Pediatric Selective IgM Immunodeficiency

• Published in: Clinical & developmental immunology Vol. 2008; no. 2008; pp. 1 - 10
• Main Authors: Goldstein, Marc F., Goldstein, Alex L., Dunsky, Eliot H., Dvorin, Donald J., Belecanech, George A., Shamir, Kfir
• Format: Journal Article
• Language: English
• Published: Cairo, Egypt Hindawi Puplishing Corporation 01.01.2008; Hindawi Publishing Corporation; Hindawi Limited
• Subjects: Adolescent; Asthma; Autoantibodies - blood; Autoimmune Diseases - complications; Autoimmune Diseases - immunology; Bacterial Infections - complications; Bacterial Infections - immunology; Celiac disease; Chickenpox - complications; Chickenpox - immunology; Child; Child, Preschool; Comparative analysis; Dermatitis; Dysgammaglobulinemia - blood; Dysgammaglobulinemia - complications; Dysgammaglobulinemia - epidemiology; Dysgammaglobulinemia - immunology; Dysgammaglobulinemia - therapy; Ear diseases; Female; Hemagglutinins - blood; Humans; Immunoglobulin M - deficiency; Immunoglobulins, Intravenous - therapeutic use; Immunology; Infant; Inflammatory bowel disease; Male; Meningitis; Mutation; PubMed; Respiratory Tract Infections - complications; Respiratory Tract Infections - immunology; Retrospective Studies; Review; Thyroid diseases; Viral infections
• ISSN: 1740-2522; 2314-8861; 1740-2530; 2314-7156
• DOI: 10.1155/2008/624850

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.