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فهرست مطالب نویسنده:

soheila alyasin

  • Hossein Esmaeilzadeh, Babak Shahhosseini *, Mohammad Amin Gholami, Hesamedin Nabavizadeh, Soheila Alyasin, Negar Mortazavi

    Chronic rhinosinusitis is divided into two groups, which are Chronic rhinosinusitis with nasal polyps (CRSwNP) and without nasal polyps (CRSsNP). The rate of post-surgical recurrence in the CRSwNP is high, and predicting factors are unknown. This study aims to identify and evaluate risk factors associated with treatment-resistant and recurrent CRSwNP.
    This cross-sectional study evaluates demographic data and atopic risk factors in patients with CRSwNP, including a high IgE level (≥100 U/mL), skin prick test (SPT) for aeroallergens, aspirin-exacerbated respiratory disease (AERD), and asthma prevalence. An oral aspirin challenge was performed to diagnose AERD. 191 patients with CRSwNP were enrolled, with 73 patients in the recurrent, and 118 patients in the non-recurrent group. The mean age of the patients in the recurrent group was 45.08±12.05. The mean age of the patients in the non-recurrent group was 42.89±11.73. 49. Asthma prevalence in recurrent- CRSwNP is significantly higher than non-recurrent CRSwNP Asthma severity in recurrent CRSwNP and AERD patients was significantly higher than in nonrecurrent CRSwNP and non-AERD patients. The level of IgE in the recurrent- CRSwNP is higher than non-recurrent CRSwNP. Positive SPT results for tree, weed, and mite allergens were higher in the non-recurrent- CRSwNP group compared to the recurrent CRSwNPgroup.
    Asthma had a significantly higher difference in AERD compared to non-AERD. The level of IgE in AERD is higher than non-AERD.
    Recurrent CRSwNP patients and AERD patients had Higher IgE levels. Asthma is more prevalent and more severe in both AERD and recurrent CRSwNP. However, a positive SPT result has been seen higher in non-recurrent CRSwNP.

    Keywords: Aspirin-Exacerbated Respiratory Disease, Chronic Rhinosinusitis With Nasal Polyps, Recurrent Nasal Polyposis, Skin Prick Test
  • Aida Askarisarvestani *, Seyed Hesamedin Nabavizadeh, Mina Esmailli, Soheila Alyasin, Hossein Esmaeilzadeh
    Background
    Multisystem Inflammatory Syndrome in Children (MIS-C) is a rather newly described entity that can potentially end in multisystem failure in children following COVID-19 infection. The prognosis of patients with MIS-C is multifactorial; consequently, many risk factors increase the risk of mortality and severity of this disease. In this study we aimed to evaluate the prognostic effect of various parameters in mortality and intensive care unit admission of patients with MIS-C.
    Methods
    in this cross-sectional study, the information of patients with MIS-C were extracted in a tertiary pediatric center during a one-year period. The relationship between mortality and ICU admission of the patients with demographic information and lab data were assessed.
    Results
    a total of 88 male-predominant (56.8% vs. 43.2%, P=0.135) entered the study. Seven patients had expired and 71 patients were discharged from the hospital. In our study, demographic information of the patients and their lab data were not associated with mortality except for Lactate Dehydrogenase (LDH) level. All of the expired patients had elevated LDH, while only 53.1% of the discharged patients showed increased LDH (P=0.016); on the other hand, LDH did not differ between patients who were managed in ICUs and the ones who were managed in wards.
    Conclusion
    LDH can be counted as a prognostic tool for mortality in MIS-C and might be regarded as a part of evaluation for ICU admission in this disease.
    Keywords: MIS-C, LDH, COVID-19
  • Hossein Esmaeilzadeh, Maryam Zare, Soheila Alyasin, Hesamedin Nabavizadeh, Negar Mortazavi, Zahra Kanannejad

    Aspirin-exacerbated respiratory disease (AERD) is a chronic inflammatory disease. It is defined by asthma, chronic rhinosinusitis with nasal polyposis, and a hypersensitivity reaction to aspirin or nonsteroidal anti-inflammatory drugs. Aspirin desensitization (AD) has been confirmed as an effective treatment to control AERD inflammation through the modulation of immune responses. We aimed to review AERD with an overview of the epidemiology, pathophysiology, and treatment. We also discussed the effect of AD on immunological markers involved in AERD pathogenesis. A search of electronic databases on AERD was performed. We included five randomized clinical trials (RCTs) on AD. We also searched databases for recent studies that investigated the effect of AD on the immunological mechanisms of AERD. RCTs have demonstrated the therapeutic effectiveness of AD on the patients’ quality of life, asthma symptom score, inhaled and oral steroid use, forced expiratory volume in 1 sec (FEV1), and inflammatory mediators. The clinical benefits of AD can occur though the regulation of innate and adaptive immune responses that are involved in the pathogenesis of AERD. In addition to the valuable effects of AD in RCTs, some side effects such as gastrointestinal bleeding, asthma exacerbation, or rash have been reported that should be considered for reaching an optimal protocol for AD.

