J Korean Med Sci.  2020 Oct;35(39):e350. 10.3346/jkms.2020.35.e350.

Epidemiology of Congenital Bleeding Disorders: a Nationwide Populationbased Korean Study

• Yoon HS
• Han Y
• Kim YJ
• Kim MJ
• Byun JM
• Youk T
• Lee JH
• Park TS
• Yoo J

• KMID: 2507611
• DOI: http://doi.org/10.3346/jkms.2020.35.e350

Abstract

Background
Except for data in the Korea Hemophilia Foundation Registry, little is known of the epidemiology of congenital bleeding disorders in Korea.
Methods
Data were obtained from the Korean Health Insurance Review and Assessment Service (HIRA) database.
Results
From 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%).
Conclusion
This 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.

Keyword

Congenital Bleeding Disorder; Epidemiology; Korea; Nationwide

Figure

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  Fig. 1 Patients with congenital bleeding disorders reported in the Korean HIRA database from 2010 to 2015.HIRA = Health Insurance Review and Assessment Service, HA = hemophilia A, HB = hemophilia B, vWD = von Willebrand disease.

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  Fig. 2 Trends in the total numbers of patients with bleeding disorders registered in the Korean HIRA database from 2010 to 2015.HIRA = Health Insurance Review and Assessment Service, HA = hemophilia A, HB = hemophilia B, vWD = von Willebrand disease.

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  Fig. 3 Distributions of patients with congenital coagulation disorders according to age during 6 years.HA = hemophilia A, HB = hemophilia B, vWD = von Willebrand disease.

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