title: Clinical characteristics and outcomes of primary immunodeficiencies in Thai children: an 18-year experience from a tertiary care center.
abstract: BACKGROUND
  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.
  
  
  CONCLUSIONS
  These results provide clinical features of PID in Thailand. Knowing these features will lead to prompt diagnosis and appropriate management.
authors.affiliation: Division of Allergy and Immunology, Department of Pediatrics, Siriraj Hospital Mahidol University, 2 Prannok Rd, Bangkoknoi, Bangkok 10700, Thailand.
authors.forename: P
authors.initials: P
authors.lastname: Benjasupattananan
authors.forename: T
authors.initials: T
authors.lastname: Simasathein
authors.forename: P
authors.initials: P
authors.lastname: Vichyanond
authors.forename: V
authors.initials: V
authors.lastname: Leungwedchakarn
authors.forename: N
authors.initials: N
authors.lastname: Visitsunthorn
authors.forename: P
authors.initials: P
authors.lastname: Pacharn
authors.forename: O
authors.initials: O
authors.lastname: Jirapongsananuruk
mesh_terms: D017668:Age of Onset; D016026:Bone Marrow Transplantation; D002648:Child; D002675:Child, Preschool; D004252:DNA Mutational Analysis; D042241:Early Diagnosis; D005260:Female; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D007153:Immunologic Deficiency Syndromes / Q000150:complications / Q000175:diagnosis* / Q000453:epidemiology / Q000503:physiopathology* / Q000628:therapy; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D009894:Opportunistic Infections / Q000150:complications / Q000276:immunology*; D015995:Prevalence; D012141:Respiratory Tract Infections / Q000150:complications / Q000276:immunology*; D013785:Thailand; D016896:Treatment Outcome
chemical_list: D016756:Immunoglobulins, Intravenous