Gotowe bibliografie tematyczne / Sickle cells diseases

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Gotowa bibliografia na temat „Sickle cells diseases”

Autor: Grafiati
Data publikacji: 12 kwietnia 2025

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Artykuły w czasopismach na temat "Sickle cells diseases"

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Ashwi, Shaima, Ahmad Alobaisy, Nawal Herzallah, Fatema Alwaheed, Ibtihal Hadi, Duaa Alabbas, Faisal Al-Rasheed, Nawaf Alshuraym i Esra Alzein. "Atopic dermatitis in sickle cell children". International Journal Of Community Medicine And Public Health 5, nr 3 (24.02.2018): 842. http://dx.doi.org/10.18203/2394-6040.ijcmph20180420.

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Sickle cell disease is an autosomal recessive disease characterized by recurrent vaso-occlusive events. Despite the genetic basis of its pathophysiology, recent researchers stated that it is an inflammatory immune-mediated disease where inflammation plays a crucial role in the initiation of adherence between sickle cells and vascular endothelial cells. Allergic, as well as infectious, inflammation is proposed to contribute to the initiation of vaso-occlusive events. Although several researchers reported an association between sickle cell disease and atopic conditions such as bronchial asthma and allergic rhino-conjunctivitis, few cases have reported an association between sickle cell disease and atopic dermatitis. Atopy was reported to be considerably linked to sickle cell disease for several reasons. Firstly, patients with sickle cell disease have higher IgE levels than the general population. Secondly, the mechanism of activation of molecular adhesion between endothelial and blood cells are similar between both sickle cell disease and atopic disease. Thirdly, the cytokines produced from platelet activation are the same cytokines that stimulate allergic inflammation in atopic diseases and promote adherence of sickle cells and endothelium in sickle cell disease. Lastly, sickle cell disease was reported to be associated with other atopic diseases.
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Akhter, Mohammad S., Hassan A. Hamali, Hina Rashid, Gasim Dobie, Aymen M. Madkhali, Abdullah A. Mobarki, Johannes Oldenburg i Arijit Biswas. "Mitochondria: Emerging Consequential in Sickle Cell Disease". Journal of Clinical Medicine 12, nr 3 (18.01.2023): 765. http://dx.doi.org/10.3390/jcm12030765.

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Advanced mitochondrial multi-omics indicate a multi-facet involvement of mitochondria in the physiology of the cell, changing the perception of mitochondria from being just the energy-generating organelles to organelles that highly influence cell structure, function, signaling, and cell fate. This sets mitochondrial dysfunction in the centerstage of numerous acquired and genetic diseases. Sickle cell disease is also being increasingly associated with mitochondrial anomalies and the pathophysiology of sickle cell disease finds mitochondria at crucial intersections in the pathological cascade. Altered mitophagy, increased ROS, and mitochondrial DNA all contribute to the condition and its severity. Such mitochondrial aberrations lead to consequent mitochondrial retention in red blood cells in sickle cell diseases, increased oxidation in the cellular environment, inflammation, worsened vaso-occlusive crisis, etc. There are increasing studies indicating mitochondrial significance in sickle cell disease, consequently providing an opportunity to target it for improving the outcomes of treatment. Identification of the impaired mitochondrial attributes in sickle cell disease and their modulation by therapeutic interventions can impart a better management of the disease. This review aims to describe the mitochondria in the perspective of sicke cell disease so as to provide the reader an overview of the emerging mitochondrial stance in sickle cell disease.
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Amorim, Maria do Socorro do N., Jerias A. Batista, Francisco Maia Junior, Adriana Fontes, Ralph Santos-Oliveira i Luciana M. Rebelo Alencar. "New Insights into Hemolytic Anemias: Ultrastructural and Nanomechanical Investigation of Red Blood Cells Showed Early Morphological Changes". Journal of Biomedical Nanotechnology 18, nr 2 (1.02.2022): 405–21. http://dx.doi.org/10.1166/jbn.2022.3267.

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Several diseases are characterized by changes in the mechanical properties of erythrocytes. Hemolytic anemias are an example of these diseases. Among the hemolytic anemias, Sickle Cell Disease and Thalassemia are the most common, characterized by alterations in the structure of their hemoglobin. Sickle cell disease has a pathological origin in synthesizing abnormal hemoglobin, HbS. In contrast, thalassemia results in extinction or decreased synthesis of α and β hemoglobin chains. This work presents a detailed study of biophysical and ultrastructural early erythrocytes membrane alterations at the nanoscale using Atomic Force Microscopy (AFM). Cells from individuals with sickle cell anemia and thalassemia mutations were studied. The analysis methodology in the AFM was given by blood smear and exposure of the inner membrane for ghost analysis. A robust statistic was used with 65,536 force curves for each map, ten cells of each type, with three individuals for each sample group. The results showed significant differences in cell rigidity, adhesion, volume, and roughness at early morphological alterations, bringing new perspectives for understanding pathogenesis. The sickle cell trait (HbAS) results stand out. Significant alterations were observed in the membrane properties, bringing new perspectives for the knowledge of this mutation. This work presents ultrastructural and biomechanical signatures of sickle cell anemia and thalassemia genotypes, which may help determine a more accurate biophysical description and clinical prognosis for these diseases.
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Lim, Teck Chwee. "Single Cell Mechanics and its connections to Human Diseases". Asia-Pacific Biotech News 09, nr 14 (30.07.2005): 674–75. http://dx.doi.org/10.1142/s0219030305001916.

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The article is about single cell mechanics and its connection to human diseases. It touches on the biomechanics used to perform quantitative study in the physical properties of cells with the progression of certain diseases such as malaria, sickle cell anemia and cancer.
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Parrish, James M., Paul A. Page, David Cohen, Mark R. Nyreen, Charles P. Kingsley, Tara Chronister, Berry F. Shesol i Richard Drew. "Prebypass Pheresis and Red Blood Cell Exchange in a Patient with Homozygous SS Sickle Cell Disease Undergoing Cardiopulmonary Bypass: A Case Report". Journal of ExtraCorporeal Technology 26, nr 3 (wrzesień 1994): 143–51. http://dx.doi.org/10.1051/ject/1994263143.

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Sickle cell disease was first described by Herrick in 1910. This disease involves an abnormality of the hemoglobin molecule which, under certain conditions, causes the red cell to take on a sickle shape. This abnormal shape and hemoglobin prevent the red cell from performing the normal respiratory functions and interfere with the normal flow through the circulatory system. Individuals demonstrate either a homozygous (dominant) SS or a heterozygous Ss state. Clinical symptoms for patients with SS disease are most often characterized by recurrent painful crises, sequestration of sickled cells, chronic hemolytic anemia, systemic diseases, vasoocclusive crises, permanent organ damage and recurrent infection. These patients usually require exchange transfusions due to the concern that the stress of surgery will precipitate a sickling crisis. Exchange transfusions require large amounts of homologous red blood cells (RBCs) to lower the percentage of the homozygous SS hemoglobin. The use of autologous pheresis in the peri operative period reduces the amount of homologous RBCs required and lowers the patient's exposure to homologous blood and its associated risks. This paper discusses the disease, the planning and the techniques for prebypass pheresis and red cell exchange in a patient with homozygous SS hemoglobin sickle cell disease undergoing cardiopulmonary bypass.
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Laure Kpoumie, Carolle. "Gene Therapy : The New Weapon Against Diseases Until There Difficult To Overcome: Some Current Facts Of Gene Therapy And Cases Of Sickle Cell Anaemia". Journal of Clinical Research and Reports 4, nr 3 (8.06.2020): 01–07. http://dx.doi.org/10.31579/2690-1919/075.

