Prevalence of Amyloidosis in Korea

Seo, Su Ra; Jang, Shin Yi; Lee, Ga Yeon; Choi, Bareun; Chun, Heeran; Cho, Eun Jeong; Cho, Sung-Il

doi:10.1186/s13023-017-0705-2

Cited by 14 publications

(17 citation statements)

References 12 publications

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“…The differences in age, socioeconomic position, and causes of death by sex were analyzed using the student's t-test for continuous variables and the χ 2 -test for categorical variables. We calculated the agestandardized incidence of amyloidosis with the direct method using the bene ciaries of health insurance from the Korean National Health Insurance Statistical Yearbook from 2006 through 2017 as the subjects and the estimated Korean population in 2015 as the reference [1,2]. The Kaplan-Meier method was also used to compare survival among patients with amyloidosis by age group and sex using log-rank tests.…”

Section: Methodsmentioning

confidence: 99%

“…Few studies have evaluated the incidence, survival rate, cause of death, and risk factors for mortality of amyloidosis. We have previously reported the prevalence of amyloidosis in Korea [1]. In the current study, we analyzed the age-standardized incidence, survival rate, cause of death, and risk factors for death in amyloidosis patients using Korean National Health Insurance Service data from 2006 through 2017.…”

Section: Introductionmentioning

confidence: 99%

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Incidence, Cause of Death, and Survival of Amyloidosis in Korea

Jang

Kim

Choi

et al. 2020

Preprint

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BackgroundWe sought to assess incidence and survival for amyloidosis.MethodsWe acquired data from newly diagnosed cases related to amyloidosis from the National Health Insurance Service in Korea from 2006 through 2017 (n=2,233; male 53.5%). We calculated the age-standardized incidence rate, analyzed the survival rate (SR) using the Kaplan-Meier method, and analyzed the death risk using Cox proportional hazards methods.ResultsThe mean age was 57.0 (±16.7) years in males and 56.8 (±15.6) years in females (p=non-significant). The proportion of death was 34.7%. The causes of death were endocrine, nutritional, and metabolic diseases (33.9%), malignant neoplasm (20.8%), diseases of the circulatory system (9.68%), and diseases of the genitourinary system (9.29%). The overall age-standardized incidence rate was 0.47 persons per 100,000 persons in 2017. Overall, the 10-year SR for amyloidosis was 57.7% (55.9% in males and 59.2% in females). Adjusted hazard ratios were 9.16 (95% confidence interval [CI] 2.23, 37.5) among 40-49 year-old, 16.1 (95% CI 4.00, 65.3) among 50-59 year-old, 30.3 (95% CI 7.53, 122.0) among 60-69 year-old, 48.7 (95% CI 12.1, 196.3) among 70-79 year-old, 80.1 (95% CI 19.6, 326.3) among people 80 years or older and 1.21 (95% CI 1.02, 1.44) in the medium-level socioeconomic position group. ConclusionsThe age-standardized incidence rate of amyloidosis was about 0.5 persons per 100,000 persons in 2017. The 10-year SR of amyloidosis was about 58%. The most common cause of death was endocrine, nutritional, and metabolic diseases. The risk of death from amyloidosis increased with age and medium socioeconomic position.

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Section: Methodsmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Incidence, Cause of Death, and Survival of Amyloidosis in Korea

Jang

Kim

Choi

et al. 2020

Preprint

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“…Systemic amyloidosis is a multisystem disorder characterized by the formation and deposition of mis-folded protein fibrils which can result in multi-organ failure and death [1,2]. This condition is associated with significant disease burden with increasing incidence and prevalence worldwide over the past decades [3][4][5]. Studies have shown that at least 20 out of one million UK residents are estimated to have systemic amyloidosis with 65% being light-chain (AL) amyloidosis.…”

Section: Introductionmentioning

confidence: 99%

Nuclear Imaging for the Diagnosis of Cardiac Amyloidosis in 2021

Uppal

Wang

et al. 2021

Diagnostics

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Cardiac amyloidosis is caused by the deposition of misfolded protein fibrils into the extracellular space of the heart. The diagnosis of cardiac amyloidosis remains challenging because of the heterogeneous manifestations of the disease. There are many different types of amyloidosis with light-chain (AL) amyloidosis and transthyretin (ATTR) amyloidosis being the most common types of cardiac amyloidosis. Endomyocardial biopsy is considered the gold standard for diagnosing cardiac amyloidosis and differentiating amyloid subtypes, but its use is limited because of the invasive nature of the procedure, with risks for complications and the need for specialized training and centers to perform the procedure. Radionuclide cardiac imaging has recently become the most commonly performed test for the diagnosis of ATTR amyloidosis but is of limited value for the diagnosis of AL amyloidosis. Positron emission tomography has been increasingly used for the diagnosis of cardiac amyloidosis and its applications are expected to expand in the future. Imaging protocols are under refinement to achieve better quantification of the disease burden and prediction of prognosis.

