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https://www.readbyqxmd.com/read/28821470/design-of-the-drepagreffe-trial-a-prospective-controlled-multicenter-study-evaluating-the-benefit-of-genoidentical-hematopoietic-stem-cell-transplantation-over-chronic-transfusion-in-sickle-cell-anemia-children-detected-to-be-at-risk-of-stroke-by-transcranial
#1
Sylvie Chevret, Suzanne Verlhac, Elisabeth Ducros-Miralles, Jean-Hugues Dalle, Regis Peffault de Latour, Mariane de Montalembert, Malika Benkerrou, Corinne Pondarré, Isabelle Thuret, Corinne Guitton, Emmanuelle Lesprit, Maryse Etienne-Julan, Gisèle Elana, Jean-Pierre Vannier, Patrick Lutz, Bénédicte Neven, Claire Galambrun, Catherine Paillard, Camille Runel, Charlotte Jubert, Cécile Arnaud, Annie Kamdem, Valentine Brousse, Florence Missud, Marie Petras, Lydia Doumdo-Divialle, Claire Berger, Françoise Fréard, Olivier Taieb, Elise Drain, Monique Elmaleh, Manuela Vasile, Yacine Khelif, Myriam Bernaudin, Philippe Chadebech, France Pirenne, Gérard Socié, Françoise Bernaudin
BACKGROUND: Children with sickle cell anemia (SCA) have an 11% risk of stroke by the age of 18. Chronic transfusion applied in patients detected to be at risk by transcranial Doppler allows a significant reduction of stroke risk. However, chronic transfusion exposes to several adverse events, including alloimmunization and iron overload, and is not curative. Hematopoietic stem cell transplantation allows termination of the transfusion program, but its benefit has not been demonstrated...
November 2017: Contemporary Clinical Trials
https://www.readbyqxmd.com/read/28759962/effects-of-hydroxyurea-on-blood-rheology-in-sickle-cell-anemia-a-two-years-follow-up-study
#2
Nathalie Lemonne, Berenike Möckesch, Keyne Charlot, Yohann Garnier, Xavier Waltz, Yann Lamarre, Sophie Antoine-Jonville, Maryse Etienne-Julan, Marie-Dominique Hardy-Dessources, Marc Romana, Philippe Connes
The aim of the present study was to test the effects of hydroxyurea (HU) therapy on clinical, hematological and hemorheological parameters in adult patients with sickle cell anemia (SCA). Hematological and hemorheological parameters were measured in 28 SCA patients before HU therapy (i.e., baseline) and at 6, 12 and 24 months of treatment. RBC deformability was determined by ektacytometry at 30 Pa. RBC aggregation properties were investigated by light-backscatter method. Blood viscosity was measured at 225 s-1 by a cone-plate viscometer...
July 25, 2017: Clinical Hemorheology and Microcirculation
https://www.readbyqxmd.com/read/28489923/differences-of-microparticle-patterns-between-sickle-cell-anemia-and-hemoglobin-sc-patients
#3
Yohann Garnier, Séverine Ferdinand, Maryse Etienne-Julan, Gisèle Elana, Marie Petras, Lydia Doumdo, Benoit Tressières, Marie-Laure Lalanne-Mistrih, Marie-Dominique Hardy-Dessources, Philippe Connes, Marc Romana
Sickle cell anemia (SCA) and hemoglobin SC (HbSC) disease are the two most common forms of sickle cell disease (SCD), a frequent hemoglobinopathy which exhibits a highly variable clinical course. Although high levels of microparticles (MPs) have been consistently reported in SCA and evidence of their harmful impact on the SCA complication occurrences have been provided, no data on MP pattern in HbSC patients has been reported so far. In this study, we determined and compared the MP patterns of 84 HbSC and 96 SCA children, all at steady-state, using flow cytometry...
2017: PloS One
https://www.readbyqxmd.com/read/28466542/association-between-oxidative-stress-and-vascular-reactivity-in-children-with-sickle-cell-anaemia-and-sickle-haemoglobin-c-disease
#4
Berenike Möckesch, Philippe Connes, Keyne Charlot, Sarah Skinner, Marie-Dominique Hardy-Dessources, Marc Romana, Stéphane Jumet, Marie Petras, Lydia Divialle-Doumdo, Cyril Martin, Benoît Tressières, Vanessa Tarer, Olivier Hue, Maryse Etienne-Julan, Sophie Antoine, Vincent Pialoux
Oxidative stress and haemolysis-associated nitric oxide (NO) depletion plays a crucial role in the development of vasculopathy in sickle cell anaemia (SS). However it remains unknown whether oxidative stress and haemolysis levels influence vascular function in patients with sickle haemoglobin C disease (SC). Microvascular response to heat (using Laser Doppler flowmetry on finger), oxidative stress biomarkers, NO metabolites, endothelin-1 and haematological parameters were compared between patients with SS and SC...
