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"Sickle Cell Anemia"

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https://www.readbyqxmd.com/read/28641096/hematopoietic-stem-cell-transplantation-for-sickle-cell-anemia-the-changing-landscape
#1
REVIEW
Adetola A Kassim, Deva Sharma
Allogeneic hematopoietic stem cell transplantation (HSCT) remains the only curative therapy for sickle cell disease (SCD); however, its use is limited by lack of suitable human leukocyte antigen (HLA)-matched donors and decreased application in older patients with significant morbidity. Myeloablative, HLA-identical sibling transplantation in children with SCD offers excellent long-term survival, with overall and event-free survival rates of 95% and 92%, respectively. However, the risk of graft-versus-host-disease, infections, infertility, and other long-term transplant complications, further limits its widespread use...
June 15, 2017: Hematology/oncology and Stem Cell Therapy
https://www.readbyqxmd.com/read/28626540/acute-crises-and-complications-of-sickle-cell-anemia-among-patients-attending-a-pediatric-tertiary-unit-in-kinshasa-democratic-republic-of-congo
#2
Michel Ntetani Aloni, Bertin Tshimanga Kadima, Pépé Mfutu Ekulu, Aléine Nzazi Budiongo, René Makuala Ngiyulu, Jean Lambert Gini-Ehungu
In the Democratic Republic of Congo, the incidence of sickle cell anemia (SCA) is estimated to affect 30,000 to 40,000 neonates per year. However, there is paucity of data on acute clinical manifestations in sickle cell children. In these circumstances, it is difficult to develop a health care policy for an adequate management of sickle cell patients. This was a seven years' retrospective study of children admitted with acute sickle cell crisis in the Department of Pediatrics in University Hospital of Kinshasa, Kinshasa, the Democratic Republic of Congo...
June 1, 2017: Hematology Reports
https://www.readbyqxmd.com/read/28618753/development-of-a-new-real-time-pcr-screening-kit-for-hbs-and-common-beta-thalassemia-mutations-observed-in-turkey
#3
Derya Kan Karaer, Mehmet Ali Ergün, Hatice Ilgın Ruhi, Jale Öztürk, Halil Kara, Deniz Reisoğlu Çakmak, Talihanur Aydoğmuş, Emriye Ferda Perçin
BACKGROUND/AIM: IVSI-110 (G>A), IVSI-6 (T>C), IVSII-1 (G>A), IVSII-745 (C>G), IVSI-1 (G>A), and HbS are mutations covering 76% of all the β-globin mutations in the Turkish population. In this study, our aim is to develop a reliable, fast, real-time kit for these mutations using the TaqMan probe method. MATERIALS AND METHODS: This study included 100 individuals with beta-thalassemia or sickle cell anemia who had unknown mutations, and 21 controls with known mutations...
June 12, 2017: Turkish Journal of Medical Sciences
https://www.readbyqxmd.com/read/28615064/anti-toxoplasma-gondii-antibodies-in-patients-with-beta-hemoglobinopathies-the-first-report-in-the-americas
#4
Marina Neves Ferreira, Claudia Regina Bonini-Domingos, Isabeth Fonseca Estevão, Clarice Lopes de Castro Lobo, Gisele Cristina Souza Carrocini, Aparecida Perpétuo Silveira-Carvalho, Octávio Ricci, Luiz Carlos de Mattos, Cinara Cássia Brandão de Mattos
BACKGROUND: In Brazil, there have been no previous studies of Toxoplasma gondii infection in sickle cell anemia patients and carriers of severe forms of beta-thalassemia. This study evaluated T. gondii infection in patients with beta-hemoglobinopathies. METHODS: A total of 158 samples, 77 (48.7%) men and 81 (51.3%) women, were evaluated. Three groups were formed: G1 (85 patients with sickle cell disease); G2 (11 patients with homozygous beta-thalassemia; G3 (62 patients with heterozygous beta-thalassemia)...
