Tehran University of Medical Sciences Immunology and Genetics Journal 2645-4831 7 2 2024 06 22 60 65 Azar Dastranji Pediatric Pulmonologist,Assistant professor ,Pediatric Health Research center of mardany azarTabriz children hospital,Tabriz university of medical Sciences Yalda Jabbri Moghaddam Tabriz University of Medical Sciences, Tabriz, Iran Mahsa Hesamifard Tabriz University of Medical Sciences, Tabriz, Iran Mahnaz Sadeghi-Shabestari Tuberculosis and Lung Disease Research Center, Immunology Research Center of Tabriz,Mardany Azar Children Hospital,Tabriz University of Medical Sciences, Tabriz, Iran 2023 05 17 2024 02 05 Background: Asthma and Allergic rhinitis (AR) are frequently concurrent diseases. Epidemiological, immunological, and clinical studies reported increasing evidence of the links between AR and asthma. Epidemiologically, up to 40% of patients with AR also have asthma, and up to 80% of patients with asthma experience nasal symptoms. Furthermore, patients with AR are at three times the risk of developing asthma compared with those without AR. Method: This cross-sectional study was carried out during one year from all patients between 0-16 years who were referred to allergy and ENT clinics with symptoms of chronic cough (more than four weeks) or upper respiratory tract symptoms such as sneezing, runny nose, and nasal congestion. Data and diagnosis were classified according to the Global Initiative for Asthma (GINA) and the AR criteria and its Impact on Asthma guidelines (ARIA), respectively. Results: 190 (120 individuals with asthma and 70 individuals with AR) were enrolled in the study. The mean age of patients with asthma was 9.14±3.13, and 43.3% were female. The severity of asthma was classified as mild (20%), moderate (70.8%), and severe (9.2%). The mean age of patients with rhinitis was 10.13±3.45 years, and 35.7% were female. Rhinitis was classified as intermittent (40%) and persistent (60%). The prevalence of AR in the asthma group was 42 individuals (35%). Twenty-nine cases of them (24.2%) had intermittent AR, and 13 (10.8%) cases had persistent AR. The prevalence of asthma in AR was 18 cases (25.7%) 3 cases (4.3%) had mild asthma, 12 cases (17/1%) had moderate asthma, and 3 cases (4.3%) had severe asthma. A significant correlation was found ( P<0.0001) between the severity of AR and asthma. Conclusion: Based on the findings of our study, there is a significant relationship between the severity of asthma and AR in patients with asthma and AR. The results show that asthma prevalence is high in patients with AR. Also, the frequency of AR in patients with asthma is significantly higher. https://igj.tums.ac.ir/index.php/igj/article/view/131 https://igj.tums.ac.ir/index.php/igj/article/download/131/142

Tehran University of Medical Sciences Immunology and Genetics Journal 2645-4831 7 2 2024 06 22 47 52 Reza Yazdani Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children's Medical Center Hospital, Tehran University of Medical Sciences, Tehran, Iran; Primary Immunodeficiency Diseases Network (PIDNet), Universal Scientific Education and Research Network (USERN), Tehran, Iran Samaneh Abdolahzade Department of Pediatric Disease, School of Medicine, Zanjan University of Medical Sciences, Zanjan, Iran Parisa Ashournia Division of Allergy and Clinical Immunology, Department of Pediatrics, Pediatrics Center of Excellence, Children's Medical Center Hospital, Tehran University of Medical Sciences, Tehran, Iran Aida Askarisarvestani Allergy Research Center, Shiraz University of Medical Sciences, Shiraz, Iran Sima Bahrami Department of Allergy and Clinical Immunology, Rasool Akram Hospital, Iran University of Medical Sciences, Tehran, Iran Maryam Behfar Pediatric Cell and Gene Therapy Research Centre, Gene, Cell & Tissue Research Institute, Tehran University of Medical Sciences, Tehran, Iran Zahra Daneshmandi Pediatric Respiratory Diseases Research Center, National Research Institute of Tuberculosis and Lung Diseases (NRITLD), Shahid Beheshti University of Medical Sciences, Tehran, Iran Mohammad Hassan Bemanian Department of Allergy and Clinical Immunology, Rasool Akram