PDF(Pubmed)
Abstract：
UNASSIGNED: We aimed to describe the epidemiology of angioid streaks (AS) and pseudoxanthoma elasticum (PXE), which are rare diseases, using a national claims database.
UNASSIGNED: This was a population-based longitudinal cohort study.
UNASSIGNED: A total of 126 million individuals were covered by the universal health coverage system in Japan.
UNASSIGNED: With permission from the Ministry of Health, Labor and Welfare, we accessed all data from the National Database of Health Insurance Claims and Specific Health Checkups of Japan, which contains the nationwide health insurance claims data for 126 million Japanese. We identified individuals with AS and PXE between January 2011 and December 2020. The incidence rates, prevalence, overlap of AS and PXE, and mean age at death were calculated.
UNASSIGNED: The incidence rates and prevalence of AS and PXE.
UNASSIGNED: A total of 6598 cases of AS and 1020 cases of PXE were identified during the 10-year study period. The incidence rates of AS and PXE were 0.52 (95% confidence interval, 0.48-0.56) and 0.08 (95% confidence interval, 0.07-0.10) per 100 000 person-years, respectively. On October 1, 2020, the prevalence of AS and PXE was 6.5 (95% confidence interval, 6.38-6.66) and 0.83 (95% confidence interval, 0.78-0.89) per 100 000 persons, respectively. The overlap of AS and PXE was 363 patients. The mean age at death of individuals with AS and PXE was 79.3 ± 0.51 and 77.1 ± 2.68 years, respectively.
UNASSIGNED: This is the first population-based study to elucidate the epidemiology of AS and PXE. The mean age of death of both AS and PXE patients was younger than the mean life expectancy of the general Japanese population, thus, appropriate diagnosis and management are important to avoid preventable death.
UNASSIGNED: Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article.
UNASSIGNED: This was a population-based longitudinal cohort study.
UNASSIGNED: A total of 126 million individuals were covered by the universal health coverage system in Japan.
UNASSIGNED: With permission from the Ministry of Health, Labor and Welfare, we accessed all data from the National Database of Health Insurance Claims and Specific Health Checkups of Japan, which contains the nationwide health insurance claims data for 126 million Japanese. We identified individuals with AS and PXE between January 2011 and December 2020. The incidence rates, prevalence, overlap of AS and PXE, and mean age at death were calculated.
UNASSIGNED: The incidence rates and prevalence of AS and PXE.
UNASSIGNED: A total of 6598 cases of AS and 1020 cases of PXE were identified during the 10-year study period. The incidence rates of AS and PXE were 0.52 (95% confidence interval, 0.48-0.56) and 0.08 (95% confidence interval, 0.07-0.10) per 100 000 person-years, respectively. On October 1, 2020, the prevalence of AS and PXE was 6.5 (95% confidence interval, 6.38-6.66) and 0.83 (95% confidence interval, 0.78-0.89) per 100 000 persons, respectively. The overlap of AS and PXE was 363 patients. The mean age at death of individuals with AS and PXE was 79.3 ± 0.51 and 77.1 ± 2.68 years, respectively.
UNASSIGNED: This is the first population-based study to elucidate the epidemiology of AS and PXE. The mean age of death of both AS and PXE patients was younger than the mean life expectancy of the general Japanese population, thus, appropriate diagnosis and management are important to avoid preventable death.
UNASSIGNED: Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article.
摘要：
■我们旨在描述血管样条纹（AS）和弹性假性黄瘤（PXE）的流行病学，这是罕见的疾病，使用国家索赔数据库。
■这是一项基于人群的纵向队列研究。
■日本的全民健康覆盖系统总共覆盖了1.26亿人。
■经卫生部许可，劳动与福利,我们访问了日本国家健康保险索赔和特定健康检查数据库的所有数据，其中包含1.26亿日本人的全国健康保险索赔数据。我们在2011年1月至2020年12月期间确定了患有AS和PXE的个体。发病率，患病率,AS和PXE的重叠，计算了平均死亡年龄。
■AS和PXE的发病率和患病率。
■在10年的研究期间，共发现6598例AS和1020例PXE。AS和PXE的发生率为0.52(95%置信区间,0.48-0.56)和0.08(95%置信区间，0.07-0.10)每100000人年，分别。2020年10月1日，AS和PXE的患病率为6.5（95%置信区间，6.38-6.66)和0.83(95%置信区间，0.78-0.89)每100000人，分别。AS和PXE重叠363例。AS和PXE患者的平均死亡年龄为79.3±0.51和77.1±2.68岁，分别。
■这是第一项基于人群的研究，以阐明AS和PXE的流行病学。AS和PXE患者的平均死亡年龄比日本普通人群的平均预期寿命年轻，因此，适当的诊断和管理对于避免可预防的死亡非常重要.
■专有或商业披露可在本文末尾的脚注和披露中找到。
■这是一项基于人群的纵向队列研究。
■日本的全民健康覆盖系统总共覆盖了1.26亿人。
■经卫生部许可，劳动与福利,我们访问了日本国家健康保险索赔和特定健康检查数据库的所有数据，其中包含1.26亿日本人的全国健康保险索赔数据。我们在2011年1月至2020年12月期间确定了患有AS和PXE的个体。发病率，患病率,AS和PXE的重叠，计算了平均死亡年龄。
■AS和PXE的发病率和患病率。
■在10年的研究期间，共发现6598例AS和1020例PXE。AS和PXE的发生率为0.52(95%置信区间,0.48-0.56)和0.08(95%置信区间，0.07-0.10)每100000人年，分别。2020年10月1日，AS和PXE的患病率为6.5（95%置信区间，6.38-6.66)和0.83(95%置信区间，0.78-0.89)每100000人，分别。AS和PXE重叠363例。AS和PXE患者的平均死亡年龄为79.3±0.51和77.1±2.68岁，分别。
■这是第一项基于人群的研究，以阐明AS和PXE的流行病学。AS和PXE患者的平均死亡年龄比日本普通人群的平均预期寿命年轻，因此，适当的诊断和管理对于避免可预防的死亡非常重要.
■专有或商业披露可在本文末尾的脚注和披露中找到。