August 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/28411485/cerebral-and-muscle-microvascular-oxygenation-in-children-with-sickle-cell-disease-influence-of-hematology-hemorheology-and-vasomotion
#5
Keyne Charlot, Sophie Antoine-Jonville, Berenike Moeckesch, Stéphane Jumet, Marc Romana, Xavier Waltz, Lydia Divialle-Doumdo, Marie-Dominique Hardy-Dessources, Marie Petras, Benoît Tressières, Vanessa Tarer, Olivier Hue, Maryse Etienne-Julan, Philippe Connes
The present study investigated cerebral and muscle hemoglobin oxygen saturation (tissue oxygen index, TOI) in children with sickle cell anemia (SS), sickle cell hemoglobin C disease (SC) and healthy children (AA). TOI was measured by near-infrared spectroscopy (NIRS) and spectral analysis of the TOI variability was used to assess flowmotion and vasomotion. Arterial oxyhemoglobin saturation (SpO2), hemorheological and hematological parameters were also measured in SS and SC children. Both TOI were lower in SS compared to both AA and SC children, with SC exhibiting lower values than AA children...
June 2017: Blood Cells, Molecules & Diseases
https://www.readbyqxmd.com/read/28340403/micro-and-macrovascular-function-in-children-with-sickle-cell-anaemia-and-sickle-cell-haemoglobin-c-disease
#6
Berenike Möckesch, Keyne Charlot, Stéphane Jumet, Marc Romana, Lydia Divialle-Doumdo, Marie-Dominique Hardy-Dessources, Marie Petras, Benoît Tressieres, Vanessa Tarer, Olivier Hue, Maryse Etienne-Julan, Philippe Connes, Sophie Antoine-Jonville
It is unclear whether vascular function is affected similarly in children with sickle cell anaemia (SS) and children with sickle haemoglobin C (SC) disease. Therefore, we compared micro and macrovascular functions in healthy (AA) children, children with SS and SC disease, and assessed their association with physical activity. Participants (24 SS, 22 SC and 16 AA), were compared in terms of 1) thermal hyperaemic response (finger pad warming to 42°C) measured by Laser Doppler techniques, 2) arterial stiffness determined by pulse wave velocity, 3) daily energy expenditure related to moderate and intense physical activities estimated by questionnaire and 4) fitness level, evaluated by the six-minute walk test...
February 4, 2017: Blood Cells, Molecules & Diseases
https://www.readbyqxmd.com/read/28243984/modern-technology-homogenizes-enological-traits-of-indigenous-saccharomyces-cerevisiae-strains-associated-with-msalais-a-traditional-wine-in-china
#7
Lixia Zhu, Julan Xue
In this study, we performed a pilot-scale evaluation of the enological characteristics of indigenous Saccharomyces cerevisiae strains associated with Msalais, a traditional Chinese wine produced by a unique technology of boiling grape juice prior to spontaneous fermentation. Technical and sensory characteristics of top ten indigenous strains previously identified by us by screening a collection of 436 indigenous S. cerevisiae strains (Zhu et al. 2016) were assayed in a traditional craft workshop (TCW) and a modern plant (MP)...
March 2017: World Journal of Microbiology & Biotechnology
https://www.readbyqxmd.com/read/28097791/alpha-thalassaemia-promotes-frequent-vaso-occlusive-crises-in-children-with-sickle-cell-anaemia-through-haemorheological-changes
#8
Céline Renoux, Philippe Connes, Elie Nader, Sarah Skinner, Camille Faes, Marie Petras, Yves Bertrand, Nathalie Garnier, Daniela Cuzzubbo, Lydia Divialle-Doumdo, Kamila Kebaïli, Cécile Renard, Alexandra Gauthier, Maryse Etienne-Julan, Giovanna Cannas, Cyril Martin, Marie-Dominique Hardy-Dessources, Vincent Pialoux, Marc Romana, Philippe Joly
BACKGROUND: Sickle cell anaemia (SCA) is a severe hereditary haemoglobinopathy characterised by haemorheological abnormalities, which play a role in the occurrence of several acute and chronic clinical complications. While β(S) -haplotypes and alpha-thalassaemia modulate SCA clinical severity, their effects on blood rheology have been incompletely described. The aim of this study was to test the effects of these genetic modifiers on the haemorheological properties and clinical complication of children with SCA...
