Dual cancers in Ataxia-Telangiectasia: a case report and literature review.

Background: Ataxia-telangiectasia (A-T) is a rare autosomal recessive disorder caused by mutations in the ATM gene, leading to defective DNA repair, genomic instability, and immune surveillance dysfunction. Therefore, A-T patients are predisposed to cancers, particularly hematological malignancies like lymphoma and leukemia.

Methods: To identify the characteristics of A-T cases with multiple cancers, we searched the Iranian A-T registry with 324 cases and conducted a systematic literature search in PubMed and Embase using appropriate keywords.

Oral Health Literacy: Decision-Making and Reading Comprehension Skills as the Determinants of Self-Reported Oral Health.

Oral health literacy (OHL) is a concept far beyond reading and including other skills such as numeracy skills, listening, and decision-making. Self-reported oral health(SROH) is a reliable and cost-effective measure of dental and periodontal conditions. The current study aimed to evaluate the association between various aspects of OHL and SROH.

NF-κB pathway variants in Iranian patients with inborn errors of immunity.

Background: Clinical and immunological manifestations associated with genetic alterations are crucial for understanding inborn errors of immunity (IEI). This study aims to characterize the clinical and immunological profiles and provide the molecular features of IEI patients from the Iranian population with IEI who harbor rare variants in the nuclear factor kappa B (NF-κB) pathway.

Research Design And Methods: Peripheral blood mononuclear cells (PBMCs) were used for immunophenotyping of B and T lymphocyte subsets via flow cytometry and for assessing T cell proliferation.

A Comparison between LTT and CFSE Proliferation Tests in Patients with Inborn Errors of Adaptive Immunity.

Background: Inborn errors of immunity (IEI) include immunodeficiencies affecting cellular and humoral immunity.

Objectives: We aimed to compare the effectiveness of the LTT and carboxyfluorescein succinimidyl ester (CFSE) assays in assessing lymphocyte proliferation in IEI patients.

Methods: We utilized radioactive [3H]-thymidine and non-radioactive CFSE to measure lymphocyte proliferation in three distinct groups: syndromic CID (SyCID), non-syndromic combined immunodeficiency (N-SyCID), and primary antibody deficiency (PAD).

Does the transition from face-to-face to face-to-screen improve oral health literacy? A non-inferiority randomized controlled trial.

Background: In recent years, more attention has been paid to virtual education methods in the field of oral health. Oral health literacy (OHL) plays a major role in reducing oral health inequalities. Individuals with inadequate health literacy are more susceptible to oral disorders.

ATM Expression and Activation in Ataxia Telangiectasia Patients with and without Class Switch Recombination Defects.

Background: Ataxia telangiectasia mutated (ATM) kinase plays a critical role in DNA double-strand break (DSB) repair. Ataxia telangiectasia (A-T) patients exhibit abnormalities in immunoglobulin isotype expression and class switch recombination (CSR). This study investigates the role of residual ATM kinase expression and activity in the severity of A-T disease.

IL-37 suppresses CNS autoimmunity by increasing the frequency of Treg cells and reducing CD4 + T cell-derived IL-10 production.

Background: Interleukin-37 (IL-37) has anti-inflammatory properties in innate and adaptive immunity. Patients with multiple sclerosis (MS), an autoimmune inflammatory demyelinating disease of the central nervous system (CNS), have increased serum levels of IL-37. However, it is unknown whether IL-37 has an inhibitory effect on ongoing autoimmune neuroinflammation, thus offering a potential MS therapy.

Cancer Trends in Inborn Errors of Immunity: A Systematic Review and Meta-Analysis.

Background: Patients with inborn errors of immunity (IEI) are susceptible to developing cancer due to defects in the immune system. The prevalence of cancer is higher in IEI patients compared to the immunocompetent population and cancers are considered as an important and common cause of death in IEI patients.

Objectives: To systematically review demographic, genetic and cancer-related data of IEI patients with a history of malignancy.

Mouse monocytes express CD127 by immune cells, not LPS.

The essential role of interleukin 7 (IL-7) signaling via its receptor (IL-7Rα; CD127) in T cell development and function has been well documented. However, CD127 expression and function in myeloid cells, including monocytes, are less clear, especially in mice. In the present study we report an inducible CD127 expression in mouse monocytes/macrophages.

The effect of physical exercise on circulating neurotrophic factors in healthy aged subjects: A meta-analysis and meta-regression.

Vascular endothelial growth factor (VEGF), brain-derived neurotrophic factor (BDNF), and insulin-like growth factor-1 (IGF-1) may help the brain resist both functional and structural neurodegeneration, which is critical for maintaining cognitive and neurological health in older adults. This meta-analysis and meta-regression seek to elucidate the impact of physical activity on these biomarker levels in healthy seniors, as well as to examine the influence of several moderator factors, including age, sex, period length, and time, for the first time. The standardized mean effect metric was used to assess the influence of weights, which reflected each group's relative importance in comparison to baseline data.

Patterns and factors associated with dental service utilization among insured people: a data mining approach.

