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Assessment of cardiac iron deposition and genotypic classification in pediatric beta-thalassemia major: the role of cardiac MRI.
BMC Med Imaging
January 2025
Department of Radiology, Shenzhen Children's Hospital, Shantou University Medical College, 7019 Yitian Road, Futian District, Shenzhen, 518038, China.
Background: Beta thalassemia major (β-TM) is a severe genetic anemia with considerable phenotypic heterogeneity. This study investigated whether genotype correlates with distinct myocardial iron overload patterns, assessed by cardiovascular magnetic resonance (CMR) T2* values.
Methods: CMR data for cardiac iron deposition evaluation, which recruited pediatric participants between January 2021 and December 2024, were analyzed with CVI42.
Maternal and Infant Outcomes in a Subset of Patients with Sickle Cell Disease in South Carolina.
South Med J
February 2025
the Department of Public Health Sciences.
Objectives: Sickle cell disease (SCD), which disproportionately affects minorities, increases complications during pregnancy. Severe maternal mortality is increased in women with SCD, including morbidity related to the disease and other nondisease-related complications. It also can have devastating complications for fetuses, with increases in premature birth and low birth weight.
View Article and Find Full Text PDFEculizumab treatment for Chinese patients with hemolytic paroxysmal nocturnal hemoglobinuria (PNH): efficacy and safety - a single-center study.
Hematology
December 2025
Department of Hematology, Peking Union Medical College Hospital, Chinese Academy of Medical Science and Peking Union Medical College, Beijing, People's Republic of China.
Objective: To evaluate the short-term efficacy and safety of eculizumab for the treatment of paroxysmal nocturnal hemoglobinuria (PNH) in China.
Method: Data were retrospectively collected from patients with PNH who received at least 3 months of full-dose eculizumab. Changes in clinical and laboratory indicators after 1, 3, and 6 months of eculizumab therapy and at the end of follow-up were documented.
Retinal and Choroidal Circulation Impairments in Fanconi Anemia.
Am J Ophthalmol
January 2025
Hacettepe University School of Medicine, Department of Ophthalmology, Ankara, Turkey.
Objective: To evaluate the effects of Fanconi anemia (FA) on retinal and choroidal microvasculature using Optical Coherence Tomography (OCT) and Optical Coherence Tomography Angiography (OCTA).
Design: Cohort study with age-matched controls.
Subjects And Participants: This study included 11 eyes from 11 patients diagnosed with FA and 12 eyes from 12 age-matched healthy controls.
Clinicopathological analysis of anti-VEGF drug-associated renal thrombotic microangiopathy: A case series and review of the literature.
Pathol Res Pract
January 2025
Department of Pathology, West China Hospital of Sichuan University, Chengdu 610041, China.
Anti-vascular endothelial growth factor-associated thrombotic microangiopathy (aVEGF-TMA) was recently discovered in patients with malignant tumors. Four aVEGF-TMA patients diagnosed by renal biopsy between 2018 and 2022 were identified, and all were females aged 30-62 years (mean age, 47 years). Two patients with malignant gastrointestinal stromal tumors who received sunitinib were analyzed.
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