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فهرست مطالب نویسنده:

mahboubeh mansouri

  • پری موسی پور میاندهی *، محبوبه منصوری

    پژوهش حاضر با هدف تحلیل محتوای کتاب های پایه پنجم ابتدایی براساس میزان توجه به مولفهای محیط زیستی انجام شده است. روش پژوهش توصیفی که از روش تحلیل محتوا به صورت کیفی، کمی انجام شده است. جامعه آماری، کتابهای پایه پنجم دوره ابتدایی در سال تحصیلی 1403 - 1402 است. در این کتابها، متن، پرسش و تصویرها تحلیل شده است. در این مطالعه 9 مولفه محیط زیستی که شامل، آب، هوا، آب و هوا، خاک، صوت، گیاهان، جانوران، زباله و آلودگی بررسی شده است. بر اساس یافته های پژوهش کتابهای مورد تحلیل از بین مولفه های محیط زیستی، 779 بار به آب، 145 بار به هوا، 52 بار به آب و هوا، 355 بار به خاک، 54 بار به صوت، 991 بار به گیاهان، 351 بار به جانوران، 34 بار به آلودگی و 8 بار به زباله توجه شده است. مولفه گیاهان با فراوانی 991 در رتبه اول و مولفه زباله با فراوانی 8 در رتبهی آخر هستند. نتایج نشان میدهد توجه به تعدادی و نادیده گرفتن مولفه های دیگر موجب اختلال در ایجاد دانش و رفتار محیط زیستی دانش آموزان میشود. همچنین، در این کتابها به تاثیرات مثبت و منفی انسان بر محیط زیست اشاره نشده است. دانشآموزان بهعنوان شهروندان جامعه و آیندهسازان باید از نتیجهی رفتار خود و دیگران با محیط زیست آگاه باشند تا بتوانند در جهت کاهش رفتار نامطلوب و افزایش رفتار مطلوب محیط زیستی گام بردارند.

    کلید واژگان: کتاب, تحلیل محتوا, مولفه های محیط زیست, پنجم ابتدایی
    Pari Mosapour Miyandehi*, Mahboubeh Mansouri

    The current research was conducted with the aim of analyzing the content of fifth grade books based on the level of attention to environmental components. Descriptive research method has been done qualitatively and quantitatively by content analysis method. The statistical society is the books of the fifth grade of the elementary school in the academic year 2023-2024. In these books, text, questions and pictures have been analyzed. In this study, 9 environmental components including water, air, climate, soil, sound, plants, animals, waste and pollution were investigated. According to the research findings, among the environmental components, attention has been paid 779 times to water, 145 times to air, 52 times to climate, 355 times to soil, 54 times to sound, 991 times to plants, 351 times to animals, 34 times to pollution and 8 times to waste. The plant component with a frequency of 991 is in the first rank and the waste component is in the last rank with a frequency of 8. The results show that paying special attention to a number of components and ignoring the others, causes disturbances in creating students' environmental knowledge and behavior. Also, the positive and negative effects of humans on the environment are not mentioned in these books. As citizens of the society and future makers, students should be aware of the result of their behavior and others with the environment so that they can take steps reducing the undesirable behavior and increasing favorable environmental behavior.

    Keywords: Book, Content Analysis, Environmental Components, Fifth Grade
  • Mitra Khalili, Zahra Chavoshzadeh, Sepideh Darougar, Mahboubeh Mansouri, Narges Eslami, Delara Babaie, Mehrnaz Mesdaghi, Abdollah Karimi, Shahnaz Armin, Alireza Fahimzad, Roxana Mansour Ghanaei, Sedigheh Rafiee Tabatabaie, Fatemeh Akrami