August 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/27984639/exacerbation-of-oxidative-stress-during-sickle-vaso-occlusive-crisis-is-associated-with-decreased-anti-band-3-autoantibodies-rate-and-increased-red-blood-cell-derived-microparticle-level-a-prospective-study
#9
Régine Hierso, Nathalie Lemonne, Rinaldo Villaescusa, Marie-Laure Lalanne-Mistrih, Keyne Charlot, Maryse Etienne-Julan, Benoit Tressières, Yann Lamarre, Vanessa Tarer, Yohann Garnier, Ada Arce Hernandez, Serge Ferracci, Philippe Connes, Marc Romana, Marie-Dominique Hardy-Dessources
Painful vaso-occlusive crisis, a hallmark of sickle cell anaemia, results from complex, incompletely understood mechanisms. Red blood cell (RBC) damage caused by continuous endogenous and exogenous oxidative stress may precipitate the occurrence of vaso-occlusive crises. In order to gain insight into the relevance of oxidative stress in vaso-occlusive crisis occurrence, we prospectively compared the expression levels of various oxidative markers in 32 adults with sickle cell anaemia during vaso-occlusive crisis and steady-state conditions...
March 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/27969229/dr%C3%A3-panocytose-et-priapisme%C3%A2-r%C3%A3-le-des-param%C3%A3-tres-h%C3%A3-matologiques-et-h%C3%A3-morh%C3%A3-ologiques
#10
L Brureau, K Cita, N Lemonne, M Billaud, P Connes, S Ferdinand, B Tressieres, V Tarer, M Etienne-Julan, P Blanchet, J Elion, M Romana
No abstract text is available yet for this article.
November 2016: Progrès en Urologie
https://www.readbyqxmd.com/read/27355589/effect-of-age-on-blood-rheology-in-sickle-cell-anaemia-and-sickle-cell-haemoglobin-c-disease-a-cross-sectional-study
#11
Céline Renoux, Marc Romana, Philippe Joly, Séverine Ferdinand, Camille Faes, Nathalie Lemonne, Sarah Skinner, Nathalie Garnier, Maryse Etienne-Julan, Yves Bertrand, Marie Petras, Giovanna Cannas, Lydia Divialle-Doumdo, Elie Nader, Daniela Cuzzubbo, Yann Lamarre, Alexandra Gauthier, Xavier Waltz, Kamila Kebaili, Cyril Martin, Arnaud Hot, Marie-Dominique Hardy-Dessources, Vincent Pialoux, Philippe Connes
OBJECTIVES: Blood rheology plays a key role in the pathophysiology of sickle cell anaemia (SS) and sickle cell haemoglobin C disease (SC), but its evolution over the lifespan is unknown. MATERIALS AND METHODS: Blood viscosity, red blood cell (RBC) deformability and aggregation, foetal haemoglobin (HbF) and haematocrit were measured in 114 healthy individuals (AA), 267 SS (161 children + 106 adults) and 138 SC (74 children + 64 adults) patients. RESULTS: Our results showed that 1) RBC deformability is at its maximal value during the early years of life in SS and SC populations, mainly because HbF level is also at its peak, 2) during childhood and adulthood, hydroxycarbamide treatment, HbF level and gender modulated RBC deformability in SS patients, independently of age, 3) blood viscosity is higher in older SS and SC patients compared to younger ones and 4) haematocrit decreases as SS patients age...
2016: PloS One
https://www.readbyqxmd.com/read/27145183/men-with-sickle-cell-anemia-and-priapism-exhibit-increased-hemolytic-rate-decreased-red-blood-cell-deformability-and-increased-red-blood-cell-aggregate-strength
#12
Kizzy-Clara Cita, Laurent Brureau, Nathalie Lemonne, Marie Billaud, Philippe Connes, Séverine Ferdinand, Benoit Tressières, Vanessa Tarer, Maryse Etienne-Julan, Pascal Blanchet, Jacques Elion, Marc Romana
OBJECTIVES: To investigate the association between priapism in men with sickle cell anemia (SCA) and hemorheological and hemolytical parameters. MATERIALS AND METHODS: Fifty-eight men with SCA (median age: 38 years) were included; 28 who had experienced priapism at least once during their life (priapism group) and 30 who never experienced this complication (control group). Twenty-two patients were treated with hydroxycarbamide, 11 in each group. All patients were at steady state at the time of inclusion...
