New Laboratory Findings in Turkish Patients with Transient Hypogammaglobulinemia of Infancy

Transient hypogammaglobulinemia (THI) of infancy is a common primary immunodeficiency usually resolves by 3 years of age. In this study, clinical, immunological data and outcome of 101 retrospectively diagnosed THI patients were evaluated. Majority of them suffered from recurrent respiratory infecti...

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Main Authors: Neslihan Edeer Karaca, Guzide Aksu, Nesrin Gulez, Basak Yildiz, Elif Azarsiz, Necil Kutukculer
Format: Article
Language:English
Published: Tehran University of Medical Sciences 2010-12-01
Series:Iranian Journal of Allergy, Asthma and Immunology
Subjects:
Online Access:https://ijaai.tums.ac.ir/index.php/ijaai/article/view/350
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author Neslihan Edeer Karaca
Guzide Aksu
Nesrin Gulez
Basak Yildiz
Elif Azarsiz
Necil Kutukculer
author_facet Neslihan Edeer Karaca
Guzide Aksu
Nesrin Gulez
Basak Yildiz
Elif Azarsiz
Necil Kutukculer
author_sort Neslihan Edeer Karaca
collection DOAJ
description Transient hypogammaglobulinemia (THI) of infancy is a common primary immunodeficiency usually resolves by 3 years of age. In this study, clinical, immunological data and outcome of 101 retrospectively diagnosed THI patients were evaluated. Majority of them suffered from recurrent respiratory infections (70.3%). Initial IgG, IgM and IgA levels were 446.7±121.5, 67.5±32.8, and 25.6±16.8 mg/dl, respectively. Patients who had lower IgG levels on admission reached normal IgG levels earlier than others. Infants who were retarded to reach age-related normal levels for IgM and IgA were found to have higher CD3+CD8+ T cells on admission. During immunoglobulin abnormalities, mean lymphocyte subset percentages and absolute counts were normal. Mean percentage of CD19+CD27+ memory B cells was 3.4±1.4% which is not significantly different from healthy children. Most of the children had protective antibody responses to tetanus (87%) and Haemophilus influenzae type B (85.7%) vaccines. Patients with low anti-tetanus responses had higher initial natural killer (NK) cell percentages probably due to recurrent viral infections or relative dominance of innate responses. Follow-up of patients with initially high NK were found to have longer duration of deficiency hence these patients' recoveries were delayed. During follow-up, 91/101 (90.1%) children produced normali levels of IgG at the end of 29.2 ± 15.2 months. The results of this study indicate that some children will achieve normal levels of IgG within 30 months of age, and some will remain IgG subclass or IgA deficient. Determination of increased NK percentages in patients with non-protective vaccine response and normal percentages of memory B cells are noteworthy novel findings.
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spelling doaj.art-ef0c5e7c1ed14ba2896621db601d6ee42022-12-22T01:21:12ZengTehran University of Medical SciencesIranian Journal of Allergy, Asthma and Immunology1735-15021735-52492010-12-01No. 4350New Laboratory Findings in Turkish Patients with Transient Hypogammaglobulinemia of InfancyNeslihan Edeer Karaca0Guzide Aksu1Nesrin Gulez2Basak Yildiz3Elif Azarsiz4Necil Kutukculer5Department of Pediatric Immunology, Ege University Medical School, Izmir, TurkeyDepartment of Pediatric Immunology, Ege University Medical School, Izmir, TurkeyDepartment of Pediatric Immunology, Ege University Medical School, Izmir, TurkeyDepartment of Pediatric Immunology, Ege University Medical School, Izmir, TurkeyDepartment of Pediatric Immunology, Ege University Medical School, Izmir, TurkeyDepartment of Pediatric Immunology, Ege University Medical School, Izmir, TurkeyTransient hypogammaglobulinemia (THI) of infancy is a common primary immunodeficiency usually resolves by 3 years of age. In this study, clinical, immunological data and outcome of 101 retrospectively diagnosed THI patients were evaluated. Majority of them suffered from recurrent respiratory infections (70.3%). Initial IgG, IgM and IgA levels were 446.7±121.5, 67.5±32.8, and 25.6±16.8 mg/dl, respectively. Patients who had lower IgG levels on admission reached normal IgG levels earlier than others. Infants who were retarded to reach age-related normal levels for IgM and IgA were found to have higher CD3+CD8+ T cells on admission. During immunoglobulin abnormalities, mean lymphocyte subset percentages and absolute counts were normal. Mean percentage of CD19+CD27+ memory B cells was 3.4±1.4% which is not significantly different from healthy children. Most of the children had protective antibody responses to tetanus (87%) and Haemophilus influenzae type B (85.7%) vaccines. Patients with low anti-tetanus responses had higher initial natural killer (NK) cell percentages probably due to recurrent viral infections or relative dominance of innate responses. Follow-up of patients with initially high NK were found to have longer duration of deficiency hence these patients' recoveries were delayed. During follow-up, 91/101 (90.1%) children produced normali levels of IgG at the end of 29.2 ± 15.2 months. The results of this study indicate that some children will achieve normal levels of IgG within 30 months of age, and some will remain IgG subclass or IgA deficient. Determination of increased NK percentages in patients with non-protective vaccine response and normal percentages of memory B cells are noteworthy novel findings.https://ijaai.tums.ac.ir/index.php/ijaai/article/view/350HypogammaglobulinemiaInfancyOutcome
spellingShingle Neslihan Edeer Karaca
Guzide Aksu
Nesrin Gulez
Basak Yildiz
Elif Azarsiz
Necil Kutukculer
New Laboratory Findings in Turkish Patients with Transient Hypogammaglobulinemia of Infancy
Iranian Journal of Allergy, Asthma and Immunology
Hypogammaglobulinemia
Infancy
Outcome
title New Laboratory Findings in Turkish Patients with Transient Hypogammaglobulinemia of Infancy
title_full New Laboratory Findings in Turkish Patients with Transient Hypogammaglobulinemia of Infancy
title_fullStr New Laboratory Findings in Turkish Patients with Transient Hypogammaglobulinemia of Infancy
title_full_unstemmed New Laboratory Findings in Turkish Patients with Transient Hypogammaglobulinemia of Infancy
title_short New Laboratory Findings in Turkish Patients with Transient Hypogammaglobulinemia of Infancy
title_sort new laboratory findings in turkish patients with transient hypogammaglobulinemia of infancy
topic Hypogammaglobulinemia
Infancy
Outcome
url https://ijaai.tums.ac.ir/index.php/ijaai/article/view/350
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