A clinical case of autosomal recessive agammaglobulinemia with B-cell deficiency
Background. Primary agammaglobulinemia is the result of specific changes in B-cells that lead to low antibody production. A preliminary diagnosis is established if there is a history of frequent bacterial infections (otitis media, sinusitis, skin abscesses), including severe course, in some cases ca...
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Association of Paediatric Allergists and Immunologists of Russia (APAIR)
2024-01-01
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| Series: | Аллергология и Иммунология в Педиатрии |
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| Online Access: | https://adair.elpub.ru/jour/article/view/120 |
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| author | E. V. Negodnova M. S. Iskandyarova E. N. Tyagusheva O. A. Radaeva G. V. Fominova |
| author_facet | E. V. Negodnova M. S. Iskandyarova E. N. Tyagusheva O. A. Radaeva G. V. Fominova |
| author_sort | E. V. Negodnova |
| collection | DOAJ |
| description | Background. Primary agammaglobulinemia is the result of specific changes in B-cells that lead to low antibody production. A preliminary diagnosis is established if there is a history of frequent bacterial infections (otitis media, sinusitis, skin abscesses), including severe course, in some cases caused by opportunistic flora and atypical mycobacteria; low levels of immunoglobulins. The main symptoms of primary immunodeficiency in a child from this clinical example were frequent recidivating bronchial obstruction with the development of pneumonia.Presentation of the clinical case.The publication presents a clinical case of autosomal recessive agammaglobulinemia with B-cell deficiency in a child of 2 years, 7 months. During the follow-up period from 4 months to 2 months, 7 months, the child had 3 episodes of pneumonia, 3 episodes of purulent otitis media. The child repeatedly underwent inpatient treatment, where he received broad-spectrum antibiotics as treatment. Based on the examination (IgA (0.02 g/l), IgG (0.3 g/l), IgM (0.07 g/l) and the absence of CD19+ cells), the diagnosis of “Primary immunodeficiency, agammaglobulinemia” was made, which was subsequently confirmed by the RDC of Moscow. From the moment of diagnosis, the child receives intravenous immunoglobulins at a dose of 7.5 g. and antibacterial therapy.Conclusion. Early recognition and diagnosis of these conditions is crucial to improve outcomes and prevent complications. |
| format | Article |
| id | doaj-art-7a14e5e5d97e4b7aaa2b8b55a948e3df |
| institution | Matheson Library |
| issn | 2500-1175 2712-7958 |
| language | Russian |
| publishDate | 2024-01-01 |
| publisher | Association of Paediatric Allergists and Immunologists of Russia (APAIR) |
| record_format | Article |
| series | Аллергология и Иммунология в Педиатрии |
| spelling | doaj-art-7a14e5e5d97e4b7aaa2b8b55a948e3df2025-08-03T13:01:11ZrusAssociation of Paediatric Allergists and Immunologists of Russia (APAIR)Аллергология и Иммунология в Педиатрии2500-11752712-79582024-01-0104515510.53529/2500-1175-2023-4-51-5599A clinical case of autosomal recessive agammaglobulinemia with B-cell deficiencyE. V. Negodnova0M. S. Iskandyarova1E. N. Tyagusheva2O. A. Radaeva3G. V. Fominova4N. P. Ogarev Mordovia State UniversityN. P. Ogarev Mordovia State UniversityN. P. Ogarev Mordovia State UniversityN. P. Ogarev Mordovia State UniversityN. P. Ogarev Mordovia State UniversityBackground. Primary agammaglobulinemia is the result of specific changes in B-cells that lead to low antibody production. A preliminary diagnosis is established if there is a history of frequent bacterial infections (otitis media, sinusitis, skin abscesses), including severe course, in some cases caused by opportunistic flora and atypical mycobacteria; low levels of immunoglobulins. The main symptoms of primary immunodeficiency in a child from this clinical example were frequent recidivating bronchial obstruction with the development of pneumonia.Presentation of the clinical case.The publication presents a clinical case of autosomal recessive agammaglobulinemia with B-cell deficiency in a child of 2 years, 7 months. During the follow-up period from 4 months to 2 months, 7 months, the child had 3 episodes of pneumonia, 3 episodes of purulent otitis media. The child repeatedly underwent inpatient treatment, where he received broad-spectrum antibiotics as treatment. Based on the examination (IgA (0.02 g/l), IgG (0.3 g/l), IgM (0.07 g/l) and the absence of CD19+ cells), the diagnosis of “Primary immunodeficiency, agammaglobulinemia” was made, which was subsequently confirmed by the RDC of Moscow. From the moment of diagnosis, the child receives intravenous immunoglobulins at a dose of 7.5 g. and antibacterial therapy.Conclusion. Early recognition and diagnosis of these conditions is crucial to improve outcomes and prevent complications.https://adair.elpub.ru/jour/article/view/120primary immunodeficiencyagammaglobulinemiaautosomal recessive formchildrenclinical case |
| spellingShingle | E. V. Negodnova M. S. Iskandyarova E. N. Tyagusheva O. A. Radaeva G. V. Fominova A clinical case of autosomal recessive agammaglobulinemia with B-cell deficiency Аллергология и Иммунология в Педиатрии primary immunodeficiency agammaglobulinemia autosomal recessive form children clinical case |
| title | A clinical case of autosomal recessive agammaglobulinemia with B-cell deficiency |
| title_full | A clinical case of autosomal recessive agammaglobulinemia with B-cell deficiency |
| title_fullStr | A clinical case of autosomal recessive agammaglobulinemia with B-cell deficiency |
| title_full_unstemmed | A clinical case of autosomal recessive agammaglobulinemia with B-cell deficiency |
| title_short | A clinical case of autosomal recessive agammaglobulinemia with B-cell deficiency |
| title_sort | clinical case of autosomal recessive agammaglobulinemia with b cell deficiency |
| topic | primary immunodeficiency agammaglobulinemia autosomal recessive form children clinical case |
| url | https://adair.elpub.ru/jour/article/view/120 |
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