2016: PloS One
https://www.readbyqxmd.com/read/27100428/serum-interleukin-il-9-and-il-10-but-not-t-helper-9-th9-cells-are-associated-with-survival-of-patients-with-acute-on-chronic-hepatitis-b-liver-failure
#13
Xueping Yu, Yijuan Zheng, Yong Deng, Julan Li, Ruyi Guo, Milong Su, Desong Ming, Zhenzhong Lin, Jiming Zhang, Zhijun Su
CD4 T helper (Th) cells are reported to be essential for initiating and maintaining an effective immune response to hepatitis B virus (HBV) infection. Th9 cells are a new subset of CD4 Th cells that produce interleukin (IL)-9 and IL-10. The present study aimed to investigate the percentage of Th9 cells relative to the number of CD4 cells in peripheral blood. We also measured serum IL-9 and IL-10 levels in different stages of HBV infection and their relationship with progress and prognosis of liver disease. Whole blood samples from 111 patients with HBV infection, including 39 chronic hepatitis B (CHB), 25 HBV-liver cirrhosis (HBV-LC), 21 acute-on-chronic liver failure (ACLF) patients, and 26 healthy controls were collected...
April 2016: Medicine (Baltimore)
https://www.readbyqxmd.com/read/27067484/association-of-adenylyl-cyclase-6-rs3730070-polymorphism-and-hemolytic-level-in-patients-with-sickle-cell-anemia
#14
Kizzy-Clara Cita, Séverine Ferdinand, Philippe Connes, Laura Brudey, Benoit Tressières, Maryse Etienne-Julan, Nathalie Lemonne, Vanessa Tarer, Jacques Elion, Marc Romana
A recent study suggested that adenosine signaling pathway could promote hemolysis in patients with sickle cell anemia (SCA). This signaling pathway involves several gene coding enzymes for which variants have been described. In this study, we analyzed the genotype-phenotype relationships between functional polymorphisms or polymorphisms associated with altered expression of adenosine pathway genes, namely adenosine deaminase (ada; rs73598374), adenosine A2b receptor (adora2b; rs7208480), adenylyl cyclase6 (adcy6; rs3730071, rs3730070, rs7300155), and hemolytic rate in SCA patients...
May 2016: Blood Cells, Molecules & Diseases
https://www.readbyqxmd.com/read/27004270/four-members-of-heat-shock-protein-70-family-in-korean-rose-bitterling-rhodeus-uyekii
#15
Jung Hyun Kim, Chun Mae Dong, Julan Kim, Cheul Min An, Hae Ja Baek, Hee Jeong Kong
Heat shock protein (HSP) 70, the highly conserved stress protein families, plays important roles in protecting cells against heat and other stresses in most animal species. In the present study, we identified and characterized four Hsp70 (RuHSP4, RuHSC70, RuHSP12A, RuGRP78) family proteins based on the expressed sequence tag (EST) analysis of the Korean rose bitterling R. uyekii cDNA library. The deduced RuHSP70 family has high amino acid identities of 72-99% with those of other species. Phylogenetic analysis revealed that RuHsp70 family clustered with fish groups (HSP4, HSC70, HSP12A, GRP78) proteins...
September 2015: Balsaeng'gwa Saengsig
https://www.readbyqxmd.com/read/26999505/carest-multilingual-regional-integration-for-health-promotion-and-research-on-sickle-cell-disease-and-thalassemia
#16
Jennifer Knight-Madden, Marc Romana, Rinaldo Villaescusa, Marvin Reid, Maryse Etienne-Julan, Laurence Boutin, Gisèle Elana, Narcisse Elenga, Gillian Wheeler, Ketty Lee, Rosa Nieves, Althea Jones Lecointe, Marie-Laure Lalanne-Mistrih, Gylna Loko, Lisiane Keclard-Christophe, Marie-Dominique Hardy-Dessources
Sickle cell disease (SCD) is a significant problem in the Caribbean, where many individuals have African and Asian forebears. However, reliable prevalence data and specific health care programs for SCD are often missing in this region. Closer collaboration between Caribbean territories initiated in 2006 to set up strategies to promote better equity in the health care system for SCD patients led to the formation of CAREST: the Caribbean Network of Researchers on Sickle Cell Disease and Thalassemia. We present the effectiveness of collaborations established by CAREST to promote SCD newborn screening programs and early childhood care, to facilitate health worker training and approaches for prevention and treatment of SCD complications, and to carry out inter-Caribbean research studies...