June 14, 2017: BMC Research Notes
https://www.readbyqxmd.com/read/28609750/association-of-plasma-cd40l-with-acute-chest-syndrome-in-sickle-cell-anemia
#5
Vanessa Tonin Garrido, Laura Sonzogni, Siana Nkya Mtatiro, Fernando F Costa, Nicola Conran, Swee Lay Thein
Platelet activation and platelet-derived cytokines contribute to the vascular inflammation and increased thrombotic activity known to occur in patients with sickle cell anemia (SCA). CD40 ligand (CD40L), a platelet-associated pro-inflammatory molecule that promotes endothelial cell activation, is elevated in the circulation of SCA patients. We sought to evaluate the association of CD40L and inflammation with sickle-related clinical complications and laboratory variables in SCA patients. Soluble CD40L, thrombospondin (TSP)-1 and tumor necrosis factor (TNF)-α were determined in the platelet-poor plasma of healthy individuals and steady-state SCA patients by ELISA...
June 10, 2017: Cytokine
https://www.readbyqxmd.com/read/28598539/feasibility-and-safety-of-home-exercise-training-in-children-with-sickle-cell-anemia
#6
Robert I Liem, Moriyike Akinosun, Devin S Muntz, Alexis A Thompson
Exercise guidelines do not exist for individuals with sickle cell anemia (SCA) despite the impact of disease-related complications on physical functioning. Thirteen subjects (mean 15.1 ± 2.8 years old) with SCA were prescribed three exercise sessions/week for 12 weeks on a stationary bicycle placed at home. In total, 77% of subjects completed 89% of prescribed sessions without exercise-related adverse events, thus meeting feasibility and safety criteria. Adherence to prescribed duration and target heart rate during training decreased during the second half of the study...
June 9, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28589652/losartan-for-the-nephropathy-of-sickle-cell-anemia-a-phase-2-multi-center-trial
#7
Charles T Quinn, Santosh L Saraf, Victor R Gordeuk, Courtney D Fitzhugh, Susan E Creary, Prasad Bodas, Alex George, Ashok B Raj, Alecia C Nero, Catherine E Terrell, Lisa McCord, Adam Lane, Hans C Ackerman, Yu Yang, Omar Niss, Michael D Taylor, Prasad Devarajan, Punam Malik
Nephropathy is a common and progressive complication of sickle cell anemia (SCA). In SCA mice, we found that hyperangiotensinemia in the absence of hypertension underlies nephropathy, and its downregulation by losartan, an angiotensin-II-receptor-1 blocker, reduced albuminuria and progression of nephropathy. Therefore, we performed a phase-2 trial of oral losartan, given for 6 months, to explore whether it reduced albuminuria in children and adults with SCA. Participants were allocated to groups defined by class of baseline urinary albumin-to-creatinine ratio (UACR): no albuminuria (NoA), microalbuminuria (MicroA), and macroalbuminuria (MacroA)...
June 7, 2017: American Journal of Hematology
https://www.readbyqxmd.com/read/28584506/prognostic-factors-and-outcome-of-management-of-ischemic-priapism-in-zaria-nigeria
#8
Muhammed Ahmed, Benjamin Augustine, Messi Matthew, Mudi Awaisu, Abdullahi Sudi, Kolapo B Hamza, Ahmad Bello, Hussaini Yusuf Maitama
OBJECTIVES: The objective of this study was to determine the nuances of management, prognostic factors, and outcome of ischemic priapism in patients seen at Ahmadu Bello University Teaching Hospital, Zaria, Nigeria. PATIENTS AND METHODS: We retrospectively studied the case notes of all patients managed for ischemic priapism in the Ahmadu Bello University Teaching Hospital, Zaria, over a period of 10 years (2006-2015). The data extracted included patients' age, occupation, duration of painful penile erection, and previous episodes...
January 2017: Nigerian Journal of Surgery: Official Publication of the Nigerian Surgical Research Society
https://www.readbyqxmd.com/read/28584135/genome-wide-association-study-to-identify-variants-associated-with-vaso-occlusive-pain-in-sickle-cell-anemia
#9
Shruti Chaturvedi, Pallav Bhatnagar, Christopher J Bean, Martin H Steinberg, Jacqueline N Milton, James F Casella, Emily Barron-Casella, Dan E Arking, Michael R DeBaun
No abstract text is available yet for this article.
June 5, 2017: Blood
https://www.readbyqxmd.com/read/28579850/coexisting-sickle-cell-anemia-and-sarcoidosis-a-management-conundrum
#10
Fnu Nutan, Nagesh S Gollahalli
Sickle cell disease and Sarcoidosis are conditions that are more common in the African American population. In this report we share an unfortunate patient who had hepatic sarcoidosis but could not receive steroids since that precipitated acute liver failure. We have discussed potential therapy options but we need more options that improve mortality.
