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Farhad Abolnezhadian,1 Seyed Ahmad Hosseini,2,3 Meysam Alipour,3 Mehrnoosh Zakerkish,4 Bahman Cheraghian,5 Pegah Ghandil,6 Makan Cheraghpour3 1Division of Immunology and Allergy, Department of Pediatrics, School of Medicine, Ahvaz Jundishapur University of Medical Sciences, Ahvaz, Iran; 2Hyperlipidemia Research Center, Ahvaz Jundishapur University of Medical Sciences, Ahvaz, Iran; 3Nutrition and Metabolic Diseases Research Center, Ahvaz Jundishapur University of Medical Sciences, Ahvaz, Iran; 4Department of Endocrinology and Metabolism, Diabetes Research Center, Health Research Institute, Ahvaz Jundishapur University of Medical Sciences, Ahvaz, Iran; 5Department of Statistics and Epidemiology, Faculty of Public Health, Ahvaz Jundishapur University of Medical Sciences, Ahvaz, Iran; 6Department of Medical Genetics, Faculty of Medicine, Ahvaz Jundishapur University of Medical Sciences, Ahvaz, IranCorrespondence: Seyed Ahmad HosseiniHyperlipidemia Research Center, Ahvaz Jundishapur University of Medical Sciences, Ahvaz, IranEmail Seyedahmadhosseini@yahoo.comBackground: The role of metabolic states in cardiovascular risks among individuals with varying degrees of obesity is unknown. The study aimed to compare cardiometabolic index (CMI), atherogenic index of plasma (AIP), lipid accumulation product (LAP) and novel anthropometric indices in metabolic and non-metabolically obese individual with regard to the role of FTO gene in Iranian adults.Methods: In total, 165 individuals were recruited into this cross-sectional study. Individuals grouped into four groups: metabolic healthy normal-weight (MHNW) individuals, metabolically unhealthy normal-weight (MUNW) individuals, metabolically healthy obese (MHO) individuals and metabolic unhealthy obese (MUO) individuals. The dietary intake was evaluated by food frequency questionnaire (FFQ). The cardiovascular indices (CMI, AIP and LAP) were calculated. A variety of anthropometric indices were calculated, including body adiposity Index (BAI), weight-adjusted-waist index (WWI), A body shape index (ABSI) and waist-height ratio (WHR). The genotypes of FTO-rs9939609 subjects were detected by the polymerase chain reaction-restriction fragment length polymorphism (PCR-RFLP).Results: The individuals with metabolically unhealthy phenotypes (MUO, MUNW) have higher levels of triglyceride and cardiovascular indices (AIP, LAP and CMI) than the individuals with metabolic healthy phenotypes (MHO, MHNW). With a similar degree of obesity, the anthropometric indices (BAI, WWI and WHR) levels were higher in metabolic unhealthy groups than metabolically healthy groups. The highest frequency of obesity-risk allele AA of FTO gene was observed in MUO, MHO, MUNW and MHNW, respectively.Conclusion: Normal-weight individuals with metabolic unhealthy status are at higher risk for cardiovascular diseases than obese individuals with metabolically healthy status. The genotype frequencies of obesity-risk allele AA of FTO gene were higher in obesity phenotypes than metabolic phenotypes.Keywords: cardiovascular, atherogenic index, obesity, FTO

BACKGROUND AND OBJECTIVE: Chronic urticaria is a common skin condition that is manifested with erythematous or white edema, itching, and transient dermis with or without angioedema. Due to insufficient knowledge of the etiology of many of these chronic lesions, this study was performed to investigate some factors related to individual and laboratory indices of patients. METHODS: In this descriptive study, 210 patients (154 females and 54 males) with chronic urticaria referred to Asthma and Allergy Clinic of Ahvaz from September 2015 to February 2019 were evaluated. Routine tests were performed for all patients including blood cell count, antinuclear antibodies, thyroxine and thyroid stimulating hormones, antithyroperoxidase (TPO) and anti-thyroglobulin (Tg) antibodies, glutamate-oxaloacetate transaminase (GOT) and glutamate-pyruvate transaminase (GPT) enzymes, autologous serum skin test and stool test. FINDINGS: Of all patients, 166 (79%) had physical urticaria, 125 (59.5%) had atopy and 158 (75%) had angioedema. Autologous serum skin test with a frequency of more than 74.28% was positive in patients. Except for 3 patients (1.42%) with low serum levels of thyroid stimulating hormone and normal thyroxine (mild hypothyroidism) and 49 patients (23.33%) with abnormal increase in anti-thyroperoxidase and anti-thyroglobulin serum antibodies, the rest of the subjects (75.23%) were healthy. The results of anti-nuclear antibody and stool tests were also generally normal. CONCLUSION: Based on the results of this study, in order to diagnose patients with chronic urticaria with or without angioedema, it is necessary to perform autologous serum skin test and thyroid function tests along with detailed clinical examinations. [ABSTRACT FROM AUTHOR]

Background: Common variable immunodeficiency (CVID) is considered the most symptomatic type of inborn errors of immunity in humans. Along with infectious complications, which have numerous consequences, noninfectious complications are a major challenge among CVID patients., Methods: All CVID patients registered in the national database were included in this retrospective cohort study. Patients were divided into 2 groups based on the presence of B-cell lymphopenia. Demographic characteristics, laboratory findings, noninfectious organ involvement, autoimmunity, and lymphoproliferative diseases were evaluated., Results: Among 387 enrolled patients, 66.4% were diagnosed with noninfectious complications and 33.6% with isolated infectious presentations. Enteropathy, autoimmunity, and lymphoproliferative disorders were reported in 35.1%, 24.3%, and 21.4% of patients, respectively. Some complications, including autoimmunity and hepatosplenomegaly, were reported to be significantly more frequent among patients with B-cell lymphopenia. As for organ involvement, the dermatologic, endocrine, and musculoskeletal systems were predominantly affected in CVID patients with B-cell lymphopenia. Among autoimmune manifestations, the frequency of rheumatologic, hematologic, and gastrointestinal autoimmunity was reported to be higher than that of other types of autoimmunity not associated with B cell-lymphopenia. Furthermore, hematological cancers, particularly lymphoma, were the most common type of malignancy. The mortality rate was 24.5%, and respiratory failure and malignancies were the most common causes of death, with no significant differences between the 2 groups., Conclusions: Considering that some of the noninfectious complications might be associated with B-cell lymphopenia, regular patient monitoring and follow-up with proper medication (in addition to immunoglobulin replacement therapy) are highly recommended to prevent sequelae and increase patient quality of life.

As an antibody-based therapy, plasma therapy has been used as an emergency therapeutic strategy against severe acute respiratory syndrome coronavirus type 2 infection. Due to the critical role of macrophages in coronavirus disease-19 (COVID-19)-associated hyperinflammation, the main objective of this study was to assess the effect of plasma transfusion on the expression levels of the inflammatory biomarkers involved in activation and pulmonary infiltration of macrophages. The target population included 50 severe hospitalized COVID-19 patients who were randomly assigned into 2 groups, including intervention and control. Serum levels of chemokine (C-C motif) ligand (CCL)-2, CCL-3, tumor necrosis factor (TNF)-α, and interleukin (IL)-6 were measured by enzyme-linked immunosorbent assay. Moreover, quantitative real-time polymerase chain reaction (PCR) was carried out to assess the relative expression of nuclear factor (NF)-κB1, NF-κB2, nuclear factor erythroid 2 p45-related factor 2 (NRF-2), and thioredoxin-interacting protein genes. Sampling was done at baseline and 72 h after receiving plasma. The intervention group demonstrated significantly lower serum levels of IL-6, TNF-α, and CCL-3. In addition, real-time PCR data analyses showed that the relative expression of NF-κB2 was significantly declined in the patients who received plasma. The use of convalescent plasma probably has a significant inhibitory effect on the cytokines, chemokines, and inflammatory genes related to macrophage activation, which are closely associated with the worsening of clinical outcomes in severe COVID-19.

