sickle cell anemia is a challenging task and despite the well understood genetic and biochemical pathway of sickle hemoglobin, current therapy continues to be limited to the symptomatic treatment of pain, supplemental oxygen, antibiotics, red blood cell transfusions and hydroxyurea. SANGUINATE is a carbon monoxide releasing molecule and oxygen transfer agent under clinical development for the treatment of sickle cell anemia and comorbidities. METHODS: An open-label randomized Phase Ib study was performed in adult sickle cell anemia patients...
January 2017: Revista Brasileira de Hematologia e Hemoterapia
https://www.readbyqxmd.com/read/28257476/cardiac-iron-overload-in-chronically-transfused-patients-with-thalassemia-sickle-cell-anemia-or-myelodysplastic-syndrome
#16
Mariane de Montalembert, Jean-Antoine Ribeil, Valentine Brousse, Agnes Guerci-Bresler, Aspasia Stamatoullas, Jean-Pierre Vannier, Cécile Dumesnil, Agnès Lahary, Mohamed Touati, Krimo Bouabdallah, Marina Cavazzana, Emmanuelle Chauzit, Amandine Baptiste, Thibaud Lefebvre, Hervé Puy, Caroline Elie, Zoubida Karim, Olivier Ernst, Christian Rose
The risk and clinical significance of cardiac iron overload due to chronic transfusion varies with the underlying disease. Cardiac iron overload shortens the life expectancy of patients with thalassemia, whereas its effect is unclear in those with myelodysplastic syndromes (MDS). In patients with sickle cell anemia (SCA), iron does not seem to deposit quickly in the heart. Our primary objective was to assess through a multicentric study the prevalence of cardiac iron overload, defined as a cardiovascular magnetic resonance T2*<20 ms, in patients with thalassemia, SCA, or MDS...
2017: PloS One
https://www.readbyqxmd.com/read/28256747/elbasvir-grazoprevir-for-patients-with-hepatitis-c-virus-infection-and-inherited-blood-disorders-a-phase-iii-study
#17
Christophe Hézode, Massimo Colombo, Marc Bourlière, Ulrich Spengler, Ziv Ben-Ari, Simone I Strasser, William M Lee, Leslie Morgan, Jingjun Qiu, Peggy Hwang, Michael Robertson, Bach-Yen Nguyen, Eliav Barr, Janice Wahl, Barbara Haber, Robert Chase, Rohit Talwani, Vito Di Marco
Direct-acting antiviral agents have not been studied exclusively in patients with inherited blood disorders and hepatitis C virus (HCV) infection. The objective of the randomized, placebo-controlled, phase III C-EDGE IBLD study was to assess the safety and efficacy of elbasvir/grazoprevir (EBR/GZR) in patients with inherited bleeding disorders and HCV infection. One hundred fifty-nine adults with HCV infection and sickle cell anemia, thalassemia, or hemophilia A/B or von Willebrand disease were enrolled at 31 study sites in the United States, Europe, Australia, Canada, Israel, and Thailand...
March 3, 2017: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://www.readbyqxmd.com/read/28250985/factors-associated-with-growth-retardation-in-children-suffering-from-sickle-cell-anemia-first-report-from-central-africa
#18
Aimé Lukusa Kazadi, René Makuala Ngiyulu, Jean Lambert Gini-Ehungu, Jean Marie Mbuyi-Muamba, Michel Ntetani Aloni
Background. The aim of this study was to investigate and determine the risk factors associated with poor growth among SCA children. Methods. A cross-sectional study was conducted in Kinshasa, the capital's country. The nutritional status was assessed using the Z scores of the anthropometric indices. Results. We gathered data on the 256 patients, 138 females (53.9%), who entered the study. The mean age at presentation was 8.4 ± 4.9 years of age. Underweight, stunting, and wasting were found, respectively, in 47...
2017: Anemia
https://www.readbyqxmd.com/read/28248201/intravascular-hemolysis-and-the-pathophysiology-of-sickle-cell-disease
#19
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/28248149/effect-of-hydroxyurea-therapy-on-pulmonary-function-in-children-with-sickle-cell-anemia
#20
Anya McLaren, Michelle Klingel, Suman Behera, Isaac Odame, Melanie Kirby-Allen, Hartmut Grasemann
No abstract text is available yet for this article.
March 1, 2017: American Journal of Respiratory and Critical Care Medicine
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