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首页> 外文期刊>Amyloid: the international journal of experimental and clinical investigation : the official journal of the International Society of Amyloidosis >Clinical features and survival in senile systemic amyloidosis: comparison to familial transthyretin cardiomyopathy.
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Clinical features and survival in senile systemic amyloidosis: comparison to familial transthyretin cardiomyopathy.

机译：老年系统性淀粉样变性病的临床特征和生存率：与家族性甲状腺素转运蛋白心肌病的比较。

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摘要

Senile systemic amyloidosis (SSA) features cardiomyopathy resulting from amyloid deposits of wild-type transthyretin (TTR). From 1994 to 2009,82 patients with SSA were diagnosed at our center; 79 were men (96%) and median age at diagnosis was 73.8 years (range, 59.1-86.0). Most patients (77/78) presented with abnormal echocardiography; median values for interventricular septal thickness and left ventricular ejection fraction were 16 mm (range, 9-24) and 50% (range, 20-70), respectively. Fat aspirates were positive for amyloid in 27% of patients. Mean levels of brain natriuretic peptide (n = 41) and troponin I (re =19) were 422 + 279 pg/ml and 0.151 +- 0.107 pg/ml. Median survival was 4.3 years (95% CI, 3.7-5.0). SSA and familial TTR cardiomyopathy were compared; survival distribution was significantly different across groups (log-rank test= 11.97, p-vahxe = 0.0075). We conclude that patients with SSA are primarily men who present with dominant cardiac involvement at an older age than patients with familial TTR cardiomyopathy.
机译：老年系统性淀粉样变性病（SSA）的特征是由野生型运甲状腺素蛋白（TTR）的淀粉样蛋白沉积引起的心肌病。从1994年到2009年，我中心共诊断出82例SSA患者。男性为79名（96％），诊断时的中位年龄为73.8岁（范围：59.1-86.0）。大多数患者（77/78）表现出超声心动图异常;室间隔厚度和左室射血分数的中位值分别为16毫米（范围9-24）和50％（范围20-70）。 27％的患者中脂肪抽吸物的淀粉样蛋白阳性。脑利钠肽（n = 41）和肌钙蛋白I（re = 19）的平均水平为422 + 279 pg / ml和0.151 +-0.107 pg / ml。中位生存期为4.3年（95％CI，3.7-5.0）。比较了SSA和家族性TTR心肌病。各组的生存率分布差异显着（对数秩检验= 11.97，p-vahxe = 0.0075）。我们得出的结论是，SSA患者主要是男性，比家族性TTR心肌病的患者年龄更大。

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