title: Pediatric selective IgM immunodeficiency.
abstract: OBJECTIVE
  Limited information exists on features of pediatric Selective IgM immunodeficiency (SIgMID). Previously published pediatric cases and 2 new cases are reviewed.
  
  
  METHODS
  English literature from PubMed and references from relevant articles were reviewed. Previously reported cases and 2 new cases from an allergy/immunology practice were analyzed.
  
  
  RESULTS
  Forty-nine reported cases of SIgMID presented with respiratory infections (77.6%), gastrointestinal disease (16.3%), skin disease (12.2%), and meningitis (8.2%). Mean serum IgM level was 16.5+/-13.8 mg/dL. Two patients were identified with SIgMID among 6300 active pediatric patients (0.03%) presenting with asthma, vasomotor rhinitis, and recurrent respiratory infections. In the 51 cases reported, none developed lymphoproliferative disease nor evolved into panhypogammaglobulinemia; four fatalities were reported.
  
  
  CONCLUSIONS
  The prevalence of SIgMID in our pediatric population was 0.03%. In general, respiratory infections are the common comorbid conditions. Death and autoimmune disease are uncommon complications of pediatric SIgMID.
authors.affiliation: The Asthma Center, Allergic Disease Associates, PC, 205 N. Broad street, Suite 300, Philadelphia, PA 19107, USA. gpike35@asthmacenter.com
authors.forename: Marc F
authors.initials: MF
authors.lastname: Goldstein
authors.forename: Alex L
authors.initials: AL
authors.lastname: Goldstein
authors.forename: Eliot H
authors.initials: EH
authors.lastname: Dunsky
authors.forename: Donald J
authors.initials: DJ
authors.lastname: Dvorin
authors.forename: George A
authors.initials: GA
authors.lastname: Belecanech
authors.forename: Kfir
authors.initials: K
authors.lastname: Shamir
mesh_terms: D000293:Adolescent; D001323:Autoantibodies / Q000097:blood; D001327:Autoimmune Diseases / Q000150:complications / Q000276:immunology; D001424:Bacterial Infections / Q000150:complications / Q000276:immunology; D002644:Chickenpox / Q000150:complications / Q000276:immunology; D002648:Child; D002675:Child, Preschool; D004406:Dysgammaglobulinemia / Q000097:blood / Q000150:complications / Q000453:epidemiology / Q000276:immunology / Q000628:therapy; D005260:Female; D006388:Hemagglutinins / Q000097:blood; D006801:Humans; D007075:Immunoglobulin M / Q000172:deficiency*; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D007223:Infant; D008297:Male; D009154:Mutation; D039781:PubMed; D012141:Respiratory Tract Infections / Q000150:complications / Q000276:immunology; D012189:Retrospective Studies
chemical_list: D001323:Autoantibodies; D006388:Hemagglutinins; D007075:Immunoglobulin M; D016756:Immunoglobulins, Intravenous