    Primary immunodeficiencies are a diverse group of rare genetic disorders, among which phagocytic dysfunction impairs neutrophil function in a wide range of inherited disorders. Due to the heterogeneity of the disorders a multidisciplinary approach is often required for early diagnosis and initiation of appropriate treatments. The aim of this study was to evaluate the imaging findings in children admitted with phagocytic primary immunodeficiencies.Thirty-five children who fulfilled the inclusion criteria for phagocytic dysfunction were enrolled in this study. The patients were under close observation and monitoring from January 2011 until data locking in December 2017. The diagnosis of phagocytic immunodeficiency was confirmed by the patient’s clinical course, presentation features, and laboratory data. Among the 35 patients studied, the most frequent condition was chronic granulomatous disease (CGD) (23 patients), followed by different types of neutropenia (8 patients) and Job’s syndrome (4 patients). Mediastinal and hilar lymphadenopathies and consolidation were the most frequent presentations. There was a significant relationship between mediastinal/hilar lymphadenopathies and fungal infections. A meaningful relationship was also found between pulmonary nodules without halo signs in patients with concomitant tuberculosis and fungal infections. A significant correlation was found between CGD, pulmonary fibrotic changes, and mediastinal lymphadenopathies.The most frequent radiological manifestations in children included mediastinal and hilar consolidations. Physicians’ awareness of the radiological and clinical manifestations of these inherited diseases may be helpful in the early diagnosis and timely initiation of specific prophylaxis measures to prevent infections and also to initiate hematopoietic stem cell transplantation as the curative management modality.

    Keywords: Chronic granulomatous disease, Lymphadenopathy, Phagocytic bactericidal dysfunction
  • Mahsa Rekabi, Parisa Honarpisheh, Mahboobeh Karimi-Galougahi, Vahab Rekabi, Maryam Vasheghani, Narges Eslami, Samin Sharafian, Hooman Sharifi, Alireza Mahdaviani, Mahboubeh Mansouri, Sasan Vasegh, Armin Shirvani, Paniz Pourpashang *
    Background

     Anxiety and depression in patients with asthma have been linked to frequent exacerbations, increased use of healthcare resources, and poor asthma control.

    Objectives

     In the current study, we examined the correlations between asthma and symptoms of depression/anxiety in adolescents with asthma referred to Masih Daneshvari and Mofid hospitals during 2020 - 2021.

    Methods

     The current observational, cross-sectional study was conducted by administering the Spence Children Anxiety Scale and a demographic information checklist among 105 subjects. Asthma severity was measured using forced expiratory volume in 1 second (FEV1), FEV1/forced vital capacity (FVC), the number of short-acting β-2 agonists used per week or month, the number of night awakenings per week or month, having asthma symptoms in daily activities, the number of asthma attacks needing oral corticosteroids per year, and the number of disease exacerbations per week or month. Data were analyzed, and the correlation between the variables was investigated through linear regression and ordinal logistic regression.

    Results

     Patients with a mean age of 11.3 ± 2.5 years showed a mean overall anxiety score of 14 ± 9 out of 114. There was a significant negative correlation between the overall score of anxiety disorders and FEV1 and FEV1/FVC (P < 0.001). There was also a direct correlation between the overall score of anxiety disorders and the frequency of using β-2 agonists, the number of night awakenings, interference with normal functions, and exacerbation frequency (P < 0.001).

    Conclusions

     Our findings indicated a significant association between anxiety disorders and asthma in children. Considering the high prevalence of asthma in Iran than the global average, studying the underlying mechanisms of anxiety and psychological and environmental variables in children with asthma can aid in developing effective psychological therapies.