Background: Severe acute respiratory syndrome coronavirus 2 was first reported in December 2019 and it has spread globally ever since. The HLA system is crucial in directing anti-viral immunity and recent studies are investigating the possible involvement of the HLA genes on the severity of immune inflammation in different phases of COVID-19., Methods: In this cross-sectional study, peripheral blood-extracted genomic DNAs of 109 COVID-19 patients and 70 healthy controls were genotyped for different alleles of HLA-A, HLA-B, and HLA-DRB1 loci using sequence-specific primer PCR method., Results: The results indicated that frequencies of HLA-DRB1*11:01 and HLA-DRB1*04:03 were significantly higher in severe patients rather than moderates (p: <0.001 and 0.004, respectively). Also, it was observed that HLA-DRB1*04:01 was more frequent in moderate patients and healthy controls (p:0.002). In addition, HLA-B*07:35, and HLA-DRB1*07:01 showed higher frequencies in patients compared with controls (p: 0.031 and 0.003 respectively). Inversely, due to the higher frequencies of HLA-B*51:01 (p:0.027), HLA-DRB1*11:05 (p:0.003), HLA-DRB1*13:05 (p:0.022), and HLA-DRB1*14:01 (p:0.006) in healthy individuals rather than patients, they may be associated with COVID-19 resistance., Conclusion: The results show that, based on the population differences, the type of alleles related to the severity of COVID-19 is different, which should be clarified by designing large-scale studies in order to develop HLA-based treatments and vaccines., Competing Interests: The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (© 2024 The Authors.)

Sanjad Sakati Syndrome (SSS) is categorized as a neuroendocrine-related disease due to disorders of the nervous and hormonal systems. Since hormonal changes in these patients may affect the nature and function of the immune system. Thus, in this study, cell count and phagocytotic function of neutrophils were evaluated which may be influenced by changes in the hormonal rate and growth factors. In this study, the neutrophil count value and the oxidative burst were evaluated in six patients diagnosed with SSS and six healthy individuals. There was a significant reduction in the neutrophil count observed in SSS patients compared to healthy controls (37.41±7.93 percent vs. 66.5±6.8 percent). However, there was no significant difference in neutrophil oxidative index between patients with SSS and control subjects (172.33±55.08 vs. 217.00±77.38). We concluded that in patients with SSS, the phagocytic activity of neutrophils was not affected by hormonal changes, while the number of neutrophils and neutrophil-to-lymphocyte ratio (NLR) index were decreased.

Background: Inborn errors of immunity (IEI) are a diverse range of genetic immune system illnesses affecting the innate and/or adaptive immune systems. Variable expressivity and incomplete penetrance have been reported in IEI patients with similar clinical diagnoses or even the same genetic mutation., Methods: Among all recorded patients in the national IEI registry, 193 families with multiple cases have been recognized. Clinical, laboratory and genetic variability were compared between 451 patients with different IEI entities., Results: The diagnosis of the first children led to the earlier diagnosis, lower diagnostic delay, timely treatment and improved survival in the second children in the majority of IEI. The highest discordance in familial lymphoproliferation, autoimmunity and malignancy were respectively observed in STK4 deficiency, DNMT3B deficiency and ATM deficiency. Regarding immunological heterogeneity within a unique family with multiple cases of IEI, the highest discordance in CD3 + , CD4 + , CD19 + , IgM and IgA levels was observed in syndromic combined immunodeficiencies (CID), while non-syndromic CID particularly severe combined immunodeficiency (SCID) manifested the highest discordance in IgG levels. Identification of the first ATM-deficient patient can lead to improved care and better survival in the next IEI children from the same family., Conclusion: Intrafamilial heterogeneity in immunological and/or clinical features could be observed in families with multiple cases of IEI indicating the indisputable role of appropriate treatment and preventive environmental factors besides specific gene mutations in the variable observed penetrance or expressivity of the disease. This also emphasizes the importance of implementing genetic evaluation in all members of a family with a history of IEI even if there is no suspicion of an underlying IEI as other factors besides the underlying genetic defects might cause a milder phenotype or delay in presentation of clinical features. Thus, affected patients could be timely diagnosed and treated, and their quality of life and survival would improve., Competing Interests: Declaration of competing interest The authors declare that they have no relevant conflicts of interest., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Allergic diseases are prevalent chronic conditions among children and can lead to significant health and economic issues. It is hypothesized that healthy and high quality diet during pregnancy can prevent the onset of allergic diseases in offspring. This study aimed to investigate the potential relationship between major dietary patterns during pregnancy and allergies in children under one year of age. This case-control study was conducted involving 244 participants (122 mothers of allergic children and 122 healthy controls) who visited pediatricians and allergy outpatient clinics in Khuzestan Province, Iran, between June 2022 and March 2023. Demographic information was recorded using a socio-demographic questionnaire. A food frequency questionnaire was used to identify the foods consumed during pregnancy. Major dietary patterns were extracted using principal component analysis, and the potential relationship between these patterns and childhood allergies was investigated using multivariable logistic regression models. The crude odds ratio (OR) analysis showed that the fourth quartile of "Nut, vegetables, fruits, and protein" dietary pattern was associated with lower occurrence of childhood allergies (OR: 0.214, 95% CI = 0.068-0.679; P trend = 0.211). After adjusting for cofactors in Model 3, this association was still observed in the fourth quartile (OR = 0.108, 95% CI = 0.019-0.613; P trend, 0.001). However, no significant association was observed between "Carbohydrate and cereals" and "Salty" dietary patterns and childhood allergies. The study findings suggest that a maternal dietary pattern rich in nuts, vegetables, and fruits during pregnancy may reduce the risk of allergic diseases in offspring., (© 2024. The Author(s).)

Competing Interests: The authors declare that they have no competing interests.

Background: The dietary approaches to stop hypertension (DASH) encourages high fruit, vegetable, and lean protein consumption and low salt, red meat, and fat intake to prevent or treat hypertension. However, besides hypertension, adherence to this diet has been shown to decrease other cardiovascular risk factors., Methods: This study assessed the relationship between the DASH diet and cardiovascular risk factors in a cross-sectional study of 2,831 adults chosen by multistage cluster sampling from 27 counties of Khuzestan province, Iran. DASH scores were calculated using data obtained from a qualitative food frequency questionnaire. Regression models were used to evaluate the association of DASH scores and common cardiovascular risk factors., Results: Significant trends were observed across quintiles of DASH scores for systolic blood pressure, fasting blood sugar, triglyceride, total cholesterol, and its components ( p < 0·05). After adjusting for potential confounders such as sex, age, ethnicity, residence, wealth score, physical activity, energy intake, and family history of heart disease, the multiple regression analysis for each cardiovascular risk factor revealed that being in the highest quintile of total DASH score (OR = 0.72, 95% CI 0.52-0.99) was negatively associated with hyperglycemia., Conclusions: This study showed a positive relationship between DASH diet adherence and lower serum levels of glucose, triglycerides, and cholesterol. Prospective studies are needed to confirm these findings., Competing Interests: There are no conflicts of interest., (Copyright: © 2023 International Journal of Preventive Medicine.)

