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Correlation study between magnetic resonance imaging-quantitated cardiac iron deposition and left ventricular function in patients with β-thalassemia major in China.
Sci Prog
January 2025
Department of Radiology, The First Affiliated Hospital of Guangxi Medical University, Nanning, Guangxi Zhuang Autonomous Region, People's Republic of China.
Objective: To explore the correlation between cardiac iron deposition and left ventricular function indicators, such as left ventricular end-diastolic volume index, left ventricular end-systolic volume index, and left ventricular ejection fraction, and to evaluate the accuracy of predicting patients with cardiac iron deposition by using left ventricular ejection fraction as an index.
Methods: This quantitative cross-sectional study involved one hundred and fifty transfusion-dependent patients with β-thalassemia major who were evaluated by magnetic resonance imaging to obtain * values, left ventricular end-diastolic volume index, left ventricular end-systolic volume index, and left ventricular ejection fraction. The relationship between cardiac * values and left ventricular end-diastolic volume index, left ventricular end-systolic volume index, and left ventricular ejection fraction was analyzed.
[A CRISPR/Cas approach to β-haemoglobinopathies].
Med Sci (Paris)
January 2025
Institut Imagine, Inserm UMR1163, université Paris Cité, Paris, France.
Beta-haemoglobinopathies are severe genetic anemias caused by mutations that affect adult haemoglobin production. Many therapeutic approaches aim to reactivate the expression of the fetal hemoglobin genes. To this end, the CRISPR/Cas9 system has recently been used to genetically modify patients' hematopoietic stem/progenitor cells ex vivo and reactivate fetal hemoglobin expression in their erythroid progeny.
View Article and Find Full Text PDFEpstein-Barr Virus-Induced Hemophagocytic Lymphohistiocytosis: A Case Report of a Rare and Life-Threatening Syndrome.
Cureus
December 2024
Internal Medicine Department, Hamad Medical Corporation, Doha, QAT.
Hemophagocytic lymphohistiocytosis (HLH) is a rare, life-threatening syndrome of excessive immune activation, leading to severe inflammation and organ damage. While more common in infants, HLH can occur at any age and is often triggered by infections such as Epstein-Barr virus (EBV). In this case, a 38-year-old man presented with a three-week history of fevers, night sweats, poor appetite, and severe anemia.
View Article and Find Full Text PDFCongenital Factor VII Deficiency: A Case Study of Four Family Members.
Cureus
December 2024
Hematology, Avicenna Military Hospital, Marrakesh, MAR.
Congenital factor VII (FVII) deficiency is a rare genetic disorder with autosomal recessive inheritance, characterized by molecular and clinical heterogeneity. This article reports four Moroccan cases of FVII deficiency within the same family, two of which were associated with Gilbert's syndrome. The index case was a 15-year-old girl with a history of menorrhagia and jaundice.
View Article and Find Full Text PDFMultiorgan Dysfunction and its Association With Congestion and Outcome in Aortic Stenosis Treated With TAVI.
JACC Adv
February 2025
Department of Internal Medicine II, Medical University of Vienna, Vienna, Austria.
Background: Degenerative severe aortic stenosis (AS) is treated by valve replacement to improve outcome. Despite diagnostic advancements, many AS patients are still diagnosed late with advanced heart failure.
Objectives: The aim of the study was to assess multiorgan dysfunction in severe AS using blood biomarkers and their association with quantitative fluid levels and clinical outcomes after transcatheter aortic valve implantation (TAVI).
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