    Keywords: Asthma, Psychiatric Morbidity, Depression, Anxiety, Adolescent
  • Rahman Pouyanfar, Mahboubeh Mansouri, Mohadese Sadat Mousavi Khorshidi, Paniz Hashemitari, Saeed Nabavi, Seyed Mohammad Norolahi, Sepideh Darougar *
    Background
    Childhood asthma is mainly developed by an interplay between genetic and environmental factors. Atopic asthma has been regarded as the most common form of asthma in the pediatric age group. Therefore, we aimed to evaluate the role of atopy in inducing uncontrolled asthma in children.
    Materials and Methods
    Seventy-five children between 1 to 14 years of age, referred to The Asthma and allergy clinics of Azad University hospitals for a period of one year because of wheezing and or chronic cough with a diagnosis of asthma were enrolled in this cross-sectional study. After scrutinizing the children’s medical history relevant to their asthmatic manifestations, they were evaluated with a skin prick test (SPT) for common aero and food allergens.
    Results
    Thirty-five asthmatic children had positive SPTs with their mean age higher than those with negative skin test results (P≤0.0001). In those with positive SPTs, the symptoms recurred if the medications were discontinued within a month of symptom improvement (P=0.001). The same results were true considering the history of previous atopic disorders in response to the discontinuation of therapy (P<0.0001).
    Conclusion
    To conclude, in most patients with negative skin tests, symptoms of asthma improved in less than a month from the initiation of appropriate therapies. However, in those with positive SPTs and a history of atopy, the symptoms recurred if the medications were discontinued within less than a month of symptom improvement (P=0.001 and P<0.0001, respectively).
    Keywords: Asthma, Hypersensitivity, Skin prick test, Atopy
  • Mitra Khalili, Hossein Farzi*, Sepideh Darougar, Fatemeh Hajijoo, Mehrnaz Mesdaghi, Mahboubeh Mansouri, Delara Babaie, Amir Hashemitari, Narges Eslami, Zahra Chavoshzadeh

    Respiratory diseases are considered as significant causes of morbidity and mortality in primary immunodeficiencies. This study aimed to reveal the radiologic patterns of thoracic involvement in these disorders. A total of 58 patients, including 38 cases with combined cellular-humoral and 20 cases with humoral immunodeficiencies, were enrolled in this study. The “combined” group consisted of 12 cases with severe combined immunodeficiency (SCID) and 26 cases with combined immunodeficiency. The “humoral” group included seven patients with Hyper IgM syndrome (HIGMs), seven cases with common variable immunodeficiency (CVID), three patients with X-linked agammaglobulinemia, and three patients with other types of humoral primary immunodeficiencies (PIDs). The mean age of patients at the time of evaluation was 3.3±3.8 and 5.3±3.9 years in combined and humoral groups, respectively. The findings of chest X-rays and CT scans were interpreted and compared. There was a significant difference for alveolar opacification between combined and humoral immunodeficiencies (58% vs. 30%). The bronchopneumonia-like pattern was detected as a significant finding in patients with SCID (42%) and HIGMs (43%). Atrophy of the thymus was detected significantly often in cases of SCID (67%). Two patients with CVID and lipopolysaccharide-responsive and beige-like anchor protein deficiency showed parenchymal changes of granulomatous lymphocytic interstitial lung disease. No significant difference was detected for bronchiectasis, bronchitis/bronchiolitis patterns, pleural effusion, and thoracic lymphadenopathy. lymphadenopathy. Distinct subtypes of primary immunodeficiency may provoke differing and comparable radiological patterns of thoracic involvement; which can clue the clinician and radiologist to the diagnosis of the disease.

    Keywords: Lung, Pediatrics, Primary immunodeficiency diseases
  • Sepideh Darougar, Arezoo Shirzani, Mahboubeh Mansouri, Masoumeh Mohkam

    Food protein-induced enterocolitis syndrome (FPIES), is a non-IgE mediated food allergy presenting in infants younger than 12 months. Diagnostic delay may occur due to overlapping clinical symptoms with several conditions. Here, we present two cases of FPIES, mistakenly diagnosed and treated as Bartter syndrome. This study aims to emphasize the several features of this syndrome that may mimic other diagnoses and sometimes leading to near-death events due to delay in the diagnosis and improper treatment. The first patient was a 30-month-old boy with multiple episodes of profuse vomiting and diarrhea within 1 hour after breastfeeding, beginning from the first month of life progressing to hypokalemia and metabolic alkalosis at the age of 5 months leading to the diagnosis of Bartter syndrome. The second patient had a history of unremitting diarrhea which had been started soon after his first breastfeeding followed by biliary vomiting on the 7th day of life. He was treated in another hospital for neonatal sepsis, however, without an appropriate response to treatment. To conclude, despite the current belief on the rarity of FPIES, it is a more prevalent disease than expected with various non-specific manifestations imitating other conditions which may result in diagnostic delay and sometimes fatalities. To shed light on the importance of the physicians’ awareness of this syndrome, these two cases are presented here as examples of FPIES imitating other disorders.