Background: Asthma essentially represents a chronic inflammatory disease that manifests as a lifelong condition with different severity throughout the life of patients with asthma. Pomegranate holds three times the antioxidant activity compared to other polyphenol-rich food sources like green tea, which may positively impact asthma. Aim of the study: This research aimed to investigate the pomegranate supplementation influences clinical symptoms, eosinophil, basophil, and neutrophil counts in patients with allergic asthma. Materials and Methods: Participants ( n = 64) suffering from mild to moderate allergic asthma were randomly divided into two groups: The control group received placebo capsules and the intervention group received 250 mg pomegranate extract capsules twice a day (for 8 weeks). To analyze the data, we used SPSS software (version 22). The significance level of p -value was considered less than 0.05. Results: The findings showed that the pomegranate extract improved patients' clinical symptoms like daily breath shortness, nocturnal breath shortness, and limitation of asthma-related activity in the intervention group compared to the control group. Furthermore, eosinophil, basophil, and neutrophil counts were significantly decreased in the intervention group. Also, by comparing the two groups, the levels of change in neutrophils and eosinophils were statistically significant. Conclusion: It appears that the pomegranate extract can ameliorate some clinical symptoms and reduce neutrophils, basophils, and eosinophils in allergic asthma patients. Clinical Trial Registration: https://www.irct.ir/trial/45612; identifier: IRCT20200205046384N1., (Copyright © 2023 Hosseini, Shateri, Abolnezhadian, Maraghi, Haddadzadeh Shoushtari and Zilaee.)

Genetic defects in the development, maturation, and/or function of the immune cells can lead to Inborn errors of immunity (IEI) which may predispose patients to malignancies. The overall risk for cancer in children with IEI ranges from 4 to 25% and the type of malignancy is highly dependent on the specific mutant gene underlying IEI. We investigated 3056 IEI patients registered in the Iranian national registry between the years 1999 and 2020 in this retrospective cohort study. The frequency of malignancy and its association with the type of IEI in these patients were evaluated. A total of 82 IEI patients with malignancy were enrolled in this study. Among them, predominantly lymphoma was the most common type of malignancy (67.1%), followed by leukemia (11%), and cancers of the head and neck (7.3%). Among identified lymphoma cancers, non-Hodgkin's lymphomas were the most frequent type (43.9%) followed by different subtypes of Hodgkin's lymphoma (23.2%). Solid tumors (18.3%) appeared to be very heterogeneous by type and localization. The correlation between the type of malignancy and survival status and the association between the type of malignancy and IEI entities were unremarkable. The awareness of the association between the presence of IEI and cancer highlights the importance of a synergistic effort by oncologists and immunologists in the early diagnosis of malignancy and personalized therapeutic strategies in IEI patients., (© 2022. The Author(s).)

Background: It is well established that upper and lower airways are often clumped together when diagnosing and treating a disease. This study was designed to determine the prevalence of upper and lower airway diseases and to assess the effect of sociodemographic factors on the prevalence and the comorbidity of these disorders. Methods: This cross-sectional population-based study included patients with ages ranging between 15 to 65 years, who were referred to allergy outpatient clinics in various provinces of Iran from April to September 2020. A modified global Allergy and Asthma European Network (GA2LEN) screening questionnaire was filled out by local allergists of the 12 selected provinces in Iran. Information about the patients and sociodemographic factors was also recorded. Statistical analysis was done by univariate statistical analyses and multiple logistic regressions in SPSS software Version 26. Results: Out of 4988 recruited patients, 1078 (21.6%) had the symptoms of allergic rhinitis (AR) and 285 (5.7%) met the criteria of asthma. The prevalence of acute rhinosinusitis (ARS) and chronic rhinosinusitis (CRS) was 21.6 % and 22%, respectively. The highest prevalence of AR and ARS was in Tehran with the arateof of 33.9% each. Asthma was more prevalent in Khuzestan (14.2%) and CRS in Baluchestan (57.5%). Our analysis showed that the patients with asthma were most likely to have other allergic diseases as well-CRS (OR = 4.8; 95% CI, 2.02- 5.82), AR (OR= 2.5, 95% CI, 2.10-3), ARS (OR = 1.8; 95% CI, 2.10-3), followed by eczema (OR = 1.4; 95% CI, 1.13-1.67).We found that those individuals with CRS were most likely to have painkiller hypersensitivity (OR= 2.1; 95% CI, 1.21-3.83). Furthermore, smoking has been found more than 1.5 folds in patients with ARS. After adjusting variables, there was no correlation between education, occupation, and ethnicity with the studied diseases. Conclusion: Rhinosinusitis is a common condition among Iranian patients. This study confirmed that inflammation of the upper and lower airways can occur simultaneously. Gender, education, occupation, and ethnicity were found to be irrelevant in the development of either AR, asthma, ARS, or CRS., Competing Interests: The authors declare that they have no competing interests., (© 2022 Iran University of Medical Sciences.)

Background: Most severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2)-infected individuals are asymptomatic or only exhibit mild disease. In about 10% of cases, the infection leads to hypoxemic pneumonia, although it is much more rare in children., Objective: We evaluated 31 young patients aged 0.5 to 19 years who had preexisting inborn errors of immunity (IEI) but lacked a molecular diagnosis and were later diagnosed with coronavirus disease 2019 (COVID-19) complications., Methods: Genetic evaluation by whole-exome sequencing was performed in all patients. SARS-CoV-2-specific antibodies, autoantibodies against type I IFN (IFN-I), and inflammatory factors in plasma were measured. We also reviewed COVID-19 disease severity/outcome in reported IEI patients., Results: A potential genetic cause of the IEI was identified in 28 patients (90.3%), including mutations that may affect IFN signaling, T- and B-cell function, the inflammasome, and the complement system. From tested patients 65.5% had detectable virus-specific antibodies, and 6.8% had autoantibodies neutralizing IFN-I. Five patients (16.1%) fulfilled the diagnostic criteria of multisystem inflammatory syndrome in children. Eleven patients (35.4%) died of COVID-19 complications. All together, at least 381 IEI children with COVID-19 have been reported in the literature to date. Although many patients with asymptomatic or mild disease may not have been reported, severe presentation of COVID-19 was observed in 23.6% of the published cases, and the mortality rate was 8.7%., Conclusions: Young patients with preexisting IEI may have higher mortality than children without IEI when infected with SARS-CoV-2. Elucidating the genetic basis of IEI patients with severe/critical COVID-19 may help to develop better strategies for prevention and treatment of severe COVID-19 disease and complications in pediatric patients., (Copyright © 2022 The Authors. Published by Elsevier Inc. All rights reserved.)

