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Outcome of hypogammaglobulinemia in children: immunoglobulin levels as predictors.

Abstract
We evaluated 131 children (M=88, F=43) with hypogammaglobulinemia. Data was analyzed mainly for delineating predictor factors for outcome. The distance from the lower limit of normal (-2SD) for any single measurement of immunoglobulins (Ig) was calculated and transformed into Ig scores. Mean age and duration of follow-up were 5.06 ± 4.05 and 3.7 ± 3.03 years, respectively. The diagnoses were: 22 CVID, 16 IgA deficiency, 33 transient hypogammaglobulinemia of childhood (THC), 3 selective IgM deficiency and 57 unclassified hypogammaglobulinemia (UCH). Low IgA scores (<-0.124) at presentation were indicative of subsequent development of IgA deficiency or CVID, whereas low IgM score (<-0.038) pointed towards more severe and persistent phenotypes. Combination of low IgM score between 2 and 5 years, impaired antibody response and low B cell counts enabled us to predict persistence of hypogammaglobulinemia beyond 5 years (specificity=90.5% and PPV=94.9%) and chronic lung disease (sensitivity=90.4% and specificity=68.3%). The set of criteria including low IgM scores, impaired antibody response and low B cell counts provided a high predictive value in detecting those with persistent hypogammaglobulinemia.
AuthorsAhmet Ozen, Safa Baris, Elif Karakoc-Aydiner, Cevdet Ozdemir, Nerin Nadir Bahceciler, Isil Berat Barlan
JournalClinical immunology (Orlando, Fla.) (Clin Immunol) Vol. 137 Issue 3 Pg. 374-83 (Dec 2010) ISSN: 1521-7035 [Electronic] United States
PMID20851686 (Publication Type: Journal Article)
CopyrightCopyright © 2010 Elsevier Inc. All rights reserved.
Chemical References
  • Biomarkers
  • Immunoglobulins
Topics
  • Adolescent
  • Agammaglobulinemia (diagnosis, immunology)
  • Biomarkers
  • Child
  • Child, Preschool
  • Female
  • Humans
  • Immunoglobulins (blood)
  • Male
  • Predictive Value of Tests
  • Prognosis
  • Retrospective Studies

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