    Keywords: Aspirin-exacerbated respiratory disease, Aspirin desensitization, Immune responses, Inflammations, Leukotrienes
  • Seyed Hesamedin Nabavizadeh, Hossein Esmaeilzadeh, Soheila Alyasin, Abbas Avazpour, Aida Askarisarvestani *

    Patients with Severe Combined Immunodeficiency are at high risk of Transfusion Associated Graft Versus Host Disease (TAGVHD) if they receive a non-radiated blood product that has a high mortality rate. This Case study reports on the case of a premature baby who had anemia of prematurity with a normal level of WBC. He received packed red blood cells, but after a while, he developed severe nausea and skin lesions. Endoscopy was done and the biopsy from the gastrointestinal lesions as well as the biopsy from the skin lesions showed graft versus host disease when he was 6 months old. However, he has received Hematopoietic Stem Cell Transplantation and is well now after about 4 months from his transplant.

    Keywords: SCID, Blood transfusion, GVHD
  • Hossein Esmaeilzadeh, Seyed Sina Dehghani *, Babak Shahhoseini, Soheila Alyasin, Sayyed Hesamedin Nabavizadeh, Aida Askari

    A novel coronavirus disease known as Coronavirus Disease 2019 (COVID-19) has spread quickly throughout the world, and it was declared a pandemic in March 2022. Chronic granulomatous disease (CGD) is a diverse group of genetic disorders characterized by recurrent bacterial and fungal infections, resulting in granulomas due to the inability of phagocytes to destroy microbes. Even though it is thought that impaired neutrophil activity is a protective mechanism against severe COVID-19-induced cytokine storms and hyper-inflammatory responses, patients with CGD have normal immunity to most viruses. Here, we present two CGD patients who were hospitalized due to severe COVID-19 infections, which suggests that COVID-19 might have a different pathogenesis than other viruses.

    Keywords: Chronic granulomatous disease, Coronavirus disease 2019
  • Sara Kashef, Mozhgan Moghtaderi, Hamid Reza Hatami, Mehdi Kalani, Soheila Alyasin, Hesamodin Nabavizadeh, Shirin Farjadian *

    A decrease in T cell count or reduced T cell function can be indicative of T cell immunodeficiency. In the present study, T-cell function was assessed using Carboxyfluorescein diacetate succinimidyl ester (CFSE) dilution test after stimulation with commonly used Phytohaemagglutinin (PHA) or anti-CD3/anti-CD28 coated beads in pediatric patients with recurrent infections. Seven infants with recurrent infections and seven sex/age-matched healthy infants were included in this study. A blood cell count, immunophenotyping, and serum immunoglobulin level were performed. The proliferation of T cells was also assessed with CFSE dilution after stimulation with PHA or anti-CD3/anti-CD28 coated beads.  This study showed increased IgA, IgG, and IgM levels in patients compared to the controls. In contrast to the controls, the immunophenotyping results showed a significant decline in the number of CD4+ T cells in patients. Although there was no difference in CD3+ T cell proliferation between patients and controls, the CD4+ and CD8+ T cell proliferation rates were significantly decreased in patients when stimulated with PHA. As a mitogen with the potential for maximum proliferation of T cells, PHA is better able to distinguish between patients with recurrent infections and controls than anti-CD3/anti-CD28, which mimics only the TCR pathway for stimulation of T cells.

    Keywords: Anti-CD3, Anti-CD28, Carboxyfluorescein diacetate succinimidyl ester, Lymphocyte transformation, Phytohaemagglutinin
  • Hossein Esmaeilzadeh, Aida Askari, Negar Mortazavi, Seyed Hesamodin Nabavizadeh, Soheila Alyasin
    Background

    Elevated serum levels of IgM and recurrent infections, mainly respiratory tract infections, could be the presenting features in some ataxia-telangiectasia (AT) patients, and may initially be misdiagnosed as hyper-IgM (HIgM) syndrome. Class switch recombination (CSR), which is defective in HIgM syndrome, is an important mechanism in the maturation of B lymphocytes to produce different isotypes of antibodies in response to antigen stimulation.

