title: Clinical and immunological features of common variable immunodeficiency in China.
abstract: BACKGROUND
  Common variable immunodeficiency (CVID) is one of the most common symptomatic primary immunodeficiency syndromes. The purpose of this article was to broaden our knowledge about CVID for better diagnosis and treatment.
  
  
  METHODS
  Clinical and immunological features of 40 Chinese patients with CVID were analyzed retrospectively.
  
  
  RESULTS
  The median age at onset was 11-year-old (range 4-51 years). The median age at diagnosis was 14.5-year-old (range 5-66 years). The average time of delay in diagnosis was 5.3 years (range 1-41 years). The most common main complaint was fever due to infections (35 cases, 87.5%). Pneumonia (28 cases, 70%) was the most common type of infections. Bronchiectasis was present in 6 patients (15%). Autoimmune disease was detected in 6 cases of CVID, and malignancy in 2 cases. The median total serum levels of IgG, IgA, and IgM at diagnosis were 1.07 g/L, 0.07 g/L, and 0.28 g/L, respectively. The percentages of CD3- /CD19 + B-cells were 1%-3.14%.
  
  
  CONCLUSIONS
  Infection is the most frequent presentation of CVID. Patients with unexplainable infections should receive further examination including serum immunoglobulin (Ig) and lymphocyte subset analysis. Regular and sufficient substitution with Ig is recommended.
authors: Lian-Jun
authors: LJ
authors: Lin
authors: Yu-Chuan
authors: YC
authors: Wang
authors: Department of Geriatrics, Peking University First Hospital, Beijing 100034, China.
authors: Xin-Min
authors: XM
authors: Liu
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D001987:Bronchiectasis / Q000188:drug therapy / Q000276:immunology / Q000473:pathology; D002648:Child; D002675:Child, Preschool; D002681:China; D017074:Common Variable Immunodeficiency / Q000188:drug therapy / Q000276:immunology* / Q000473:pathology*; D006801:Humans; D007136:Immunoglobulins / Q000378:metabolism; D016756:Immunoglobulins, Intravenous; D008875:Middle Aged; D055815:Young Adult
chemical_list: D007136:Immunoglobulins; D016756:Immunoglobulins, Intravenous
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