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Children
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Advances in Research on Childhood Immune and Allergic Diseases
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Advances in Research on Childhood Immune and Allergic Diseases

A special issue of Children (ISSN 2227-9067). This special issue belongs to the section "Pediatric Allergy and Immunology".

Deadline for manuscript submissions: closed (20 March 2025) | Viewed by 3332

Special Issue Editors

Dr. Aleksandra Szczawińska-Popłonyk

E-Mail Website
Guest Editor
Department of Pediatric Pneumonology, Allergy and Clinical Immunology, Institute of Pediatrics, Karol Marcinkowski University of Medical Sciences, 60-572 Poznań, Poland
Interests: inborn errors of immunity; immune dysregulation; immunogenetics; syndromic immunodeficiencies; atopy; organ-specific immunopathology
Special Issues, Collections and Topics in MDPI journals
Dr. Paulina Sobkowiak

E-Mail Website1 Website2
Guest Editor
Department of Pediatric Pneumonology, Allergy and Clinical Immunology, Institute of Pediatrics, Karol Marcinkowski University of Medical Sciences, 60-572 Poznań, Poland
Interests: atopy; asthma; alimentary allergy; atopic dermatitis; inflammatory cytokines; immunotherapy

Special Issue Information

Dear Colleagues,

Childhood immune diseases comprise a wide range of conditions, including inborn errors of immunity, and allergic, immune dysregulation, autoinflammatory, autoimmune and lymphoproliferative disorders as well as organ-specific immunopathology. Attempts are being made to identify the prognosis, patterns, severity scale of outcomes, and the global social and psychological burden of inborn errors of immunity and allergic diseases among pediatric patients. Significant progress has been made recently in uncovering the genetic, molecular, immunological and environmental underpinnings of childhood immune and allergic diseases. These discoveries have contributed to elucidating the phenotype–genotype correlations and clinical manifestation heterogeneity, defining and delineating disease entities, and paving the way to elaborate targeted immunotherapy. The aim of this Special Issue is to better understand the immunogenetic background and clinico-pathological interconnections of childhood immune and allergic diseases. The thematic scope of articles will encompass the clinical, diagnostic and therapeutic aspects of these disorders.

In this Special Issue, original research articles and reviews are particularly welcome. Instructive case reports on unusual immune and allergic conditions in children are also accepted.

Dr. Aleksandra Szczawińska-Popłonyk
Dr. Paulina Sobkowiak
Guest Editors

Manuscript Submission Information

Manuscripts should be submitted online at www.mdpi.com by registering and logging in to this website. Once you are registered, click here to go to the submission form. Manuscripts can be submitted until the deadline. All submissions that pass pre-check are peer-reviewed. Accepted papers will be published continuously in the journal (as soon as accepted) and will be listed together on the special issue website. Research articles, review articles as well as short communications are invited. For planned papers, a title and short abstract (about 100 words) can be sent to the Editorial Office for announcement on this website.

Submitted manuscripts should not have been published previously, nor be under consideration for publication elsewhere (except conference proceedings papers). All manuscripts are thoroughly refereed through a single-blind peer-review process. A guide for authors and other relevant information for submission of manuscripts is available on the Instructions for Authors page. Children is an international peer-reviewed open access monthly journal published by MDPI.

Please visit the Instructions for Authors page before submitting a manuscript. The Article Processing Charge (APC) for publication in this open access journal is 2400 CHF (Swiss Francs). Submitted papers should be well formatted and use good English. Authors may use MDPI's English editing service prior to publication or during author revisions.

Keywords

  • asthma
  • atopy
  • autoimmunity
  • autoinflammation
  • immune dysregulation
  • immunosuppression
  • immunotherapy
  • inborn errors of immunity
  • lymphoproliferation

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Further information on MDPI's Special Issue policies can be found here.