    Methods

    The clinical manifestations and laboratory findings of 16 cases with low IgA and IgG levels, and normal to elevated IgM levels with CSR defect are reported.

    Results

    In 16 cases, the median age at onset of the diseases, and median age at the time of the diagnosis were 1 year (interquartile range [IQR] = 1.6), and 4 years (IQR = 3.1), respectively. Two of the patients (12.5 %) died due to respiratory infection. In this study, Out of the studied population, four were male (25%), and 12 were female (75%). Most of the patients had consanguineous parents (81.3 %). All of the patients had ataxia, and 15 patients had telangiectasia (93.8 %), and one of the cases had malignancy (dermatofibroma). Also, 15 patients presented infections (93.8 %). Autoimmunity was seen in three patients (18.8 %). In addition, some of the patients manifested hepatosplenomegaly (31.3 %) and thrombocytopenia (18.8 %). Neurological manifestations, such as visual impairment (12.5 %), epilepsy (6.3 %), and tremor (12.5 %), were also present.

    Conclusion

    AT patients with HIgM phenotype and CSR defect, compared to other AT patients, may present different clinical manifestations, such as various infections. Considering their manifestations, the management and treatment of these patients are necessary.

    Keywords: Ataxia-Telangiectasia, Case Series, Class Switch Recombination Defect, Hyper-IgM
  • Maryam Babaei, Zahra Kanannejad, Najmeh Sepahi, Soheila Alyasin *

    Both adaptive and innate immune responses are essential for an effective defense against the severe acute respiratory syndrome Coronavirus 2 (SARS-CoV-2) infection. We aimed to investigate the effect of the coronavirus disease 2019 (COVID-19) pandemic on patients with primary immunodeficiency (PID). This study was performed on patients who were diagnosed with PID by immunologist specialists and referred to Imam Reza Clinic of Asthma and Allergy, affiliated with Shiraz University of Medical Sciences, (Shiraz, Iran) for regular check-ups. The patients were enrolled in this cohort study and followed for any sign of COVID-19 from March 2020 to May 2021. COVID-19 infection was confirmed using a real-time polymerase chain reaction (RT-PCR) assay of nasal and pharyngeal swabs. Among the 90 PID patients under study, nine patients (10%) were diagnosed positive for COVID-19 infection. Five out of these nine patients belonged to the combined immunodeficiency (CID) category, while four patients were categorized as having primary antibody deficiencies (PADs). Eight patients with COVID-19 were required to be admitted to the hospital, and three patients died after hospitalization due to COVID-19 infection. It seems that patients with CID are at a higher risk of mortality, due to COVID-19 infection, that other types of PID.

    Keywords: COVID-19, Mortality, Primary immunodeficiency diseases
  • Najmeh Sepahi, Soheila Alyasin, Zahra Kanannejad, Hossein Esmaeilzadeh, Farzaneh Mohammadalizadeh Shirazi, Maryam Babaei, Shirin Farjadian*

    Pollens have been identified as potent inducers of allergic diseases worldwide. Acer velutinum (Persian maple) tree is an important source of allergic pollens in Iran. This study aimed to identify the immunoglobulin E (IgE)-reactive components of A. velutinum pollen extract in patients with maple allergy. We aimed to evaluate its allergenic components; using IgE in the serum of patients with maple allergy. Twenty-two patients with a clinical history of reaction and a positive skin-prick test to maple pollen extract were included in this study. Identification of IgE-binding proteins in A. velutinum pollen extract was performed by immunoblotting using sera from sensitive patients. A protein band with a molecular weight of around 70 kDa was the most IgE-reactive allergen in A. velutinum pollen extract detected by this method. Identification of a protein with a molecular weight of about 70kDa, as the most reactive allergen of A. velutinum pollen extract, can be considered as a potential allergen for designing diagnostic kits or as a target for immunotherapy of allergic patients with maple allergy.

    Keywords: Hypersensitivity, Immunoglobulin E, Pollen
  • Soheila Alyasin, Zahra Kanannejad, Hossein Esmaeilzadeh, Hesamedin Nabavizadeh, Reza Amin

    Asthma is one of the most common respiratory diseases caused by chronic airway inflammation. A complex network of cytokines could affect asthma development.IL-4, IL-13, IL-17, and IL-33 have been identified as cytokines associated with asthma severity and these cytokines can be considered as candidate biomarkers for predicting the asthma severity while the IL-10 is lower in asthmatics compared with healthy subjects. There are many controversies about the IL-22, IL-25, and TGF-β levels between the Iranian publications. No significant differences have been observed between the healthy subjects and the asthmatic cases regarding the IL-6 and IL-8 levels.