    Keywords: Bartter syndrome, Failure to thrive, Food hypersensitivity
  • Parnia Poursadri, Mahboubeh Mansouri, Amirnezam Mir, Seyedeh Niloofar Hashemi, Seyed Karen Hashemitari, Sepideh Darougar *

    Background:

     Different risk factors including previous history of food allergy may play an important role in predicting the development and the outcome of asthma in children. This study aims to assess the relationship of previous food allergy in infancy with asthma severity later in childhood.

    Materials and Methods

    Infants and children of up to 14 years old referred to the Allergy Clinics of Azad University Hospitals, Tehran, Iran, between October 2018 and October 2019 for asthma were enrolled in this cross-sectional study. After confirming the diagnosis by a physician expert in this field and determining the level of severity, the patients’ caregivers were asked to fill out an eight-item researcher made questionnaire. The questionnaire focused on family history of allergy, as well as that of the child, specifically a previous history of physician-diagnosed food allergy.

    Results

    170 asthmatic patients were enrolled in the study with a mean age of 7.1±3 years. A meaningful relationship between food allergy and asthma (p < 0.001) was found. There was also a significant relationship according to history of food allergy in children with moderate, and severe asthma comparing to those with mild asthma (p <0.001). But a meaningful relationship was not found when comparing moderate and severe asthma (p=0.6).

    Conclusion 

    To conclude, food allergy in conjunction with a positive family history is suggested as a risk factor for persistent problematic asthma, which places patients at greater risk for morbidities. A history of previous food allergy was significantly associated with moderate to severe asthma compared to mild asthma.

    Keywords: Asthma, Children, food allergy, Severity
  • Shima Baradaran, Zahra Chavoshzadeh, Roxana Ghanaie, Seyed Alireza Mahdaviani, Sepideh Darougar *, Mehrnaz Mesdaghi, Seyed Alireza Fahimzad, Sedigheh Rafiei Tabatabaei, Abdollah Karimi, Shahnaz Armin, Mahboubeh Mansouri, Delara Babaie
    Background

    One of the most serious complications of BCG immunization is disseminated BCG infection, which is suggested to occur in immunized children with underlying primary immunodeficiencies.

    Objectives

    This study aimed to assess the clinical manifestations and underlying primary immunodeficiencies associated with disseminated BCG infection.

    Methods

    The study enrolled 47 patients suspected of disseminated BCG infection referring to Mofid Children Hospital and Masih Daneshvari Hospital for 12 years. The patients’ records were reviewed and patients were classified into three distinctive groups of definitive, probable, or possible disseminated BCG infection.

    Results

    Twenty-five (53.2%) patients were male and twenty-two (46.8%) were female. The mean age at onset of clinical manifestations was 4.83 months. The first presentation of the disease occurred within one year of vaccination in 28 (60%) patients. Clinical manifestations included lymphadenopathies (61%), fever (38%), hepatosplenomegaly (36%), failure to thrive (23%), skin rash (14%), chronic cough (10%), ascites (6%), and clubbing (6%). The confirmed underlying primary immunodeficiency detected in these patients were Mendelian Susceptibility to Mycobacterial Disease (MSMD; 69.56%), Severe Combined Immunodeficiency (SCID; 26%) and Chronic Granulomatous Disease (CGD; 4.3%).

    Conclusions

    Disseminated BCG infection may be a devastating complication and an important preliminary manifestation of underlying primary immunodeficiency. Because of the wide spectrum of mortality and morbidity, as well as the socioeconomic burden on the health system, it is worth to take a careful medical history before BCG immunization particularly in families with a history of consanguineous marriage and death due to unknown etiology.

