Estimated Prevalence of Cronkhite-Canada Syndrome,Chronic Enteropathy Associated With SLCO2A1 Gene,and Intestinal Behçet’s Disease in Japan in 2017: A Nationwide Survey |
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| Authors: | Mari S Oba Yoshitaka Murakami Yuji Nishiwaki Keiko Asakura Satoko Ohfuji Wakaba Fukushima Yosikazu Nakamura Yasuo Suzuki |
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| Institution: | 1.Department of Medical Statistics, Toho University, Tokyo, Japan;2.Department of Environmental and Occupational Health, Toho University, Tokyo, Japan;3.Department of Public Health, Osaka City University Graduate School of Medicine, Osaka, Japan;4.Department of Public Health, Jichi Medical University, Tochigi, Japan;5.Department of Internal Medicine, Toho University Sakura Medical Center, Chiba, Japan |
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| Abstract: | BackgroundCronkhite-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.MethodsWe 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.ResultsThe 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.ConclusionsEstimates of the national prevalence of CCS, CEAS, and intestinal BD in Japan were generated and found to be higher than those previously reported.Key words: Cronkhite-Canada syndrome, chronic enteropathy associated with SLCO2A1 gene, intestinal Behçet’s disease, nationwide survey, prevalence |
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