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Sickle cell anaemia is an inherited genetic disease that affects the hemoglobin chains of red blood cell hemoglobin, carrying oxygen less well through the body. It is a rare disease, however, it is the most widespread genetic disease in the world and especially widespread in sub-Saharan Africa. It causes anemia, painful seizures that affect several organs, it is also called sickle cell anemia, this disease results in a deformation of red blood cells in the form of sickle or a crescent moon, which prevents normal circulation in the blood vessels. This will cause blood flow to be blocked. It is a disease that is geographically concentrated in certain areas such as Africa, India, Brazil, the Mediterranean Basin, but it is currently found everywhere because of mass migration and has been considered since 2008 by United Nations as a public health priority. Sickle cell disease affects black people and accounts for 50% of deaths in childhood.
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Darghouth, Dhouha, Bérengère Koehl, Geoffrey Madalinski, Jean-François Heilier, Petra Bovee, Ying Xu, Marie-Françoise Olivier i in. "Pathophysiology of sickle cell disease is mirrored by the red blood cell metabolome". Blood 117, nr 6 (10.02.2011): e57-e66. http://dx.doi.org/10.1182/blood-2010-07-299636.

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Abstract Emerging metabolomic tools can now be used to establish metabolic signatures of specialized circulating hematopoietic cells in physiologic or pathologic conditions and in human hematologic diseases. To determine metabolomes of normal and sickle cell erythrocytes, we used an extraction method of erythrocytes metabolites coupled with a liquid chromatography-mass spectrometry–based metabolite profiling method. Comparison of these 2 metabolomes identified major changes in metabolites produced by (1) endogenous glycolysis characterized by accumulation of many glycolytic intermediates; (2) endogenous glutathione and ascorbate metabolisms characterized by accumulation of ascorbate metabolism intermediates, such as diketogulonic acid and decreased levels of both glutathione and glutathione disulfide; (3) membrane turnover, such as carnitine, or membrane transport characteristics, such as amino acids; and (4) exogenous arginine and NO metabolisms, such as spermine, spermidine, or citrulline. Finally, metabolomic analysis of young and old normal red blood cells indicates metabolites whose levels are directly related to sickle cell disease. These results show the relevance of metabolic profiling for the follow-up of sickle cell patients or other red blood cell diseases and pinpoint the importance of metabolomics to further depict the pathophysiology of human hematologic diseases.
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Simões, Belinda Pinto, Fabiano Pieroni, Thalita Costa, George Navarro Barros, Guilherme Darrigo Jr., Carlos Settani Grecco, Juliana Elias Bernardes i in. "Allogenic bone narrow transplantation in sickle-cell diseases." Revista da Associação Médica Brasileira 62, suppl 1 (październik 2016): 16–22. http://dx.doi.org/10.1590/1806-9282.62.suppl1.16.

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SUMMARY Sickle-cell diseases are the most common inherited hemoglobinopathies worldwide. Improvement in survival has been seen in the last decades with the introduction of careful screening and prevention of complications and the introduction of hydroxyurea. Stem-cell transplantation is currently the only curative option for these patients and has been indicated for patients with neurological events, repeated vaso-occlusive crisis, any organ damage or presence of red blood cell antibodies. Related bone-marrow or cord-blood transplant has shown an overall survival of more than 90% with a disease-free survival of 90% in 1,000 patients transplanted in the last decades. The use of unrelated donors unfortunately has not shown the same good results, but better typing methods and improved support may improve the outcome with this source of stem cells in the future. In Brazil, only recently stem cell transplant from related donors has been included in the procedures performed in the public health system. The use of related bone marrow or cord blood and a myeloablative conditioning regimen are considered standard of care for patients with sickle-cell diseases. Transplants with non-myeloablative regimens, unrelated donors or haploidentical donors should be performed only in controlled clinical trials.
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Pryzhkova, Marina, Xuan Yuan, Abhai Tripathi, David Sullivan i Elias Zambidis. "Efficient Erythroid Differentiation of a PGD-Derived Human Pluripotent Stem Cell Line Affected with Sickle Cell Hemoglobinopathy". Blood 112, nr 11 (16.11.2008): 539. http://dx.doi.org/10.1182/blood.v112.11.539.539.

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Abstract The technology of preimplantation genetic diagnosis (PGD) screens IVF-derived cleavage-stage embryos or oocytes from parents with inherited disorders, and is routinely used to avoid births with severe genetic disorders. More than one hundred testable genetic conditions, including severe hematologic diseases such as beta thalassemia, sickle cell anemia, and Fanconi anemia can be PCR-screened from either a micro-manipulated blastomere, or a pre-fertilization ovarian polar body. Derivation of human embryonic stem cell (hESC) lines from diseased IVF blastocysts has recently been reported, and these PGD-hESC are untested yet potentially valuable tools for investigating cellular and molecular events of human embryogenesis in diseased states. For example, a great deal of interest has recently been generated in treating hematologic diseases with genetically-corrected hematopoietic stem cells (HSC) derived from patient-specific pluripotent stem cells. Generation of hematopoietic progenitors from PGD-hESC affected with genetic syndromes may thus provide novel opportunities for testing cell-based and gene therapeutic strategies. An important candidate for such cell-based therapy includes sickle cell disease (SSD) hemoglobinopathy, a classic inherited single gene disorder resulting from the substitution of glutamic acid by valine at position 6 of the beta chain of hemoglobin. Human pluripotent stem cells derived from PGD-selected blastocysts or induced pluripotency (iPS; e.g., using patient’s somatic cells), will serve as critical reagents for testing such therapeutic strategies. In these studies, we report the characterization and erythropoietic differentiation of a novel PGD-hESC line affected with SSD hemoglobinopathy. The sickle point mutation was confirmed in this PGD-hESC line with direct genomic sequencing of the beta globin locus. This hESC line possesses typical pluripotency characteristics and forms multilineage teratomas in vivo. SSD-hESC can be efficiently differentiated to the hematopoietic lineage under serum-free conditions, and gave rise to robust primitive and definitive erythropoieses. The expression of embryonic, fetal and adult globin genes in SSD PGD-hESC derived erythroid cells was confirmed by qRT-PCR, intracytoplasmic FACS, and in situ immunostaining of PGD-hESC teratoma sections. Moreover, we defined culture conditions for massive, long-term liquid culture expansion of sickle affected erythroid progenitors that remained in an undifferentiated erythroblastic phenotype for at least two months. These sickle erythroblasts were continuously maintained as a primary cell line that could be frozen and thawed without loss of viability. In vitro-expanded sickle erythroblasts expressed CD71+CD36+ and CD71+CD235a+ phenotypes, and underwent developmentally appropriate embryonic, fetal, and adult hemoglobin switching over a period of several months. Moreover, hESC-derived erythroblasts were readily infected with Plasmodium falciparum malaria parasites, thus demonstrating their potential utility in studying the effects of this important pathogen in normal and diseased erythropoiesis. These data demonstrate the utility of using patient-specific, hemoglobinopathic hESC for generating significant numbers of erythroid progenitors for molecular, developmental, gene therapeutic, pharmacologic, and microbiological studies. We are currently conducting a comparative erythropoietic differentiation study using SSD PGD-hESC vs. SSD iPS that were generated from somatic fibroblasts using defined pluripotency factors.
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Williams, David A., i Erica Esrick. "Investigational curative gene therapy approaches to sickle cell disease". Blood Advances 5, nr 23 (14.12.2021): 5452. http://dx.doi.org/10.1182/bloodadvances.2021005567.