2017: Clinical Medicine Insights. Blood Disorders
https://www.readbyqxmd.com/read/28579187/functional-mri-bold-response-in-sickle-mice-with-hyperalgesia
#11
Ying Wang, Xiao Wang, Wei Chen, Kalpna Gupta, Xiao-Hong Zhu
Patients with sickle cell anemia (SCA) have abnormal hemoglobin (sickle hemoglobin S) leading to the crystallization of hemoglobin chains in red blood cells (RBCs), which assume sickle shape and display reduced flexibility. Sickle RBCs (sRBCs) adhere to vessel walls and block blood flow, thus preventing oxygen delivery to the tissues leading to vaso-occlusive crises (VOC), acute pain and organ damage. SCA patients often have chronic pain that can be attributed to inflammation, vasculopathy, neuropathy, ischemia-reperfusion injury and organ damage...
June 2017: Blood Cells, Molecules & Diseases
https://www.readbyqxmd.com/read/28578536/population-pharmacokinetic-and-exposure-response-analyses-of-prasugrel-in-pediatric-patients-with-sickle-cell-anemia
#12
Brian A Moser, Elizabeth S LaBell, Emmanuel Chigutsa, Joseph A Jakubowski, David S Small
BACKGROUND AND OBJECTIVE: Prasugrel, a P2Y12 adenosine diphosphate (ADP) receptor antagonist, inhibits ADP-mediated platelet activation and aggregation in patients with sickle cell anemia (SCA). We developed a population pharmacokinetic (popPK) model in pediatric patients from 2 to <18 years of age with SCA, and performed exposure-response evaluations to characterize the effects of prasugrel in a subset of these patients who weighed 19 kg or more and experienced at least two episodes of vaso-occlusive crises (VOC) in the past year...
June 3, 2017: Clinical Pharmacokinetics
https://www.readbyqxmd.com/read/28577650/physical-activity-level-and-performance-in-the-six-minute-walk-test-of-children-and-adolescents-with-sickle-cell-anemia
#13
Hugo Nivaldo Melo, Simone Joanna-Maria Stoots, Marijn Aimee Pool, Vitor Oliveira Carvalho, Lucas Oliveira Carvalho Almeida, Max Luan De Carvalho Aragão, Charles Agyemang, Rosana Cipolotti
BACKGROUND: To establish determinants of maximum walking distance in the 6-minute walk test of children and adolescents with sickle cell anemia, and to compare the performance in this test with physical activity level between patients and healthy controls. METHODS: A cross-sectional study was performed in which the participants answered the Physical Activity Questionnaire for Older Children and Adolescents, and completed the 6-minute walk test. MAIN RESULTS: Fifty-seven patients and 58 controls were studied...
April 2017: Revista Brasileira de Hematologia e Hemoterapia
https://www.readbyqxmd.com/read/28577646/interleukin-10-haplotypes-are-not-associated-with-acute-cerebral-ischemia-or-high-risk-transcranial-doppler-in-a-newborn-cohort-of-395-children-with-sickle-cell-anemia
#14
André Rolim Belisário, Rahyssa Rodrigues Sales, Nayara Evelin Toledo, Cibele Velloso-Rodrigues, Célia Maria Silva, Marcos Borato Viana
BACKGROUND: The etiology of stroke, a severe complication of sickle cell anemia, involves inflammatory processes. However, the pathogenetic mechanisms are unknown. The aim of this study was to evaluate the influence of interleukin-10 polymorphisms and haplotypes on the risk of acute cerebral ischemia and high-risk transcranial Doppler in 395 children with sickle cell anemia from the state of Minas Gerais, Brazil. METHODS: Interleukin-10 haplotypes were determined by polymerase chain reaction-restriction fragment length polymorphism and sequencing...
