Journal of Clinical Immunology

, Volume 25, Issue 2, pp 162–173 | Cite as

Akhil Kakroo

  • Wen-I Lee
  • Ming-Ling Kuo
  • Jing-Long Huang
  • Syh-Jae Lin
  • Cheng-Jang Wu
Article

Abstract

Recent advances in immunologic techniques have lead to increased recognition of primary immunodeficiencies. A review of patients with suspected immunodeficiencies in a Taiwan tertiary hospital from January 1985 to October 2004 and molecular/genetic analyses done on some patients were investigated. Of the 403 patients selected based on the International Classification of Disease, Ninth Revision, 37 patients with PID (8 females and 29 males) were identified: 17 (46%) with antibody production deficiencies, nine (24%) with defective phagocyte function, four (11%) with combined B and T cell immunodeficiencies, seven (19%) with T cell deficiencies, but none with primary complement deficiencies. Those with secondary immunodeficiencies were excluded from the study. Recurrent sinopulmonary infections (62%) were the most common clinical manifestation, followed by sepsis (57%), severe skin infection (40%), splenomagaly/hepatomegaly (27%), central nervous system dysfunction (22%), chronic diarrhea (22%), and failure to thrive (19%). Seven (19%) patients died, five of infections, one of disseminated intravascular coagulopathy and one of hepatocellular carcinoma. Six novel mutations were found from 11 agreed patients. This is the first report on primary immunodeficiencies in Taiwan covering a 20-year period.

Primary immunodeficiency agammaglobulinemia recurrent sinopulmonary infections unrelated umbilical cord stem cell transplantation Taiwan 

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Copyright information

© Springer Science + Business Media, Inc. 2005

Authors and Affiliations

  • Wen-I Lee
    • 1
    • 2
    • 4
  • Ming-Ling Kuo
    • 3
    • 4
  • Jing-Long Huang
    • 2
  • Syh-Jae Lin
    • 2
  • Cheng-Jang Wu
    • 2
  1. 1.Graduate Institute of Clinical Medical SciencesChang Gung University, Chang Gung Children’s HospitalTaiwan
  2. 2.Department of Pediatric Allergy, Immunology and RheumatologyChang Gung University, Chang Gung Children’s HospitalTaiwan
  3. 3.Department of Microbiology and ImmunologyGraduate Institute of Basic Medical Sciences, Chang Gung University, Chang Gung Children’s HospitalTaiwan
  4. 4.Department of Pediatric Allergy, Immunology, and RheumatologyChang Gung University and Chang Gung Children’s HospitalKwei-ShanTaiwan

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