May 2016: American Journal of Public Health
https://www.readbyqxmd.com/read/26856407/kinetic-properties-and-substrate-inhibition-of-%C3%AE-galactosidase-from-aspergillus-niger
#17
Julan Liao, Masayuki Okuyama, Keigo Ishihara, Yoshinori Yamori, Shigeo Iki, Takayoshi Tagami, Haruhide Mori, Seiya Chiba, Atsuo Kimura
The recombinant AglB produced by Pichia pastoris exhibited substrate inhibition behavior for the hydrolysis of p-nitrophenyl α-galactoside, whereas it hydrolyzed the natural substrates, including galactomanno-oligosaccharides and raffinose family oligosaccharides, according to the Michaelian kinetics. These contrasting kinetic behaviors can be attributed to the difference in the dissociation constant of second substrate from the enzyme and/or to the ability of the leaving group of the substrates. The enzyme displays the grater kcat/Km values for hydrolysis of the branched α-galactoside in galactomanno-oligosaccharides than that of raffinose and stachyose...
September 2016: Bioscience, Biotechnology, and Biochemistry
https://www.readbyqxmd.com/read/26631808/microarray-analysis-of-gene-expression-in-olive-flounder-liver-infected-with-viral-haemorrhagic-septicaemia-virus-vhsv
#18
Hyun Kook Cho, Julan Kim, Ji Young Moon, Bo-Hye Nam, Young-Ok Kim, Woo-Jin Kim, Jung Youn Park, Cheul Min An, Jaehun Cheong, Hee Jeong Kong
The most fatal viral pathogen in olive flounder Paralichthys olivaceus, is viral hemorrhagic septicemia virus, which afflicts over 48 species of freshwater and marine fish. Here, we performed gene expression profiling on transcripts isolated from VHSV-infected olive flounder livers using a 13 K cDNA microarray chip. A total of 1832 and 1647 genes were upregulated and down-regulated over two-fold, respectively, after infection. A variety of immune-related genes showing significant changes in gene expression were identified in upregulated genes through gene ontology annotation...
February 2016: Fish & Shellfish Immunology
https://www.readbyqxmd.com/read/26603723/which-side-of-the-balance-determines-the-frequency-of-vaso-occlusive-crises-in-children-with-sickle-cell-anemia-blood-viscosity-or-microvascular-dysfunction
#19
Keyne Charlot, Marc Romana, Berenike Moeckesch, Stéphane Jumet, Xavier Waltz, Lydia Divialle-Doumdo, Marie-Dominique Hardy-Dessources, Marie Petras, Benoît Tressières, Vanessa Tarer, Olivier Hue, Maryse Etienne-Julan, Sophie Antoine-Jonville, Philippe Connes
Vascular resistance and tissue perfusion may be both affected by impaired vascular function and increased blood viscosity. Little is known about the effects of vascular function on the occurrence of painful vaso-occlusive crises (VOC) in children with sickle cell anemia (SCA). The aim of the present study was to determine which side of the balance (blood viscosity or vascular function) is the most deleterious in SCA and increases the risk for frequent hospitalized VOC. Microvascular function, microcirculatory oxygenation and blood viscosity were determined in a group of 22 SCA children/adolescents at steady state and a group of 13 healthy children/adolescents...
January 2016: Blood Cells, Molecules & Diseases
https://www.readbyqxmd.com/read/26287186/molecular-and-functional-characterization-of-thioredoxin-1-from-korean-rose-bitterling-rhodeus-uyekii
#20
Julan Kim, Ji Young Moon, Woo-Jin Kim, Dong-Gyun Kim, Bo-Hye Nam, Young-Ok Kim, Jung Youn Park, Cheul Min An, Hee Jeong Kong
Thioredoxin is a multifunctional antioxidant enzyme that belongs to the reductase family. In this study, we cloned and characterized thioredoxin 1 cDNA from the Korean rose bitterling Rhodeus uyekii (RuTrx). The full-length RuTrx cDNA consists of 674 bp with a 324 nt open reading frame (ORF) encoding a 107 aa protein. The deduced RuTrx amino acid sequence indicated a characteristic redox active site, (31)WCGPC(35). Pairwise alignment revealed RuTrx amino acid identity (55.1%-83.2%) with orthologs from various species of mammalia, amphibia, fish and bird...
2015: International Journal of Molecular Sciences
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