    Keywords: Disseminated BCG Infection, BCGosis, Primary Immunodeficiency
  • Delara Babaie, Zahra Daneshmandi, Sara Jafarian, Zahra Chavoshzadeh, Shima Rsouli, Mahboubeh Mansouri, Aliakbar Sayyari, Farid Imanzadeh, Naghi Dara, Pejman Rouhani, Katayoun Khatami, Maryam Kazemi-Aghdam, Yalda Nilipour, Maliheh Khoddami, Reza Gholami, Reihane Motaghinezhad, Shima Rasouli, Mehrnaz Mesdaghi *
    Background
    Eosinophilic Gastrointestinal Disorders (EGID) are a heterogeneous group of gastrointestinal disorders, associated with an increase of the eosinophils in the gastrointestinal mucosal tissue. Regulatory T cells (Tregs), as a subset of T cells, have a proven prominent role in immunopathology and protection against allergic diseases. Also, they appear to play a role in EGID pathogenesis. In the present study, serum levels of Tumor Growth Factor (TGF)-β and interleukin (IL)-10 were measured in patients with EGID compared to patients with Gastroesophageal Reflux Disease (GERD) and healthy subjects.
    Materials and Methods
    A total of 34 patients with EGID, 23 with GERD, and 25 healthy controls were included in the study. The diagnoses of EGID and GERD were made based on the patients’ clinical symptoms, endoscopic findings, and biopsy confirmation. A questionnaire of demographic information, allergy history, as well as endoscopic-pathological and skin prick test results were completed and performed. The serum levels of TGF-β and IL-10 were measured using the ELISA method.
    Results
    Family history of allergic disorders in patients with EGID or GERD was significantly high compared to healthy controls (P=0.010, P=0.005, respectively). There was a statistically significant increase in serum levels of TGF-β1 (P=0.025), but no significant difference was observed in serum level of IL-10 among three groups. However, the serum level of IL-10 was significantly high in a subgroup of patients with upper gastrointestinal eosinophilic involvement compared to the healthy controls (P=0.018).
    Conclusion
    Significant increase in the serum level of IL-10 and TGF- β might be due to the Tregs dysfunction in EGID patients. Further studies should determine the role of Tregs in the pathogenesis of EGID.
    Keywords: Eosinophilic gastrointestinal disorders (EGID), Gastroesophageal reflux disease (GERD), Tumor growth factor (TGF)-β, Interleukin (IL)-10, Eosinophilic Esophagitis
  • Zahra Chavoshzadeh *, Azadeh Zeinab Mirzaee, Mehrnaz Mesdaghi, Sepideh Darougar, Mahboubeh Mansouri, Delara Babaie
    Background
    Primary immunodeficiency disorders are a diverse group of rare genetic diseases that are often under-recognized. Therefore, significant morbidities and mortalities ensue due to diagnosis delay. This is an evaluation of clinical presentations and laboratory data of children referred to Mofid University Hospital during a 10-year period. The aim of this study is to provide epidemiologic data for early diagnosis and proper management of these patients.
    Methods
    A total of 80 children referred to Mofid’s Children Hospital suspected to primary immunodeficiencies (PIDs) were evaluated clinically. The demographic, clinical, and laboratory data were collected in detail and were analyzed as a cross-sectional study. Missing data were completed in follow-up visits.
    Results
    The prevalence of antibody deficiencies, phagocytic defects, and combined immunodeficiencies in 80 children with primary immunodeficiency syndromes were respectively 36.25%, 32.5%, and 31.25%. Common variable immunodeficiency (13.75%) and severe combined immunodeficiency (12.5%) had the highest prevalence among these disorders and the lowest prevalence were reported for cyclic neutropenia (5%), ataxia-telangiectasia (5%), and transient hypogammaglobulinemia of infancy (3.75%). The most common mode of inheritance detected was an autosomal recessive pattern. Pneumonia, otitis media, and diarrhea were the most common complications in the course of these disorders, which were seen respectively in 30%, 18.8%, and 15% of the patients.