    Keywords: Cytokine, Level, Asthma, Biomarker, Iran
  • Maryam Babaei, Zahra Kanannejad, Soheila Alyasin *
    Background

    Bronchiectasis is a clinical syndrome characterized by chronic cough, sputum production, recurrent respiratory infections, and permanent bronchial dilation.  The association between the level of alpha-1 antitrypsin (AAT) and bronchiectasis is controversial. In this study, we aimed to investigate this association in children with idiopathic bronchiectasis.

    Methods

      The study was conducted on 20 patients with idiopathic bronchiectasis as the case group (mean age 15.9±2.1) and 20 healthy individuals as the control group (mean age 14.9±2.6). Serum AAT level was measured using nephelometric analysis (g/L). Other criteria including sex, parent consanguinity, number of hospitalization, age of the first symptom were evaluated in both groups related to AAT level.

    Results

    The mean serum level of AAT in the case and control groups were 1.3±0.29; 1.5 ±0.59, respectively with statistical significance (P=0.001). There was a significant difference between the two groups in the AAT level distribution, according to AAT normal range (p=0.01). The case group had a more positive attitude toward consanguinity than the control group (66.7% versus 33.3%; P<0.001). The results showed that 80% of patients had the first symptom of disease under 1 year of age, 6.6% 1- 5 years, 6.6% 5- 10 years, and 6.6% in more than 10 years old. In the case group, 53.3% had a history of medical hospitalization for one time, 26.7% two times, while 20% of the patients had no medical hospitalization.

    Conclusion

    Decreased AAT serum level and high consanguinity rates may be considered as two risk factors for idiopathic bronchiectasis occurrence in children.

    Keywords: Alpha-1 antitrypsin, Idiopathic bronchiectasis, Nephelometry
  • Heidar Ali Kafashan, Ahmad Reza Khosravi, Soheila Alyasin*, Najmeh Sepahi, Zahra Kanannejad, Farzaneh Mohammad Ali Zadeh Shirazi, Sahar Karami

    Airborne pollen is considered one of the causative agents of hay fever, allergic rhinitis, conjunctivitis, and asthma. We aimed to investigate airborne pollens in the context of Shiraz located in the southwest of Iran and find their association with meteorological parameters.  The survey was conducted from October 2017 to September 2018, using seven days of volumetric Burkard spore trap, located in the center of the city. A total of 5810 pollen grains/m3 belonging to 15 taxa were identified and recorded. Among them, 73.8% was the tree, while the grass, shrub, and weed constituted 13.56%, 3.5%, and 9.2% of total reported pollens, respectively. The major pollen types were Platanaceae (28.39%), Oleaceae (21.17%), Pinaceae (15.11%), Amaranthaceae (9.29%), and Brassicaceae (8.02%). A higher number of pollen counts and types were recorded in March, followed by September, while it was lower in May. Meteorological parameters were correlated with the monthly pollen counts. Wind speed was found to have a positive correlation with Platanaceae concentration. The significant correlation between pollen concentration and the temperature was positive for Poaceae, Amaranthaceae, and Plantaginaceae and negative for Rosaceae, Oleaceae, and Ulmaceae.  Poaceae and Amaranthaceae were negatively correlated with humidity and positively with Rosaceae, Oleaceae, and Plantaginaceae. A negative correlation was found between rainfall and Poaceae and Amaranthaceae, while Plantaginaceae had a positive correlation with this parameter. The results of this study may be helpful for allergologists in the diagnosis and treatment of airborne allergic disorders due to pollen grains.

    Keywords: Hypersensitivity, Meteorological factors, Polle
  • Soheila Alyasin, Zahra Kanannejad*, Hossein Esmaeilzadeh, Hesamedin Nabavizadeh, Mohammad Amin Ghatee, Reza Amin

    Bacillus Calmette Guerin (BCG) was designed for protecting children against tuberculosis. Also, it can protect against other infectious diseases through the induction of trained immunity. Due to its heterologous protective effects, the BCG vaccine has been proposed as a treatment option for coronavirus disease-2019 (COVID-19). Epidemiological studies have found that countries without BCG vaccination policy have experienced higher mortality rates related to COVID-19 infection than those with BCG vaccination policy. However, there are some confounding factors such as age, population intensity, immigration, the pandemic phase, and data accuracy that may affect these results. Therefore, this hypothesis should be evaluated by clinical trial studies. Large-scale clinical trials are in progress to investigate if the BCG vaccine could be used as a useful tool for protection against COVID-19 infection.

