A population-based cohort study of the epidemiology of light-chain amyloidosis in Taiwan

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作者
Hsin-An Hou
Chao-Hsiun Tang
Choo Hua Goh
Shih-Pei Shen
Kuan-Chih Huang
Hong Qiu
Sarah Siggins
Lee Anne Rothwell
Yanfang Liu
机构
[1] National Taiwan University Hospital,Division of Hematology, Department of Internal Medicine
[2] Taipei Medical University,School of Health Care Administration, College of Management
[3] Johnson & Johnson,Epidemiology, Office of the Chief Medical Officer
[4] Johnson & Johnson,Epidemiology, Office of the Chief Medical Officer
[5] Johnson & Johnson,Epidemiology, Office of the Chief Medical Officer
[6] Janssen Medical Affairs Asia Pacific, Global Real World Evidence, GDCS, GCSO
[7] Janssen Research & Development LLC,undefined
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Scientific Reports | / 12卷
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摘要
The incidence rate of AL (light-chain) amyloidosis is not known in Asia. We conducted a retrospective cohort study using the Taiwan National Healthcare Insurance Research database and Death Registry to estimate incidence and all-cause case fatality rates, and characteristics of patients with AL amyloidosis in Taiwan. All patients with confirmed, newly diagnosed AL amyloidosis from 01-Jan-2016 until 31-Dec-2019 were enrolled and followed up until dis-enrolment, death or study end (31-Dec-2019). There were 841 patients with newly diagnosed AL amyloidosis with median age of 61.4 years and 58.7% were men. At diagnosis, cardiac, renal and liver-related diseases were present in 28.54%, 23.19% and 2.14% of patients, respectively. AL amyloidosis age-adjusted annual incidence was 5.73 per million population in 2016 and 5.26 per million population in 2019. All-cause case fatality ranged from 1.7 to 2.9% over the study period and was highest (~10%) in patients ≥ 80 years. Survival was significantly lower in patients with co-morbid cardiac, renal, or liver-related diseases which could indicate organ involvement. The incidence of AL amyloidosis in Taiwan appears to be similar to Western countries. The poor prognosis in patients with co-morbid diseases highlights the need for earlier diagnosis.
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