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Abstract Sickle cell disease (SCD) is an inherited blood condition resulting from abnormal hemoglobin production. It is one of the most common genetic diseases in the world. The clinical manifestations are variable and range from recurrent acute and debilitating painful crises to life-threatening pulmonary, cardiovascular, renal, and neurologic complications. The only curative treatment of SCD at this time is bone marrow transplantation (also called hematopoietic stem cell transplantation) using healthy blood stem cells from an unaffected brother or sister or from an unrelated donor if one can be identified who is a match in tissue typing. Unfortunately, only a minority of patients with sickle cell has such a donor available. The use of autologous hematopoietic stem cells and alternative types of genetic modifications is currently under study in clinical research trials for this disease. The approaches include the use of viral vectors to express globin genes that are modified to prevent sickle hemoglobin polymerization or to express interfering RNAs to “flip the switch” in adult red cells from adult β-sickle hemoglobin to fetal hemoglobin using a physiologic switch, and several gene editing approaches with the goal of inducing fetal hemoglobin or correcting/modifying the actual sickle mutation. In this audio review, we will discuss these different approaches and review the current progress of curative therapy for SCD using gene therapy.
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Rozprawy doktorskie na temat "Sickle cells diseases"

1

Wu, Li-Chen. "Correction of sickle cell disease by homologous recombination". Thesis, Birmingham, Ala. : University of Alabama at Birmingham, 2008. https://www.mhsl.uab.edu/dt/2008p/wu.pdf.

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Tozatto, Maio Karina. "Immunogenetics in sickle cell disease". Thesis, Sorbonne Paris Cité, 2019. http://www.theses.fr/2019USPCC093.

