Background:
There is growing interest in the observed significant incidence of transthyretin cardiac
amyloidosis in elderly patients with aortic stenosis. Approximately 16% of patients with severe aortic
stenosis undergoing aortic valve replacement have transthyretin cardiac amyloidosis. Outcomes after
aortic valve replacement appear worse in patients with concomitant transthyretin cardiac amyloidosis.
Method:
Publications in PubMed, Cochrane Library, and Embase databases were systematically
searched from January 2012 to September 2018 using the keywords transthyretin, amyloidosis, and
aortic stenosis. All studies published in English that reported the prevalence, association and outcomes
of transthyretin cardiac amyloidosis in patients with aortic stenosis undergoing were included.
Results/Conclusion:
The relationship between aortic stenosis and transthyretin cardiac amyloidosis is
not well understood. A few studies have proven successful surgical management when both
conditions coexist. This systematic review suggests that transthyretin cardiac amyloidosis is common
in elderly patients with aortic stenosis and tend to have high mortality rates after AVR. The
significant incidence of the two diseases occurring simultaneously warrants further investigation to
improve management strategies in the future.
Apoptosis has a major role in molding the embryo, in the maintenance of tissue homeostasis, and in the defense against pathogens, while its disgregulation is strongly implicated in cancer as well as in autoimmune and degenerative diseases. The opposite action of anti-apoptotic proteins (Bcl-2 family) and pro-apoptotic proteins (p53, Bax, Bak) regulates the activation of caspases that are the effectors proteases of the cell suicide. Bcl-W is a pro-survival protein, recently discovered, related to the Bcl-2 family. The presence of Bcl-W is fundamental for spermatogenesis in rats. Caspases are cysteine-dependent aspartate-specific proteases, and their over-expression can result in apoptotic cell death. Normally, caspases exist in cells as inactive pro-enzymes and can be activated by 2 distinct mechanisms: the FADD/caspase 8 cascade, and the Apaf-1/caspase 9 cascade. These 2 mechanisms are used extensively by cells for the activation of the effectors caspases: caspase 3, caspase 6, and/or caspase 7. Bcl-W and caspases might have a pivotal role in maintenance of Sertoli cells integrity. In this study, we demonstrate that both Bcl-W mRNA and caspase 3 mRNA are expressed in isolated Sertoli cells of pre-puberal rat testes. This finding might be crucial in clarifying whether Sertoli cells die by an apoptotic mechanism. Further studies are required to understand whether the expression of Bcl-W and caspases is different before and after puberty in rat testis and/or in pathological conditions, that lead to an increased cell apoptosis.
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