Background: The main aim of the present study is to investigate the independent association objectively measured level of physical activity (PA) and serum concentration of liver aminotransferases (alanine aminotransferase [ALT] and aspartate aminotransferase [AST]) among seemingly healthy individuals., Materials and Methods: The current secondary study was conducted in the framework of Khuzestan Comprehensive Health Study, a large population-based multicentric cross-sectional study, conducted between 2016 and 2019 on 18,966 individuals living in Khuzestan province, southwestern Iran. International PA Questionnaire was used for evaluating PA levels, and participants were divided into three groups: low, moderate, and high PA, and ALT and AST were compared between these groups., Results: The mean ± standard deviation age of participants was 38.65 ± 11.40 years. The majority of participants were female (71%). The mean concentration of ALT in total sample was 18.22 ± 13.06 (male: 23.65 ± 16.26 and female: 15.57 ± 10.06), while the mean concentration of ALT in total sample was 19.61 ± 8.40 (male: 22.44 ± 10.03 and female: 18.23 ± 7.08). A statistically significant inverse correlation was found between AST ( r = -0.08, P = 0.02) and ALT ( r = -0.038, P < 0.001) with total PA score. The mean concentration of ALT was 19.96 ± 13.63 in people with low PA, 17.62 ± 12.31 with moderate PA, and 18.12 ± 13.47 with high PA ( P < 0.001). The mean concentration of AST in total sample was 20.37 ± 8.85 in people with low PA, 19.21 ± 8.83 with moderate PA, and 19.75 ± 8.85 with high PA ( P < 0.001). The difference between people in different levels of PA in terms of mean concentration of AST was remained significant ( P = 0.003); however, the difference for ALT was not remained significant after adjusting potential confounders., Conclusion: The current study based on large sample showed that PA had a statistically negative association with the concentration of liver aminotransferases in the seemingly healthy individuals; however, the observed associations were weak. People in the lowest levels of PA had the highest levels of ALT and AST., Competing Interests: There are no conflicts of interest., (Copyright: © 2022 Journal of Research in Medical Sciences.)

Existing asthma treatments are associated with side effects and limitations, which has led to an interest in alternative and complementary therapies. Given the anti-inflammatory properties of pomegranate, the present study aimed to determine the impact of pomegranate extract supplementation on lung function parameters evaluated through spirometry, high-sensitivity C-reactive protein (hs-CRP), pro-oxidant antioxidant balance, and interleukin 35) (IL-35) in participants with mild and moderate allergic asthma (based on forced expiratory volume in 1 second (FEV 1 ) and clinical symptoms). Participants with mild and moderate allergic asthma ( n = 64) were randomly assigned to two groups: the intervention group, which received two pomegranate extract capsules (500 mg/day), or the control group for eight weeks. Also, the physician prescribed similar drugs to the participants in the study. Independent samples T -test and Mann-Whitney U were used to compare the quantitative outcomes between the intervention group and the comparison group. The Wilcoxon test and the paired T -test were applied for within-group comparisons. A p -value <0.05 was considered significant. At the end of the study, the change levels of IL-35 in the intervention group increased significantly compared to the control group. In terms of the lung function parameters, FEV 1 / forced vital capacity (FVC) (FEV 1 /FVC) ratio enhanced significantly in the intervention group compared to the control group. Also, the pomegranate extract significantly improved forced expiratory flow 25-75% (FEF 25-75% ), FEV 1 /FVC ratio, and FEV 1 in the intervention group. No significant changes in FEV 1 values were observed between the two groups at the end of the study. Also, no significant changes were seen in other indicators. It seems that pomegranate extract can improve lung function parameters and IL-35 expression in mild and moderate allergic asthma., Clinical Trial Registration: https://www.irct.ir/trial/45612; identifier: IRCT20200205046384N1., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2022 Shateri, Hosseini, Abolnezhadian, Maraghi, Haddadzadeh Shoushtari and Zilaee.)

Background: Transmission routes of COVID-19 have been well identified and documented. Considering the high prevalence of the Covid-19 and its impacts on the population, this study aimed to assess the status of preventive behaviors against coronavirus infection and estimate the odds of its transmission routes among people. Methods: A comparative study was conducted from March to April 2021. A total of 1256 participants were randomly selected, including 262 COVID-19 patients and 994 healthy people from 10 counties in Khuzestan, southwest Iran. A two-part questionnaire was used for data collection that included items on demographic and adherence to preventive behaviors. Statistical analysis was performed using the statistical software SPSS 18.0.0 applying logistic regression. Results: The mean age of participants was 37.60±11.48 years (female: 36.49±11.15 years; male: 38.86±11.74 years). The results showed that having contact with infected patient at home (OR = 4.90, 95%CI = 3.32-7.25), going to the hospital for not-necessary medical reasons (OR = 4.47, 95%CI = 3.05-6.55), leaving home for essential daily services (OR = 2.49, 95% CI = 1.63-3.81), and going to doctors' office (OR = 1.78, 95% CI = 1.20-2.63) could increase the odds of infection. Conclusion: The findings suggest that different factors are responsible for the increased spread of the Covid-19. Indeed, since the intervention for every single factor will have a small contribution to reducing the prevalence of the disease, it seems essential to design comprehensive interventions while emphasizing isolation and contacts tracing. The study provides evidence for multi-level and multi-faceted policy and interventions for promoting adherence to COVID-19 preventive behaviors., Competing Interests: The authors declare that they have no competing interests., (© 2022 Iran University of Medical Sciences.)

Follicular helper T (TFH) cells are a subset of effector CD4+ T cells that support the differentiation of antigen-specific B cells in the germinal center. TFH cells are distinct from other established CD4+ T cell subsets and possess a list of transcription factors, including BCL6, IRF4, c-Maf, Batf, NFAT1-2, and STAT3. The mentioned factors direct several activities such as cell differentiation, migration to the follicles, cell-to-cell interaction, as well as cell programming. Given that TFH cells are essential for the germinal center formation, affinity maturation and the development of most high-affinity antibodies. TFH cells may play crucial roles in different pathologic conditions, particularly autoimmune diseases. However, the mechanisms that cause functional differences of TFH cell responses are not exactly defined. In this review first the immunological profile of TFH cells will be discussed then attempts will be made to give a broad picture on the role of this key subset of T cells in autoimmune diseases.

Background: Pharmaceutical medications are considered an important component of healthcare delivery and there is a need for its rational use; although there have been major improvements in the pharmaceutical sector in the recent past, inappropriate medication use is considered a major threat to patients' health. Therefore, the pattern of medication use should be monitored routinely. The aim of this study was to investigate the population-based prevalence of polypharmacy and the patterns of medication use in southwestern Iran. We also investigated correlates between polypharmacy and patterns of medication use., Methods: Data from the Khuzestan Comprehensive Health Study (KCHS) which consists information of 30 506 with age range to 20 to 65 years were used. Crude and age-standardized prevalence of polypharmacy and their 95% confidence intervals were estimated for different subpopulations. Multivariable Poisson regression modeling was applied, and adjusted prevalence ratios and their 95% confidence intervals were estimated. The Anatomical Therapeutic Chemical classification system (first and second levels) was used for drug classification in different groups based on age, gender, and socioeconomic status., Results: Out of 30 506 adults, 93.3% were not on any medications, and age- and gender-standardized prevalence of polypharmacy was 0.24%. The lowest estimated prevalence was among Arab participants (0.13%). The prevalence of polypharmacy was 1.57% among elderly people, and 4.78% among patients with heart diseases. Socioeconomic status was not associated with polypharmacy (prevalence ratio: 0.95; p = 0.573) but was significantly associated with patterns of medications use for alimentary tract and metabolism and nervous system diseases. The most common drug classes were cardiovascular system (3.93%), alimentary tract and metabolism (2.79%), nervous system (1.01%), systemic hormonal preparations (0.69%), and blood and blood-forming organs (0.38%)., Conclusions: The prevalence of polypharmacy is very low in Khuzestan province, especially in the Arab population. Future research is needed to investigate the causes of medication underuse in this population., (© 2022 John Wiley & Sons Ltd.)

