Introduction Sickle cell anemia (SCA) in adults has many clinical manifestations. These manifestations are due to effects of recurrent hemolysis, anemia, and ischemia-reperfusion injury on various organs, including the heart. These factors determine the severity of the disease. Objectives The aim of the study was to assess the severity of SCA using a scoring system consisting of clinical and laboratory parameters. In addition, the study aimed to determine the electrocardiographic abnormalities in the adult SCA population. Study design This was a cross-sectional, observational study conducted in the medical outpatient clinic of Irrua Specialist Teaching Hospital, Irrua, Nigeria. Methodology Sixty SCA patients who were older than 18 years old were recruited for this study between February 2017 and January 2018. Sixty healthy individuals matched for age and sex were recruited to serve as controls. Patients who were pregnant or having an acute crises were excluded from the study. Each participant had an electrocardiogram and a SCA severity score was calculated using their clinical history and complete blood count. Data analysis was carried out using the IBM Statistical Package for Social Sciences Statistics® software, version 21 (IBM SPSS Statistics for Windows, Armonk, NY) and statistical significance assigned to p-values less than 0.05. Results Severity scores for SCA ranged between 7 and 24, with a mean score of 14.5 ± 4.04. Out of the 60 patients, 14 (23.3%), 39 (65%), and seven (11.7%) participants met criteria for mild, moderate, and severe disease, respectively. Tachycardia, prolonged QTc, and the presence of ST-segment and T-wave abnormalities were significantly associated with severe SCA (p = 0.024, p = 0.027, and p = 0.018, respectively). There was positive correlation between SCA severity scores and P-wave duration (r = 0.327, p = 0.011), QRS dispersion (r = 0.298, p = 0.021), QTc interval (r = 0.332, p = 0.010), and QTc dispersion (r = 0.320, p = 0.013). Conclusion This study demonstrated that moderate and severe forms of SCA are common in our region. Tachycardia, left atrial abnormality, prolonged corrected QT interval, and the presence of ST-segment and T-wave changes are electrocardiographic findings associated with more severe forms of the disease. These abnormalities are significant etiologies of cardiac morbidity and mortality in SCA.
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http://dx.doi.org/10.7759/cureus.15296 | DOI Listing |
Haematologica
January 2025
Division of Hematology, Cincinnati Children's Hospital Medical Center, Cincinnati OH; University of Cincinnati College of Medicine, Cincinnati OH; Global Health Center, Cincinnati Children's Hospital Medical Center, Cincinnati OH.
Over the past 40 years, the introduction and refinement of hydroxyurea therapy has led to remarkable progress for the care of individuals with sickle cell anemia (SCA). From initial small proof-of-principle studies to multi-center Phase 3 controlled clinical trials and then numerous open-label studies, the consistent benefits of once-daily oral hydroxyurea have been demonstrated across the lifespan. Elevated fetal hemoglobin (HbF) serves as the most important treatment response, as HbF delays sickle hemoglobin polymerization and reduces erythrocyte sickling.
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January 2025
Department of Pathology, Keck School of Medicine, University of Southern California, Los Angeles, California, USA.
Serous cystadenoma (SCA) of the pancreas is a benign nonmucinous cystic pancreatic neoplasm and the second most common type of pancreatic cystic neoplasm. Conservative management is advocated in asymptomatic cases as they have indolent clinical behavior and risk for postoperative morbidity, making an exact diagnosis essential. Morphologically, serous cystadenoma has a prominent subepithelial capillary meshwork causing the aspirate to be paucicellular and nondiagnostic.
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December 2024
Department of Gastrointestinal Surgery, Weihai Central Hospital, Qingdao University, Weihai, Shandong, China.
Background: Sarcomatoid carcinoma of the small bowel is an exceedingly rare gastrointestinal tumor characterized by a biphasic cellular pattern of epithelioid and mesenchymal-like cells. Due to its rarity and non-specific clinical presentation, it is frequently misdiagnosed, and there is a lack of standardized management guidelines. We report a case of multiple sarcomatoid carcinoma of the small intestine, presenting initially with gastrointestinal perforation.
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January 2025
Department of Clinical Medicine and Surgery, University of Naples Federico II, Naples, Naples, Italy.
Familial hypercholesterolemia (FH) is a genetic disease, usually with onset during childhood, characterized by elevated blood LDL cholesterol levels and potentially associated with severe cardiovascular complications. Concerning mutated genes in FH, such as , a small subset of FH patients presents a homozygous genotype, resulting in homozygous FH (HoFH) disease with a generally aggressive phenotype. Besides statins, ezetimibe and PCSK9 inhibitors, lomitapide (an anti-ApoB therapy) was also approved in 2012-2013 as an adjunctive treatment for HoFH.
View Article and Find Full Text PDFSci Rep
January 2025
Institute of Physics, Faculty of Physics, Astronomy and Informatics, Nicolaus Copernicus University in Toruń, ul. Grudziądzka 5, 87-100, Toruń, Poland.
Due to their simple structure (two bond species randomly arranged on a cubic lattice), the zincblende ABC semiconductor alloys (zb-SCA) set a benchmark to explore how physical properties are impacted by disorder. A longstanding controversy was whether the lattice dynamics (phonons), governed by the bond force constant, i.e.
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