April 2017: Revista Brasileira de Hematologia e Hemoterapia
https://www.readbyqxmd.com/read/28576754/stroke-avoidance-for-children-in-rep%C3%A3%C2%BAblica-dominicana-sacred-protocol-for-a-prospective-study-of-stroke-risk-and-hydroxyurea-treatment-in-sickle-cell-anemia
#15
Neelum D Jeste, Luisanna M Sánchez, Gabriela S Urcuyo, Melissa E Bergés, Judy P Luden, Susan E Stuber, Teresa S Latham, Rafael Mena, Rosa M Nieves, Russell E Ware
BACKGROUND: In the Dominican Republic, where the burden of sickle cell anemia (SCA) is high, many children lack access to routine screening and preventative care. Children with SCA are at risk for stroke, an event that leads to significant morbidity and mortality. In the United States, screening via transcranial Doppler (TCD) identifies children with SCA at highest stroke risk, allowing early intervention with blood transfusions. The need for indefinite transfusions for primary stroke prevention limits their practicality in limited-resource countries...
June 2, 2017: JMIR Research Protocols
https://www.readbyqxmd.com/read/28569575/airway-hyperresponsiveness-does-not-predict-morbidity-in-children-with-sickle-cell-anemia
#16
Shaina M Willen, Mark Rodeghier, Robert C Strunk, Carol L Rosen, Fenella J Kirkham, Joshua J Field, Michael R DeBaun, Robyn T Cohen
No abstract text is available yet for this article.
June 1, 2017: American Journal of Respiratory and Critical Care Medicine
https://www.readbyqxmd.com/read/28567595/do-alpha-thalassemia-fetal-hemoglobin-and-the-ugt1a1-polymorphism-have-an-influence-on-serum-bilirubin-levels-and-cholelithiasis-in-patients-with-sickle-cell-disease
#17
Laura Alencastro de Azevedo, Joyce Bonazzoni, Sandrine Comparsi Wagner, Mariela Granero Farias, Christina M Bittar, Liane Daudt, Simone Martins de Castro
BACKGROUND: Increased destruction of erythrocytes in patients with sickle cell disease results in chronic hyperbilirubinemia and leads to the formation of gallstones. OBJECTIVES: The objective of this study was to determine the combined influence of alpha thalassemia, fetal hemoglobin, and the UGT1A1 polymorphism on serum bilirubin levels and cholelithiasis in patients with sickle cell disease. METHODS: We analyzed 72 patients treated in the outpatient hematology unit of the Clinical Hospital of Porto Alegre...
May 31, 2017: Molecular Diagnosis & Therapy
https://www.readbyqxmd.com/read/28566841/new-hope-in-fight-against-sickle-cell-anemia
#18
EDITORIAL
S G Damle
No abstract text is available yet for this article.
January 2017: Contemporary Clinical Dentistry
https://www.readbyqxmd.com/read/28552477/genome-wide-association-study-of-erythrocyte-density-in-sickle-cell-disease-patients
#19
Yann Ilboudo, Pablo Bartolucci, Alicia Rivera, Josepha-Clara Sedzro, Mélissa Beaudoin, Marie Trudel, Seth L Alper, Carlo Brugnara, Frédéric Galactéros, Guillaume Lettre
Deoxy-hemoglobin S polymerization into rigid fibers is the direct cause of the clinical sequelae observed in sickle cell disease (SCD). The rate of polymerization of sickle hemoglobin is determined primarily by intracellular hemoglobin concentration, itself dependent on the amount of sickle hemoglobin and on red blood cell (RBC) volume. Dense, dehydrated RBC (DRBC) are observed in SCD patients, and their number correlates with hemolytic parameters and complications such as renal dysfunction, leg ulcers and priapism...
June 2017: Blood Cells, Molecules & Diseases
https://www.readbyqxmd.com/read/28542880/suicidal-death-of-erythrocytes-in-cancer-and-its-chemotherapy-a-potential-target-in-the-treatment-of-tumor-associated-anemia
#20
REVIEW
Elisabeth Lang, Rosi Bissinger, Syed M Qadri, Florian Lang
In analogy to apoptosis of nucleated cells, erythrocytes may enter eryptosis characterized by cell shrinkage and cell membrane scrambling. Eryptotic erythrocytes are rapidly cleared from circulating blood and may adhere to the vascular wall. Stimulation of eryptosis thus impairs microcirculation and leads to anemia as soon as the loss of erythrocytes cannot be fully compensated by enhanced erythropoiesis. Signaling stimulating eryptosis includes increase of cytosolic Ca(2+) -activity, ceramide, caspases, calpain, p38-kinase, protein-kinase C, Janus-activated kinase 3, casein-kinase 1α, and cyclin-dependent kinase 4...
May 23, 2017: International Journal of Cancer. Journal International du Cancer
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