Abstract:
:Objectives: Ataxia-telangiectasia (A-T) is an autosomal recessive neurodegenerative disorder with multisystem involvement caused by homozygous or compound heterozygous mutations in the ataxia telangiectasia mutated (ATM) gene which encodes a serine/threonine protein kinase. The aims of this study were to investigate class switch recombination (CSR) and to review the clinical and immunologic phenotypes of 3 groups of A-T patients, including A-T patients with CSR defects (CSR-D), A-T patients with selective immunoglobulin A deficiency (IgA-D) and A-T patients with normal Ig level. Methods: In this study, 41 patients with confirmed diagnosis of A-T (16 A-T patients with HIgM, 15 A-T patients with IgA-D, and 10 A-T patients with normal Ig levels) from Iranian immunodeficiency registry center were enrolled. B-cell proliferation, in vitro CSR toward IgE and IgA were compared between three groups as well as G2 radiosensitivity assay. Results: Earliest presentation of telangiectasia was a significant hallmark in A-T patients with CSR-D (p = .036). In this investigation, we found that the frequency of respiratory infection (p = .002), pneumonia (p = .02), otitis media (p = .008), chronic fever (p < .001), autoimmunity (p = .02) and hepatosplenomegaly (p = .03) in A-T patients with HIgM phenotype were significantly higher than the other groups. As expected IgE production stimulation and IgA CSR were perturbed in HIgM patients that were aligned with the higher readiosenstivity scores in this group. Conclusion: A-T patients with HIgM compared to other A-T patients presenting more infections and noninfectious complications, therefore, early detection and careful management of these patients is necessary.
journal_name
Immunol Investjournal_title
Immunological investigationsauthors
Amirifar P,Mozdarani H,Yazdani R,Kiaei F,Moeini Shad T,Shahkarami S,Abolhassani H,Delavari S,Sohani M,Rezaei A,Hassanpour G,Akrami SM,Aghamohammadi Adoi
10.1080/08820139.2020.1723104subject
Has Abstractpub_date
2021-02-01 00:00:00pages
201-215issue
2-3eissn
0882-0139issn
1532-4311journal_volume
50pub_type
杂志文章abstract::The phenomenon of apoptosis observed in lymphoid cells from HIV+ subjects is an important factor contributing to their massive depletion. Several studies have identified nitric oxide (NO) as one of the molecules involved in the apoptosis phenomenon observed during HIV infection. It has been shown that HIV-derived gp12...
journal_title:Immunological investigations
pub_type: 杂志文章
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journal_title:Immunological investigations
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journal_title:Immunological investigations
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journal_title:Immunological investigations
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journal_title:Immunological investigations
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journal_title:Immunological investigations
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journal_title:Immunological investigations
pub_type: 杂志文章,评审
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更新日期:2006-01-01 00:00:00
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