Background: Despite all recent health-related improvements, anemia remains an extensive global public health issue affecting the lives of about one-fourth of the world population in a geographically heterogeneous pattern. We, therefore, aimed to illustrate the prevalence, severity, most common types, and major determinants of anemia among adults in Khuzestan, Iran, from 2016 to 2019., Methods: In a large population-based cross-sectional study comprising of a diverse population, each participant underwent a questionnaire-based interview and laboratory testing for hematological analysis. A hemoglobin (HGB) concentration of < 12 g/dL in non-pregnant women and < 13 g/dL in men were defined anemic. The multivariate logistic regression analysis was performed to explore the association between anemia and its potential determinants., Results: Data on 29,550 (96.87%) males and non-pregnant females between 20-65 years of age (mean age: 41.90 ± 11.88 years; female sex: 63.58%; Arab ethnicity: 48.65%), whose HGB level was available, were included in the study. The mean ± SD HGB concentration was 13.75 ± 1.65 g/dL. The age- and sex-standardized prevalence rate of anemia was 10.86% (95% CI: 10.51-11.23%). The most prevalent degree was mild anemia (7.71%, 95% CI: 7.40-8.03%) and only 0.17% were severely anemic. Of those considered anemic, the highest proportion was related to normochromic/microcytic (50.65%), followed by hypochromic/microcytic (30.29%). In the multiple logistic regression, the parameters of female gender (OR: 3.17, 95% CI: 2.68-3.76), age group of 35-49 years (OR: 1.66, 95% CI: 1.52-1.82), being underweight (OR: 1.58, 95% CI: 1.29-1.93), being unemployed or retired (OR: 1.55, 95% CI: 1.33-1.81), and living in urban areas (OR: 1.18, 95% CI: 1.09-1.29) were major determinants of anemia. Additionally, we observed a minor but significant positive association between anemia status and CKD, older ages, increased night sleep duration, being a housewife and married, as well as a negative association between anemia and factors including hookah smoking, presence of metabolic syndrome, and overweight and obesity., Conclusions: Taken together, the anemia prevalence in this study population was of mild public health significance. The major suspected causes might be iron deficiency and chronic disease anemias. Comparably higher rates of anemia were observed amongst women, individuals aged 35-49 years, underweights, unemployed or retired subjects, and urban residents., (© 2022. The Author(s).)

In this study, the therapeutic efficacy of quercetin in combination with remdesivir and favipiravir, were evaluated in severe hospitalized COVID-19 patients. Our main objective was to assess the ability of quercetin for preventing the progression of the disease into critical phase, and reducing the levels of inflammatory markers related to SARS-Cov-2 pathogenesis. Through an open-label clinical trial, 60 severe cases were randomly divided into control and intervention groups. During a 7-day period, patients in the control group received antivirals, i.e., remdesivir or favipiravir, while the intervention group was treated with 1000 mg of quercetin daily in addition to the antiviral drugs. According to the results, taking quercetin was significantly associated with partial earlier discharge and reduced serum levels of ALP, q-CRP, and LDH in the intervention group. Furthermore, although the values were in normal range, the statistical outputs showed significant increase in hemoglobin level and respiratory rate in patients who were taking quercetin. Based on our observations, quercetin is safe and effective in lowering the serum levels of ALP, q-CRP, and LDH as critical markers involved in COVID-19 severity. However, according to the non-significant borderline results in comparing the mortality, the ICU-admission rate, and the duration of ICU-admission, further studies can be helpful to compensate the limitations of our study and clarify the therapeutic potential of quercetin in COVID-19 treatments., (Copyright © 2021 Elsevier B.V. All rights reserved.)

Aims: Bleomycin, an important toxic anti-cancer agent, induces pulmonary fibrosis. The significance of oxidative stress and inflammation in promoting of bleomycin-induced idiopathic pulmonary fibrosis (IPF) has been reported. Thus, we evaluated the protective effects of carnosol as a robust natural antioxidant and anti-inflammatory agent for bleomycin-related IPF in rats., Main Methods: Male Wistar rats (n = 40) were randomly assigned to five groups. Group 1 was administrated with saline (intratracheally) on day 7 and oral gavage of dimethyl sulfoxide (DMSO, 0.05%) from day 1 to day 28. Group 2 received a single dose of bleomycin (intratracheally, 7.5 UI/kg) on day 7 and oral gavage of saline for 28 days. Groups 3, 4 and 5 were administrated with bleomycin (single dose) on day 7, along with oral administration of carnosol (at doses 10, 20 and 40 mg/kg, respectively) from day 1 to day 28. The lungs were isolated to measure the histopathological and biochemical and inflammatory markers., Key Findings: Carnosol treatment significantly reduced malondialdehyde, nitric oxide, protein carbonyl, tumor necrosis factor- α, interleukin-6 levels and myeloperoxidase activity in the lungs of rats exposed to bleomycin. Also, lung glutathione content, catalase, glutathione peroxidase and superoxide dismutase activities significantly increased in the carnosol/bleomycin-treated group than the bleomycin group. Lung index, hydroxyproline content, fibrosis and histopathological changes, also significantly decreased by carnosol therapy., Significance: Treatment with carnosol can modulate biochemical and histological alterations caused by bleomycin. Thus, it can be regarded as an appropriate therapeutic approach for IPF., (Copyright © 2021. Published by Elsevier Inc.)

Background: Little is known regarding the impact of quantity and quality of sleep on the incidence of cardiovascular disease. The aim of this study was to investigate the possible independent association of late bedtime and premature coronary artery disease (PCAD)., Methods: Between October 2016 and November 2019, we conducted a cross-sectional population-based study on 30101 participants aged 20-65 years in Khuzestan Comprehensive Health Study (KCHS). Data on major risk factors of cardiovascular disease, habit history, physical activity, and sleep behavior was gathered and participants underwent blood pressure, anthropometric, and serum lipid and glucose profile measurements. PCAD was defined as documented history of developing obstructive coronary artery disease before 45 years in men and before 55 years in women., Results: Of a total of 30101 participants (64.1% female, mean age: 41.7±11.7 years) included in this study, 1602 (5.3%, 95% confidence interval: 5.1%-5.6%) had PCAD. Late bedtime was reported in 7613 participants (25.3%, 95% confidence interval: 24.9%-25.8%). Age-sex standardized prevalence for PCAD and late bedtime were 3.62 (3.43-3.82) and 27.8 (27.2-28.4), respectively. There was no significant difference ( P =0.558) regarding prevalence of PCAD between those with late bedtime (5.5%, 95% CI: 4.9%-6.0%) and those with early bedtime (5.3%, 95% CI: 5.0%-5.6%). However, after adjustment for potential confounders, late bedtime was independently associated with PCAD (OR=1.136, 95% CI=1.002-1.288, P =0.046)., Conclusion: In this study, late bedtime was significantly associated with presence of PCAD. Future prospective studies should elucidate the exact role of late bedtime in developing coronary atherosclerosis prematurely., (© 2021 The Author(s). This is an open-access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/4.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.)

Background: Chronic kidney disease (CKD) is a growing global health problem with faster progression in developing countries such as Iran. Here we aimed to evaluate the prevalence and determinants of CKD stage III+., Methods: This research is part of the Khuzestan Comprehensive Health Study (KCHS), a large observational population-based cross-sectional study in which 30,041 participants aged 20 to 65 were enrolled. CKD was determined with estimated glomerular filtration rate (eGFR) less than 60 ml/min/1.73m 2 , based on two equations of Modification of Diet in Renal Disease (MDRD) and Chronic Kidney Disease Epidemiology Collaboration (CKD-EPI). The multivariate logistic regression was used to evaluate the CKD stage III+ determinants., Results: Prevalence of CKD stage III+ is estimated to be 7.1, 5.5, and 5.4% based on MDRD, CKD-EPI, and combination of both equations, respectively. More than 89% of CKD subjects aged higher than 40 years. In regression analysis, age more than 40 years had the strongest association with CKD stage III+ probability (OR: 8.23, 95% CI: 6.91-9.18). Higher wealth score, hypertension, High-Density Lipoprotein levels less than 40 mg/dl, and higher waist to hip ratio were all associated with CKD stage III+ while Arab ethnicity showed a protective effect (OR: 0.69, 95% CI: 0.57-0.78)., Conclusion: Our findings provide detailed information on the CKD stage III+ and its determinants in the southwest region of Iran. Due to strong association between age and CKD stage III+, within a few decades we might expect a huge rise in the CKD prevalence., (© 2021. The Author(s).)

