A case of primary immunodeficiency with immune dysregulation: features of clinical manifestation and diagnostic difficulties

The article describes a case of a rare primary immunodeficiency with immune dysregulation — CD25 deficiency with manifestation in the neonatal period in the form of severe chronic diarrhea, susceptibility to viral, bacterial, and fungal infections, autoimmune manifestations, malabsorption syndrome,...

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Main Authors: E. N. Serebryakova, T. V. Shilova, A. S. Kozhevnikov, E. I. Kilmukhametova
Format: Article
Language:Russian
Published: LLC "Publishing House OKI" 2024-06-01
Series:Антибиотики и Химиотерапия
Subjects:
Online Access:https://www.antibiotics-chemotherapy.ru/jour/article/view/1125
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author E. N. Serebryakova
T. V. Shilova
A. S. Kozhevnikov
E. I. Kilmukhametova
author_facet E. N. Serebryakova
T. V. Shilova
A. S. Kozhevnikov
E. I. Kilmukhametova
author_sort E. N. Serebryakova
collection DOAJ
description The article describes a case of a rare primary immunodeficiency with immune dysregulation — CD25 deficiency with manifestation in the neonatal period in the form of severe chronic diarrhea, susceptibility to viral, bacterial, and fungal infections, autoimmune manifestations, malabsorption syndrome, hypotrophy, skin lesions, confirmed by a homozygous mutation in the IL2-RA gene detected by DNA analysis. The article discusses the role of IL2-RA in the maturation and differentiation of T-lymphocytes, modern approaches to the classification of primary immunodeficiencies with immune dysregulation, principles of diagnosis and treatment of CD25 deficiency, as well as analyzes and compares other cases of CD25 deficiency described in the literature.
format Article
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institution Kabale University
issn 0235-2990
language Russian
publishDate 2024-06-01
publisher LLC "Publishing House OKI"
record_format Article
series Антибиотики и Химиотерапия
spelling doaj-art-ceea7e4feeb6439eaec3d47ba530625a2025-08-20T03:59:08ZrusLLC "Publishing House OKI"Антибиотики и Химиотерапия0235-29902024-06-01693-4515710.37489/0235-2990-2024-69-3-4-51-571030A case of primary immunodeficiency with immune dysregulation: features of clinical manifestation and diagnostic difficultiesE. N. Serebryakova0T. V. Shilova1A. S. Kozhevnikov2E. I. Kilmukhametova3South Ural State Medical University of the Ministry of Health of the Russian Federation; Chelyabinsk Regional Children's Clinical HospitalSouth Ural State Medical University of the Ministry of Health of the Russian Federation; Chelyabinsk Regional Children's Clinical HospitalSouth Ural State Medical University of the Ministry of Health of the Russian Federation; Chelyabinsk Regional Children's Clinical HospitalSouth Ural State Medical University of the Ministry of Health of the Russian Federation; Chelyabinsk Regional Children's Clinical HospitalThe article describes a case of a rare primary immunodeficiency with immune dysregulation — CD25 deficiency with manifestation in the neonatal period in the form of severe chronic diarrhea, susceptibility to viral, bacterial, and fungal infections, autoimmune manifestations, malabsorption syndrome, hypotrophy, skin lesions, confirmed by a homozygous mutation in the IL2-RA gene detected by DNA analysis. The article discusses the role of IL2-RA in the maturation and differentiation of T-lymphocytes, modern approaches to the classification of primary immunodeficiencies with immune dysregulation, principles of diagnosis and treatment of CD25 deficiency, as well as analyzes and compares other cases of CD25 deficiency described in the literature.https://www.antibiotics-chemotherapy.ru/jour/article/view/1125cd25 deficiencyprimary immunodeficiency with immune dysregulationdiagnosisclinical manifestations
spellingShingle E. N. Serebryakova
T. V. Shilova
A. S. Kozhevnikov
E. I. Kilmukhametova
A case of primary immunodeficiency with immune dysregulation: features of clinical manifestation and diagnostic difficulties
Антибиотики и Химиотерапия
cd25 deficiency
primary immunodeficiency with immune dysregulation
diagnosis
clinical manifestations
title A case of primary immunodeficiency with immune dysregulation: features of clinical manifestation and diagnostic difficulties
title_full A case of primary immunodeficiency with immune dysregulation: features of clinical manifestation and diagnostic difficulties
title_fullStr A case of primary immunodeficiency with immune dysregulation: features of clinical manifestation and diagnostic difficulties
title_full_unstemmed A case of primary immunodeficiency with immune dysregulation: features of clinical manifestation and diagnostic difficulties
title_short A case of primary immunodeficiency with immune dysregulation: features of clinical manifestation and diagnostic difficulties
title_sort case of primary immunodeficiency with immune dysregulation features of clinical manifestation and diagnostic difficulties
topic cd25 deficiency
primary immunodeficiency with immune dysregulation
diagnosis
clinical manifestations
url https://www.antibiotics-chemotherapy.ru/jour/article/view/1125
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AT eikilmukhametova acaseofprimaryimmunodeficiencywithimmunedysregulationfeaturesofclinicalmanifestationanddiagnosticdifficulties
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