Transient hypogammaglobulinemia of infancy

OvadiaAdi,; DalalIlan,

doi:10.14785/lpsn-2014-0006

Cited by 4 publications

(2 citation statements)

References 50 publications

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“…Predominantly, immunoglobulin deficiencies are the most common forms of PIDs [22, 23]. One of them, transient hypogammaglobulinaemia of infancy (THI), is a heterogeneous disorder characterised by reduced serum IgG (often IgA and sometimes also IgM) level in early childhood and recurrent infections, mostly of the respiratory tract [24-26]. A putative diagnosis is initially made after exclusion of other causes of hypogammaglobulinaemia, while a definitive diagnosis of THI can only be made retrospectively in patients with normalised IgG levels and withdrawal of clinical symptoms, which occurs usually between the second and fourth year of life [27].…”

Section: Introductionmentioning

confidence: 99%

The level of myeloid-derived suppressor cells positively correlates with regulatory T cells in the blood of children with transient hypogammaglobulinaemia of infancy

Siemińska

Rutkowska-Zapała

Bukowska-Straková

et al. 2018

cejoi

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IntroductionTransient hypogammaglobulinaemia of infancy (THI) is a primary immunodeficiency characterised by low levels of immunoglobulin G (often with concomitant decrease of IgA and sometimes also of IgM) with still unknown exact reason. A delayed normalisation of the immunoglobulin level in THI may be associated with a transiently elevated number of regulatory T cells (Treg). Although in cancer and chronic inflammation it was shown that the level of Treg cells can be increased by myeloid-derived suppressor cells (MDSCs), until now no studies have been performed in the context of the role of MDSCs in THI and their correlation with Treg cells. Consequently, we aimed to determine the occurrence of MDSCs in the peripheral blood of children with THI and correlate their level with the level of Treg cells.Material and methodsFlow cytometry analyses of Mo-MDSCs and Gr-MDSCs, characterised as HLA-DR–CD11b+CD15–CD14+ and HLA-DR–CD11b+CD15+CD14–, respectively, and Treg (CD4+CD25+Foxp3+) cells were performed.ResultsThe proportion of Mo-MDSCs and Gr-MDSCs was significantly higher in the group of THI patients with elevated level of Treg cells (from the 95% confidence interval level of healthy controls). The cells with Mo-MDSC and Gr-MDSC characteristics positively correlated with the level of Treg cells. Moreover, children with a higher proportion of circulating Treg cells, and thereby higher level of MDSCs, showed delayed normalisation of IgG level and recovery.ConclusionsThese findings show for the first time that MDSCs may be involved in the pathomechanism of THI, probably acting through the induction of Treg cells.

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Section: Introductionmentioning

confidence: 99%

The level of myeloid-derived suppressor cells positively correlates with regulatory T cells in the blood of children with transient hypogammaglobulinaemia of infancy

Siemińska

Rutkowska-Zapała

Bukowska-Straková

et al. 2018

cejoi

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“…In 1D1a transgenic mice at all the studied ages, significant suppression of the humoral response was observed. Interestingly, similar to transient hypogammaglobulinemia in infant humans [ 31 ], decreased levels of IgM and IgG were registered in young (3-month-old) TG mice. This, however, could not be the sole reason for inhibition of the humoral response, as immunoglobulin levels in aged transgenic mice were restored to those in age-matched wild-type animals.…”

Section: Discussionmentioning

confidence: 66%

Physiological and Functional Effects of Dominant Active TCRα Expression in Transgenic Mice

Kalinina

Ziganshin

Silaeva

et al. 2023

IJMS

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A T cell receptor (TCR) consists of α- and β-chains. Accumulating evidence suggests that some TCRs possess chain centricity, i.e., either of the hemi-chains can dominate in antigen recognition and dictate the TCR’s specificity. The introduction of TCRα/β into naive lymphocytes generates antigen-specific T cells that are ready to perform their functions. Transgenesis of the dominant active TCRα creates transgenic animals with improved anti-tumor immune control, and adoptive immunotherapy with TCRα-transduced T cells provides resistance to infections. However, the potential detrimental effects of the dominant hemi-chain TCR’s expression in transgenic animals have not been well investigated. Here, we analyzed, in detail, the functional status of the immune system of recently generated 1D1a transgenic mice expressing the dominant active TCRα specific to the H2-Kb molecule. In their age dynamics, neither autoimmunity due to the random pairing of transgenic TCRα with endogenous TCRβ variants nor significant disturbances in systemic homeostasis were detected in these mice. Although the specific immune response was considerably enhanced in 1D1a mice, responses to third-party alloantigens were not compromised, indicating that the expression of dominant active TCRα did not limit immune reactivity in transgenic mice. Our data suggest that TCRα transgene expression could delay thymic involution and maintain TCRβ repertoire diversity in old transgenic mice. The detected changes in the systemic homeostasis in 1D1a transgenic mice, which are minor and primarily transient, may indicate variations in the ontogeny of wild-type and transgenic mouse lines.

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Transient Immunodeficiency of Infancy

Sokol¹

2021

Primary and Secondary Immunodeficiency

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Transient hypogammaglobulinemia of infancy

Cited by 4 publications

References 50 publications

The level of myeloid-derived suppressor cells positively correlates with regulatory T cells in the blood of children with transient hypogammaglobulinaemia of infancy

The level of myeloid-derived suppressor cells positively correlates with regulatory T cells in the blood of children with transient hypogammaglobulinaemia of infancy

Physiological and Functional Effects of Dominant Active TCRα Expression in Transgenic Mice

Transient Immunodeficiency of Infancy

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