• Hoi Soo Yoon, Yujin Han, Young Jin Kim, Min Jin Kim, Ja Min Byun, Taemi Youk, Jae Hee Lee, Tae Sung Park, and Jongha Yoo.
• Department of Pediatrics, Kyung Hee University School of Medicine, Seoul, Korea.
• J. Korean Med. Sci. 2020 Oct 12; 35 (39): e350.

BackgroundExcept for data in the Korea Hemophilia Foundation Registry, little is known of the epidemiology of congenital bleeding disorders in Korea.MethodsData were obtained from the Korean Health Insurance Review and Assessment Service (HIRA) database.ResultsFrom 2010 to 2015, there were 2,029 patients with congenital bleeding disorders in the Korean HIRA database: 38% (n = 775) of these patients had hemophilia A (HA), 25% (n = 517) had von Willebrand disease (vWD), 7% (n = 132) had hemophilia B (HB), and 25% (n = 513) had less common factor deficiencies. The estimated age-standardized incidence rate (ASR) of HA and HB was 1.78-3.15/100,000 and 0.31-0.51/100,000, respectively. That of vWD was 1.38-1.95/100,000. The estimated ASR of HA showed increase over time though the number of new patients did not increase. Most patients with congenital bleeding disorders were younger than 19 years old (47.8%), and most were registered in Gyeonggi (22.1%) and Seoul (19.2%).ConclusionThis is the first nationwide population-based study of congenital bleeding disorders in Korea. This study provides data that will enable more accurate estimations of patients with vWD. This information will help advance the comprehensive care of congenital bleeding disorders. We need to continue to obtain more detailed information on patients to improve the management of these diseases.© 2020 The Korean Academy of Medical Sciences.

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