    Keywords: COVID-19, Mycobacterium bovis
  • Seyed Hesam Nabavizadeh, Davoud Almasi, Aida Askari, Soheila Alyasin, Negar Mortazavi, Hossein Esmaeilzadeh *
    Background
    Asthma is chronic respiratory inflammatory disease with prevalence of 10-20% in children and 5-10% in adults. Main treatment of asthma constituted of inhaled corticosteroids (ICS) which can impede the progression of pathology in asthmatic patients. Potentially side effects of corticosteroids may prevent some patients from taking medication, which can increase exacerbation and and severity of asthma. We aimed to investigate the prevalence and reasons of fear towards corticosteroids in asthma patients.
    Methods
    In this cross-sectional study asthma patients viewpoint regarding inhaled and oral corticosteroids in the management of asthma was assessed using questionnaire. Exclusion criteria were the patients afflicted with chronic respiratory diseases other than asthma and the ones who were taking corticosteroids for other reasons. The data were analyzed using SPSS version 25.
    Results
    50.7% of patients were male. The mean age of the subjects was 10.71 years and the mean duration of disease and treatment course were 3.2 and 2.2 years respectively. 75.4% of patients had regular follow-up. 56.6% did not know the mechanism of action of corticosteroids in asthma and 75.4% of patients had concerns regarding the adverse effects of corticosteroids. The most common concerns reported were drug dependency (14.9%) followed by osteoporosis (14.3%). 40.8% of patients claimed that they use corticosteroids with peace of mind if their physician prescribes them.
    conclusion
    75%of patients had concerns regarding the adverse effects of corticosteroids and the most common concerns reported were drug dependency (14.9%).
    Keywords: Asthma, corticosteroid, adverse effect, phobia
  • Soheila Alyasin, Zahra Kanannejad*, Hossein Esmaeilzadeh, Hesameddin Nabavizadeh, Reza Amin

    Context:

     Chronic airway inflammation in asthma is affected by a complex network of interacting cytokines. The exact role of each cytokine in childhood asthma development has remained poorly understood. In this study, we aimed to review articles investigating cytokine levels and polymorphisms in childhood asthma among the Iranian population to assess susceptible cytokines related to childhood asthma.

    Evidence Acquisition: 

    We performed a literature search in PubMed, Scopus, Science direct, and Embase databases to find articles that have evaluated the cytokine levels and gene polymorphisms in childhood asthma among the Iranian population until March 2, 2020. The terms used to search included “cytokine”, “interleukin”, “polymorphism”, “Asthma”, and “Iran” in the international databases. Only case-control studies with a healthy control group that investigated cytokine levels and polymorphism in childhood asthma among the Iranian population have been included.

    Results

    Considering these criteria, we extracted 7 articles from international databases and included them in the current review. We investigated 4 cytokine levels and 4 cytokine polymorphism patterns in asthmatic and non-asthmatic subjects in Iran. Interleukin (IL)-23, IL-17, and IL-33 levels were statistically higher in asthmatic children, and also IL-33, IL-17 levels were associated with asthma severity. There were no significant differences between healthy and asthmatic subjects regarding IL-35 levels. Polymorphisms in cytokine IL-4, IL-10, tumor necrosis factor (TNF)-α, and IL-2 were susceptible to childhood asthma in the Iranian population. 

    Conclusions

    Increased IL-33 and IL-17 levels were related to disease severity in childhood asthma. Four cytokine polymorphisms (IL-4, IL-10, IL-2, and TNF-α) were associated with the risk of pediatric asthma in the Iranian population.

