The Prevalence of Marfan Syndrome in Korea

Shin Yi Jang1, Su Ra Seo2, Seung Woo Park1

  • 1Division of Cardiology, Department of Medicine, Heart Vascular Stroke Institute, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.

Insights

The prevalence of Marfan syndrome (MFS) in Korean adults increased from 0.90 to 2.27 per 100,000 people between 2006 and 2013. This rise was particularly notable in adolescent males and females.

Area of Science:

  • Genetics and Hereditary Diseases
  • Epidemiology
  • Public Health

Background:

  • Marfan syndrome (MFS) is a rare genetic disorder affecting connective tissue.
  • Accurate prevalence data is crucial for understanding disease burden and healthcare needs.
  • Previous MFS prevalence studies in Korea are limited, necessitating updated epidemiological assessment.

Purpose of the Study:

  • To determine the age-standardized prevalence of Marfan syndrome (MFS) in the adult Korean population.
  • To analyze trends in MFS prevalence from 2006 to 2013.
  • To identify demographic variations in MFS prevalence within specific age and sex groups.

Main Methods:

  • Utilized data from the Korean National Health Insurance Service (2006-2013).
  • Identified MFS cases using the ICD-10 code Q87.4 for primary diagnoses.
  • Calculated age-standardized prevalence rates based on the 2010 Korean population.

Main Results:

  • The overall age-standardized prevalence of MFS in Korean adults was 0.90 per 100,000 in 2006, rising to 2.27 per 100,000 in 2013.
  • In 2013, MFS prevalence was 2.61 per 100,000 overall for males and 4.32 per 100,000 for males aged 15-19.
  • In 2013, MFS prevalence was 1.92 per 100,000 overall for females and 3.02 per 100,000 for females aged 10-14.

Conclusions:

  • The age-standardized prevalence of Marfan syndrome in Korean adults significantly increased between 2006 and 2013.
  • Adolescent males (15-19 years) and young females (10-14 years) showed a particularly pronounced increase in MFS prevalence.
  • These findings highlight the need for continued surveillance and potentially targeted screening for MFS in the Korean population.

Related Concept Videos

Prevalence and Incidence01:08

Prevalence and Incidence

In statistical epidemiology and health sciences, two essential metrics—prevalence and incidence—are fundamental for understanding disease dynamics within a population. These measures enable public health officials, epidemiologists, and researchers to assess the burden of diseases, allocate resources effectively, and design impactful public health policies and interventions.
Prevalence indicates the proportion of individuals in a population who have a specific disease or health...
2.1K
Aneurysm I: Introduction01:30

Aneurysm I: Introduction

An aortic aneurysm is a localized outpouching or dilation at a weak point in the artery wall. It may involve different parts of the aorta, such as the abdominal aorta, aortic arch, or thoracic aorta.Etiological factorsSeveral disorders are associated with aortic aneurysms.Congenital causes, such as primary connective tissue disorders like Marfan syndrome, impact the integrity and strength of connective tissues, notably affecting the aorta. Marfan syndrome is a genetic disorder that specifically...
563
Mitral Valve Prolapse I: Introduction01:27

Mitral Valve Prolapse I: Introduction

IntroductionThe mitral valve, one of the heart's four valves, regulates blood flow. These valves have flaps that open and close to direct blood properly through the heart and body. During each heartbeat, the flaps open for blood to pass through and seal shut to prevent backflow. Specifically, the mitral valve opens to allow blood flow from the heart's upper left chamber to the lower left chamber. It then closes securely as the lower left chamber contracts to pump blood to the body, preventing...
798
Mitral Stenosis I: Introduction01:22

Mitral Stenosis I: Introduction

Mitral Valve Stenosis (MVS) is a heart condition where the mitral valve narrows, impeding blood circulation from the left atrium to the left ventricle. The etiology and pathophysiology of this condition are multifaceted, leading to a cascade of cardiovascular complications.Causes of Mitral Valve StenosisRheumatic Heart Disease: It is the main cause of mitral valve stenosis, particularly in developing nations. This condition arises from rheumatic fever, an inflammatory illness resulting from...
1.0K
Mitral Stenosis II: Clinical features and Diagnostic Tests01:23

Mitral Stenosis II: Clinical features and Diagnostic Tests

Mitral stenosis is a heart condition in which the mitral valve, which allows blood to flow from the left atrium to the left ventricle, becomes narrowed or stenotic. This narrowing hinders blood flow and leads to clinical symptoms requiring specific medical evaluations and management strategies. The following overview outlines the clinical symptoms, assessments, diagnostic findings, prevention methods, and treatments for mitral stenosis.Clinical ManifestationsDyspnea (shortness of breath): This...
371
Cystic Fibrosis: Pathogenesis01:23

Cystic Fibrosis: Pathogenesis

Cystic fibrosis (CF), an autosomal recessive disorder, significantly affects the function of exocrine glands. This genetically inherited disease is characterized by the production of thick and sticky mucus, which can severely affect various organs and systems in the body.
CF is primarily caused by a genetic mutation in a chromosome 7 gene coding for the cystic fibrosis transmembrane conductance regulator (CFTR) protein. The most common gene mutation leading to CF is the ΔF508 mutation,...
966