    Conclusions
    Issues regarding clinical presentation and management of primary immunodeficiencies continue to puzzle the clinical practitioner. Epidemiological data are needed to increase the awareness of the physicians regarding primary immunodeficiency disorders and to support the benefits of early diagnosis and treatment. Diagnosis delay is associated with increased morbidities and even mortalities.
    Keywords: Primary Immunodeficiency, Delayed Diagnosis, Morbidity
  • Shahrzad Fallah, Mehrnaz Mesdaghi, Mahboubeh Mansouri, Delara Babaei, Abdollah Karimi, Seyed Alireza Fahimzad, Shahnaz Armin, Sedigheh Rafiei Tabatabaei, Roxana Azma, Ghamartaj Khanbabaee, Bahram Bashardoost, Mehrdad Amirmoeini, Saeed Sadr
    Severe combined immunodeficiency syndrome (SCID) is a life-threatening condition leading to early infant death as a result of severe infection, due to impaired cellular and humoral immune systems. Various forms of SCID are classified based on the presence or absence of T cells, B cells and natural killer cells. Patients usually present with recurrent infections and failure to thrive. Definitive treatment is hematopoietic stem cell transplantation. To achieve the best outcome, it should be performed prior to the development of severe infection. In This study, we described 10 patients (6 male and 4 female) with SCID who were admitted to Mofid Children Hospital, Tehran, Iran, from 2006 to 2013. We reviewed patients’ clinical manifestation, laboratory data, family history and outcome. The mean age at the time of diagnosis was 131.8 days. One patient had non-consanguineous parents. Seven patients received BCG vaccine before the diagnosis of SCID, three of them showed disseminated BCG infection. One patient presented with invasive pulmonary aspergillosis. Flow cytometric analysis showed T⁻B⁺NK⁻ in three patients, T⁻B⁻NK⁺ in five patients, T⁻B⁻NK⁻ in one patient, and T⁻B⁺NK⁺ in one patient. This study highlights the importance of early diagnosis and patient referral before the occurrence of serious infection.
    Keywords: Neonatal screening, Primary immunodeficiency disorder, Severe combined immunodeficiency
  • Maryam Alamzadeh, Zahra Chavoshzadeh, Mohammadreza Alaei, Mahboubeh Mansouri, Eznollah Azargashb, Maryam Boloursaz, Sepideh Darougar
    Background
    Leptin is a pro-inflammatory cytokine produced by adipose tissue. Considering the association between obesity and asthma, the current study aimed at investigating if leptin was in the pathway of obesity-asthma relationship in children and if it played a distinctive role in children with asthma and obesity versus the ones with obesity but without asthma.
    Methods
    The current case-control study was conducted on 23 children with asthma and obesity and 13 children with obesity but without asthma (body mass index ≥ 95%) aged 6 to 15 years from October 2011 to October 2012 in Mofid Children’s Hospital, Tehran, Iran. Group 1 included 23 children with asthma and obesity with a mean BMI of 24.3 kg/m2, while group 2, included 13 cases with obesity but without asthma and a mean BMI of 26.6 kg/m2. Both groups were evaluated for their serum leptin, triglyceride, cholesterol, and IgE mean levels. The serum leptin levels were measured by the enzyme-linked immunosorbent assay (ELISA) technique. The results were analyzed with SPSS version 19. The Mann-Whitney test was employed to compare the results.
    Results
    The mean serum leptin level in the children with asthma and obesity was 2.19 ng/mL and in the ones with obesity but without asthma was 2.85 ng/mL (P = 0.006). The mean serum triglyceride and cholesterol levels in the group 1 were 175.4 mg/dL and 189 mg/dL, respectively, while in the group 2 were 175.4 mg/dL and 226.2 mg/dL, respectively. A significant difference was observed in serum leptin levels between the children in groups 1 and 2 (P = 0.006), but surprisingly the increased leptin was detected in the group 2 subjects.