Hospital, Iran University of Medical Sciences, Tehran, Iran Taher Cheraghi Department of Pediatrics, 17th Shahrivar Children's Hospital, Guilan University of Medical Sciences, Rasht, Iran Kian Darabi Department of Allergy and Clinical Immunology, Rasool Akram Hospital, Iran University of Medical Sciences, Tehran, Iran Sepideh Darougar Department of Pediatrics, Faculty of Medicine, Tehran Medical Sciences, Islamic Azad University, Tehran, Iran Samaneh Delavari Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children's Medical Center Hospital, Tehran University of Medical Sciences, Tehran, Iran; Primary Immunodeficiency Diseases Network (PIDNet), Universal Scientific Education and Research Network (USERN), Tehran, Iran Sarehsadat Ebrahimi Division of Allergy and Clinical Immunology, Department of Pediatrics, Pediatrics Center of Excellence, Children's Medical Center Hospital, Tehran University of Medical Sciences, Tehran, Iran Shabnam Eskandarzadeh Department of Pediatrics, Pediatric Health Center, Tabriz University of Medical Sciences, Tabriz, Iran Golnaz Eslamian Department of Allergy and Clinical Immunology, Shahid Beheshti University of Medical Sciences, Tehran, Iran Narges Eslami Department of Allergy and Clinical Immunology, Shahid Beheshti University of Medical Sciences, Tehran, Iran Shahrzad Fallah Immunology and Allergy Department, Mofid Children's Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran Morteza Fallahpour Department of Allergy and Clinical Immunology, Rasool Akram Hospital, Iran University of Medical Sciences, Tehran, Iran Saba Fekrvand Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children's Medical Center Hospital, Tehran University of Medical Sciences, Tehran, Iran; Primary Immunodeficiency Diseases Network (PIDNet), Universal Scientific Education and Research Network (USERN), Tehran, Iran Ali Haghbin Department of Pediatrics, North Khorasan University of Medical Sciences, Bojnurd, Iran Amir Ali Hamidieh Pediatric Cell and Gene Therapy Research Centre, Gene, Cell & Tissue Research Institute, Tehran University of Medical Sciences, Tehran, Iran Zohre Hassanpoor Allergy Research Center, Mashhad University of Medical Sciences, Mashhad, Iran Zahra Hamidi Esfahani Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children's Medical Center Hospital, Tehran University of Medical Sciences, Tehran, Iran; Primary Immunodeficiency Diseases Network (PIDNet), Universal Scientific Education and Research Network (USERN), Tehran, Iran Tolue Mahdavi Department of Allergy and Clinical Immunology, Rasool Akram Hospital, Iran University of Medical Sciences, Tehran, Iran Mehrnaz Mesdaghi Department of Allergy and Clinical Immunology, Shahid Beheshti University of Medical Sciences, Tehran, Iran Homa Hatefi Minaei Dorsa Pharmaceutical Co, Alborz, Iran Majid Marjani Clinical Tuberculosis and Epidemiology Research Center, National Research Institute of Tuberculosis and Lung Diseases, Shahid Beheshti University of Medical Sciences, Tehran, Iran Shahla Mirazizi Pharmaceutical Sciences Research Center, Health Institute, Kermanshah University of Medical Sciences, Kermanshah, Iran Mohammadreza Modaresi Pediatric Pulmonary Disease and Sleep Medicine Research Center, Pediatric Center of Excellence, Children's Medical Center Hospital, Tehran University of Medical Sciences, Tehran, Iran Mahshid Movahedi Division of Allergy and Clinical Immunology, Department of Pediatrics, Pediatrics Center of Excellence, Children's Medical Center Hospital, Tehran University of Medical Sciences, Tehran, Iran Mohammamd Nabavi Department of Allergy and Clinical Immunology, Rasool Akram Hospital, Iran University of Medical Sciences, Tehran, Iran Mojtaba Ranjbar Children’s Medical Center Hospital, Pediatric Center of Excellence, Tehran University of Medical Sciences, Tehran, Iran Anahita Razaghian Division of Allergy and Clinical Immunology, Department of Pediatrics, Pediatrics Center of Excellence, Children's Medical Center Hospital, Tehran University of Medical Sciences, Tehran, Iran Mohammad Saberi Department of Medical Genetics, School of Medicine, Tehran University of Medical Sciences, Tehran, Iran