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明尼苏达州奥姆斯特德县 1990 年至 2015 年 AL 淀粉样变性的发病率。

Incidence of AL Amyloidosis in Olmsted County, Minnesota, 1990 through 2015.

机构信息

Division of Hematology, Mayo Clinic, Rochester, MN.

Division of Biostatistics, Mayo Clinic, Rochester, MN.

出版信息

Mayo Clin Proc. 2019 Mar;94(3):465-471. doi: 10.1016/j.mayocp.2018.08.041. Epub 2019 Jan 31.

Abstract

OBJECTIVE

To determine the incidence of immunoglobulin light chain amyloidosis (AL amyloidosis) in a strictly defined geographic area from 1990 through 2015.

PATIENTS AND METHODS

We searched a computerized database for the records of all Olmsted County, Minnesota, residents with a diagnosis of AL amyloidosis from January 1, 1990, through December 31, 2015. In addition, records of all residents with a mention of amyloidosis were obtained from the Rochester Epidemiology Project, which contains the medical records of Mayo Clinic and Olmsted Medical Group. The diagnosis of AL amyloidosis was determined by mass spectrometry, immunohistochemical analysis, or positive Congo red staining.

RESULTS

Thirty-five patients were identified as having AL amyloidosis. The median age at diagnosis was 76 years (range, 38-90 years), with men accounting for 54%. The incidence rate of AL amyloidosis from 1990 through 2015 adjusted for age and sex was 1.2 per 100,000 person-years (95% CI, 0.8-1.6 per 100,000 person-years). Rates were similar across the decades 1990-1999, 2000-2009, and 2010-2015 at 1.1, 0.9, and 1.6 per 100,000 person-years, respectively, with no suggestion of an increasing rate during the 26 years. There was a trend toward an increasing incidence over time from 1950 through 2015 in Olmsted County, but it was not significant (P=.15). Applying the rate of 1.2 per 100,000 person-years to the US population of 321 million in 2015, one would expect 3852 new cases of AL amyloidosis in the United States each year.

CONCLUSION

The incidence of AL amyloidosis in Olmsted County has not changed significantly in the past 66 years.

摘要

目的

确定 1990 年至 2015 年期间在一个严格界定的地理区域内免疫球蛋白轻链淀粉样变性(AL 淀粉样变性)的发病率。

患者和方法

我们检索了明尼苏达州奥姆斯特德县的计算机数据库,以获取 1990 年 1 月 1 日至 2015 年 12 月 31 日期间所有被诊断为 AL 淀粉样变性的居民的记录。此外,还从罗切斯特流行病学项目中获取了所有提到淀粉样变性的居民的记录,该项目包含梅奥诊所和奥姆斯特德医疗集团的病历。AL 淀粉样变性的诊断通过质谱分析、免疫组织化学分析或刚果红染色阳性确定。

结果

确定了 35 名患有 AL 淀粉样变性的患者。诊断时的中位年龄为 76 岁(范围,38-90 岁),男性占 54%。1990 年至 2015 年期间,经年龄和性别调整后的 AL 淀粉样变性发病率为 1.2/100,000 人年(95%CI,0.8-1.6/100,000 人年)。20 世纪 90 年代至 1999 年、2000 年至 2009 年和 2010 年至 2015 年期间,各十年的发病率分别为 1.1、0.9 和 1.6/100,000 人年,在 26 年期间没有提示发病率增加的趋势。在奥姆斯特德县,从 1950 年到 2015 年,发病率呈上升趋势,但没有统计学意义(P=.15)。如果将 1.2/100,000 人年的发病率应用于 2015 年 3.21 亿美国人口,则预计每年美国会有 3852 例新的 AL 淀粉样变性病例。

结论

在过去的 66 年中,奥姆斯特德县 AL 淀粉样变性的发病率没有明显变化。

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