Published Papers (2 papers)

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15 pages, 4195 KiB  
Article
Down Syndrome in Children: A Primary Immunodeficiency with Immune Dysregulation
by Aleksandra Szczawińska-Popłonyk, Natalia Popłonyk and Karina Awdi
Children 2024, 11(10), 1251; https://doi.org/10.3390/children11101251 - 17 Oct 2024
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Abstract
Background: The multisystemic features of Down syndrome (DS) in children are accompanied by immunodeficiency, making them susceptible to infections and immune dysregulation with autoimmune, allergic, inflammatory, and hematological complications. This study was aimed at a better understanding of the abnormalities within the B [...] Read more.
Background: The multisystemic features of Down syndrome (DS) in children are accompanied by immunodeficiency, making them susceptible to infections and immune dysregulation with autoimmune, allergic, inflammatory, and hematological complications. This study was aimed at a better understanding of the abnormalities within the B and T cell compartments and their correlations with clinical immunophenotypes. Methods: Medical records of 35 DS children were retrospectively reviewed, referring to clinical symptomatology including history of infections, immune dysregulation disorders, and humoral and cellular immune response. Results: While the etiology of respiratory tract infections included typical viral and bacterial pathogens, SARS-CoV2-induced inflammatory disease and syndromic immunodeficiency contributed significantly to the deterioration of the clinical course. Allergic diseases in the form of asthma, allergic rhinitis, and alimentary allergy were the most frequent manifestations of immune dysregulation and were followed by autoimmune disorders, such as Crohn’s disease, celiac disease, autoimmune thyroiditis, and alopecia, as well as inflammatory disorders, balanitis xerotica obliterans and lymphadenopathy, and a hematological disorder of myelopoiesis. Deficiency of serum immunoglobulin levels, reduced numbers of naïve B cells, and non-switched memory B cells along with low naïve T helper cells and significantly reduced regulatory T helper cells were the most prominent immune abnormalities. Conclusions: The loss of naïveté in B and T lymphocyte compartments with a deficiency of regulatory T cells may be underpinning pathomechanisms for the skewed immune response. The clinical immunophenotype in DS is complex and represents syndromic primary immunodeficiency with immune dysregulation. Full article
(This article belongs to the Special Issue Advances in Research on Childhood Immune and Allergic Diseases)
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31 pages, 1809 KiB  
Systematic Review
The Management of IgG4-Related Disease in Children: A Systematic Review
by Evdoxia Sapountzi, Eleni P. Kotanidou, Vasiliki-Rengina Tsinopoulou, Lampros Fotis, Liana Fidani and Assimina Galli-Tsinopoulou
Children 2025, 12(2), 213; https://doi.org/10.3390/children12020213 - 11 Feb 2025
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Abstract
Background/Objectives: IgG4-related disease (IgG4-RD) is a multi-organ disease with greatly varying therapeutic approaches and a lack of specific treatment algorithms. This systematic review aimed to determine the therapeutic approaches for pediatric IgG4-RD in real-word practice. Methods: We searched PubMed and Google [...] Read more.
Background/Objectives: IgG4-related disease (IgG4-RD) is a multi-organ disease with greatly varying therapeutic approaches and a lack of specific treatment algorithms. This systematic review aimed to determine the therapeutic approaches for pediatric IgG4-RD in real-word practice. Methods: We searched PubMed and Google Scholar for articles on pediatric IgG4-RD cases published in English from 2012 to August 2024, focusing on treatments and outcomes. Study type, treatment(s), dose/regimen, age and sex, organ(s) involved, and treatment outcomes were manually extracted from each study. Results: Of the 219 studies identified, we analyzed 81 studies, including 114 pediatric IgG4-RD cases. Fifty-seven percent of patients suffered from multi-organ disease and required several treatment schemes. Around 75% received steroids, alone or in combination, regardless of the organ affected. The treatment outcomes were positive in most cases, although relapses occurred in approximately 30% of patients, usually upon steroid tapering. Other common therapeutic approaches included immunosuppressants, often used as steroid-sparing agents, with azathioprine and mycophenolate mofetil being the most common; surgery for localized disease; and biologics, mainly rituximab, used in more severe/refractory cases. Uncommon but effective therapies included adalimumab and ruxolitinib. Drug combinations seemed to be more efficacious than monotherapies across studies. Patients > 10 years old more frequently received aggressive approaches (surgery and rituximab) and more often experienced relapses. Relapse rates were higher among females. Conclusions: This review highlights the use of systemic steroids as an effective first-line treatment for pediatric IgG4-RD, but also underscores the use of non-steroid-based alternatives in combination with steroids or other immunosuppressants for the effective management of IgG4-RD. Full article
(This article belongs to the Special Issue Advances in Research on Childhood Immune and Allergic Diseases)
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