Mahnaz Sadeghi-Shabestari Department of Immunology and Allergy, Tabriz University of Medical Sciences, Tabriz, Iran Mitra Sahragard Department of Immunology and Allergy, Tabriz University of Medical Sciences, Tabriz, Iran Fereshte Salami Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children's Medical Center Hospital, Tehran University of Medical Sciences, Tehran, Iran; Primary Immunodeficiency Diseases Network (PIDNet), Universal Scientific Education and Research Network (USERN), Tehran, Iran Sahar Seraj Department of Immunology and Allergy, Shahid Beheshti University of Medical Sciences, Tehran, Iran Seyedehshabnam Seyedsalehi Abuzar Children’s Medical Center, Ahvaz Jundishapur University of Medical Sciences, Ahvaz, Iran Alireza Shafiei Department of Immunology, Bahrami Hospital, Tehran University of Medical Sciences, Tehran, Iran Roya Sherkat mmunodeficiency Diseases Research Center, Isfahan University of Medical Sciences , Isfahan , Iran Reza Shiari Division of Pediatric Rheumatology, Department of Pediatrics, Mofid Children's Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran Sima Shokri Department of Allergy and Clinical Immunology, Rasool Akram Hospital, Iran University of Medical Sciences, Tehran, Iran Saman Tavakoli Department of Pediatric, School of Medicine, Isfahan University of Medical Sciences, Isfahan, Iran Ahmad Vosughimotlagh Immunology and Allergy Department, Mofid Children's Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran Niloufar Yazdanpanah Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children's Medical Center Hospital, Tehran University of Medical Sciences, Tehran, Iran; Network of Immunity in Infection, Malignancy and Autoimmunity (NIIMA), Universal Scientific Education and Research Network (USERN), Tehran, Iran Vahid Ziaee Pediatric Rheumatology Research Group, Rheumatology Research Center, Tehran University of Medical Sciences, Tehran, Iran Nima Rezaei Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children's Medical Center Hospital, Tehran University of Medical Sciences, Tehran, Iran; Primary Immunodeficiency Diseases Network (PIDNet), Universal Scientific Education and Research Network (USERN), Tehran, Iran 2025 01 12 2025 01 12 At present, a national consensus or guideline for diagnosing and managing patients suspected of having predominantly antibody deficiencies (PADs) is lacking. This consensus is written based on a combination of scientific literature and comments from the expert panel of Iranian immunologists. A group of clinical immunologists reviewed the current consensus, presented their comments at a meeting titled 'First Meeting on the Diagnosis of Inborn Errors of Immunity (IEI) by IEI Experts,' and agreed on this consensus. This consensus guideline provides recommendations on the diagnosis, antimicrobial prophylaxis, management of clinical manifestations, and immunoglobulin replacement therapy (IgRT) for patients with PAD. https://igj.tums.ac.ir/index.php/igj/article/view/167 https://igj.tums.ac.ir/index.php/igj/article/download/167/141

Tehran University of Medical Sciences Immunology and Genetics Journal 2645-4831 7 2 2024 06 22 82 86 Mostafa Kamali Network of Immunity in Infection, Malignancy and Autoimmunity (NIIMA), Universal Scientific Education and Research Network (USERN), Tehran, Iran Sepideh Shahkarami Department of Pediatrics, Dr. von Hauner Children's Hospital, University Hospital, Ludwig-Maximilians-Universität München (LMU), Munich, Germany; Medical Genetics Network (MeGeNe), Universal Scientific Education and Research Network (USERN), Munich, Germany Elham Rayzan Research Center for Immunodeficiencies, Children’s Medical Center, Tehran University of Medical Sciences, Tehran, Iran; International Hematology/Oncology of Pediatrics Experts (IHOPE), Universal Scientific Education and Research Network (USERN), Tehran, Iran. Iraj Mohammazadeh Noncommunicable Pediatric Diseases Research Center, Amirkola Hospital, Babol University of Medical Sciences, Babol, Iran Meino Rohlfs Department of Pediatrics, Dr. von Hauner Children's Hospital, University Hospital, Ludwig-Maximilians-Universität München (LMU), Munich, Germany Christoph Klein Department of Pediatrics, Dr. von Hauner Children's Hospital, University Hospital, Ludwig-Maximilians-Universität München (LMU), Munich, Germany Nima Rezaei Network of Immunity in Infection, Malignancy and Autoimmunity (NIIMA), Universal Scientific Education and Research Network (USERN), Tehran, Iran; Research Center for Immunodeficiencies, Children’s Medical Center, Tehran University of Medical Sciences, Tehran, Iran; Department of Immunology, School of Medicine, Tehran University of Medical Sciences, Tehran, Iran 2025 01 08 2025 01 12 Background: Griscelli syndrome type II is a primary immunodeficiency disorder caused by RAB27A gene mutation and is inherited in an autosomal recessive manner and characterized by oculocutaneous hypopigmentation and various cellular immune system deficiencies. Case Presentation: Herein, we report a 5-year-old girl with silvery-gray hair, eyebrows, and eyelashes who were referred to our primary immune deficiency clinic because of recurrent oral thrush. Further investigations were performed to uncover the probable underlying genetic disorder. Whole-exome sequencing revealed a novel mutation in the RAB27A gene (c.137T>G) and confirmed the diagnosis of Griscelli syndrome type 2. Conclusions: Due to the poor prognosis nature of this disorder and also its need for differential diagnosis with some other conditions with hypopigmentation, prompt diagnosis, genetic analysis, and proper treatment are necessary for avoiding serious complications. https://igj.tums.ac.ir/index.php/igj/article/view/163 https://igj.tums.ac.ir/index.php/igj/article/download/163/146

Tehran University of Medical Sciences Immunology and Genetics Journal 2645-4831 7 2 2024 06 22 66 71 Reza Shiari Department of Pediatric Rheumatology, School of Medicine, Shahid Beheshti University of Medical Sciences, Tehran, Iran Navid Roshanzamir Department of Internal Medicine, School of Medicine, Shahid Beheshti University of Medical Sciences, Tehran, Iran Mahsa Erfanian-Salim Department of Pediatrics, School of Medicine, Shahid Beheshti University of Medical Sciences, Tehran, Iran 2025 01 12 2025 01 12 Background: Systemic juvenile idiopathic arthritis (sJIA) is determined by prominent multisystemic involvements with onset before the age of 16 years of which IL-6 has a significant role in inflammatory process. Tocilizumab (TCZ) is a human anti-IL-6 receptor monoclonal antibody, which modulates IL-6 activity by blocking its binding to the soluble and membrane-bound IL-6 receptor. No comprehensive study has been done regarding the use of IL-6 inhibitor in the treatment of SJIA patients in Iran. This study was conducted with the aim of investigating the effectiveness and outcome of IL-6 inhibitor treatment in Iranian children with sJIA. Methods and Materials: This is a case-control study including twenty patients diagnosed with sJIA divided equally in two groups of case and control who received TCZ and corticosteroids respectively. Subsequently, various clinical and laboratory features were compared amongst these two groups. Results: A significant difference before and after TCZ has been seen in terms of height increase, weight gain, and decrease in leukocytosis, thrombocytosis and ESR, CRP negativity and arthritis improvement. Conclusions: Considering the favorable effects of TCZ in the control of sJIA, by producing this drug in the country and making it available to patients at a lower cost, it is possible to better control the disease and reduce the harmful effects caused by the disease and reduce the long-term use of corticosteroids. https://igj.tums.ac.ir/index.php/igj/article/view/166 https://igj.tums.ac.ir/index.php/igj/article/download/166/143

