Symptomatic hypogammaglobulinemia in infancy and childhood – clinical outcome and in vitro immune responses
<p>Abstract</p> <p>Background</p> <p>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...
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| Format: | Article |
| Language: | English |
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BMC
2004-10-01
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| Series: | BMC Family Practice |
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| Online Access: | http://www.biomedcentral.com/1471-2296/5/23 |
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| author | Stein Michael Altboum Irit Schwartz Rivka Handzel Zeev T Kidon Mona Zan-Bar Israel |
| author_facet | Stein Michael Altboum Irit Schwartz Rivka Handzel Zeev T Kidon Mona Zan-Bar Israel |
| author_sort | Stein Michael |
| collection | DOAJ |
| description | <p>Abstract</p> <p>Background</p> <p>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.</p> <p>Methods</p> <p>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.</p> <p>Results</p> <p>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.</p> <p>Conclusions</p> <p>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.</p> |
| first_indexed | 2024-12-12T16:56:00Z |
| format | Article |
| id | doaj.art-271cf642afd24da78cc70d3b86d98f27 |
| institution | Directory Open Access Journal |
| issn | 1471-2296 |
| language | English |
| last_indexed | 2024-12-12T16:56:00Z |
| publishDate | 2004-10-01 |
| publisher | BMC |
| record_format | Article |
| series | BMC Family Practice |
| spelling | doaj.art-271cf642afd24da78cc70d3b86d98f272022-12-22T00:18:13ZengBMCBMC Family Practice1471-22962004-10-01512310.1186/1471-2296-5-23Symptomatic hypogammaglobulinemia in infancy and childhood – clinical outcome and in vitro immune responsesStein MichaelAltboum IritSchwartz RivkaHandzel Zeev TKidon MonaZan-Bar Israel<p>Abstract</p> <p>Background</p> <p>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.</p> <p>Methods</p> <p>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.</p> <p>Results</p> <p>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.</p> <p>Conclusions</p> <p>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.</p>http://www.biomedcentral.com/1471-2296/5/23Humoral immunodeficiencyTransient hypogammaglobulinemiaMitogens |
| spellingShingle | Stein Michael Altboum Irit Schwartz Rivka Handzel Zeev T Kidon Mona Zan-Bar Israel Symptomatic hypogammaglobulinemia in infancy and childhood – clinical outcome and in vitro immune responses BMC Family Practice Humoral immunodeficiency Transient hypogammaglobulinemia Mitogens |
| 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 | Humoral immunodeficiency Transient hypogammaglobulinemia Mitogens |
| url | http://www.biomedcentral.com/1471-2296/5/23 |
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