The plasma levels of some blood coagulation parameters; prothrombin time, (PT), partial thromboplastin time with kaolin (PTTK), thrombin clotting time, (TCT), fibrinogen and factor X assay were determined in 50 Nigerian homozygous (HbSS) patients and 50 HbAA healthy individuals for the purpose of assessing their baseline values and susceptibility of patients with sickle cell disease (SCD) to hyper coagulability. Standard procedures were used for all variables. The mean age of the study participants was 21.7 +/- 5.0 years. The mean PT of 13.7 +/- 1.4 s in HbSS patients was found to be significantly longer than the mean PT value of 12.9 +/- 1.0 in HbAA control subjects (p < 0.001). The mean PTTK values of 46.0 +/- 9.6 s in HbSS patients was also found to be significantly higher than the 41.0 +/- 3.7 s recorded among the control subjects (p < 0.001). The mean TCT of 6.4 +/- 0.8 s in HbSS patients was however found to be significantly lower than the mean value of 10.6 +/- 0.8 s obtained in the control group. Fibrinogen level (4.1 +/- 2.1 g/l) in HbSS patients and 2.8 +/- 0.9 g/l in HbAA controls was also found to be significantly different (p < 0.001). Factor X level in the sickle cell patients, (64.6 +/- 14.9%) was equally found to be significantly lower than that of the apparently healthy HbAA control individuals (95.2 +/- 7.2%) (p < 0.001).
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Sickle cell disease (SCD) is the most common genetic disease in the world and a societal challenge. SCD is characterized by multi-organ injury related to intravascular hemolysis. To understand tissue-specific responses to intravascular hemolysis and exposure to heme, we present a transcriptomic atlas in the primary target organs of HbSS vs HbAA transgenic SCD mice.
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Article Synopsis
- Sickle cell disease (SCD) is a genetic disorder caused by a mutation in the HBB gene, resulting in abnormal hemoglobin S that deforms red blood cells, leading to severe health complications such as pain, anemia, and organ damage.
- The disease is prevalent, especially in sub-Saharan Africa, where over 7.5 million people are affected, often facing underdiagnosis and poor management, particularly in children.
- This study in Ghana aims to identify specific circulating biomarkers in pediatric SCD patients that could help predict disease outcomes and improve patient care by assessing various serum markers associated with inflammation and neuronal injury.
Background Sickle cell disease (SCD) is a hereditary disorder marked by abnormal hemoglobin (HbS), leading to chronic hemolytic anemia, vaso-occlusive crises (VOCs), and multi-organ complications. In India, the prevalence of SCD is highest among tribal populations in states like Madhya Pradesh, Maharashtra, Odisha, and Assam, with the disease burden exacerbated by limited healthcare access, especially in rural regions. This study provides a comprehensive analysis of the demographic profile, clinical features, and treatment patterns of SCD patients at a tertiary healthcare center in Upper Assam, where the prevalence of SCD is high among the tea tribe communities.
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