Background: Insurance databases contain valuable information related to the use of dental services. This data is instrumental in decision-making processes, enhancing risk assessment, and predicting outcomes. The objective of this study was to identify patterns and factors influencing the utilization of dental services among complementary insured individuals, employing a data mining methodology.

Immunopathogenesis of multiple sclerosis: molecular and cellular mechanisms and new immunotherapeutic approaches.

Background: Multiple sclerosis (MS) is a central nervous system (CNS) demyelinating autoimmune disease with increasing global prevalence. It predominantly affects females, especially those of European descent. The interplay between environmental factors and genetic predisposition plays a crucial role in MS etiopathogenesis.

Exploring the Challenges in Covering Dental Services through Complementary Insurance in Iran: A Qualitative Study.

Background: Financial protection is crucial for attaining universal health coverage. The inclusion of costly dental services in insurance plans poses a significant challenge for all parties involved in the insurance sector. This study aimed to investigate the challenges of covering dental services by complementary insurance in Iran during 2020-2021.

Oral Health Literacy and Its Determinants in Young Couples.

Oral health literacy (OHL) is an interplay of cultural, social and individual factors and plays an effective role in public health promotion. This study aimed to assess OHL and its socioeconomic and demographic determinants among young couples. This analytical cross-sectional study was conducted on 828 adults between 15 to 35 years in 2018 in Zanjan city.

Assessment of the first presentations of common variable immunodeficiency in a large cohort of patients.

Background: Common Variable Immunodeficiency (CVID) is a primary immunodeficiency syndrome resulting in recurrent infections, autoimmunity, and granulomatous manifestations.

Methods And Materials: This retrospective study was conducted on an Iranian national registry of immunodeficient patients from 2010 to 2021. The frequency of first presentations of CVID and its association with sex, age of onset, and family history of CVID was evaluated.

B cells and T cells abnormalities in patients with selective IgA deficiency.

Background: Selective IgA deficiency (SIgAD) is the most prevalent inborn errors of immunity with almost unknown etiology. This study aimed to investigate the clinical diagnostic and prognostic values of lymphocyte subsets and function in symptomatic SIgAD patients.

Methods: A total of 30 available SIgAD patients from the Iranian registry and 30 age-sex-matched healthy controls were included in the present study.

Designing and Evaluation of an Elective Oral Health Course for Fifth-Year Medical Students of Tehran University of Medical Sciences.

This study aimed to evaluate the effect of an educational intervention on oral health-related knowledge, attitude, and practice of medical students. This study was performed on the fifth-year medical students attending an elective oral health course at the Faculty of Dentistry of Tehran University (intervention group) and 25 other students attending another elective course (control group) in 2018. A 2-week internship program including 6 sessions of a workshop program plus 2 days of school field and 2 days of attending dental departments was designed for the intervention group.

The Autoimmune Manifestations in Patients with Genetic Defects in the B Cell Development and Differentiation Stages.

Purpose: Primary B cell defects manifesting as predominantly antibody deficiencies result from variable inborn errors of the B cell lineage and their development, including impairments in early bone marrow development, class switch recombination (CSR), or terminal B cell differentiation. In this study, we aimed to investigate autoimmunity in monogenic patients with B cell development and differentiation defects.

Methods: Patients with known genetic defects in the B cell development and differentiation were recruited from the Iranian inborn errors of immunity registry.

Autoimmunity in monogenic combined immune deficiencies with associated or syndromic features.

Background: Combined immune deficiencies (CIDs) with associated or syndromic features are a highly heterogeneous subgroup of inherited immune disorders. These patients represent specific clinical complications with an increased risk of autoimmune conditions.

Methods: We analyzed data of monogenic patients with syndromic CIDs adopted from the Iranian inborn errors of immunity registry up to January 2022.

Novel mutation of SLC37A4 in a glycogen storage disease type Ib patient with neutropenia, horseshoe kidney, and arteriovenous malformation: a case report.

Glycogen storage disease type Ib (GSDIb) is an autosomal recessive disorder caused by mutations of SLC37A4 gene, which encodes glucose 6-phosphate translocase (G6PT). Malfunction of G6PT leads to excessive fat and glycogen in liver, kidney, and intestinal mucosa. The clinical manifestations of GSD1b include hepatomegaly, renomegaly, neutropenia, hypoglycemia, and lactic acidosis.

Evaluation of effective factors on IL-10 signaling in B cells in patients with selective IgA deficiency.

Background: Selective IgA deficiency is the most prevalent form of primary immunodeficiencies. The pathogenesis of the disease is still unknown. Several studies have suggested a defect in B cell responses to IL-10; however, the main reason for this defect has not been reported.

Psychometric Properties of a New Questionnaire Assessing the Mothers' Knowledge and Perception about the Oral Health of Their School Children.

The aim of the present study was to develop and evaluate the psychometric properties of an instrument in Persian to assess the mothers' knowledge, and perception about oral health of school children. A sequential exploratory mixed method design consisting of qualitative and quantitative phases was performed. We developed the questionnaire by inductive-deductive method, through a synthesis of literature review and a qualitative study with semi-structured interviews and focus group discussions.