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La drépanocytose est l’hémoglobinopathie héréditaire la plus fréquente, causée par un polymorphisme unique d’un nucléotide (SNP) dans le gène de la beta-globine (HBB). Ce SNP détermine la synthèse de l’hémoglobine S, qui polymérise lorsqu’elle est soumise au stress, et ceci change la forme des hématies drépanocytaires en faucille. Les drépanocytes sont moins déformables, plus adhérents à l’endothélium, et plus susceptibles à l’hémolyse. Les complications cliniques de la drépanocytose peuvent être expliquées par l’interaction entre la vaso-occlusion, l’hémolyse et l’activation inflammatoire résultant de la présence des drépanocytes dans la circulation. Les patients drépanocytaires peuvent présenter de nombreuses complications, qui touchent tous les organes. La présentation clinique de cette maladie est très hétérogène, variant entre des patients qui ont très peu de symptômes à des patients qui décèdent de la maladie. Sachant que l’inflammation joue un rôle majeur dans la physiopathologie de la drépanocytose, des polymorphismes dans les gènes inflammatoires peuvent être évoqués pour expliquer cette hétérogénéité. La greffe de cellules souches hématopoïétiques est la seule thérapie curative disponible actuellement pour la drépanocytose, avec des bons résultats démontrés après la greffe d’un donneur apparenté HLA identique. Néanmoins, la plupart des patients n’a pas de donneur apparenté. Les patients drépanocytaires sont d’origine africaine, le groupe ethnique le moins représenté dans les registres de donneurs non apparentés de cellules souches. A ce jour, peu d’études, utilisant des registres locaux, ont été faites pour estimer la probabilité des patients drépanocytaires de trouver un donneur potentiel non apparenté dans les registres internationaux.Cette étude a eu pour objectif d’évaluer le rôle de quelques gènes inflammatoires liés aux Toll-like récepteurs (TLR) dans la survenue des infections bactériennes chez les patients drépanocytaires, vu que les infections sont une cause majeure de mortalité chez ces patients, et les TLR sont impliqués dans la reconnaissance de plusieurs types de bactéries. Les patients inclus avaient des échantillons d’ADN et des données cliniques disponibles. Les SNPs ont été génotypés par réaction en chaîne par polymérase en temps réel (RT-PCR). Quatre-cents trente patients, la plupart d’origine brésilienne ou africaine subsaharienne, ont été divisés en deux groupes : infectés (n=235, patients qui ont eu au moins un épisode d’infection bactérienne documentée) et non infectés (n=195, patients qui n’ont jamais présentés d’infections sévères). Le génotype T/A du SNP rs4696480 in TLR2 a été plus fréquent chez les patients non infectés (50% versus 67%, OR=0.50, 95% CI 0.34-0.75, p<0.001). En outre, le génotype T/T de ce SNP a été plus fréquent chez les patients infectés (15% versus 5%, OR=0.50, 95% CI 0.34-0.75, p<0.001). Des études précédentes ont démontré que les individus A/A avaient plus de sécrétion de marqueurs inflammatoires, lorsque l’allèle T était associé à moins de fréquence et de sévérité des maladies inflammatoires. Cette étude a également eu pour objectif d’estimer la probabilité de trouver un donneur potentiel non apparenté, antigène leucocytaire humain (HLA) allélique identique pour les loci HLA-A, HLA-B et HLA-DRB1. Dans cette étude, 185 patients ont été inclus, 116 suivis dans un centre brésilien et 69 greffés d’un donneur apparenté ou non apparenté dans des centres de greffe qui reportent leurs données à la Société Européenne de Greffe de Cellules Souches (EBMT). Les patients inclus avaient des données HLA testés en moyenne ou haute résolution. Les haplotypes HLA ont été estimés à travers un logiciel, HaploStats, et classifiés selon l’ethnicité. Ensuite, nous avons recherché des potentiels donneurs HLA alléliques identiques pour les loci HLA-A, HLA-B et HLA-DRB1 (6/6) dans des registres internationaux (WMDA)
Sickle cell disease (SCD) is the most common inherited hemoglobinopathy, caused by a single nucleotide polymorphism (SNP) in the beta-globin (HBB) gene. This SNP determines the synthesis of S haemoglobin (HbS), which polymerizes under stress conditions, sickling the red blood cell (RBC). Sickle RBC are less deformable, more adherent to the endothelium, and more susceptible to haemolysis. SCD complications are explained by the interaction between haemolysis, vaso-occlusion and inflammatory activation, determined by the RBC sickling. Patients with SCD may present several complications, affecting all organs. Clinical presentation is very heterogeneous, ranging from patients who have mild symptoms to patients who die from disease complications. Because inflammation plays a major role in SCD, polymorphisms in inflammatory genes are potential targets to explain this heterogeneity. Haematopoietic stem cell transplantation (HSCT) is the only curative therapy currently available for SCD, with good results shown after human leukocyte antigen (HLA) identical sibling HSCT. However, most patients will not have a matched sibling donor. Patients with SCD are mostly from African origin, the less represented ethnic group in stem cell donor registries. To date, few studies using local registries were performed to find the probability of having a potential unrelated donor in SCD settings. This study aimed to assess the role of inflammatory genes encoding Toll-like receptors (TLR) in the occurrence of bacterial infections in patients with SCD, because infection is a leading cause of mortality in SCD, and TLR recognize a wide range of bacteria. Patients included had DNA samples and clinical data available. SNPs were genotyped by real-time polymerase chain reaction (RT-PCR). Four hundred thirty patients, mostly from Brazilian and Sub-Saharan African origin, were divided in two groups: infected (n=235, patients who presented at least one episode of bacterial infection), and non-infected (n=195, patients who never presented bacterial infections). The T/A genotype of SNP rs4696480 in TLR2 was less frequent in infected patients (50% versus 67%, OR=0.50, 95% CI 0.34-0.75, p<0.001). In addition, the T/T genotype of this SNP was more frequent among infected patients (15% versus 5%, OR=0.50, 95% CI 0.34-0.75, p<0.001). Previous reports in other settings showed that A/A carriers had higher secretion of inflammatory markers, while T allele was associated with less occurrence and severity of inflammatory diseases. Hence, T/A genotype might express the ideal inflammatory response to defeat bacteria, while the weaker inflammatory response determined by the T/T genotype increases susceptibility to bacterial infections in SCD settings
A doença falciforme (DF) é a hemoglobinopatia hereditária mais frequente, causada por um polimorfismo de nucleotídeo único (SNP) no gene da betaglobina (HBB). A ocorrência desse SNP determina a síntese de hemoglobina S, que polimeriza sob condições de stress, alterando a conformação das hemácias, que adquirem forma de drepanócitos. Os drepanócitos são menos deformáveis, mais aderentes ao endotélio e mais suscetíveis à hemolise. As complicações clínicas da DF podem ser explicadas pela interação entre a vasoclusão, hemólise e ativação inflamatória resultantes da presença dos drepanócitos na circulação. Os pacientes com DF podem apresentar numerosas complicações, que afetam todos os órgãos. A apresentação clínica da DF é muito heterogênea, variando de pacientes pouco sintomáticos a pacientes que falecem por complicações da doença. Visto que a inflamação tem um papel importante na fisiopatologia da DF, polimorfismos em genes inflamatórios poderiam explicar essa heterogeneidade.O transplante de células tronco hematopoiéticas (TCPH) é a única terapia curativa disponível atualmente para a DF, com bons resultados demonstrados em TCPH de doador aparentado antígeno leucocitário humano (HLA) idêntico. Não obstante, a maioria dos pacientes não dispõe de doador aparentado HLA idêntico. A DF ocorre em pacientes normalmente de origem africana, o grupo étnico menos representado em registro de doadores de células tronco. Nos dias de hoje, poucos estudos, utilizando registros locais, avaliaram a probabilidade de encontrar potenciais doadores não aparentados para pacientes com DF. Este estudo teve por objetivo avaliar o papel de genes inflamaórios que codificam receptores Toll-like (TLR) na ocorrência de infecções bacterianas em pacientes com DF, visto que infecção é uma das principais causas de mortalidade em DF, e os TLR reconhecem diversos tipos de bactérias. Os pacientes incluídos no estudo tinham amostras de DNA e dados clínicos disponiveis. Os SNPs foram genotipados por reação em cadeia de polimerase em tempo real (RT-PCR). Quatrocentos e trinta pacientes, a maioria de orgem brasileira ou africana subsaariana, foram divididos em dois grupos, infectados (n=235, pacientes que apresentaram ao menos um episodio de infecção bacteriana), e não infectados (n=195, pacientes que nunca tiveram tais infecções). O genótipo T/A do SNP rs4696480 foi menos frequente em pacientes infectados (50% versus 67%, OR=0.50, 95% CI 0.34-0.75, p<0.001). Além disso, o genótipo T/T do mesmo SNP foi mais frequente em pacientes infectados (15% versus 5%, OR=0.50, 95% CI 0.34-0.75, p<0.001). Estudos prévios mostraram que indivíduos com genótipo A/A apresentavam mais secreção de marcadores inflamatórios, enquanto o alelo T foi associado a menor ocorrência e menor gravidade de doenças inflamatórias
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Cytlak, Urszula Malgorzata. "Phosphatidylserine exposure in red blood cells from patients with sickle cell disease". Thesis, University of Cambridge, 2015. https://ethos.bl.uk/OrderDetails.do?uin=uk.bl.ethos.708601.

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4

Hays, Mary Margaret. "Stem cell transplant for sickle cell disease". Thesis, Boston University, 2013. https://hdl.handle.net/2144/12117.

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Thesis (M.A.)--Boston University
Background: Sickle cell disease (SCD) is the most common inherited blood disorder in the United States. As SCD can cause significant morbidity and decrease in life expectancy, further research on curative options is of great interest. Hematopoietic stem cell transplant (HSCT) is the only treatment option offering a chance of cure, but the risks of treatment are not negligible. Because the outcomes of HSCT are best when the procedure is performed at a younger age, understanding what parents know about transplant, their opinion on this option and the risks they are willing to take to achieve a cure is of great value. As sickle cell disease has changed in the United States from a life-threatening condition of childhood to a chronic condition with most of the burden of morbidity and mortality shifted towards adulthood, it is necessary for parents to be fully aware of long term risks and educated on all therapeutic options, so the optimal decision can be made. Objectives: (i) To learn about parents’ recollection and pursuit of further information after undergoing an educational session on risks and benefits of HSCT. (ii) To learn about their worries about transplant and the highest mortality and infertility risks they are willing to accept in order to achieve a cure for their child. (iii) To learn about parents’ readiness to proceed to transplant based on a hypothetical scenario. [TRUNCATED]
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5

Belfield, Helen. "Expression of adhesion molecules on the erythroid cells of patients with sickle cell disease". Thesis, University of Bristol, 2005. http://ethos.bl.uk/OrderDetails.do?uin=uk.bl.ethos.411024.

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Kawadler, J. M. "Neuroimaging biomarkers in paediatric sickle cell disease". Thesis, University College London (University of London), 2015. http://discovery.ucl.ac.uk/1464063/.

