Dataset Information

Estimated prevalence of Cronkhite-Canada Syndrome, Chronic Enteropathy Associated with SLCO2A1 Gene, and Intestinal Behcet's Disease in Japan in 2017: A Nationwide Survey.

ABSTRACT:

Background

Cronkhite-Canada syndrome (CCS), chronic enteropathy associated with SLCO2A1 gene (CEAS), and intestinal Behçet's disease (BD) are classified as intractable intestinal disorders in Japan. However, the national prevalence of these diseases remains unknown. We performed a nationwide survey to estimate the patient numbers and prevalence rates of these diseases throughout Japan in 2017.

Methods

We conducted a mail-based survey targeting hospitals across Japan to estimate the annual numbers of patients with CCS, CEAS, and intestinal BD in 2017. Using a stratified random sampling method, we selected 2979 hospital departments and asked them to report the number of patients who met specific diagnostic criteria. The total number of patients for each disease was estimated by multiplying the reported numbers by the reciprocal of the sampling rate and response rate. The corresponding prevalence rates per 1,000,000 population were calculated based on the mid-year population of Japan in 2017.

Results

The overall survey response rate was 68.1% (2029 departments). The estimated numbers of patients with CCS, CEAS, and intestinal BD were 473 (95% confidence interval: 357-589), 388 (289-486), and 3139 (2749-3529), respectively; the prevalence rates per 1,000,000 population were 3.7 (male: 4.0; female: 3.5), 3.1 (male: 3.0; female: 3.1), and 24.8 (male: 24.5; female: 25.0), respectively. The male-to-female ratios were 1.10, 0.94, and 0.93 for patients with CCS, CEAS, and intestinal BD, respectively.

Conclusions

Estimates of the national prevalence of CCS, CEAS, and intestinal BD in Japan were generated and found to be higher than those previously reported.

SUBMITTER: Oba MS

PROVIDER: S-EPMC7813772 | biostudies-literature | 2020 Feb

REPOSITORIES: biostudies-literature

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Publications

Estimated Prevalence of Cronkhite-Canada Syndrome, Chronic Enteropathy Associated With SLCO2A1 Gene, and Intestinal Behçet's Disease in Japan in 2017: A Nationwide Survey.

Oba Mari S MS Murakami Yoshitaka Y Nishiwaki Yuji Y Asakura Keiko K Ohfuji Satoko S Fukushima Wakaba W Nakamura Yosikazu Y Suzuki Yasuo Y

Journal of epidemiology 20200222 2

<h4>Background</h4>Cronkhite-Canada syndrome (CCS), chronic enteropathy associated with SLCO2A1 gene (CEAS), and intestinal Behçet's disease (BD) are classified as intractable intestinal disorders in Japan. However, the national prevalence of these diseases remains unknown. We performed a nationwide survey to estimate the patient numbers and prevalence rates of these diseases throughout Japan in 2017.<h4>Methods</h4>We conducted a mail-based survey targeting hospitals across Japan to estimate th ...[more]

PMID: 32092751

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