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“…Several studies have attempted to understand the epidemiology of rare diseases. For example, Seo et al, using NHIS data, reported that the prevalence of amyloidosis was 0.93 (95% confidence interval [CI]: 0.81–1.04) in Korea [17]. Ryder et al reported that the point prevalence of Duchenne muscular dystrophy per 100,000 men in France, the USA, UK, and Canada was 10.9, 1.9, 2.2, and 6.1, respectively [18].…”

Section: Introductionmentioning

confidence: 99%

“…Furthermore, Orphanet conducted a systemic review of literature to estimate the incidence and prevalence of rare diseases [20]. However, most of the studies did not use the rare disease register data, but rather the medical claim data [17, 18]; thus, it is not easy to obtain the real incidence of each rare disease [21]. Therefore, population-based administrative records of rare disease registries are appropriate sources of data to conduct a passive surveillance of rare diseases to maximize valid case detection [21].…”

Section: Introductionmentioning

confidence: 99%

The cumulative incidence and trends of rare diseases in South Korea: a nationwide study of the administrative data from the National Health Insurance Service database from 2011–2015

Lim

Lee

Kim

et al. 2019

Orphanet J Rare Dis

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Background The burden of rare diseases on society and patients’ families has increased in Korea. However, because of the infrequency of rare diseases, there is a lack of resources and information to address these cases and inadequate funding for the management of these patients. We investigated the average annual cumulative incidence of rare diseases and the trends in annual cumulative incidence from 2011 to 2015 in Korea by using nationwide administrative data from the Korean National Health Insurance Service (NHIS) database for patients registered with the co-payment assistance policy for rare and incurable diseases. Annual cumulative incidence per 10,000,000 was calculated as the total number of newly enrolled patients with the Korean Standard Classification of Diseases (KCD)-7 code in the register, divided by the number of residents with health insurance coverage during each year. We employed simple linear regression analysis to evaluate the trends in annual cumulative incidence/10,000,000 population per year for each rare disease. Results Overall, national support was provided for patients with 415 KCD codes listed among the targeted rare diseases. The total number of newly enrolled patients with rare diseases was 53,831 in 2011, 52,658 in 2012, 52,955 in 2013, 71,530 in 2014, and 70,559 in 2015. The number of rare diseases with an average annual cumulative incidence of 100/10,000,000 and above was 22 (5.30%), while there were 227 (54.70%) and 148 (35.66%) with an average cumulative incidence between 1/10,000,000 and 100/10,000,000 and less than 1/10,000,000, respectively. The trends in the annual cumulative incidence for 43 rare diseases were statistically significant ( p -value < 0.05). The rare diseases for which the incremental trend per year was statistically significant were sarcoidosis (D86, D86.0, D86.1, D86.2, D86.3, D86.8, D86.9), Parkinson’s disease (G20), Guillain-Barré syndrome (G61.0), primary biliary cirrhosis (K74.3) and Sjogren’s syndrome (M35.0). Conclusions The number of rare diseases showing an increasing trend in annual cumulative incidence was higher than the number of diseases showing a decreasing trend in annual cumulative incidence. Given that the definition and diagnosis vary based on country and that there is difficulty in identifying valid cases, further detection strategies are needed to establish the incidence of each rare disease considering the importance of establishing a health policy based on the actual incidence of the targeted diseases. Electronic supplementary material The online version of this article (10.1186/s13023-019-1032-6) contains supplementary material, which is available to authorized users.

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Prevalence of Amyloidosis in Korea

Cited by 14 publications

References 12 publications

Incidence, Cause of Death, and Survival of Amyloidosis in Korea

Incidence, Cause of Death, and Survival of Amyloidosis in Korea

Nuclear Imaging for the Diagnosis of Cardiac Amyloidosis in 2021

The cumulative incidence and trends of rare diseases in South Korea: a nationwide study of the administrative data from the National Health Insurance Service database from 2011–2015

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