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Although the life expectancy of patients with sickle cell disease (SCD) has increased significantly with medical development, the prognosis is still poor and the average life expectancy is still around 40 years.
autopsy study suggested that the main causes of death in SCD patients were acute chest syndrome (ACS), pulmonary hypertension (PH) and sudden death.
addition, studies have reported that the common QT interstitia in SCD patients is prolonged, suggesting an increased risk of fatal arrhyth arrhythmics.
previous reports suggest that IL18 is a new candidate for dysfunction in the throesopathy period associated with sickle cell disease (SCD).
such as Akash speculated that IL-18 was involved in the development of SCD cardiomyopathy and roomary tatcardia (VT).
Compared with the control group (CTR) mice, the "humanized" SCD mouse model had increased cardiomyospheric fibrosis, extended motion potentior time period (APD), increased VT induction, elevated levels of Icardial NF-B phosphorylation and circulating IL-18 levels, and reduced expression of voltage-gated potassium channels, resulting in a decrease in the external potassium ion current (Ito) of myocardial cells.
the heart of mice treated with IL-18 induces cerial tatiac tatia (VT) originating in the right cardiac chamber and further reduces ito in the heart muscle cells of SCD mice.
Continuous inhibition of IL-18 by IL-18 binding protein can reduce myocardial fibrosis and NF-B phosphorylation, improve espressosis function, normalize electrical reconstruction, and reduce IL-18-mediated roomal tachycardia (VT).
expression of the IL18 gene was enhanced in patients with myocardial fibrosis or QTc-enhanced SCD, and QTc was closely related to plasma IL-18 levels.
expression levels of the IL18 gene, which is the source of blood monocytes in dead individuals, were higher than those of living individuals.
, the study suggests that IL-18 may be a mediated factor for sickle cell cardiomyopathy and roomular titration, as well as a new therapeutic target for patients at risk of sudden death.
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