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"Sickle Cell Anemia"

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https://www.readbyqxmd.com/read/28730452/transcranial-color-doppler-in-stroke-free-adult-patients-with-sickle-cell-disease
#1
G Graziadei, F M Casoni, F Annoni, I Cortinovis, P Ridolfi, I Gandolfi, A Marcon, E Di Pierro, M D Cappellini
The threshold velocity ≥200 cm/s at transcranial Doppler (TCD) evaluation is a useful cut-off for preventing the stroke (STOP trial) in pediatric patients with sickle cell disease (SCD), term including different types of sickle genotypes. Scanty data are available for adult SCD patients. We compared intracranial blood flow velocities between adult SCD patients and controls using transcranial color Doppler (TCCD), measuring the peak of systolic velocity (PSV) with the insonation angle correction and the pulsatility index (PI), an indicator of endothelial elasticity...
July 20, 2017: Annals of Hematology
https://www.readbyqxmd.com/read/28705443/-impact-of-sickle-cell-trait-on-arterial-stiffness-in-african-subjects
#2
V Ouédraogo, R Soleti, I Signolet, M Diaw, M Hallab, A Samb, R Andriantsitohaina, A Ba, G Lefthériotis
Sickle cell trait (SCT) is the benign condition of sickle cell disease. Often asymptomatic, the carriers of the sickle cell trait have hemorheological disturbances with increased oxidative stress compared to healthy subjects. These disturbances can lead to structural and functional changes in large vessels. The aim of the study was to measure arterial stiffness, an independent marker of subclinical atherosclerosis, SCT carriers compared to sickle cell anemia (SCA) subjects. Nine SCT carriers aged 32±9 years (7 men) were compared to 14 SCA subjects aged 29±9 years (2 men) and 22 control subjects aged 34±9 years (11 men) recruited by the National blood transfusion center (CNTS) in Dakar (Senegal)...
February 2017: J Med Vasc
https://www.readbyqxmd.com/read/28700924/mesoscopic-adaptive-resolution-scheme-toward-understanding-of-interactions-between-sickle-cell-fibers
#3
Lu Lu, He Li, Xin Bian, Xuejin Li, George Em Karniadakis
Understanding of intracellular polymerization of sickle hemoglobin (HbS) and subsequent interaction with the membrane of a red blood cell (RBC) is important to predict the altered morphologies and mechanical properties of sickle RBCs in sickle cell anemia. However, modeling the integrated processes of HbS nucleation, polymerization, HbS fiber interaction, and subsequent distortion of RBCs is challenging as they occur at multispatial scales, ranging from nanometers to micrometers. To make progress toward simulating the integrated processes, we propose a hybrid HbS fiber model, which couples fine-grained and coarse-grained HbS fiber models through a mesoscopic adaptive resolution scheme (MARS)...
July 11, 2017: Biophysical Journal
https://www.readbyqxmd.com/read/28699284/a-monocyte-tnf-endothelial-activation-axis-in-sickle-transgenic-mice-therapeutic-benefit-from-tnf-blockade
#4
Anna Solovey, Arif Somani, John D Belcher, Liming Milbauer, Lucile Vincent, Rafal Pawlinski, Karl A Nath, Robert J Kelm, Nigel Mackman, M Gerard O'Sullivan, Kalpna Gupta, Gregory M Vercellotti, Robert P Hebbel
Elaboration of tumor necrosis factor (TNF) is a very early event in development of ischemia/reperfusion injury pathophysiology. Therefore, TNF may be a prominent mediator of endothelial cell and vascular wall dysfunction in sickle cell anemia, a hypothesis we addressed using NY1DD, S+S(Antilles) , and SS-BERK sickle transgenic mice. Transfusion experiments revealed participation of abnormally activated blood monocytes exerting an endothelial activating effect, dependent upon Egr-1 in both vessel wall and blood cells, and upon NFκB(p50) in a blood cell only...
July 11, 2017: American Journal of Hematology
https://www.readbyqxmd.com/read/28687959/stem-cell-therapy-for-fanconi-anemia
#5
Qing-Shuo Zhang
Stem cell therapy is the administration of stem cells to a patient to treat or prevent a disease. Since stem cells possess the long-term self-renewal capacity and provide daughter cells that differentiate into the specialized cells of each tissue, stem cell therapy will theoretically improve the disease condition for the lifetime of the patient. As the most widely used stem cell therapy, bone marrow transplantation is the treatment of choice for many kinds of blood disorders, including anemias, leukemias, lymphomas, and rare immunodeficiency diseases...