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Sickle Cell Disease (SCD) is a collection of genetic haemoglobinopathies, the most common and severe being homozygous sickle cell anaemia. In the UK, it has been estimated that 1 in 2000 children are born with SCD. The disease is characterised by chronic anaemia, recurrent pain crises and vascular occlusion. Neurologically, there is a high incidence of stroke in childhood, as well as cognitive dysfunction. Newborn screening programmes and preventative treatments have allowed a much longer lifespan; however recently, neurological research has shifted to characterising subtler aspects of brain development and functioning that may be critically important to the individual’s quality of life. This thesis overviews the neurological and neurocognitive complications of SCD, and how magnetic resonance imaging (MRI) can provide biomarkers for severity of disease. During the PhD, retrospective and prospective cognitive and MRI data were collected and analysed. Diagnostic clinical MRI sequences and advanced MRI sequences were applied, as well as a neuropsychological test battery aimed at intelligence and executive function. First, this thesis reviews the intelligence literature in SCD and includes previously unreported data, finding patients, regardless of abnormality seen on conventional MRI, have lowered full-scale intelligence quotient than controls. Then, to determine imaging biomarkers, volumetric differences and diffusion characteristics were identified. Patients were found to have decreased volumes of subcortical structures compared to controls, in groups corresponding to disease severity. Results from a three-year longitudinal clinical trial suggest evidence of atrophy in paediatric patients, with no apparent protective effect of treatment. Diffusion tensor imaging revealed reduced white matter integrity across the brain, correlating with recognised markers of disease severity (i.e. oxygen saturation and haemoglobin from a full blood count). Overall, the four experiments bridge a gap in the cognitive and neuroimaging literature of the extent of neurological injury in children with SCD.
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Gavlak, J. C. D. "Sleep in children with sickle cell disease". Thesis, University College London (University of London), 2016. http://discovery.ucl.ac.uk/1528622/.

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BACKGROUND: Sickle Cell Anaemia (SCA) or homozygosity for the sickle haemoglobin gene (HbSS) is the most common genetic condition in the UK. A high prevalence of Sleep Disordered Breathing (SDB) in SCA is widely accepted but there is a lack of unselected population based studies. As Polysomnography (PSG) is expensive, screening for SDB using a robust tool, e.g. the Delta 12 (Δ12) index and 3% Oxygen Desaturation Indices (ODI) calculated from home pulse oximetry, should be validated. Elevated Cerebral Blood Flow Velocity (CBFV) is a predictor of stroke in SCA, and may be associated with SDB. METHODS AND RESULTS: Prevalence of OSA was assessed and compared with the general paediatric population from Polysomnography (SCA n=195) analysed using the American Academy of Sleep Medicine (AASM) criteria. Interobserver reliability between two observers was excellent. At all ages, the prevalence of OSA was higher in SCA. OSA prevalence was highest in young children, with 50% of 4-6 year olds having an obstructive apnoea hyopnoea index (OAHI) of >1. A zero inflated model was fitted for prediction of OAHI from Δ12 or ODI; for predicting OAHI zero or >1, 3% ODI alone had the best fit. In 83 London patients, OSA was associated with elevated CBFV. Mean SpO2, ODI and CAI were predictors of basilar velocity independent of age. In the final model, mean SpO2 remained a predictor (B-3.1, beta -0.41, t -4.2, p < 0.0005) independent of age (B- 2.9, beta -0,453, t -4.7, p < 0.0005). CONCLUSION: Despite the difficulties in comparing across populations, there is a higher prevalence of OSA in unselected children with SCA than in typically developing children with no SDB symptoms, screening with overnight pulse oximetry to select those with OAHI>1 is feasible, and this may be important as there is an association between OSA and basilar CBFV in this population.
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Gardner, Catherine Joanne. "Genotype-phenotype correlation in sickle cell disease". Thesis, King's College London (University of London), 2017. https://kclpure.kcl.ac.uk/portal/en/theses/genotypephenotype-correlation-in-sickle-cell-disease(07a190be-c88a-41f2-8e74-e063d85919a3).html.

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Sickle cell disease (SCD) has a complex pathophysiology initiated by the polymerisation of deoxy-sickle-haemoglobin. The single nucleotide change underpinning SCD does not account for the vast range and severity of SCD complications. This clinical heterogeneity is only partly explained by the genetic variability of fetal haemoglobin gene levels and co-inheritance of α- thalassemia. Although environmental factors also contribute to the clinical complexity of SCD, further genetic modifiers of SCD severity exist but are yet to be determined. Genetic association studies have been boosted recently not only with the advent of new genotyping tools, but also with the development of increasingly sophisticated analytical methods. New developments in phenotyping, genotyping and genotype-phenotype association approaches allow us to disentangle true genetic associations from hits due to chance. This thesis seeks to investigate biomarkers of sickle severity and to use these clinical markers in genotype-phenotype correlation studies. I have investigated three key markers of disease severity: haemolysis, frequency of acute pain episodes and mortality. Estimated median survival of 67 years in HbSS disease in our UK cohort is a significant improvement in survival compared to other recent estimates in the USA and Jamaica. I have undertaken genome-wide micro-array scanning and created an imputed genotype dataset of over 15,000,000 genetic variants. I have used these phenotype and genotype datasets to conduct genetic association studies, both genome-wide and candidate gene association studies. These analyses are based on linear mixed modelling to account for relatedness (including population stratification) within the cohort. In addition to the severity outcomes, I have also evaluated the known genetic loci for HbF and created a genetic “summary statistic” to quantify the effects of these three loci. Finally, I have also assessed the role of the erythroid regulator KLF1 in HbF levels in SCD with two laboratory-based projects.
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Barber, Latorya Arnold. "The Activity of Lipid Transport Proteins in Normal and Sickle Red Blood Cells". University of Cincinnati / OhioLINK, 2009. http://rave.ohiolink.edu/etdc/view?acc_num=ucin1243353188.

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Mackey, Michelle Noble. "Understanding Parents' Disease-Managing Strategies for Children With Sickle Cell Disease". ScholarWorks, 2019. https://scholarworks.waldenu.edu/dissertations/6610.

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Sickle cell disease (SCD) is one of the most difficult and stressful chronic diseases for parents of afflicted children to manage. Managing SCD can be traumatic for parents particularly if they have no specific coping strategies for managing the disease or ensuring the child visits the doctor as scheduled. The use of certain coping strategies may affect the parents' and patients' perceptions of the illness and influence their decisions regarding treatment, which can have a lasting impact on their lives. Effective parental strategies such as positive thinking can aid in disease management, but there is limited research on the coping strategies used by parents of children with SCD specifically. The purpose of this phenomenological study, which was guided by Thompson and Gustafson's transactional stress and coping model, was to describe parents' coping strategies in managing their young child's SCD as it relates to use of health services. Data collection included one-to-one, open-ended interviews with 10 parents of children with SCD. Colaizzi's method of phenomenological data analysis was used to identify themes. Five themes emerged from data analysis and they are: parental methods of coping with SCD, participants' understanding of SCD, SCD family and support, managing SCD with hydration and medication, and experience accessing healthcare. These results indicated the participants' coping strategies varied according to their individual situations. Insight from this study could lead to positive social change by helping to identify specific coping strategies parents can use to better manage their child's disease and effectively access available health services.
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Książki na temat "Sickle cells diseases"

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Alvin, Silverstein, Silverstein Virginia B i Nunn Laura Silverstein, red. What you can do about sickle cell disease. New York, NY: Enslow Publishing, 2016.

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Peak, Lizabeth. Sickle cell disease. Detroit: Lucent Books, 2008.

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Peak, Lizabeth. Sickle cell disease. Detroit: Lucent Books, 2008.

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Serjeant, Graham R. Sickle cell disease. Wyd. 2. Oxford: Oxford University Press, 1992.

