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

Authors :
Mari S. Oba
Yoshitaka Murakami
Yuji Nishiwaki
Keiko Asakura
Satoko Ohfuji
Wakaba Fukushima
Yosikazu Nakamura
Yasuo Suzuki
Source :
Journal of Epidemiology, Vol 31, Iss 2, Pp 139-144 (2021)
Publication Year :
2021
Publisher :
Japan Epidemiological Association, 2021.

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 2,979 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% (2,029 departments). The estimated numbers of patients with CCS, CEAS, and intestinal BD were 473 (95% confidence interval [CI], 357–589), 388 (95% CI, 289–486), and 3,139 (95% CI, 2,749–3,529), 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.

Details

Language :
English
ISSN :
09175040 and 13499092
Volume :
31
Issue :
2
Database :
Directory of Open Access Journals
Journal :
Journal of Epidemiology
Publication Type :
Academic Journal
Accession number :
edsdoj.8c2bbb31f33c4911bee64532a903b1c9
Document Type :
article
Full Text :
https://doi.org/10.2188/jea.JE20190349