Background: The Middle East and North Africa (MENA) is postulated to have the highest increase in the prevalence of diabetes by 2030; however, studies on the epidemiology of diabetes are rather limited across the region, including in Iran., Methods: This study was conducted between 2016 and 2018 among Iranian adults aged 20 to 65 years residing in Khuzestan province, southwestern Iran. Diabetes was defined as the fasting blood glucose (FBG) level of 126 mg/dl or higher, and/or taking antidiabetic medications, and/or self-declared diabetes. Prediabetes was defined as FBG 100 to 125 mg/dl. Multinomial logistic regression models were used to examine the association of multiple risk factors that attained significance on the outcome., Results: Overall, 30,498 participants were recruited; the mean (±SD) age was 41.6 (±11.9) years. The prevalence of prediabetes and diabetes were 30.8 and 15.3%, respectively. We found a similar prevalence of diabetes in both sexes, although it was higher among illiterates, urban residents, married people, and smokers. Participants aged 50-65 and those with Body Mass Index (BMI) 30 kg/m 2 or higher were more likely to be affected by diabetes [RR: 20.5 (18.1,23.3) and 3.2 (3.0,3.6)]. Hypertension [RR: 5.1 (4.7,5.5)], waist circumference (WC) equal or more than 90 cm [RR: 3.6 (3.3,3.9)], and family history [RR: 2.3 (2.2,2.5)] were also significantly associated with diabetes. For prediabetes, the main risk factors were age 50 to 65 years [RR: 2.6 (2.4,2.8)], BMI 30 kg/m 2 or higher [RR: 1.9 (1.8,2.0)], hypertension and WC of 90 cm or higher [RR: 1.7 (1.6,1.8)]. The adjusted relative risks for all variables were higher in females than males, with the exception of family history for both conditions and waist circumference for prediabetes., Conclusions: Prediabetes and diabetes are prevalent in southwestern Iran. The major determinants are older age, obesity, and the presence of hypertension. Further interventions are required to escalate diabetes prevention and diagnosis in high-risk areas across Iran.

Background: In 2017, the American College of Cardiology/American Heart Association (ACC/AHA) provided a new guideline for hypertension prevention and management. We aimed to update the prevalence, awareness, control, and determinants of hypertension based on this guideline in Khuzestan province, southwest of Iran, and to estimate the number of people who are eligible for non-pharmacologic and pharmacologic intervention., Methods: This population-based cross-sectional study was conducted in Khuzestan, a large province in the southwest of Iran. Comprehensive information about the potential relating factors of hypertension was collected, blood pressure was measured, and anthropometric measurements were obtained. Moreover, the dietary pattern was evaluated in 2830 individuals, using a qualitative food frequency questionnaire., Results: Among 30,506 participants, 30,424 individuals aged 20-65 years were eligible for the study. In comparison with the previous guideline released by the Joint National Committee (JNC8), the prevalence of hypertension in Khuzestan dramatically increased from 15.81 to 42.85% after implementation of the ACC/AHA guideline, which was more dominant in the male population and the 45-54 age group. The sex and age adjustment of the hypertension prevalence was estimated to be 39.40%. The percentage of hypertension awareness, treatment, and control were 45.85%, 35.42%, and 59.63%, which dropped to 22.72%, 26.37%, and 28.94% after implementation of new guideline, respectively., Conclusions: In the ACC/AHA guideline, a higher number of individuals with the pre-hypertension condition were shifted into the hypertension category and the level of awareness, treatment, and control were dramatically decreased, which highlight a great need to expand the public health infrastructure for further managing the substantial increased burden on healthcare system. However, further studies with population over 65 years are required to estimate the eligibility for antihypertensive treatment in this province after implementation of new guideline.

Purpose: Metabolic syndrome (MetS) is a cluster of risk factors, mainly central obesity, insulin resistance, and dyslipidemia, leading to life-threatening cardiovascular diseases. The prevalence of MetS can vary based on different ethnicities and many cultural and lifestyle factors., Aims: We aimed to evaluate the prevalence of MetS and possible correlations with lifestyle-associated factors among different ethnicities in Khuzestan, Iran., Methods: This cross-sectional study was conducted in Khuzestan province of Iran, among 30,504 participants aged 20-65 years, between October 2016 and November 2019. Data was collected through questionnaires along with anthropometric and biological measurements. The National Cholesterol Education Program Adult Treatment Panel III definition was used to estimate MetS prevalence., Results: Overall, 31.9% (95% CI 31.4-32.4) had MetS (34.2% [95% CI 33.3-35.1] among males; 30.7% [95% CI 30.0-31.3] among females [p < 0.001]). Central obesity, elevated fasting blood sugar levels, and dyslipidemia were the most common abnormalities among those with MetS. The risk of MetS was estimated to increase by age, male gender, residing in urban regions, lower educational levels, lower physical activity levels, lower sleep time, and a positive family history of diabetes mellitus (p < 0.001). Individuals of the Arab and Bakhtiary ethnicities had the highest and lowest risk of MetS, respectively., Conclusion: MetS prevalence varied among different ethnicities. Aging and some lifestyle-associated factors such as physical activity and sleep time were related to the risk of MetS. Raising awareness about risk factors of MetS would be of great value in setting new health policies to manage the rising trend of MetS., Competing Interests: Conflicts of interestThe authors have no relevant financial or non-financial interests to disclose., (© Springer Nature Switzerland AG 2021.)

Although it is estimated that COVID-19 life-threatening conditions may be diagnosed in less than 1:1000 infected individuals below the age of 50, but the real impact of this pandemic on pediatric patients with different types of primary immunodeficiency (PID) is not elucidated. The current prospective study on a national registry of PID patients showed that with only 1.23 folds higher incidence of infections, these patients present a 10-folds higher mortality rate compared to population mainly in patients with combined immunodeficiency and immune dysregulation. Therefore, further management modalities against COVID-19 should be considered to improve the survival rate in these two PID entities using hematopoietic stem cell transplantation and immunomodulatory agents.

Background: X-linked chronic granulomatous disease (X-CGD) is an immunodeficiency disorder caused by defects in the gp91 phox subunit that leads to life-threatening infections. We aimed to identify CYBB gene mutations and study clinical phenotypes in Iranian patients with probable X-CGD., Methods: We studied four unrelated Iranian patients with probable X-CGD and their families recruited in several years. We isolated genomic DNA from whole blood and performed Sanger sequencing in the CYBB gene's coding and flanking regions. We also performed pathogenicity predictions using in silico tools., Results: We detected four different mutations, including a novel insertion mutation in exon 5 (p.Ile117AsnfsX6), in the patient. Bioinformatics analysis confirmed the pathogenic effect of this mutation. We predicted protein modeling and demonstrated lost functional domains. The patient with the insertion mutation presented pneumonia and acute sinusitis during his life. We also detected three other known nonsense mutations (p.Arg157Ter, p.Arg226Ter, and p.Trp424Ter) in the CYBB gene. The patient with p.Arg157Ter developed lymphadenitis and pneumonia. Moreover, the patient with inflammatory bowel disease showed p.Arg226Ter and the patient with tuberculosis presented p.Trp424Ter. We detected different clinical features in the patients compared to other Iranian patients with the same mutations., Conclusion: Our results expand the genetic database of patients with X-CGD from Iran and make it much easier and faster to identify patients with X-CGD. Our results also help to detect carriers and enable prenatal diagnosis in high-risk families as a cost-effective strategy., (© 2020 The Authors. Journal of Clinical Laboratory Analysis published by Wiley Periodicals LLC.)

