Prevalence of primary immunodeficiency in Korea

Area of Science:

• Immunology
• Epidemiology
• Pediatrics

Background:

• Primary immunodeficiencies (PID) are a group of rare genetic disorders affecting the immune system.
• Epidemiological data on PID prevalence in Korea were previously limited.
• Understanding PID distribution is crucial for healthcare planning and early diagnosis.

Purpose of the Study:

• To establish the first national registry for primary immunodeficiencies in Korea.
• To determine the period prevalence and epidemiological characteristics of PID in Korean children.
• To identify the most common types and initial manifestations of PID in the studied population.

Main Methods:

• Data collection from 23 major Korean hospitals for patients under 19 years of age.
• Inclusion of 152 patients diagnosed with PID between 2001 and 2005.
• Analysis of patient data to determine PID types, frequencies, and clinical presentations.

Main Results:

• The period prevalence of PID in Korea in 2005 was 11.25 per million children.
• Antibody deficiencies constituted the largest group (53.3%), followed by phagocytic disorders (28.9%).
• Congenital agammaglobulinemia and selective IgA deficiency were the most frequent antibody deficiencies; pneumonia was the most common initial symptom.

Conclusions:

• This study provides the first comprehensive epidemiological data on PID in Korea.
• The findings highlight the significant burden of antibody deficiencies among Korean children with PID.
• Accurate estimations of PID patient numbers are now possible, aiding future research and clinical management.