Pediatric selective IgM immunodeficiency.
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.
Goldstein
Marc F
MF
The Asthma Center, Allergic Disease Associates, PC, 205 N. Broad street, Suite 300, Philadelphia, PA 19107, USA. gpike35@asthmacenter.com
Goldstein
Alex L
AL
Dunsky
Eliot H
EH
Dvorin
Donald J
DJ
Belecanech
George A
GA
Shamir
Kfir
K
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
D001323:Autoantibodies; D006388:Hemagglutinins; D007075:Immunoglobulin M; D016756:Immunoglobulins, Intravenous