Document Detail


Impaired specific antibody response and increased B-cell population in transient hypogammaglobulinemia of infancy.
MedLine Citation:
PMID:  17165264     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
BACKGROUND: Transient hypogammaglobulinemia of infancy (THI) is a heterogeneous disorder with poorly understood pathophysiology. OBJECTIVES: To better characterize THI and improve understanding of its pathophysiology. METHODS: Twenty-four children with hypogammaglobulinemia defined by an IgG level less than 2 SDs below the mean on 2 occasions, who did not have other immunologic diagnoses, were followed and retrospectively reviewed. RESULTS: The average z-score for IgG level at presentation was -2.4 (mean age, 12 months; median age, 8 months), with a mean level of 254 mg/dL. Thirteen of 24 patients had IgA levels less than 2 SDs below the mean, 5 had IgM levels less than 2 SDs below the mean, and 7 of 23 had elevated IgE levels. Eighteen were followed up until their IgG levels normalized (mean age, 27 months; median age, 23 months), with 12 of 18 normalizing by 24 months and the remainder by 59 months. There was a significant association between presenting IgG z-score and duration of disease (P = .05). Five of the 18 patients had absolute CD19+ B-cell counts greater than the 95% percentile for age (P < .001), and the mean percentage and absolute CD19+ B-cell count across all patients were greater than those of the age-matched controls (P = .02). Most patients had nonprotective titers to Haemophilus influenzae type b vaccine, and one third had nonprotective titers to tetanus vaccine. Twenty patients carried at least one atopic diagnosis, and 13 of those had recurrent wheezing. CONCLUSIONS: THI is associated with a number of immunologic abnormalities beyond just hypogammaglobulinemia. These abnormalities include impaired specific antibody responses and increased proportions of CD19+ B cells and may be suggestive of particular immunologic mechanisms that result in hypogammaglobulinemia.
Authors:
Morna J Dorsey; Jordan S Orange
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Publication Detail:
Type:  Journal Article; Research Support, N.I.H., Extramural    
Journal Detail:
Title:  Annals of allergy, asthma & immunology : official publication of the American College of Allergy, Asthma, & Immunology     Volume:  97     ISSN:  1081-1206     ISO Abbreviation:  Ann. Allergy Asthma Immunol.     Publication Date:  2006 Nov 
Date Detail:
Created Date:  2006-12-14     Completed Date:  2007-01-17     Revised Date:  2007-11-14    
Medline Journal Info:
Nlm Unique ID:  9503580     Medline TA:  Ann Allergy Asthma Immunol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  590-5     Citation Subset:  IM    
Affiliation:
Division of Immunology, Children's Hospital Boston, Harvard Medical School, Boston, Massachusetts, USA. MDorsey@hsc.usf.edu
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MeSH Terms
Descriptor/Qualifier:
Agammaglobulinemia / blood,  immunology*
Antibody Formation / immunology*
B-Lymphocytes / cytology,  immunology*
Child, Preschool
Humans
Immunity, Cellular / immunology
Immunoglobulins / blood
Infant
T-Lymphocyte Subsets / cytology,  immunology
Grant Support
ID/Acronym/Agency:
T32-AI-007512/AI/NIAID NIH HHS
Chemical
Reg. No./Substance:
0/Immunoglobulins
Comments/Corrections
Erratum In:
Ann Allergy Asthma Immunol. 2006 Dec;97(6):819

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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