Clinical Characteristics and Outcomes of Primary Immunodeficiencies in Thai Children: An 18-year Experience from a Tertiary Care Center

Introduction  Early diagnosis and treatment are keys to improve survival of patients with primary immunodeficiency diseases (PID). The clinical characteristics of these patients in Thailand were not well defined. Objective  This study aimed to determine the clinical characteristics and outcomes of patients with PID in Thailand. Methods  Medical records of PID patients in the past 18 years were reviewed. Results  Sixty-seven children were registered. Antibody deficiencies were the most common PID (52.2%), followed by combined T cell and B cell immunodeficiencies (25.4%), other well-defined immunodeficiency syndromes (11.9%), and phagocytic defects (10.4%). The most common presentations of antibody deficiencies, combined T cell and B cell immunodeficiencies, and phagocytic defects were infection in the upper respiratory tract (74.3%), gastrointestinal tract (82.4%), and skin (85.7%), respectively. The highest mortality rate (52.9%) was found in severe combined immunodeficiency. Conclusion  These results provide clinical features of PID in Thailand. Knowing these features will lead to prompt diagnosis and appropriate management.