Department of Pediatrics, Kyung Hee University School of Medicine, Seoul, Korea.
Department of Laboratory Medicine, Kyung Hee University School of Medicine, Seoul, Korea.
J Korean Med Sci. 2020 Oct 12;35(39):e350. doi: 10.3346/jkms.2020.35.e350.
Except for data in the Korea Hemophilia Foundation Registry, little is known of the epidemiology of congenital bleeding disorders in Korea.
Data were obtained from the Korean Health Insurance Review and Assessment Service (HIRA) database.
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%).
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.
除了韩国血友病基金会登记处的数据外，对韩国先天性出血性疾病的流行病学知之甚少。
数据来自韩国健康保险审查与评估服务（HIRA）数据库。
2010 年至 2015 年，韩国 HIRA 数据库中有 2029 例先天性出血性疾病患者：38%（n=775）为甲型血友病（HA），25%（n=517）为血管性血友病（vWD），7%（n=132）为乙型血友病（HB），25%（n=513）为其他少见因子缺乏症。HA 和 HB 的估计年龄标准化发病率（ASR）分别为 1.78-3.15/10 万和 0.31-0.51/10 万。vWD 的估计 ASR 为 1.38-1.95/10 万。HA 的估计 ASR 随时间呈上升趋势，但新发病例数并未增加。大多数先天性出血性疾病患者年龄小于 19 岁（47.8%），且大多数登记在京畿道（22.1%）和首尔（19.2%）。
这是韩国首例全国性先天性出血性疾病人群研究。本研究提供了数据，将有助于更准确地估计 vWD 患者人数。这些信息将有助于推进先天性出血性疾病的全面护理。我们需要继续获取患者更详细的信息，以改善这些疾病的管理。
