keyword
https://read.qxmd.com/read/38497419/transfusing-children-with-sickle-cell-disease-using-blood-group-genotyping-when-the-pool-of-black-donors-is-limited
#1
JOURNAL ARTICLE
Gabriel André Leiva-Torres, Maude Cigna, Jessica Constanzo-Yanez, Maryse St-Louis, Josée Perreault, Josée Lavoie, Geneviève Laflamme, Antoine Lewin, Yves Pastore, Nancy Robitaille
BACKGROUND: Red blood cell transfusion is an effective treatment for patients with sickle cell disease (SCD). Alloimmunization can occur after a single transfusion, limiting further usage of blood transfusion. It is recommended to match for the ABO, D, C, E, and K antigens to reduce risks of alloimmunization. However, availability of compatible blood units can be challenging for blood providers with a limited number of Black donors. STUDY DESIGN AND METHODS: A prospective cohort of 205 pediatric patients with SCD was genotyped for the RH and FY genes...
March 18, 2024: Transfusion
https://read.qxmd.com/read/38497171/stroke-without-cerebral-arteriopathy-in-sickle-cell-disease-children-causes-and-treatment
#2
JOURNAL ARTICLE
Sarah Liane Linguet, Suzanne Verlhac, Florence Missud, Laurent Holvoet-Vermaut, Valentine Brousse, Ghislaine Ithier, Alexandra Ntorkou, Emmanuelle Lesprit, Malika Benkerrou, Manoëlle Kossorotoff, Berengere Koehl
Cerebral arteriopathy (CA) in children with sickle cell disease (SCD) is classically described as chronic stenosis of arteries in the anterior brain circulation, leading to ischemic stroke. Some studies have however reported strokes in children with SCD but without CA. In order to better understand the etiology and risk factors of these strokes, we retrospectively analyzed ischemic strokes occurring in a large cohort of children over a 13 year-period. Between 2007 and 2020, 25/1500 children with SCD had an ischemic stroke in our center...
March 14, 2024: Haematologica
https://read.qxmd.com/read/38496096/the-influence-of-abstinence-interval-on-semen-quality-and-its-effect-on-an-assisted-reproductive-technology-art-pregnancy-a-case-report
#3
Rokaiya Shaikh, Akash More, Shilpa Dutta, Namrata Choudhary, Shivani Khemani
This case report focuses on a couple facing primary infertility, where the male partner exhibited asthenoteratozoospermia and high DNA fragmentation. The treatment approach involved three cycles of intracytoplasmic sperm injection (ICSI), an assisted reproductive technology (ART), to indicate and address the specific challenges posed by male factor infertility. The initial two attempts failed as DNA fragmentation was high, which was observed on days 4 and 3 of abstinence, respectively. In the third cycle, DNA fragmentation was low on day 2 of the abstinence period, resulting in the successful formation and cryopreservation of embryos...
February 2024: Curēus
https://read.qxmd.com/read/38494406/sickle-cell-disease-and-increased-adverse-maternal-and-perinatal-outcomes-in-different-genotypes
#4
JOURNAL ARTICLE
Camilla Olivares Figueira, José Paulo S Guida, Fernanda G Surita, Arthur Antolini-Tavares, Sara T Saad, Fernando F Costa, Kleber Y Fertrin, Maria Laura Costa
BACKGROUND: Sickle cell disease (SCD) comprises a heterogeneous group of inherited hemolytic disorders that increases the risk of maternal and perinatal complications due to chronic systemic inflammatory response, endothelial damage and vaso-occlusion. The contribution of genotypes to the severity of outcomes during pregnancy is not completely established. METHODS: A retrospective study of medical charts was performed to compare maternal and perinatal outcomes in Hb SS, Hb SC disease and sickle-beta thalassemia (Hb Sβ) pregnancies followed at a high-risk antenatal care unit over a 6-year period...
March 14, 2024: Hematology, Transfusion and Cell Therapy
https://read.qxmd.com/read/38493007/revolutionising-healing-gene-editing-s-breakthrough-against-sickle-cell-disease
#5
REVIEW
Marija Dimitrievska, Dravie Bansal, Marta Vitale, John Strouboulis, Annarita Miccio, Kypros H Nicolaides, Sara El Hoss, Panicos Shangaris, Joanna Jacków-Malinowska
Recent advancements in gene editing illuminate new potential therapeutic approaches for Sickle Cell Disease (SCD), a debilitating monogenic disorder caused by a point mutation in the β-globin gene. Despite the availability of several FDA-approved medications for symptomatic relief, allogeneic hematopoietic stem cell transplantation (HSCT) remains the sole curative option, underscoring a persistent need for novel treatments. This review delves into the growing field of gene editing, particularly the extensive research focused on curing haemoglobinopathies like SCD...