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F, Whitten Charles, Bertles John F. 1925-, National Association for Sickle Cell Disease (U.S.) i New York Academy of Sciences., red. Sickle cell disease. New York, N.Y: New York Academy of Sciences, 1989.

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Eboh, Winifred Oluchukwu. Sickle cell disease. (Birmingham): Birmingham Sickle Cell & Thalassaemia Centre, 1993.

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Jones, Phill. Sickle cell disease. New York: Chelsea House, 2008.

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Samuel, Charache, i Johnson Cage S, red. Sickle cell disease. Philadelphia: W.B. Saunders, 1996.

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McCormick, Marie, Henrietta Awo Osei-Anto i Rose Marie Martinez, red. Addressing Sickle Cell Disease. Washington, D.C.: National Academies Press, 2020. http://dx.doi.org/10.17226/25632.

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Beshore, George. Sickle cell anemia. New York: F. Watts, 1994.

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Części książek na temat "Sickle cells diseases"

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Álvarez, Ofelia, i María Angélica Wietstruck. "Sickle Cell Disease". W Pediatric Respiratory Diseases, 529–41. Cham: Springer International Publishing, 2020. http://dx.doi.org/10.1007/978-3-030-26961-6_52.

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Kothari, Nikisha, i Amir Mohsenin. "Sickle Cell Retinopathy". W Manual of Retinal Diseases, 347–50. Cham: Springer International Publishing, 2016. http://dx.doi.org/10.1007/978-3-319-20460-4_69.

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Dworkis, Daniel A., i Martin H. Steinberg. "Sickle Cell Disease". W Metabolism of Human Diseases, 289–93. Vienna: Springer Vienna, 2014. http://dx.doi.org/10.1007/978-3-7091-0715-7_42.

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Mallouh, Ahmad A. "Sickle Cell Disease". W Textbook of Clinical Pediatrics, 3005–22. Berlin, Heidelberg: Springer Berlin Heidelberg, 2012. http://dx.doi.org/10.1007/978-3-642-02202-9_324.

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Kashani, John, Richard D. Shih, Thomas H. Cogbill, David H. Jang, Lewis S. Nelson, Mitchell M. Levy, Margaret M. Parker i in. "Sickle Cell Disease". W Encyclopedia of Intensive Care Medicine, 2075–77. Berlin, Heidelberg: Springer Berlin Heidelberg, 2012. http://dx.doi.org/10.1007/978-3-642-00418-6_342.

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Fischer, Matt, Harsh Sachdeva i Alaa Abd-Elsayed. "Sickle Cell Disease". W Pain, 1279–81. Cham: Springer International Publishing, 2019. http://dx.doi.org/10.1007/978-3-319-99124-5_274.

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Roth, Elliot J. "Sickle-Cell Disease". W Encyclopedia of Clinical Neuropsychology, 3180–81. Cham: Springer International Publishing, 2018. http://dx.doi.org/10.1007/978-3-319-57111-9_2197.

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Chaudhury, Sonali, i Shalini Shenoy. "Sickle cell disease". W Clinical Manual of Blood and Bone Marrow Transplantation, 236–45. Chichester, UK: John Wiley & Sons, Ltd, 2017. http://dx.doi.org/10.1002/9781119095491.ch27.

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Glassberg, Jeffrey, i Michael R. DeBaun. "Sickle Cell Disease". W Respiratory Medicine, 131–38. Cham: Springer International Publishing, 2016. http://dx.doi.org/10.1007/978-3-319-43447-6_11.

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Orellana, Juan, i Alan H. Friedman. "Sickle Cell Disease". W Clinico-Pathological Atlas of Congenital Fundus Disorders, 101–4. New York, NY: Springer New York, 1993. http://dx.doi.org/10.1007/978-1-4613-9320-7_19.

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Streszczenia konferencji na temat "Sickle cells diseases"

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Liang, Jiebin, Yunhyuk Chang, Jinhyeok Kim, Zhiyuan Li, John Canevari i Sasan Haghani. "Sickle Cell Disease Patient/Provider Match Tool". W 2024 IEEE International Humanitarian Technologies Conference (IHTC), 1–6. IEEE, 2024. https://doi.org/10.1109/ihtc61819.2024.10855129.

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Highland, Robert E., Cindy X. Chen, David A. Miller, Chao-Chieh Lin, Jen-Tsan A. Chi i Adam Wax. "Examining sickle cell disease using high-throughput holographic cytometry". W Quantitative Phase Imaging XI, redaktorzy YongKeun Park i Yang Liu, 34. SPIE, 2025. https://doi.org/10.1117/12.3044015.

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Lodonio, Larissa Lacerda, Livia Romana Lima Gonçalves Arrais, David Nilson Gondim Alves, Valéria Sampaio Freire Alencar, Eva Cristina Lopes Vieira Torres, Estefani Gonçalves de Almeida Grangeiro, Jackeline Lima Vidal, Orleudo Ferreira Teixeira, Gessyca Tavares Feitosa i Joanderson Nunes Cardoso. "Acute chest syndrome in sickle cell anemia: Diagnostic challenges and therapeutic strategies". W VI Seven International Multidisciplinary Congress. Seven Congress, 2024. http://dx.doi.org/10.56238/sevenvimulti2024-066.

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Sickle cell disease is a genetic blood disorder caused by a mutation in the amino acid glutamic acid, which is replaced by valine in the beta chain of the hemoglobin molecule. In this context, acute chest syndrome is a serious disease with a high rate of morbidity and mortality, accounting for approximately 25% of deaths in patients with sickle cell anemia. Thus, the objective of this study is to describe the main aspects of Acute Chest Syndrome (ACS) in patients with sickle cell anemia. This is a review study of a scoping review in which data collection was carried out on the research portal of the Virtual Health Library (BVS). The databases used were: Online Medical Literature Search and Analysis System ( MEDLINE ) and Latin American and Caribbean Literature in Health Sciences (LILACS ) , where the following descriptors were used for the search: “Sickle Cell Anemia” AND“Acute Chest Syndrome”. Using the descriptors, 451 articles were found. The inclusion criteria were: articles published between 2019 and 2024, available in full and free of charge; and the exclusion criteria were repeated articles, paid articles, and methods with an emphasis on literature review. Through the established criteria, 20 studies were included in the final sample. Acute chest syndrome is characterized by fever and/or respiratory symptoms with pulmonary infiltrates, which can lead to sepsis and cause stroke in patients with sickle cell anemia. In addition, hemolysis increases during sickle cell crises, causing a faster depletion of nitric oxide, which is a potent vasodilator of metabolism and a factor for cardiopulmonary hemodynamics. In this context, ultrasound is recognized as the gold standard in the diagnosis of the syndrome, due to the absence of radiation, with high accuracy, sensitivity, and specificity. Changes in lung function in acute chest syndrome cause variations in the levels of inflammatory markers that can help to recognize the condition and treat it more effectively, such as phosphatidylserine, a specific type of phospholipid that is essential in cell membranes, serum ferritin, which increases in an attempt to compensate for the increase in hemolysis, and IL-6, which reflects the recruitment of monocytes and other innate immune cells in the lungs. In view of the intense hemolysis, red blood cell transfusions can be seen as a protective factor for acute chest syndrome and can even be considered the definitive therapy for the syndrome, as it improves the supply of oxygen to the tissues, increases the overall level of hemoglobin and reduces the fraction of sickle-shaped red blood cells. This allows the patient to have relief from respiratory symptoms more quickly. Therefore, acute chest syndrome in patients with sickle cell anemia requires appropriate interventions and should be treated as rare diseases in health units, whether in the basic health unit or in emergency care, in order to ensure adequate treatment without delays. Thus, the management that should be carried out is analgesia, hydration, antibiotic therapy, bronchodilators, ventilation, which may be invasive or non-invasive, oxygen and blood transfusion.
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Galpayage Dona, Kalpani Nisansala Udeni, Jia Liu, Yuhao Qiang, E. Du i A. W. C. Lau. "Electrical Equivalent Circuit Model of Sickle Cell". W ASME 2017 International Mechanical Engineering Congress and Exposition. American Society of Mechanical Engineers, 2017. http://dx.doi.org/10.1115/imece2017-70677.

