Cardiovascular Sequelae of Sickle Cell Disease
Sickle cell disease (SCD) is one of the most common hereditary hemoglobinopathies worldwide. It is a multisystem disease that causes considerable patient morbidity. Despite advances in medical treatment, cardiopulmonary complications remain the most common cause of death in individuals with SCD. A growing body of evidence has shown that SCD results in a spectrum of cardiovascular complications through a variety of mechanisms, including chronic hemolysis, local tissue hypoxia, increased oxidative stress, and autonomic instability. Herein, we will examine the pathophysiology of sickle cell vasculopathy and discuss the spectrum of cardiovascular sequelae of the disease, while highlighting the impact of SCD on the cardiovascular health of the patients.
CONCLUSIONS: These results suggest that circulating EVs can modulate endothelial integrity contributing to the development of ACS in SCD patients by altering cadherin-containing intercellular junctions. IMPACT: Sickle cell disease patients have circulating extracellular vesicles (EVs) that modulate endothelial integrity by altering cadherin-containing intercellular junctions.Disruption is more severe by EVs obtained during acute chest syndrome (ACS).These results expand our knowledge of the pathophysiology of acute chest syndrome and the vasculopathies of sickle cell disease. PMID: 32454519 [PubMed - as supplied by publisher]
A significant amount of clinical and research interest in thrombosis is focused on large vessels (e.g., stroke, myocardial infarction, deep venous thrombosis, etc.); however, thrombosis is often present in the microcirculation in a variety of significant human diseases, such as disseminated intravascular coagulation, thrombotic microangiopathy, sickle cell disease, and others. Further, microvascular thrombosis has recently been demonstrated in patients with COVID-19, and has been proposed to mediate the pathogenesis of organ injury in this disease.
MICROVASCULAR THROMBOSIS: EXPERIMENTAL AND CLINICAL IMPLICATIONS. Transl Res. 2020 May 23;: Authors: Bray MA, Sartain SA, Gollamudi J, Rumbaut RE Abstract A significant amount of clinical and research interest in thrombosis is focused on large vessels (e.g., stroke, myocardial infarction, deep venous thrombosis, etc.); however, thrombosis is often present in the microcirculation in a variety of significant human diseases, such as disseminated intravascular coagulation, thrombotic microangiopathy, sickle cell disease, and others. Further, microvascular thrombosis has recently been demonstrated in patie...
Sickle cell anaemia (SCA) is characterized by attendant ischemia-reperfusion injury especially to the heart.
Abstract Sickle cell disease (SCD) is an inherited hemoglobinopathy that is caused by the presence of abnormal hemoglobin S (HbS) in red blood cells, leading to alterations in red cell properties and shape, as the result of HbS dexoygenation and subsequent polymerization. SCD pathophysiology is characterized by chronic inflammatory processes, triggered by hemolytic and vaso-occlusive events, which lead to the varied complications, organ damage and elevated mortality seen in individuals with the disease. In association with activation of the endothelium and leukocytes, hemostatic alterations and thrombotic events a...
2'-O-methoxyethyl splice-switching oligos correct splicing from IVS2-745 β-thalassemia patient cells restoring HbA production and chain rebalance. Haematologica. 2020 May 21;: Authors: Dong A, Ghiaccio V, Motta I, Guo S, Peralta R, Freier SM, Watt A, Damle S, Ikawa Y, Jarocha D, Chappell M, Stephanou C, Delbini P, Chen C, Christou S, Kleanthous M, Smith-Whitley K, Manwani D, Casu C, Abdulmalik O, Cappellini MD, Rivella S, Breda L Abstract β-thalassemia is a disorder caused by altered hemoglobin protein synthesis and affects individuals worldwide. Severe forms of the disease, left untreated, ...
Chronic pain is a complex integration of biological, psychological, and social variables. Multidisciplinary pain management experts design interventions that treat the multidimensional experience. Children and adolescents with sickle cell disease (SCD) are at risk for chronic pain. Increased risk is associated with multiple characteristics including sickle cell genotype, age, gender, frequency of hospitalization, duration of hospitalization, and certain comorbid diagnoses. Referral to pain management professionals for this population is often delayed.
To examine mental health diagnoses, health care utilization, and receipt of age-appropriate preventive care, including antibiotic prophylaxis, hydroxyurea therapy, and transcranial Doppler (TCD) screenings among children with sickle cell anemia (SCA).