March 7, 2024: Blood Reviews
https://read.qxmd.com/read/38491245/plant-produced-therapeutic-crizanlizumab-monoclonal-antibody-binds-p-selectin-to-alleviate-vaso-occlusive-pain-crises-in-sickle-cell-disease
#6
REVIEW
Taewon Yang, Hyunjoo Hwang, Kibum Kim, Yerin Kim, Richard D Cummings, Yong Kyoo Shin, Taejin Lee, Kisung Ko
Sickle Cell Disease (SCD) is a severe genetic disorder causing vascular occlusion and pain by upregulating the adhesion molecule P-selectin on endothelial cells and platelets. It primarily affects infants and children, causing chronic pain, circulatory problems, organ damage, and complications. Thus, effective treatment and management are crucial to reduce SCD-related risks. Anti-P-selectin antibody Crizanlizumab (Crimab) has been used to treat SCD. In this study, the heavy and light chain (HC and LC) genes of anti-P-Selectin antibody Crimab were cloned into a plant expression binary vector...
March 15, 2024: Molecular Biotechnology
https://read.qxmd.com/read/38491086/the-roxyscan-is-a-novel-measurement-of-red-blood-cell-deformability-under-oxidative-and-shear-stress
#7
JOURNAL ARTICLE
Sandra K Larkin, Carolina Hernández, Eduard J van Beers, Richard van Wijk, Frans A Kuypers
Exposure to both oxidative and shear stress, a condition that the red blood cell (RBC) continuously experiences in the circulation in vivo can be mimicked in a Couette type viscometer and monitored by ektacytometry. RBCs maintain their deformation and orientation under shear stress and oxidative stress until a threshold is reached at which these conditions appear to overwhelm the elaborate and complex pathways that maintain a proper redox environment in the cell. Oxidative stress under shear alters the ability of the cell to deform, changes cell morphology, its orientation in the shear stress field, and appears to alter intracellular and membrane characteristics...
March 15, 2024: Scientific Reports
https://read.qxmd.com/read/38490797/association-between-social-capital-and-quality-of-life-in-older-adults-with-subjective-cognitive-decline-a-cross-sectional-study
#8
JOURNAL ARTICLE
Yiping Chen, Wei Li, Xin Zhang, Hui Cheng, Yuling Tian, Hui Yang
BACKGROUND: Subjective cognitive decline (SCD) is increasingly recognized as a clinical and medical risk factor for mild cognitive impairment (MCI) and dementia. Currently, there is little evidence regarding the quality of life (QoL) in older adults with SCD and the impact of social capital on their QoL. AIMS: To examine the perceptions of social capital and QoL among older adults with SCD. METHODS: A total of 325 participants (92.9 % response rate) with a self-reported diagnosis of SCD completed the Chinese version of the 36-item Short-Form Health Survey, the Chinese Shortened Social Capital Scale and the Generalized Anxiety Disorder Scale...
February 2024: Applied Nursing Research: ANR
https://read.qxmd.com/read/38488719/the-relationship-between-cumulative-risk-and-health-related-quality-of-life-in-youth-with-sickle-cell-disease-moderating-effects-of-secondary-control-engagement-coping
#9
JOURNAL ARTICLE
Elise M Belkin, Natalie Koskela-Staples, Elise Turner, L Vandy Black, David A Fedele
BACKGROUND: Youth with sickle cell disease (SCD) often experience low health-related quality of life (HRQOL). Engagement in resilience-promoting processes, such as secondary control engagement (SCE) coping, or adapting to stressors, may be linked to contextual risk factors (e.g., poverty status). This study aims to illuminate relationships between a cumulative risk index (CRI), SCE coping, and HRQOL in youth with SCD and test whether SCE coping moderates the relationship between CRI and HRQOL...
March 15, 2024: Pediatric Blood & Cancer
https://read.qxmd.com/read/38488686/favorable-outcome-of-non-myeloablative-allogeneic-transplantation-in-adult-patients-with-severe-sickle-cell-disease-a-single-center-experience-of-200-patients
#10
JOURNAL ARTICLE
Moussab Damlaj, Bader Alahmari, Ahmed Alaskar, Ayman Alhejazi, Husam Alsadi, Mazin Ahmed, Tahani Alanazi, Rasha Ahmed, Amani Alharbi, Inaam Shehabeddine, Afnan Alzaidi, Suha Alkhuraisat, Isam Mahassnah, Hamza Alquraan, Maybelle Ballili, Mohsen Alzahrani
Allogeneic hematopoietic stem cell transplant (HSCT) for adults with severe sickle cell disease (SCD) is potentially curative but not commonly utilized therapy due to complications such as graft failure (GF) and organ toxicity. Herein, we are reporting our long-term outcome data of non-myeloablative (NMA) HSCT in adults with severe SCD with emphasis on factors predicting event free survival (EFS). Adults with severe SCD undergoing NMA match-related donor allogeneic HSCT from 2015 to 2021 with at least 12 months of follow-up were included...
