Sickle hemoglobin (Hb S; beta Glu6Val) is due to an A
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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.
Sickle 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 PDFPrevalence and Spectrum of β-Thalassemia Mutations in Baghdad, Iraq: Data from the Premarital Screening Program.
Hemoglobin
January 2025
Department of Pathology, School of Medicine, University of Duhok, Duhok, Iraq.
The knowledge of the prevalence and molecular basis of β-hemoglobinopathies constitutes an important prerequisite for an effective prevention program. To address this issue in Iraq's capital, Baghdad, a total of 12526 individuals (6263 couples) attending three main Premarital Screening centers were enrolled. Individuals were labeled as β-hemoglobin disorders based on full blood counts and high-performance liquid chromatography.
View Article and Find Full Text PDFArtificial Oxygen Carriers Save Lives in Expanded Access Use: Three Cases when Blood Was Not an Option.
Am J Ther
January 2025
Anesthesiology and Perioperative Medicine, David Geffen School of Medicine at UCLA, Los Angeles, CA.
Clinical Features: Sickle cell patients may develop a multitude of antibodies and experience life-threatening events with transfusion such as hyperhemolysis syndrome or delayed hemolytic transfusion reaction. Further transfusion may not be possible in such cases.
Therapeutic Challenge: When conventional blood products are not available for transfusion yet the patient requires additional oxygen-carrying support, artificial oxygen carriers may be required.
Knowledge of Sickle Cell Disease, Awareness of Sickle Cell Trait Status and Its Impact on Relationships Among Students at a Historically Black College.
Hemoglobin
January 2025
Howard University College of Pharmacy, Washington, DC, USA.
Sickle cell disease (SCD) primarily affects people of African American descent in the United States. Many individuals do not know their sickle cell trait (SCT) status or might not be aware of SCD. The purpose of this study was to assess SCD knowledge, awareness of SCT status, and its impact on relationships.
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