    Keywords: Cytokine, Asthma, Children, Polymorphism
  • Zahra Hojaji *, Sayyed Hesamedin Nabavizadeh, Hossein Esmaeilzadeh, Soheila Alyasin, Mohammad Paktinat
    Introduction
    Because some children have allergies to egg as an important food source, finding the right alternative is essential. To date, the interaction between other birds’ eggs and hen egg has not been investigated in Iran. Because of the common usage of eggs from birds other than hens in Iran, the present study aimed to compare hens’ eggs with the eggs of other birds.
    Methods
    In this case-control study, 70 children who referred to our allergy clinic were evaluated. In patients who had a history of egg allergy, SPT and specific IgE Ab analyses were done, and then an oral food challenge (OFC) was performed. Patients with egg allergy underwent skin prick tests with eggs from birds such as turkey, goose, duck, quail, partridge, and pigeon.
    Results
    From 40 patients with egg allergy, 8 patients passed the OFC. The greatest cross-reactions were seen in turkey and quail egg extracts. The least cross-reactions were reported in partridge and pigeon. Among patients who had negative SPT with partridge egg (N=3), one patient passed and two failed the OFC. Three of the patients who had negative pigeon SPT (N=5) passed the OFC with pigeon egg.
    Conclusion
    In conclusion, turkey, goose, duck, and quail eggs cannot be used as an alternative to hen eggs; only partridge or pigeon eggs can be considered as a suitable alternative to hen eggs.
    Keywords: Hen Egg, Cross-Reaction, Egg Allergy
  • Ali Aghebati Maleki, Tina Shahani, Tooba Momen, Soheila Alyasin, Majid Changi Ashtiani, Alireza Biglari, Mohammad Shahrooei, Asiyeh Sadat Javanian, Suzan Amini, Xavier Bossuyt, Hassan Rokni Zadeh*

    The prevalence of primary immunodeficiency (PID) is rather high in Iran compared to the world average, mainly due to the high rate of consanguineous marriage. Despite that, little genetic information is available about primary immunodeficiencies in Iran. Autosomal recessive hyper IgE syndrome (AR-HIES) is a severe type of immunodeficiency, mainly caused by mutations in the dedicator of cytokinesis 8 (DOCK8). Rapid and precise diagnoses of patients suffering from AR-HIES can help to manage the patients and reach properly the treatment decision. However, in regions with low financial resources and limited expertise, deep phenotyping is uncommon. Therefore, an exome-first approach is helpful to make a genetic-based diagnosis. In the present study, whole-exome sequencing (WES) was applied to detect causative mutations in three unrelated primary immunodeficient patients with poor clinical information. One of the cases was a deceased patient with suspected hyper IgE syndrome (HIES) whose parents were subjected to WES. As a result, three novel pathogenic variants were detected in the DOCK8 gene, including two splicing sites (c.4241+1G>T and c.4886+1G>T) and one-stop-gain (c.4201G>T, p.Glu1401Ter) variants. Sanger sequencing confirmed the mutations’ segregation in corresponding families. Further immunological investigations confirmed that HIES in the studied probands. The presence of frontal bossing and broad nose in one of the studied cases, in addition to the typical clinical presentation of DOCK8-AR-HIES, is notable. This work suggests that an exome-first approach can be a valuable alternative strategy for precise diagnosis of primary immunodeficiency patients.

    Keywords: Dedicator of cytokinesis 8, Exome-first approach, Hyper IgE syndrome, Wholeexome sequencing
  • Soheila Alyasin, Hossein Esmaeilzadeh*, Narjes Ebrahimi, Seyed Hesamedin Nabavizadeh, Hamid Nemati
    Background

    Ataxia-telangiectasia is a multi-system disorder in which neurologic impairment and immune deficiency are observed. In the present study, patients with ataxia-telangiectasia were followed to provide information regarding clinical and immunological features.

    Methods

    We report a case series of 18 patients diagnosed with ataxia-telangiectasia, who were referred to a tertiary center of clinical immunology from 2008–2018. Clinical presentations, medical records and lab data were observed during this period with a mean follow-up time of 4.57 ± 2.66 years.

    Results

    The mean age of the patients was 10.92 ± 3.24 years (11 females and 7 males). Thirteen patients (72.22%) were from families with consanguinity. Ataxia was the most common clinical feature, observed in 18 (100%) patients. The predominant clinical presentations were tremor and oculocutaneous telangiectasia, observed in 14 (77.8%) patients; dysarthria and oculomotor apraxia, observed in 13 (72.2%) patients. Infections were recorded in 12 (70.6%) patients. Decreased IgG level and IgA levels were observed in 5 (33.3%) and 6 (40.0%) patients, respectively. Decreased B-cell number and T-cell number were noted in 7 (46.67%) and 11 (73.33%) patients, respectively. Three (16.7%) patients were diagnosed with acute lymphoblastic leukemia and two of them expired subsequently.

    Conclusion

    Ataxia-telangiectasia is a progressive disease with no established therapy; so, it necessitates early diagnosis and follow-up of the patients. The presented clinical and immunological data in this study may help with diagnosis and management of the disease complications.