March 15, 2024: American Journal of Hematology
https://read.qxmd.com/read/38487952/current-state-of-gene-therapy-in-sickle-cell-disease
#11
REVIEW
Mei San Tang, Hua Shan
Sickle cell disease (SCD) is a type of hemoglobinopathy due to an autosomal recessive genetic defect, causing significant red cell sickling, multi-organ damage and long-term severe morbidities. Due to its complicated care and the impact on quality of life, a curative treatment for SCD is highly desirable. In recent years, gene therapy is emerging as a curative option for SCD, where autologous haematopoietic stem cells are collected from SCD patients and genetically modified ex vivo to reduce its sickling tendency before reinfusion...
March 15, 2024: Vox Sanguinis
https://read.qxmd.com/read/38487039/exploring-factors-associated-with-quality-of-life-in-caregivers-of-children-and-adolescents-with-sickle-cell-disease-and-hiv-a-comparative-analysis
#12
JOURNAL ARTICLE
Charlotte Eposse Ekoube, Dora Mbonjo Bitsie, Erero F Njiengwe, Edgar Mandeng Ma Linwa, Christian Eyoum, Ritha Mbono Betoko, Jeannette Disso Massako, Emmanuel Heles Nsang, Abba Soumaiyatou, Callixte Tegueu Kuate
INTRODUCTION: Paediatric HIV and sickle cell disease (SCD) are two stigmatising and potentially fatal illnesses that place a significant burden on families. HIV patients benefit from a longstanding free-service national programme in Cameroon, and this could considerably alleviate burden of care on HIV caregivers, possibly leading to better quality of life (QoL) in HIV caregivers compared to SCD caregivers. Our study aimed to compare the QoL between caregivers of children and adolescents with SCD and HIV and explore factors associated with this QoL in Cameroon...
2024: Anemia
https://read.qxmd.com/read/38486359/the-effects-of-exergame-telerehabilitation-in-persons-with-subjective-cognitive-decline
#13
JOURNAL ARTICLE
Dereck L Salisbury, Keenan A Pituch, Fang Yu
BACKGROUND AND OBJECTIVES: The primary aim of this Stage IB randomized controlled trial (RCT) was to test the preliminary effects of a dual-task exergaming telerehabilitation intervention on cognition and aerobic fitness, compared to aerobic exercise (AEx) only and attention control (stretching) in older adults with SCD. RESEARCH DESIGN AND METHODS: This RCT randomized 39 participants on a 2:1:1 allocation ratio to supervised exergame (n=20), AEx (n=11), and stretching (n=8) for 12 weeks...
March 15, 2024: Gerontologist
https://read.qxmd.com/read/38486114/durable-engraftment-after-pharmacological-pre-transplant-immune-suppression-followed-by-reduced-toxicity-myeloablative-haploidentical-stem-cell-transplantation-in-highly-hla-immunized-adults-with-sickle-cell-disease
#14
JOURNAL ARTICLE
Sabine Fürst, Emmanuelle Bernit, Faezeh Legrand, Angela Granata, Samia Harbi, Raynier Devillier, Valerio Maisano, Benjamin Bouchacourt, Thomas Pagliardini, Djamel Mokart, Claude Lemarié, Boris Calmels, Christophe Picard, Agnès Basire, Borje S Andersson, Didier Blaise
Allogeneic stem cell transplantation (Allo-SCT) is the only rapidly available curative treatment modality in patients with severe sickle cell disease (SCD). The development of reduced-toxicity myeloablative conditioning (RT-MAC) regimen and the use of partially matched family donors with post-transplantation cyclophosphamide (PT-Cy) have widened the access to Allo-SCT. Antibodies against donor-specific HLA (DSA) increase the risk of engraftment failure in HLA mismatched Allo-SCT. We report the results of five patients with SCD, whereas three with DSA, who underwent an unmanipulated haploidentical stem cell transplantation (Haplo-SCT) after a busulfan-based RT-MAC regimen with PT-Cy...
