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淀粉样变的感知流行病学变化:托斯卡纳三级转诊中心 20 年经验。

Changes in the perceived epidemiology of amyloidosis: 20 year-experience from a Tertiary Referral Centre in Tuscany.

机构信息

Tuscan Regional Amyloidosis Centre, Careggi University Hospital, Florence, Italy; Cardiomyopathy Unit, Careggi University Hospital, Florence, Italy.

Tuscan Regional Amyloidosis Centre, Careggi University Hospital, Florence, Italy.

出版信息

Int J Cardiol. 2021 Jul 15;335:123-127. doi: 10.1016/j.ijcard.2021.04.023. Epub 2021 Apr 15.

DOI:10.1016/j.ijcard.2021.04.023
PMID:33865873
Abstract

BACKGROUND

Amyloidosis is considered a rare heterogeneous condition comprising different entities. Epidemiological data are limited and often controversial. We aimed to examine epidemiological changes in amyloidosis diagnosed over a 20-year period at a tertiary referral centre for amyloidosis.

METHODS

We retrospectively reviewed medical files from all patients diagnosed with amyloidosis between January 2000 and December 2019, at Careggi University Hospital, Florence, Italy. Diagnosis of amyloidosis was performed as per current clinical practice and scientific evidence at the time of patient evaluation.

RESULTS

We reported data on 654 consecutive patients: 274 (42%) wild type transthyretin amyloidosis (wtATTR), 68 (10%) genetic variant amyloidosis (vATTR), 281 (43%) light-chain amyloidosis (AL) and 31 (5%) serum amyloid A amyloidosis (AA). With limited fluctuations, the absolute number of new AL diagnosis increased during the 20-year period. wtATTR was unrecognized before 2009 but represented by far the most common aetiology at the end of the observation period. AA represented a residual diagnosis throughout the entire examined period.

CONCLUSIONS

Following a rapid and marked increase in the number of new diagnoses over the last decade, ATTR represents by far the most common type of amyloidosis in our regional centre. These data contrasts with recent reports from national referral institutions and may help shed light on the epidemiology of the disease at the community level.

摘要

背景

淀粉样变性被认为是一种罕见的异质疾病,包含不同的实体。流行病学数据有限,且往往存在争议。我们旨在研究在一家淀粉样变性三级转诊中心 20 年间诊断出的淀粉样变性的流行病学变化。

方法

我们回顾性地审查了 2000 年 1 月至 2019 年 12 月期间在意大利佛罗伦萨卡雷吉大学医院诊断出的所有淀粉样变性患者的病历。淀粉样变性的诊断是根据当时患者评估时的临床实践和科学证据进行的。

结果

我们报告了 654 例连续患者的数据:274 例(42%)野生型转甲状腺素蛋白淀粉样变性(wtATTR)、68 例(10%)遗传变异淀粉样变性(vATTR)、281 例(43%)轻链淀粉样变性(AL)和 31 例(5%)血清淀粉样蛋白 A 淀粉样变性(AA)。在 20 年期间,新的 AL 诊断的绝对数量呈有限波动增长。wtATTR 在 2009 年前未被认识,但在观察期结束时是最常见的病因。AA 在整个研究期间一直是残留诊断。

结论

在过去十年中,新诊断数量迅速显著增加后,ATTR 目前是我们地区中心最常见的淀粉样变性类型。这些数据与来自国家转诊机构的最近报告形成对比,可能有助于阐明社区层面疾病的流行病学。

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