    Keywords: Ataxia telangiectasia, Clinical manifestations, Immunologic factors
  • Hossein Esmaeilzadeh, Shirin Farjadian *, Soheila Alyasin, Hamid Nemati, Hesamodin Nabavizadeh, Elmira Esmailzadeh
    Severe cutaneous adverse drug reaction (SCAR) is considered to be a multifactorial drug side effect. This study was designed to investigate the epidemiology and human leukocyte antigen (HLA)-A and -B gene polymorphisms in pediatric patients with SCAR admitted in tertiary referral center, southwestern of Iran from 2013 to 2017. Demographic data, past allergy and autoimmune history, clinical presentations, drugs confirmed to be the cause of SCAR as well as its therapy were reviewed for each patient. HLA-A and -B allele frequencies were determined in 40 of the patients using polymerase chain reaction based on sequence specific primers (PCR-SSP) and compared with 40 healthy individuals as control group.Sixty-one patients with mean age of 6 years old and boy to girl ratio was 1.2/1 in this study. The most common type of SCAR in our patients was Steven Johnson Syndrome (SJS)/Toxic Epidermal Necrosis (TEN) mainly caused by beta-lactam antibiotics. Carbamazepine was the second cause of drug–induced SCAR. Moreover, HLA-A*02:01 and A*51:01 were related to the increased risk of SCAR while A*11:01 seemed to be protective against SCAR. HLA-A*02:01, HLA-A*24:02, and HLA-B*51:01 showed associations to the increased risk of SJS. Based on our results, beta-lactam antibiotics and antiepileptic drugs are the most common causes of severe adverse drug reaction in southwestern Iranian pediatric patients. Moreover, some HLA-A alleles can influence risk of SCAR.
    Keywords: DHR, SCAR, STS-TEN, DRESS, AGEP, HLA
  • Negar Mortazavi, Hossein Esmaeilzadeh, Mohammad Abbasinazari, Delara Babaie, Soheila Alyasin, Hesamodin Nabavizadeh, Elmira Esmailzadeh
    This study aimed to investigate the efficacy and the underlining mechanism of aspirin desensitization among patients with Aspirin Exacerbated Respiratory Disease (AERD). Thirtyeight patients, who had undergone an aspirin challenge test and were diagnosed as having AERD, were engaged in a double-blind randomized clinical trial. They were divided into two groups—an active group of patients who went through aspirin desensitization, and the control group, receiving placebo. Clinical symptoms and the quality of life of the patients—in addition to the levels of interleukin 4 and 5 (IL4), (IL5)—were documented at the beginning of the study and again after six months of aspirin desensitization. The quality of life of the patients was significantly higher in the active group after six months (P = 0.001). Medication requirements and symptom score were manifested to be significantly lower in the active group after six months than at the beginning of the study (P = 0.005, 0.017 respectively). Forcedexpiratory volume in the second one (FEV1) was, also, significantly higher in the active group after six months of the study (P = 0.032). IL5 was found to be significantly lower in the active group after six months (P = 0.019). However, no significant difference was observed in the levels of IL4 between the two groups (P = 0.152). The study revealed that aspirin desensitization can improve the quality of life of patients with AERD, lessen their symptoms and medication requirements, lower their levels of IL5, and improve some pulmonary function tests such as FEV1.
    Keywords: AERD, Aspirin, Desensitization, Interleukin-5, Interleukin-4
  • Soheila Alyasin, Reza Amin, Ali Fazel *, Mohammad Hossein Karimi, Seyed Hesamedin Nabavizadeh, Hossein Esmaeilzadeh, Maryam Babaei
    Background
    Asthma is the chronic inflammation of airways characterized by eosinophilic infiltration, mucus overproduction, airway hyper-responsiveness and airway remodeling. These changes are induced mostly by cytokines which are produced by T helper (Th) 2 cells. Recently, the role of interleukin-23 (IL-23) in the pathogenesis of adult allergic asthma has been studied.
    Objective
    To explore IL-23 serum levels and its expression in persistent asthma compared with healthy children younger than five years old.
    Method
    Blood samples of 40 children with mild and severe persistent asthma were compared to 34 healthy children regarding IL-23 serum levels and gene expression using enzyme-linked immunosorbentassay (ELISA) and real time quantitative polymerase chain reaction (PCR).
    Results
    The IL-23 gene expression level was significantly different in the 25 children with mild persistent asthma and the 15 children with severe persistent asthma compared to the control group (p=0.001). There was no significant difference in IL-23 gene expression level between the two groups of patients with mild and severe persistent asthma. A significant difference was seen in IL23 serum levels between the 25 children with persistent asthma and control group (p=0.002).