Background: Irritable bowel syndrome (IBS) is a functional disease with no exact laboratory or imaging findings. IBS is more common in areas with a history of psychological trauma and war. This study aims to report the prevalence and possible determinants of IBS in southwestern Iran, an area with a notable history of war., Methods: We randomly enrolled 1849 permanent residents in 29 cities aged 20 to 65 years. A validated for Farsi version Rome III criteria and a questionnaire, including demographic data and health history, were administered to each subject. Participants who fulfilled the Rome III criteria were categorized into three groups: Diarrhea dominant (IBS-D), Constipation dominant (IBS-C), and Mixed type (IBS-M)., Results: The total prevalence of IBS was 3.2%, with 70% of subjects being of Arab descent ( P =0.004). IBS was more common in females, singles, illiterate subjects, and people younger than 30 years; however, none of these differences were statistically significant. People with depression, anxiety, self-report of psychological disorders, and very low socioeconomic status had a significantly higher prevalence of IBS ( P <0.05). After multivariable logistic regression analysis, very low socioeconomic status had an independent role in IBS predictivity (OR: 2.28, 95% CI: 1.01-5.15)., Conclusion: This study shows a higher prevalence of IBS symptoms in a population-based study in the region compared to counterparts in other regions of Iran. Considering the higher prevalence of self-reported psychological disorders, further studies are recommended to focus on the exact diagnosis of mental disorders and their influence on IBS., (© 2020 The Author(s). This is an open-access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/4.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.)

The emergence of a highly pathogenic virus named severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) accounts for severe pneumonia throughout the world. More than 7 million world population have been infected with SARS-CoV-2, and the number of deaths is increasing every day. This study aimed to evaluate the frequency of SARS-CoV-2 in hospitalized patients with an acute respiratory infection (ARI). During an outbreak of the SARS-CoV-2, the nasopharyngeal and oropharyngeal swabs were collected from 909 hospitalized patients with severe pneumonia, including 517 (56.9%) males and 392 (43.1%) females. All the collected samples were from different cities of Khuzestan province from 19 February to- 27 March 2020. The RNA was extracted from samples and subjected to real-time polymerase chain reaction (PCR) tests for the detection of the SARS-CoV-2. Simultaneously, the computerized tomography (CT) scan was tested for the presence of ground-glass opacity in the lung among the patients. Of the total number of 909 specimens, 328 (36.08%) cases, including 185 (20.35%) females and 143 (15.73%) males, were positive for the SARS-CoV-2 while, 581 (63.9%) cases, including 374 (41.14%) males and 207 (22.77%) were negative for the SARS-CoV-2 by real-time PCR (p=0.001).Four hundred sixteen (45.76%) cases were positive for ground-glass opacity in the lung by CT scan, while 328/909 (36.08%) trials proved positive for SARS-CoV-2 by the real-time PCR (p=0.003).  In this study, 36.08% of patients were positive for SARS-CoV-2. Although the results of positive cases by CT scan showed higher than real-time PCR, screening the SARS-COV-2 with a real-time PCR method is the first line of choice.

Recurrence of severe microbial infections results from a primary immunodeficiency disorder known as major histocompatibility complex class II deficiency or bare lymphocyte syndrome type II. Immunologic function is severely impaired due to the absence of MHC class II molecules on the surface of immune cells. Here, we report a 5-year-old boy with a novel homozygous mutation in RFXANK gene that negatively affects the proper expression of MHC class II molecules by antigen presenting cells. The frame shift mutations in RFXANK gene and negative HLA-DR proteins expression on peripheral blood mononuclear cells were identified and confirmed by whole exome sequencing, Sanger sequencing, and flow cytometry. The patient was referred with long-term severe prolonged diarrhea, fever, coughing, and vomiting. Also, antibiotic resistance, normal T cell, and NK cell counts with low B cell count and reduced serum immunoglobulin level were observed. The patient did not give a dramatic response to intravenous immunoglobulin infusion. The significancy of this report is the novelty of mutation and low B cell count which is not commonly expected in such patients. The final diagnosis of BLS type II is based on WES, Sanger sequencing, and flow cytometric evaluation. Moreover, it seems that the only therapeutic choice is hematopoietic stem cell transplantation.

Poikiloderma is a hereditary pathologic situation in which the appearance of skin rash is associated with epidermal atrophy, telangiectasia, and reticular dyspigmentation skin symptoms of poikiloderma are usually caused by sun damage. The main reason forpoikiloderma is unknown. We introduce a 14- month-old boy who referred to our center with a complaint of fever and cough. Furthermore, hepatosplenomegaly symptoms had been presented at the time of birth and were continuously observed at age one. He had transient thrombocytopenia when he was born due to his prematurity condition, which was resolved during Intravenous Immunoglobin (IVIG) treatment. Therefore, the presence of various mutation scan lead to distinct clinical symptoms. Immunohematologic abnormalities such as increased level of IgM and IgE antibodies, as well as increased C-reactive protein (CRP) and Erythrocyte sedimentation rate (ESR), have been reported. However, mutation of the C16orf57 gene was identified in this patient. We also introduced a new genetic mutation in a particular part of DNA sequence (NM&#95;001195302: exon6: c.T703C) that leads to new clinical finding in PN.

Background: Predominantly antibody deficiencies (PADs) are the most common primary immunodeficiencies, characterized by hypogammaglobulinemia and inability to generate effective antibody responses., Objective: We intended to report most common monogenic PADs and to investigate how patients with PAD who were primarily diagnosed as suffering from agammaglobulinemia, hyper-IgM (HIgM) syndrome, and common variable immunodeficiency (CVID) have different clinical and immunological findings., Methods: Stepwise next-generation sequencing and Sanger sequencing were performed for confirmation of the mutations in the patients clinically diagnosed as suffering from agammaglobulinemia, HIgM syndrome, and CVID., Results: Among 550 registered patients, the predominant genetic defects associated with agammaglobulinemia (48 Bruton's tyrosine kinase [BTK] and 6 μ heavy chain deficiencies), HIgM syndrome (21 CD40 ligand and 7 activation-induced cytidine deaminase deficiencies), and CVID (17 lipopolysaccharides-responsive beige-like anchor deficiency and 12 atypical Immunodeficiency, Centromeric instability, and Facial dysmorphism syndromes) were identified. Clinical disease severity was significantly higher in patients with μ heavy chain and CD40 ligand mutations compared with patients with BTK (P = .003) and activation-induced cytidine deaminase (P = .009) mutations. Paralysis following live polio vaccination was considerably higher in patients with μ heavy chain deficiency compared with BTK deficiency (P < .001). We found a genotype-phenotype correlation among patients with BTK mutations regarding clinical manifestation of meningitis and chronic diarrhea. Surprisingly, we noticed that first presentations in most patients with Immunodeficiency, Centromeric instability, and Facial dysmorphism were respiratory complications (P = .008), whereas first presentations in patients with lipopolysaccharides-responsive beige-like anchor deficiency were nonrespiratory complications (P = .008)., Conclusions: This study highlights similarities and differences in the clinical and genetic spectrum of the most common PAD-associated gene defects. This comprehensive comparison will facilitate clinical decision making, and improve prognosis and targeted treatment., (Copyright © 2018 American Academy of Allergy, Asthma & Immunology. Published by Elsevier Inc. All rights reserved.)

