Prevalence of Marfan syndrome from 2006 to 2013 in the Korean

Purpose The aim of this study is to assess changes in the causes of marfan syndrome between 2006 and 2013 in Korean adults.

Methods Data were collected from the National Health Insurance Corporation in Korea from 2006 through 2013. The data consisted of primary diagnoses related to marfan syndrome (Q87.4) diagnosed according to the 10th revision of the International Statistical Classification of Diseases and Related Health Problems. The age-standardized prevalence of congenital heart diseases in adults was calculated with the direct method using the estimated Korean population in 2010 as the reference.

Results Overall, the age-standardized cumulative prevalence of marfan syndrome was 0.92 per 100,000 persons in 2006 and 2.32 in 2013. For male in 2006, 0.77 in 0-4 years, 1.41 in 5-9 years, 2.63 in 10-14 years, 3.13 in 15-19 years, 1.19 in 20-44 years, 0.37 in 45-64 years, and 0.30 in older than 65 years. For male in 2013, 0.34 in 0-4 years, 3.31 in 5-9 years, 3.95 in 10-14 years, 4.35 in 15-19 years, 3.50 in 20-44 years, 1.80 in 45-64 years, and 0.49 in over 65 years. For female in 2006, 0.56 in 0-4 years, 1.19 in 5-9 years, 2.23 in 10-14 years, 1.61 in 15-19 years, 0.77 in 20-44 years, 0.35 in 45-64 years, and 0 in older than 65 years. For female in 2013, 1.17 in 0-4 years, 2.27 in 5-9 years, 3.08 in 10-14 years, 2.40 in 15-19 years, 2.35 in 20-44 years, 1.88 in 45-64 years, and 0.24 in over 65 years.

Conclusions The overall age-standardized cumulative prevalence of marfan syndrome increased between 2006 and 2013, especially in 15-19 years males and 10-14 years females.