    Conclusions
    The current study findings indicated that serum levels of leptin were significantly higher in the cases with obesity but without asthma. Therefore, other cytokines appeared to play a role in the children with asthma and obesity.
    Keywords: Obesity, Asthma, Leptin, Children, Adipose Tissue
  • Arezou Rahimi, Mehrnaz Mesdaghi*, Nima Rezaei, Mahboubeh Mansouri, Iraj Mohammadzadeh, Hamid Farajifard, Mehrdad Amirmoini, Delara Babaie, Reza Alimohammadi, Zahra Chavoshzadeh
    Background And Aims
    Patients with Hyper-IgE syndrome suffer from fungal and bacterial infections, especially Candida albicans and Staphylococcus aureus. Due to the important role of T helper17 (Th17) lymphocytes in defense against fungal infections, the percentage of Th17 lymphocytes was studied in the patients with autosomal recessive hyper-IgE syndrome (AR-HIES).
    Materials And Methods
    In this case-control study, six patients with AR-HIES (with DOCK-8 mutation) and seven healthy age and sex-matched controls were included. Peripheral blood mononuclear cells were isolated from their venous blood and the percentage of Th17 lymphocytes were determined by flow cytometry.
    Results
    There was no statistical difference between the percentage of Th17 lymphocytes (p=0.15) in the case and control groups. Also in comparison to the control subjects, the numbers of eosinophils were dramatically increased (p=0.000). Also, there was a significant negative correlation between serum IgE levels and Th17 lymphocytes’ percentage (r=-0.927, p=0.006) and a significant positive correlation between eosinophils number and Th17 lymphocytes’ percentage (r=0.557, p=0.01). Serum IgE levels showed a significant positive correlation with the numbers of eosinophils in the patients’ peripheral blood with AR-HIES (r=0.961, p=0.003).
    Conclusions
    The numbers of Th17 in the patients with AR-HIES may not show statistical differences between the cases and controls. The numbers of eosinophils significantly increased in the patients AR-HIES compare to the controls.
    Keywords: AR-HIES, Hyper IgE Syndrome, Th17
  • Seyed Hasan Tonekaboni, Narjes Jafari, Mahboubeh Mansouri, Sayena Jabbehdari, Rahil Eftekhari, Zahra Chavoshzadeh, Fatemeh Abdollah Gorji, Mehrnaz Mesdaghi*
    Objective
    Anticonvulsant drugs can cause various forms of skin drug reactions, ranging from exanthema to severe blistering reactions. An association between HLA-B*1502 allele and severe skin reactions have been reported.
    Materials and Methods
    Fifteen patients with severe skin reactions following treatment with anticonvulsant drugs (Carbamazepine, lamotrigine, phenobarbital, primidone) and 15 controls (age-matched epileptic patients taking similar anticonvulsants without drug eruption) were included. They were referred to Mofid Children’s Hospital in Tehran, Iran, between Jan 2012 to Jan 2014. Genomic DNA was extracted from peripheral blood of all patients and HLA- B*1502 genotype was detected by real-time PCR.
    Results
    None of the patients was positive for HLA- B*1502, but two patients in control group had positive HLA- B*1502.
    Conclusion
    The HLA- B*1502 is not correlated with severe anticonvulsant drugs -induced skin reactions in Iranian children.
    Keywords: Drug eruption, HLA, B*1502, Anticonvulsants
  • Hoshang Gorjipour, Golnaz Eslamyan, Mehrnaz Mesdaghi*, Mahboubeh Mansouri, Abdollah Karimi, Payman Eshghi, Delara Babaie, Mehrdad Amirmoeni, Bahram Bashardust, Marjan Shakiba, Zahra Chavoshzadeh