Background: Asthma is a heterogeneous disease which is usually associated with chronic airway inflammation. Saffron has anti-inflammatory effects and may has beneficial effects on asthma., Hypothesis: The present study was intended to survey the effects of saffron supplementation on blood pressure, lipid profiles, basophils, eosinophils and clinical symptoms in patients with allergic asthma., Study Design: Our study was a clinical trial., Methods: Subjects (N = 80, 32 women and 48 men, 41.25 ± 9.87 years old) with mild and moderate allergic asthma were randomized into two groups: the intervention group who received two capsules of saffron (100 mg/d), and the control group who received two capsules of placebo for 8 weeks. SPSS software (version 16.0) was used for the data analysis., Results: Saffron improved the frequency of clinical symptoms of the patients (i.e., frequency of the shortness of breath during the day and night time, use of salbutamol spray, waking up due to asthma symptoms and activity limitation) in comparison to the placebo (p < 0.001). Besides, asthma severity decreased almost significantly in the saffron group (p = 0.07). It was also found that saffron, in comparison with the placebo, significantly reduced the systolic and diastolic blood pressure, triglycerides and low density lipoprotein cholesterol. Moreover, eosinophils and basophils concentration reduced in the saffron group (p = 0.06 and 0.05 respectively)., Conclusion: Saffron seems to be an effective and safe option (in 8 weeks supplementation) to improve clinical symptoms of patients with allergic asthma but the toxicity and/or long-term effects of saffron intake are not known. Registration ID in IRCT (IRCT2017012132081N2).

Major histocompatibility complex (MHC) class II deficiency is a rare primary immunodeficiency disorder (PID) with less than 200 cases worldwide. Here, we report an 8 month-old girl with MHC class II deficiency with a novel homozygous mutation in RFXANK gene (NM&#95;001278728: exon 5: c.495G>A: p.Trp165*) and normal CD4+ T cell counts, diagnosed by whole exome sequencing (WES) and negative HLA-DR proteins on peripheral blood mononuclear cell (PBMC) in flow cytometry. She was referred with pneumonia, prolonged fever, resistance to antibiotics (ceftriaxone, clindamycin, and vancomycin), and low serum immunoglobulin (IG) levels, while natural killer (NK), B, and T cells were normal. She received intra-venous immune-globulin (IVIG) replacement, broad spectrum antibiotics, and anti-fungal treatments. The presented case report is interesting not only because of the rarity of the PID but also due to normal CD4+ T cell counts. According to our experience, we suggest that physicians consider MHC class II deficiency in families with consanguineous marriages, even with normal CD4+ T cell counts. At the first, the diagnosis of the disease could be successfully perform using WES, and finally, treatment with hematopoietic stem cell transplantation can save the patients' lives.

Background: The number of inherited diseases and the spectrum of clinical manifestations of primary immunodeficiency disorders (PIDs) are ever-expanding. Molecular diagnosis using genomic approaches should be performed for all PID patients since it provides a resource to improve the management and to estimate the prognosis of patients with these rare immune disorders., Method: The current update of Iranian PID registry (IPIDR) contains the clinical phenotype of newly registered patients during last 5 years (2013-2018) and the result of molecular diagnosis in patients enrolled for targeted and next-generation sequencing., Results: Considering the newly diagnosed patients (n = 1395), the total number of registered PID patients reached 3056 (1852 male and 1204 female) from 31 medical centers. The predominantly antibody deficiency was the most common subcategory of PID (29.5%). The putative causative genetic defect was identified in 1014 patients (33.1%) and an autosomal recessive pattern was found in 79.3% of these patients. Among the genetically different categories of PID patients, the diagnostic rate was highest in defects in immune dysregulation and lowest in predominantly antibody deficiencies and mutations in the MEFV gene were the most frequent genetic disorder in our cohort., Conclusions: During a 20-year registration of Iranian PID patients, significant changes have been observed by increasing the awareness of the medical community, national PID network establishment, improving therapeutic facilities, and recently by inclusion of the molecular diagnosis. The current collective study of PID phenotypes and genotypes provides a major source for ethnic surveillance, newborn screening, and genetic consultation for prenatal and preimplantation genetic diagnosis.

Limited evidence has been obtained concerning the beneficial effects of autologous serum therapy in treatment of skin disorders particularly chronic urticaria. In the present study, we have assessed the effect of this treatment method in patients with chronic urticaria (CU). This randomized single-blind controlled trial was performed on fifty consecutive patients with chronic urticaria. The patients were randomly assigned to receive autologous serum (as the case group, n=35) or normal saline (as the control group, n=15) and treated with monthly autologous serum therapy or normal saline for 6 months. The considered study endpoint was changes in total severity score (TSS) at the 6 months follow-up visit. The TSS score was assessed at baseline as well as at the ninth week and the sixth month of interventions. The mean±SD of TSS at the ninth week of intervention was 10.94±3.92 in autologous serum therapy group and 11.67±2.72 in the normal saline group (p=0.458). Furthermore, the mean values of TSS at the sixth month of treatment in the study groups were 8.29±6.29 and 9.27±4.89 respectively (p=0.593). A downward trend in TSS, from baseline to the end of treatment, was seen in the case and control groups (p<0.001 for both), however the trend of this decline was insignificant between the two groups (p=0.592). The change in the trend of TSS after 6 months of treatment was independent from the administration of autologous serum when compared with normal saline administration (beta=-0.962, p=0.630). Multivariate linear regression model with the presence of baseline factors including gender, age, disease duration and history of atopy was performed to assess difference in TSS at six-month follow-up visit compared with the baseline value. Only young age was associated with more reduction of TSS (beta=0.163, p=0.023). We found no difference in the effects of autologous serum therapy and normal saline on the trend of the changes in disease severity in patients with chronic urticaria.

Major histocompatibility complex (MHC) class II deficiency is a primary immunodeficiency disease characterized by abnormality of MHC class II molecules surface expression on peripheral blood lymphocytes and monocytes. Clinical manifestations include extreme susceptibility to viral, bacterial, and fungal infections but the immunodeficiency is not as severe as SCID (severe combined immunodeficiency), as evidenced by failure to develop disseminated infection after BCG vaccination. Therefore, MHC II deficiency with BCGosis, that is disseminated BCGitis, is not reported commonly. We report an interesting case of BCGosis after vaccination that was diagnosed to have probable MHC II deficiency.

Background: Ataxia telangiectasia (AT) is one of the combined immunodeficiency syndromes with immunologic, neurologic, endocrinologic, hepatic and cutaneous abnormalities. Regarding the fact that autoimmune disorders; such as autoimmune hemolytic anemia (AIHA), are not generally expected in the course of AT, we present a patient with an unusual presentation of these two conditions., Case Presentation: An otherwise seemingly normal girl, who had developed limping at the age of 11 months old, referred to Namazi Hospital, Shiraz, Iran, due to pallor and latitude at the age of 3 yrs and was diagnosed of AIHA. After 2 years of therapeutic course she developed ocular telangiectasia and ataxic gate., Conclusion: This case emphasizes on the possibility of ataxia telangiectasia coexistence with autoimmune disorders that must be taken into consideration by physicians.