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[Extramedullary hematopoiesis, a rare complication of sickle cell disease: A six-case series and literature review].
Rev Med Interne
January 2025
Service de médecine interne, centre national de référence des syndromes drépanocytaires majeurs de l'adulte, hôpital européen Georges-Pompidou, AP-HP, 20, rue Leblanc, 75015 Paris, France; Inserm U1163, laboratoire « Mécanismes cellulaires et moléculaires des désordres hématologiques et implications thérapeutiques », institut Imagine, université Paris-Cité, 75015 Paris, France; Laboratoire d'Excellence GR-Ex, 75015 Paris, France; Faculté de santé, université Paris-Cité, 75006 Paris, France. Electronic address:
Introduction: Extramedullary hematopoiesis (EMH) is very rarely described during sickle cell disease (SCD). Our aim was to describe six cases of EMH occurring in adult SCD patients and to conduct a literature review.
Methods: Retrospective, descriptive, and monocentric study, identifying all cases of EMH recorded in our cohort of adult SCD patients, up to April 2024.
Effectiveness of implementation of sickle cell disease referral guidelines and other measures in paediatric department at a tertiary hospital in Saudi Arabia.
BMJ Open Qual
January 2025
Pediatric Hematology Oncology, Al Hada Armed Forces Hospital, Taif, Makkah, Saudi Arabia.
Background: Sickle cell disease (SCD) is an autosomal recessive genetic blood disorder. It affects up to 2.6% of the Kingdom of Saudi Arabia population.
View Article and Find Full Text PDFSickle Lung Disease Long-Term Consequences and Prevention.
Pediatr Pulmonol
January 2025
Department of Child Health, School of Medical Science, Kwame Nkrumah University of Science and Technology, Kumasi, Ashanti Region, Ghana.
Sickle cell disease (SCD) is of global significance due to its severity and occurrence worldwide. Inheritance of the abnormal hemoglobin structure contributes to microvascular events that underlie the development of the multi-systemic complications seen in the disease pathogenesis. Pulmonary complications are common and heterogeneous including pulmonary hypertension, sleep-disordered breathing and lung function abnormalities.
View Article and Find Full Text PDFMissing the mark(ers): circulating endothelial cells and endothelial-derived extracellular vesicles are elevated in sickle cell disease plasma.
Front Immunol
January 2025
Department of Medicine, Division of Hematology, Oncology and Transplantation, University of Minnesota, Minneapolis, MN, United States.
Sickle cell disease (SCD) is a devastating hemolytic disease, marked by recurring bouts of painful vaso-occlusion, leading to tissue damage from ischemia/reperfusion pathophysiology. Central to this process are oxidative stress, endothelial cell activation, inflammation, and vascular dysfunction. The endothelium exhibits a pro-inflammatory, pro-coagulant, and enhanced permeability phenotype.
View Article and Find Full Text PDFMetabolic stimulation improves bioenergetics and haematologic indices of circulating erythrocytes from sickle cell mice.
J Physiol
January 2025
Department of Perioperative Medicine, National Institutes of Health Clinical Center, National Institutes of Health, Bethesda, MD, USA.
Circulating mature red blood cells (RBCs) from patients and mice with sickle cell disease (SCD) abnormally retain mitochondria, a factor shown to contribute to the disease's pathobiology. To further understand the functional implications of RBC mitochondria retention in SCD, we used mitochondria inhibitors and metabolites/substrates from the tricarboxylic acid cycle, oxidative phosphorylation and glycolysis pathways (ADP, glutamate, malate, pyruvate, succinate or all metabolites combined) and examined RBC bioenergetics, reactive oxygen species (ROS) levels, calcium flux and hydration. In RBCs from sickle mice, mitochondria inhibition reduced ATP levels by 30%-60%, whereas control RBCs were unaffected.
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