July 8, 2017: Advances in Experimental Medicine and Biology
https://www.readbyqxmd.com/read/28685705/chronic-active-parietal-osteomyelitis-due-to-salmonella-typhi-in-a-patient-with-sickle-cell-anemia
#6
Ahmad Antar, George Karam, Maurice Kfoury, Nadim El-Majzoub
No abstract text is available yet for this article.
July 4, 2017: Turkish Journal of Haematology: Official Journal of Turkish Society of Haematology
https://www.readbyqxmd.com/read/28682672/sickle-cell-anemia-and-comorbid-leg-ulcer-treated-with-curative-peripheral-blood-stem-cell-transplantation
#7
Joseph L Connor, Caterina P Minniti, John F Tisdale, Matthew M Hsieh
Allogeneic bone marrow transplantation or peripheral blood stem cell transplantation (PBSCT) are the only curative therapies for patients with sickle cell disease (SCD). Once the patients have successfully undergone transplantation and engrafted, the hallmark of hemolytic anemia resolves, and normal hemoglobin levels are achieved. Some transplant protocols exclude patients with open wounds, including leg ulcers, because of infection risks associated with transplantation and long-term immunosuppression required to prevent graft-versus-host disease...
March 2017: International Journal of Lower Extremity Wounds
https://www.readbyqxmd.com/read/28667380/variants-in-the-non-coding-region-of-the-tlr2-gene-associated-with-infectious-subphenotypes-in-pediatric-sickle-cell-anemia
#8
Susana David, Pedro Aguiar, Liliana Antunes, Alexandra Dias, Anabela Morais, Anavaj Sakuntabhai, João Lavinha
Sickle cell anemia (SCA) is characterized by chronic hemolysis, severe vasoocclusive crises (VOCs), and recurrent often severe infections. A cohort of 95 SCA pediatric patients was the background for genotype-to-phenotype association of the patient's infectious disease phenotype and three non-coding polymorphic regions of the TLR2 gene, the -196 to -174 indel, SNP rs4696480, and a (GT)n short tandem repeat. The infectious subphenotypes included (A) recurrent respiratory infections and (B) severe bacterial infection at least once during the patient's follow-up...
June 30, 2017: Immunogenetics
https://www.readbyqxmd.com/read/28663878/measuring-sickle-cell-morphology-during-blood-flow
#9
Inna Kviatkovsky, Adel Zeidan, Daniella Yeheskely-Hayon, Eveline L Shabad, Eldad J Dann, Dvir Yelin
During a sickle cell crisis in sickle cell anemia patients, deoxygenated red blood cells may change their mechanical properties and block small blood vessels, causing pain, local tissue damage, and possibly organ failure. Measuring the structural and morphological changes in sickle cells is important for understanding the factors contributing to vessel blockage and for developing an effective treatment. In this work, we image blood cells from sickle cell anemia patients using spectrally encoded flow cytometry, and analyze the interference patterns between reflections from the cell membranes...
March 1, 2017: Biomedical Optics Express
https://www.readbyqxmd.com/read/28662541/reproductive-endocrine-issues-in-men-with-sickle-cell-anemia
#10
REVIEW
A W Huang, O Muneyyirci-Delale
In patients with sickle cell anemia, the sickling of red blood cells is known to cause end-organ damage by infarction. In some men who are affected by sickle cell anemia, the obstruction of venous outflow of the penis causes priapism, which could lead to erectile dysfunction. There is also evidence that the disease is linked to other reproductive issues in men-specifically delayed puberty, low testosterone, and sperm abnormalities-although the causes of these problems are less clear. Treatment of sickle cell anemia can have effects on the reproductive system as well...
June 29, 2017: Andrology
https://www.readbyqxmd.com/read/28641096/hematopoietic-stem-cell-transplantation-for-sickle-cell-anemia-the-changing-landscape
#11
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
#12
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
#13
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
#14
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
#15
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
#16
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
#17
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
#18
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
#19
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
#20
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
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