    Conclusion
    For pre-school children with history and physical exam in favor of asthma which cannot be tested by spirometry, IL-23 serum levels may be an auxiliary biomarker for the diagnosis of asthma.
    Keywords: Asthma, Childhood, Interleukin-23
  • Soheila Alyasin*, Marzieh Adab*, Asieh Hosseinpour, Reza Amin, Maryam Babaei
    Background
    Systemic Lupus Erythematosus (SLE) is a chronic autoimmune disease which is characterized by B-cell abnormality and auto-antibody generation. Since bacterial infections are the most important causes of mortality in these patients, pneumococcal vaccination is recommended for children with SLE.
    Objective
    To investigate humoral immunity and specific-antibody formation in response to a 23-valent polysaccharide pneumococcal vaccination in SLE children and asthmatic control group.
    Method
    The case and control groups consisted of 30 children with the mean age of 13 years who were matched by sex and age. Anti-pneumococcal antibody titers were determined using Enzyme-Linked Immunosorbent Assay (ELISA) before the vaccination with the 23-valent pneumococcal vaccine and 3 weeks later in both groups. Also the correlation between anti-pneumococcal antibody titer and different factors including age, sex, lupus activity, disease duration, medications, history of recurrent infections, and laboratory data were investigated.
    Results
    Both groups showed significant increases in anti-pneumococcal antibody level after vaccination (p≤0.001). The increase in antibody level were almost the same in both groups (p≥0.05) such that 77.7% of SLE children and 86.2% of control children showed at least 2-fold increase in anti-pneumococcal antibody titer following immunization. Significant correlations were seen between the level of post-immunization anti-pneumococcal antibody with the age of children with SLE (p=0.02) and their age of disease onset (p=0.02).
    Conclusion
    It is concluded that pneumococcal vaccination is generally immunogenic in children with SLE. However, a small group of patients show impaired response to the vaccine.
    Keywords: 23, Valent Pneumococcal Vaccine, Anti, Pneumococcal Antibody, Children, Immunogenicity, Systemic Lupus Erythematosus (SLE)
  • Farhad Abolnezhadian, Soheila Alyasin, Reza Amin, Maryam Babaei
    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
    Keywords: Angioedema, Antihistamines, Urticaria
  • Short Time Efficiency of Rhinophototherapy in Management of Patients with Allergic Rhinitis Resistant to Medical Therapy
    Soheila Alyasin, Seyed Hesamedin Nabavizadeh, Hamidreza Houshmand, Hossein Esmaeilzadeh, Sina Jelodar, Reza Amin
    Allergic rhinitis is one of the most common health problems with a major effect on the quality of life. We intended to treat Allergic Rhinitis (AR) in patients who are either unresponsive to antihistamines or their job requires optimal alertness that may be disturbed by antihistamine’s side effects and those who do not comply with the regular use. We tried short term phototherapy and evaluated its effect on AR. As phototherapy is effective in the treatment of atopic dermatitis (AD) and the same allergens can produce both AD and AR, phototherapy is proposed as a new tool in the AR treatment. In AD, phototherapy causes induction of apoptosis in infiltrating T cells and other immunomodulatory effects. We performed a randomized single-blind study to investigate the effect of low-dose phototherapy in AR patients. Among AR patients who did not respond to local and systemic therapy, we chose 62 allergic patients all above 25 years of age with moderate to severe AR whose disease was verified by allergy skin test or specific IgE to allergens; then, they were randomly divided into 31 patients as treatment group and 31 patients as control group. In treatment groups, we used a mixture of UVA, UVB and visible light. In the control group, we used visible light alone as placebo. Then we evaluated the level of response to treatment in two groups and compared them according to Total Nasal Symptom scores (TNSS) and Global Severity Scores (GSS) and Rhinoconjunctivitis Quality of Life Questionnaires (RQLQ) symptom scores. We found out that phototherapy in the treatment group in comparison with placebo was effective in treatment of AR (p-value
    Keywords: Allergic rhinitis, Phototherapy, Quality of life, Treatment
  • Soheila Alyasin, Maryam Khoshkhui, Farhad Abolnezhadian
    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 with AIHA. After 2 years of therapeutic course she developed ocular telangiectasia and ataxic gate.
    Conclusion
    This case emphasizes the possibility of ataxia telangiectasia coexistence with autoimmune disorders and must be taken into consideration by physicians.
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