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Mature red blood cell (RBC) consists of cytoplasm, mainly normal hemoglobin (HbA) within a plasma membrane. In sickle cell disease, abnormal sickle hemoglobin (HbS) molecule polymerizes and forms into rigid fibers at low oxygen tension, which contributes to variation in the biophysical properties of sickle cells from healthy RBCs. This paper presents an electrical equivalent circuit (EEC) model of sickle cell that considers the phase transition of oxy-HbS solution to deoxy-HbS polymers. Briefly, we model the oxy-HbS solution following healthy RBCs using a resistor and deoxy-HbS fibers as a capacitor. To validate the model, electrical impedance measurements of cell suspensions for normal RBCs and sickle cells are performed, using a multi-channel lock in amplifier in the frequency range of 1 kHz to 10 MHz in a customized microfluidic chamber. Quantitative measurements of the classical components of EEC model are extracted using the developed EEC sickle cell model, allowing us to better understand the biophysics of cell sickling event in sickle cell disease.
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Alade, Oluwatomi. "Brief Review: Regional Anesthesia for Vaso-occlusive Pain Crises". W 28th Annual Rowan-Virtua Research Day. Rowan University Libraries, 2024. http://dx.doi.org/10.31986/issn.2689-0690_rdw.stratford_research_day.41_2024.

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Vaso-occlusive pain crisis occurs with obstruction of blood vessels from sickled red blood cells. This results in ischemic injury causing in pain. Acute vasoocclusive pain crisis is one of the most common reasons for patients with sickle cell disease to present to the hospital for medical attention. Acute treatment involves IV opioid therapy, non-opioid therapy, and IV hydration. There is a known lack of trust between a patient in acute pain and a provider in the emergency department (ED) and hospital secondary to stereotypes regarding pain seeking behavior. Here we discuss a case of vasoocclusive pain crisis refractory to opioid therapy and local regional anesthesia as an alternative treatment.
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Liu, Jia, Yuhao Qiang i E. Du. "Measurement of Electrical Properties of Sickle Cells From Electrical Impedance of Cell Suspension". W ASME 2017 International Mechanical Engineering Congress and Exposition. American Society of Mechanical Engineers, 2017. http://dx.doi.org/10.1115/imece2017-71734.

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Analysis of the electrical properties of a biological cell can provide useful information about its characteristic features, such as the intracellular composition, charge distribution and composition changes in cell membrane, as well as the extracellular environment. Electrical impedance spectroscopy of a cell suspension can be used to extract an average measure of the electrical properties of single cells. In sickle cell disease, the disease state of a sickle red blood cell is closely related to the intracellular hemoglobin composition and concentration. This study presents an electrical impedance measurement of sickle cell suspension with normal red blood cells as control. Electrical impedance spectra of cell suspensions are obtained in the range of 1000 Hz to 1MHz. Based on Maxwell’s mixture theory, average values of membrane capacitance and cytoplasm resistance of single cells are extracted for both normal and sickle blood samples. Comparing to traditional parallel-plate setup for cell suspension subjected to frequency sweep, this method requires low quantity of blood specimens and can be potentially valuable for patients that are already anemic.
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Chamone, D. A. F., A. Y. Hoshikawa-Fujimura, C. Massumoto, G. Bellotti, F. Arashiro i M. Jamra. "ABNORMALITIES OF PLATELET AGGREGATION AND ENHANCED FACTOR X ACTIVATOR ACTIVITY OF WASHED PLATELETS IN SICKLE CELL DISEASE". W XIth International Congress on Thrombosis and Haemostasis. Schattauer GmbH, 1987. http://dx.doi.org/10.1055/s-0038-1644544.

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The occurence of microvascular occlusion is one of the most prominent pathologic features of sickle cell anemia. The mechanism of vaso occlusion has generally been attributed to the abnormal shape and reduced deformability of the sickled erithrocy tes. However, the involvement of vascular endothelium, platelets and their interactions with coagulation factors may also be of pathogenic significance in microvascular occlusive crises.We investigated the interaction between vascular endothelium, platelets and blood coagulation factors in 23 patients with Sickle Cell Disease (SCD) and in normal volunteers.Factor X activator activity in washed platelets was performed according to Semeraro and Vermylen (1977), thromboxane B2 (TXB2) and 6-keto-PGF1β were determined using specific radioimmunoassays.. PAF-acether from platelets was determined according to Chignard et al (Nature, 1979, 279:799). Platelet aggregation was performed with a Chrono-Log Aggregometer (Model 440) on platelet rich plasma (PRP) using the Born method. Prostacyclin release from endothelium was performed according to Mon-cada et al (Lancet i:18, 1977).Our results showed that platelets from patients with SCD ha ve enhanced factor X activator activity (p < 0.0001), produce mo re PAF-acether than controls (p < 0.02) and showed hyperaggregability in these patients as compared to normal volunteers (p < 0.00001).We concluded that platelets from homozygous sicklers have enhanced factor X activator activity as well as increased capacity for PAF-acether production. These abnormalities may contribute to the incidence of vaso occlusive crises in these patients.
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Ferrant, A., N. Leners, E. K. Gini, J. P. Osselaerey i J. Sonnet. "EFFECT OF PIRACETAM ON THE MEAN INTRASPLENIC RED CELL TRANSIT TIME (MST) IN SICKLE CELL DISEASE AND SICKLE CELL THALASSAEMIA". W XIth International Congress on Thrombosis and Haemostasis. Schattauer GmbH, 1987. http://dx.doi.org/10.1055/s-0038-1644215.

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The effect of Piracetam on the MST has been evaluated in 3 children with homozygous sickle cell disease, and in 1 child and 2 adults with sickle cell β thalassaemia. The MST was measured using 99 Tern labelled autologous red cells before and during treatment with Piracetam (160 mg/kg/d). Tests for in vitro fi1terabi1ity of red cells were performed and an improvement of the in vitro deformabi1ity was observed in all the patients.A shortening of the MST was also observed :
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Glas-Greenwalt, P., J. Palascak, R. Gruppo, D. Stroop i V. Pollak. "DEFECTIVE FIBRINOLYSIS IN SICKLE CELL DISEASE". W XIth International Congress on Thrombosis and Haemostasis. Schattauer GmbH, 1987. http://dx.doi.org/10.1055/s-0038-1644838.

