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Minimal assessment of the index case: the point of view of the cardiologist

机译:对索引病例的最小评估:心脏病专家的观点

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TTR familial amyloidosis is a systemic disease, with afrequent cardiac involvementFor clinical reasons – related to its prolonged latency -and historical reasons – as the first cases where diagnosedby neurologists, in young patients who died fromneurological disease before cardiac complicationsoccurred- familial cardiac amyloidosis (TTR-FAC) hasbeen and still remains underestimated. It is known to beresponsible of 40% of mid-term deaths after liver transplantation.TTR-FAC appears irreversible; it can progressafter liver transplantation, and is associated with a poorprognosis. Hence the heart deserves a special attentionwhen taking in charge patients with familial amyloidosis,especially since there are several new promising therapeuticoptions.
机译:TTR家族性淀粉样变性病是一种系统性疾病,心脏受累频繁由于临床原因–与其潜伏期延长有关–历史原因–作为神经科医生诊断的首例病例,是在发生心脏并发症之前因神经系统疾病死亡的年轻患者–家族性心脏淀粉样变性病(TTR- FAC)仍然被低估。众所周知,肝移植后中期死亡的40%是由TTR-FAC引起的。它可以在肝移植后发展,并且与不良预后有关。因此,在接受家族性淀粉样变性病患者的治疗时,心脏尤其值得关注,特别是因为有几种新的有希望的治疗选择。

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