Tehran University of Medical Sciences Immunology and Genetics Journal 2645-4831 7 2 2024 06 22 72 76 Shamsolmolouk Najafi Dental Research Center, School of Dentistry, Tehran University of Medical Sciences, Tehran, Iran Farzaneh Bolandparva Department of Oral Medicine, School of Dentistry, Tehran University of Medical Sciences, Tehran, Iran Arezou Rezaei Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children's Medical Center, Tehran University of Medical Sciences, Tehran, Iran Mohammad Javad Kharrazifard Department of Oral Medicine, School of Dentistry, Tehran University of Medical Sciences, Tehran, Iran Nima Rezaei Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children's Medical Center, Tehran University of Medical Sciences, Tehran, Iran; Department of Immunology, School of Medicine, Tehran University of Medical Sciences, Tehran, Iran; Network of Immunity in Infection, Malignancy and Autoimmunity (NIIMA), Universal Scientific Education and Research Network (USERN), Stockholm, Sweden 2025 01 08 2025 01 12 Background: Recurrent aphthous stomatitis (RAS) is an inflammatory nature, while interleukin 17 (IL-17) has a pre-inflammatory role. In the present study, serum level of IL-17 was measured in a group of individuals with RAS, compared to healthy individuals. Methods: This case-control study was done on 36 patients with RAS as cases and 36 healthy subjects as controls. The level of IL-17 was measured, using ELISA method. Results: The mean serum level of IL-17 in cases was 0.14±0.21 pg/ml, which was insignificantly lower than 0.21±0.17 pg/ml in control group (p=0.12). Meanwhile there was a significant reverse correlation between age and serum level of IL-17 (r=-0.32, p=0.006). Conclusion: Although the serum level of IL-17 in RAS patients was lower than the controls, the difference was not significant. However, significantly lower serum level of IL-17 in older subjects. https://igj.tums.ac.ir/index.php/igj/article/view/164 https://igj.tums.ac.ir/index.php/igj/article/download/164/145

Tehran University of Medical Sciences Immunology and Genetics Journal 2645-4831 7 2 2024 06 22 176 176 Pardis Shirkani Department of Science, University of British Columbia, Vancouver, British Columbia V6T 1Z4, Canada Fatemeh Dehghani Department of Immunology, Asthma and Allergy, The Persian Gulf Tropical Medicine Research Center, The Persian Gulf Biomedical Sciences Research Institute, Bushehr University of Medical Sciences, Bushehr, Iran Batoul Amiri Clinical Research Development Center, The Persian Gulf Martyrs Hospital, Bushehr University of Medical Sciences, Bushehr, Iran Afshin Shirkani Allergy and Clinical Immunology Department, Bushehr University of Medical Science, School of Medicine, Bushehr, Iran 2025 02 13 2025 02 13 Background: Asthma and allergic rhinitis are prevalent respiratory conditions influenced by genetic, environmental, and immunological factors. Understanding the distribution of these conditions and their associated allergens is essential for effective management and prevention strategies. This study aimed to investigate the prevalence of asthma, allergic rhinitis, and their co-occurrence in a specific population, with a focus on allergen sensitivity patterns. Methods: A retrospective analysis was conducted on patients diagnosed with asthma, allergic rhinitis, or both. Allergen sensitivity was assessed using skin prick tests for common environmental allergens, including weeds, mites, cockroaches, and mixed allergens. Data analysis included prevalence rates, allergen distribution, and a heatmap visualization of allergen-patient associations. Results: Males demonstrated a higher prevalence of asthma (68.8%), allergic rhinitis (64.4%), and combined conditions (67.0%) compared to females. Weeds were the most common allergen among asthma patients (40.0%), while mixed allergens were predominant among allergic rhinitis (45.7%) and coexisting conditions (50.0%). Heatmap analysis revealed strong associations between mites, cockroaches, and respiratory conditions, reinforcing their role as significant allergens. The high prevalence of mixed allergens in patients with both conditions suggests the need for comprehensive allergy management approaches. Conclusion: This study highlights a male predominance in asthma and allergic rhinitis cases and underscores the role of environmental allergens in disease manifestation. The findings support targeted allergen avoidance and personalized immunotherapy strategies for optimal respiratory disease management. https://igj.tums.ac.ir/index.php/igj/article/view/176