March 14, 2024: Bone Marrow Transplantation
https://read.qxmd.com/read/38485505/androgen-signaling-inhibits-de-novo-lipogenesis-to-alleviate-lipid-deposition-in-zebrafish
#15
JOURNAL ARTICLE
Jing-Yi Jia, Guang-Hui Chen, Ting-Ting Shu, Qi-Yong Lou, Xia Jin, Jiang-Yan He, Wu-Han Xiao, Gang Zhai, Zhan Yin
Testosterone is closely associated with lipid metabolism and known to affect body fat composition and muscle mass in males. However, the mechanisms by which testosterone acts on lipid metabolism are not yet fully understood, especially in teleosts. In this study, cyp17a1 -/- zebrafish ( Danio rerio ) exhibited excessive visceral adipose tissue (VAT), lipid content, and up-regulated expression and activity of hepatic de novo lipogenesis (DNL) enzymes. The assay for transposase accessible chromatin with sequencing (ATAC-seq) results demonstrated that chromatin accessibility of DNL genes was increased in cyp17a1 -/- fish compared to cyp17a1 +/+ male fish, including stearoyl-CoA desaturase ( scd ) and fatty acid synthase ( fasn )...
March 18, 2024: Zoological Research
https://read.qxmd.com/read/38483250/the-hypotheses-of-oral-disease-associated-perioprosthetic-joint-infections-to-understand-the-role-of-oral-health-for-endoprostheses-a-narrative-review
#16
REVIEW
Gerhard Schmalz, Dirk Ziebolz
AIMS: Periprosthetic infections are a severe complication, causing enormous morbidity. The role of oral diseases in the development of such infections has been discussed controversially, showing a large discrepancy between prevalence of oral foci and infections of endoprostheses (EP). This narrative review aimed in explaining and discussing two hypotheses for the occurrence of oral disease-associated EP infections. METHODS AND RESULTS: The hypotheses include two mechanisms...
2024: Special Care in Dentistry
https://read.qxmd.com/read/38483203/survey-on-the-attitudes-and-perceptions-of-swiss-dental-practitioners-and-dental-students-toward-treating-patients-with-disabilities-and-sensory-impairments
#17
JOURNAL ARTICLE
Lisa Takeshita, Murali Srinivasan
AIMS: This study evaluated the attitudes and perceptions of private dental practitioners (PPs) and students on treating patients with disabilities and sensory impairments. METHODS AND RESULTS: A multifaceted questionnaire was used to assess the participants' attitudes on providing care to persons with disabilities and sensory impairments [visual (VI) and hearing (HI)]. The survey evaluated different aspects of the participants' attitudes that included integrity, altruism, perceived barriers, and theory of planned behavior (TPB)...
March 14, 2024: Special Care in Dentistry
https://read.qxmd.com/read/38481370/bone-pattern-changes-in-post-liver-transplant-patients-using-bisphosphonates
#18
JOURNAL ARTICLE
Marcus Vinícius Bueno, Luciana Munhoz, Karem L Ortega, Maria Paula Siqueira de Melo Peres, Juliana Bertoldi Franco
OBJECTIVE: To identify radiographic findings suggestive of drug-induced osteonecrosis and evaluate radiomorphometric patterns indicative of changes in bone mineral density in individuals transplanted for liver disorders using bisphosphonates. STUDY DESIGN: The study group included panoramic x-rays of liver transplant patients who are being monitored and who present a clinical status of osteoporosis and use bisphosphonates. The control group was made up of liver transplant patients who did not have osteoporosis...
March 14, 2024: Special Care in Dentistry
https://read.qxmd.com/read/38481237/effects-of-antioxidant-vitamins-a-d-e-and-trace-elements-cu-mn-se-zn-administration-on-gene-expression-metabolic-antioxidants-and-immunological-profiles-during-transition-period-in-dromedary-camels
#19
JOURNAL ARTICLE
Ahmed El-Sayed, Eman Ebissy, Ragab Mohamed, Ahmed Ateya
BACKGROUND: Nutrition has a primary role for optimum expression of genetic potential, and most of the farmers have limited resources of green fodder. Hence, a fat-soluble vitamin, especially vitamin A and E and trace elements remained most critical in the animal's ration and affects their productive and reproductive performance adversely. Animals cannot be able to produce these vitamins in their bodies; hence, an exogenous regular supply is needed to fulfil the physiological needs and to maintain high production performance...
March 13, 2024: BMC Veterinary Research
https://read.qxmd.com/read/38480484/effectiveness-of-oral-health-interventions-among-children-and-adolescents-with-mental-disorders-a-systematic-review
#20
REVIEW
Noor Baiti Bab, Raja Nur Adilah Raja Abdul Rahman, Salina Mohamed, Nawwal Alwani Mohd Radzi, Norashikin Yusof
BACKGROUND: The rising percentage of children and adolescents experiencing mental disorders brought attention to the emerging opportunities for proactive oral health interventions in this population. Currently, existing guidelines focus mainly on oral health in general practice and on adults residing in care homes. This report aims to provide a broad overview of the effectiveness of oral health interventions for children and adolescents with mental disorders. METHOD: This review followed the PRISMA (Preferred Reporting Items for Systematic Review and Meta-Analyses 2020) statement...
March 13, 2024: Special Care in Dentistry
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