title: [Overview of clinical occurrence of primary immunodeficiency disorders in children].
abstract: OBJECTIVE
  More than one hundred primary immunodeficiency disorders have been discovered so far. But the incidence of these disorders in our country is still not clear, so we analyzed the clinical data of 93 children with primary immunodeficiency disorders seen in our hospital in recent 30 years to understand the occurrence of primary immunodeficiency disorders in children, to promote the clinicians to become familiar with these disorders, to improve the nationwide registry system and to establish the basis for the treatment and prevention in future.
  
  
  METHODS
  To analyze the constituent ratio of the 93 children with primary immunodeficiency disorders seen in our hospital from 1974 to 2003, diagnostic and classification criteria were set by taking the proposal by International Union of Immunological Societies (IUIS) PID classification committee in 2003 into account. All the data were analyzed retrospectively.
  
  
  RESULTS
  In the 93 children with primary immunodeficiency disorders, antibody deficiencies were the most frequent (39.8%) finding, followed by combined immunodeficiency, combined T- and B-cell disorders (22.6%), and T lymphocytic deficiencies alone (14.0%). Immunodeficiency with other major defects accounted for 12.9%, phagocytic disorders 9.7%, and complement deficiencies 1.1%. Thus, there seemed to be a tendency that the incidence increased with time. The incidence of these disorders has increased significantly as shown by 50 diagnosed cases in children with these disorders since 1996. Sixteen children died, with the highest mortality occurred with combined immunodeficiency. Seven children developed bronchiectasis. Two children suffered from persistent diarrhea while one of the two was complicated with persistent intestinal fistula. One child developed juvenile rheumatoid arthritis, another one with granulocytopenia and iridocyclitis, and the other with allergic purpura. The boys: girls ratio for all disorders was 3:1. The age of onset ranged from 10 days to 37 years of age.
  
  
  CONCLUSIONS
  There are vast variety of primary immunodeficiency disorders in our area and antibody deficiency is the most common abnormality. Combined immunodeficiency has early onset age and high mortality rate. With the great improvement of the diagnostic techniques, these disorders have become a group of important disorders and all the clinicians should pay great attention to these disorders.
authors.affiliation: Department of Immunology/Oncology, Shanghai Institute for Pediatric Research, Xinhua Hospital, Shanghai Jiaotong University Medical School, Shanghai 200092, China.
authors.forename: Hui-jun
authors.initials: HJ
authors.lastname: Zhao
authors.forename: Tong-xin
authors.initials: TX
authors.lastname: Chen
authors.forename: Yi-qun
authors.initials: YQ
authors.lastname: Hao
authors.forename: Yun-fang
authors.initials: YF
authors.lastname: Zhou
authors.forename: Da-ming
authors.initials: DM
authors.lastname: Ying
mesh_terms: D000293:Adolescent; D000328:Adult; D000361:Agammaglobulinemia / Q000453:epidemiology / Q000276:immunology; D002648:Child; D002675:Child, Preschool; D002681:China / Q000453:epidemiology; D005260:Female; D006761:Hospitals; D006801:Humans; D007153:Immunologic Deficiency Syndromes / Q000145:classification / Q000175:diagnosis / Q000453:epidemiology* / Q000276:immunology*; D015994:Incidence; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D012042:Registries; D012189:Retrospective Studies; D012307:Risk Factors; D016511:Severe Combined Immunodeficiency / Q000453:epidemiology / Q000276:immunology; D012737:Sex Factors; D013997:Time Factors