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Symptomatic hypogammaglobulinemia in infancy and childhood – clinical outcome and in vitro immune responses

BACKGROUND: Symptomatic hypogammaglobulinemia in infancy and childhood (SHIC), may be an early manifestation of a primary immunodeficiency or a maturational delay in the normal production of immunoglobulins (Ig). We aimed to evaluate the natural course of SHIC and correlate in vitro lymphoproliferat...

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Autores principales: Kidon, Mona Iancovici, Handzel, Zeev T, Schwartz, Rivka, Altboum, Irit, Stein, Michael, Zan-Bar, Israel
Formato: Texto
Lenguaje:English
Publicado: BioMed Central 2004
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC529469/
https://www.ncbi.nlm.nih.gov/pubmed/15498106
http://dx.doi.org/10.1186/1471-2296-5-23
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author Kidon, Mona Iancovici
Handzel, Zeev T
Schwartz, Rivka
Altboum, Irit
Stein, Michael
Zan-Bar, Israel
author_facet Kidon, Mona Iancovici
Handzel, Zeev T
Schwartz, Rivka
Altboum, Irit
Stein, Michael
Zan-Bar, Israel
author_sort Kidon, Mona Iancovici
collection PubMed
description BACKGROUND: Symptomatic hypogammaglobulinemia in infancy and childhood (SHIC), may be an early manifestation of a primary immunodeficiency or a maturational delay in the normal production of immunoglobulins (Ig). We aimed to evaluate the natural course of SHIC and correlate in vitro lymphoproliferative and secretory responses with recovery of immunoglobulin values and clinical resolution. METHODS: Children, older than 1 year of age, referred to our specialist clinic because of recurrent infections and serum immunoglobulin (Ig) levels 2 SD below the mean for age, were followed for a period of 8 years. Patient with any known familial, clinical or laboratory evidence of cellular immunodeficiency or other immunodeficiency syndromes were excluded from this cohort. Evaluation at 6- to 12-months intervals continued up to 1 year after resolution of symptoms. In a subgroup of patients, in vitro lymphocyte proliferation and Ig secretion in response to mitogens was performed. RESULTS: 32 children, 24 (75%) males, 8 (25%) females, mean age 3.4 years fulfilled the inclusion criteria. Clinical presentation: ENT infections 69%, respiratory 81%, diarrhea 12.5%. During follow-up, 17 (53%) normalized serum Ig levels and were diagnosed as transient hypogammaglobulinemia of infancy (THGI). THGI patients did not differ clinically or demographically from non-transient patients, both having a benign clinical outcome. In vitro Ig secretory responses, were lower in hypogammaglobulinemic, compared to normal children and did not normalize concomitantly with serum Ig's in THGI patients. CONCLUSIONS: The majority of children with SHIC in the first decade of life have THGI. Resolution of symptoms as well as normalization of Ig values may be delayed, but overall the clinical outcome is good and the clinical course benign.
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spelling pubmed-5294692004-11-21 Symptomatic hypogammaglobulinemia in infancy and childhood – clinical outcome and in vitro immune responses Kidon, Mona Iancovici Handzel, Zeev T Schwartz, Rivka Altboum, Irit Stein, Michael Zan-Bar, Israel BMC Fam Pract Research Article BACKGROUND: Symptomatic hypogammaglobulinemia in infancy and childhood (SHIC), may be an early manifestation of a primary immunodeficiency or a maturational delay in the normal production of immunoglobulins (Ig). We aimed to evaluate the natural course of SHIC and correlate in vitro lymphoproliferative and secretory responses with recovery of immunoglobulin values and clinical resolution. METHODS: Children, older than 1 year of age, referred to our specialist clinic because of recurrent infections and serum immunoglobulin (Ig) levels 2 SD below the mean for age, were followed for a period of 8 years. Patient with any known familial, clinical or laboratory evidence of cellular immunodeficiency or other immunodeficiency syndromes were excluded from this cohort. Evaluation at 6- to 12-months intervals continued up to 1 year after resolution of symptoms. In a subgroup of patients, in vitro lymphocyte proliferation and Ig secretion in response to mitogens was performed. RESULTS: 32 children, 24 (75%) males, 8 (25%) females, mean age 3.4 years fulfilled the inclusion criteria. Clinical presentation: ENT infections 69%, respiratory 81%, diarrhea 12.5%. During follow-up, 17 (53%) normalized serum Ig levels and were diagnosed as transient hypogammaglobulinemia of infancy (THGI). THGI patients did not differ clinically or demographically from non-transient patients, both having a benign clinical outcome. In vitro Ig secretory responses, were lower in hypogammaglobulinemic, compared to normal children and did not normalize concomitantly with serum Ig's in THGI patients. CONCLUSIONS: The majority of children with SHIC in the first decade of life have THGI. Resolution of symptoms as well as normalization of Ig values may be delayed, but overall the clinical outcome is good and the clinical course benign. BioMed Central 2004-10-21 /pmc/articles/PMC529469/ /pubmed/15498106 http://dx.doi.org/10.1186/1471-2296-5-23 Text en Copyright © 2004 Kidon et al; licensee BioMed Central Ltd. http://creativecommons.org/licenses/by/2.0 This is an open-access article distributed under the terms of the Creative Commons Attribution License ( (http://creativecommons.org/licenses/by/2.0) ), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Research Article
Kidon, Mona Iancovici
Handzel, Zeev T
Schwartz, Rivka
Altboum, Irit
Stein, Michael
Zan-Bar, Israel
Symptomatic hypogammaglobulinemia in infancy and childhood – clinical outcome and in vitro immune responses
title Symptomatic hypogammaglobulinemia in infancy and childhood – clinical outcome and in vitro immune responses
title_full Symptomatic hypogammaglobulinemia in infancy and childhood – clinical outcome and in vitro immune responses
title_fullStr Symptomatic hypogammaglobulinemia in infancy and childhood – clinical outcome and in vitro immune responses
title_full_unstemmed Symptomatic hypogammaglobulinemia in infancy and childhood – clinical outcome and in vitro immune responses
title_short Symptomatic hypogammaglobulinemia in infancy and childhood – clinical outcome and in vitro immune responses
title_sort symptomatic hypogammaglobulinemia in infancy and childhood – clinical outcome and in vitro immune responses
topic Research Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC529469/
https://www.ncbi.nlm.nih.gov/pubmed/15498106
http://dx.doi.org/10.1186/1471-2296-5-23
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