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Vasocclusive crises (VOC) cause significant morbidity and mortality in sickle cell disease (SCD). Although sickling is thought to be the predominant factor in VOC, investigators have examined the possible role of the hemostatic mechanism in the process. The data are, however, inconsistent. We studied, functionally with the fibrin plate method, the fibrinolytic system in 36 adults in the steady state and in 8 children, 7 of whom suffered from painful crises. Values in 240 normal blood donors were: tissue-type plasminogen activator activity (t-PA); 3-25 activator units/ml, corresponding to 0.04 to 0.4 ng/ml; plasminogen activator inhibitor (PA-I): 649-885 inhibitor units/ml; and α2-antiplasmin (α2-AP) : 718-970 inhibitor units/ml. In patients with sickle cell disease t-PA levels were below the normal range in 24/44. PA-I and α2-AP levels were elevated in 35/44 and 23/44 respectively. The prevalence was similar in patients in crises and quiescent. Functionally impaired t-PA was associated with either low, normal or high t-PA antigen levels measured immunologically with an ELISA technique (normal range: 3-6 ng/ml). This indicates various degrees of endothelial dysfunction, ranging from impaired synthesis to functionally defective protein to complex formation with PA-I. Fibrin has been implicated in the intravascular sludging of sickle cells. It is suggested that a defective fibrin-clearing system contributes to the syndrome of SCD.
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Nguka, Prof Gordon. "Nutrition and Dietetics Lens in the Management Sickle Cell Disease". W 3rd International Nutrition and Dietetics Scientific Conference. KENYA NUTRITIONISTS AND DIETICIANS INSTITUTE, 2023. http://dx.doi.org/10.57039/jnd-conf-knt-2023-004.

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SICKLE CELL disease is the most common severe monogenic disorder in humans. In Africa, 50–90% of children born with sickle cell disease die before they reach their fifth birthday. A cure for sickle cell anemia (SCA) is not available to all who have inherited this devastating genetically inherited disease. However, increasing knowledge that nutritional imbalances are fundamental to the severity of the disease, has produced interest in promoting dietary and nutrition intervention for treating these patients. This review seeks to emphasize the understanding that both children and adults with sickle cell disease require much higher energy and protein consumption (more macronutrient intake) than healthy individuals and tend to suffer from under nutrition if energy intake is consistently low. Shortages may also exist for micronutrients, e.g., Glutathione, which has both anti-inflammatory and antioxidant properties. Both chronic inflammation and oxidative stress are central issues for increased sickle cell disease severity. In conclusion, dedicating more effort and resources to establishing recommended dietary reference intakes (DRIs)/recommended dietary allowances (RDAs) for SCA patients is essential, and nutritional intervention should be integrated as an important treatment in tandem with standard practice.
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Raporty organizacyjne na temat "Sickle cells diseases"

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Santos Garcia, João Batista, i Ana Laura Schwartzmann Bruno. Pain in Sickle Cell Disease and Acute Varicella Zoster Pain. World Federation of Societies of Anaesthesiologists, grudzień 2024. https://doi.org/10.28923/atotw.537.

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This tutorial addresses the etiology and management of pain associated with sickle cell disease and acute pain related to varicella zoster. Both are severe pain conditions with a significant impact on individuals' functionality and the potential to cause substantial suffering.
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Paul, Satashree. Turning Back the Sickle Cell Disease: A New Drug into Play. Science Repository OÜ, maj 2021. http://dx.doi.org/10.31487/sr.blog.38.

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Researchers at the Fulcrum Therapeutics developed a bioavailable drug candidate called FTX 6058 – (a novel small molecular fetal haemoglobin inducer for sickle cell disease) that can restore the body’s ability to produce fetal haemoglobin
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Krishnamurti, Lakshmanan, Deepika Darbari, Victor Gordeuk, Torria Beasely, Clark Brown, Syed Nouraie i Gregory Kato. Can Personalized Encouragement Help People With Sickle Cell Disease Take Hydroxyurea Therapy Regularly? Patient-Centered Outcomes Research Institute® (PCORI), maj 2020. http://dx.doi.org/10.25302/05.2020.ce.13046859em.

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Yu, Winifred W., Tanvi Reddy i Melissa Barnhart. Dental Care for People With Sickle Cell Disease: A Rapid Response Literature Review. Agency for Healthcare Research and Quality (AHRQ), lipiec 2024. http://dx.doi.org/10.23970/ahrqepcrapid_dental_sicklecell.

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Lanzkron, Sophie, Jane Little, Joshua Field, J. Ryan Shows, Carlton Haywood Jr, Ravi Varadhan, Mustapha Saheed i in. Comparing Pain Management for Sickle Cell Disease Crises in Emergency Rooms and Infusion Centers. Patient-Centered Outcomes Research Institute (PCORI), sierpień 2020. http://dx.doi.org/10.25302/08.2020.ihs.140311888.

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Krishnamurti, Lakshmanan, Diana Ross, Nitya Bakshi, Cynthia Brown Sinha i Geoerge Loewenstein. An Online Decision Aid to Help Patients and Caregivers Decide on Treatments for Sickle Cell Disease. Patient-Centered Outcomes Research Institute® (PCORI), styczeń 2020. http://dx.doi.org/10.25302/1.2020.ce.12114318em.

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Marques, Carla, Larissa Lopes, Rita Lucena i Abrahão Baptista. Brain morphofunctional changes associated with pain in children, adolescents and young adults with sickle cell disease. INPLASY - International Platform of Registered Systematic Review and Meta-analysis Protocols, grudzień 2022. http://dx.doi.org/10.37766/inplasy2022.12.0022.

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Crosby, Lori, Constance Mara, Yolanda Johnson, Rogelle Hackworth i Charles Quinn. Comparing Ways to Help Parents of Children with Sickle Cell Disease Decide on Treatment -- The ENGAGE HU Study. Patient-Centered Outcomes Research Institute (PCORI), luty 2024. http://dx.doi.org/10.25302/02.2024.cdr.160936055.

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Amponsah, Isaac, Denzel Opoku-Kwabi, Francis Ackah Armah, John Nii Addotey, Bernard Kofi Turkson i Emmanuel Quaye Kontoh. A systematic review of validated medicinal plants and their compounds as agents for the management of sickle cell disease. INPLASY - International Platform of Registered Systematic Review and Meta-analysis Protocols, kwiecień 2024. http://dx.doi.org/10.37766/inplasy2024.4.0121.

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Desai, Payal, Raymona Lawrence, Myra Robinson, Michelle Wallander, Jennifer Cornette, Azizi Coleman, James Symanowski, Charity Patterson i Ifeyinwa Osunkwo. Comparing the Effects of Peer Mentoring on Care Transitions in Emerging Adults with Sickle Cell Disease: The ST3P-UP Study. Patient-Centered Outcomes Research Institute (PCORI), listopad 2024. http://dx.doi.org/10.25302/11.2024.mcsc.160835861.

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