    Nijmegen breakage syndrome is a rare autosomal recessive congenital disorder causing chromosomal instability, characterized by short stature, microcephaly, distinctive facial features, recurrent respiratory tract infections, an increased risk of cancer, intellectual disability, and other health problems. People with Nijmegen breakage syndrome have immunodeficiency. Some patients with ataxia telangiectasia-like syndromes (about 10%) have decreased serum IgA and IgG levels with normal or raised IgM level, a phenotype reminiscent of hyper IgM syndrome, which is due to class switch recombination defect. The case presented in this report was an eight-year-old female with related parents, who had been admitted to the hospital several times with recurrent infections (pneumonia and sinusitis). In immunological workups, she had high IgM, low IgG, and IgA levels. According to high α-fetoprotein level and her microcephaly, Nijmegen breakage syndrome was suggested. She was receiving IVIg monthly for two years, when she developed hypersplenism and pancytopenia. Her bone marrow aspiration and biopsy was reported normal twice. The patient underwent Rituximab therapy (375mg/m2) weekly for four weeks, with good response and improvement of splenomegaly and pancytopenia. Class switch recombination defects should be considered in patients with ataxia telangiectasia variants, especially when they have hyper IgM phenotype, and if they present lymphoproliferation, Rituximab therapy could be an effective treatment.
    Keywords: Nijmegen Breakage Syndrome, Rituximab
  • بررسی درصد لنفوسیت های Th1 در خون محیطی بیماران مبتلا به سندرم هایپر IgE اتوزومال مغلوب
    آرزو رحیمی، زهرا چاوش زاده، نیما رضایی، محبوبه منصوری، دلارا بابایی، محمد نبوی، حمید فرجی فرد، امیر عطشی، مهرداد امیرمعینی، راضیه رضایی، رضا علی محمدی، مهرناز مصداقی
    مقدمه
    سندرم هایپر IgE نقص ایمنی اولیه است که با افزایش استعداد ابتلا به طیف محدودی از عفونت های قارچی و باکتریایی به خصوص کاندیدا آلبیکنس و استافیلوکوک اورئوس همراه است. بررسی تغییرات زیر گروه های لنفوسیت ها در بیماران در یافتن پاتوژنز بیماری می تواند کمک کننده باشد. در این مطالعه درصد لنفوسیت های Th1 در بیماران مبتلا به سندرم هایپر IgE اتوزومال مغلوب مورد بررسی قرار گرفت.
    مواد و روش ها
    در این مطالعه مورد-شاهدی شش بیمار مبتلا به سندرم هایپر IgE اتوزومال مغلوب و هفت فرد سالم به عنوان گروه کنترل که با گروه بیماران از لحاظ سن و جنس همسان سازی شده بودند، مورد مطالعه قرار گرفتند. پس از خونگیری وریدی، سلول های تک هسته ای خون محیطی جدا گردیدند و پس از تحریک سلولی و کشت 12 ساعت، درصد سلول های Th1 به روش فلوسایتومتری مورد سنجش قرار گرفتند.
    نتایج
    نتایج حاصل از این مطالعه نشان داد که درصد سلول های Th1 در بیماران در مقایسه با گروه شاهد به طور معنی داری کاهش یافته است (008/0P<).
    نتیجه گیری
    با توجه به نتایج این مطالعه به نظر می رسد که تغییر درصد زیر گروه های لنفوسیت Th1 ممکن است نقش مهمی در پاتوژنز این بیماری داشته باشد و کاهش درصد این لنفوسیت ها می تواند در افزایش استعداد ابتلا به عفونت های قارچی و باکتریایی سهیم باشد.
    کلید واژگان: سندرم هایپر IgE, IFپN-γ, Th1, اتوزومال مغلوب
    Investigating the Percentage of Th1 lymphocytes in Peripheral Blood of Patients with Autosomal Recessive Hyper-IgE Syndrome
    Arezou Rahimi, Zahra Chavoshzad, Nima Rezaei, Mahboubeh Mansouri, Delara Babaie, Mohammad Nabavi, Hamid Farajifard, Amir Atashi, Mehrdad Amirmoini, Razeye Rezaei, Reza Alimohammadi, Mehrnaz Mesdaghi
    Introduction
    Hyper-IgE syndrome is a primary immunodeficiency disease, characterized by increased susceptibility to a limited range of fungal and bacterial infections, especially Candida Albicans and Staphylococcus Aureus. The study of different subtypes of lymphocytes would be helpful in understanding of the disease pathogenesis. In this study, the percentage of Th1 lymphocytes in peripheral blood of patients with autosomal recessive hyper-IgE syndrome was investigated.
    Methods
    In this case-control study, six patients with autosomal recessive hyper IgE syndrome and seven healthy controls, which were age and sex matched, were studied. Peripheral blood mononuclear cells were isolated from venous blood. After cell stimulation and culture for 12 hours, the percentage of Th1 cells was evaluated by flow cytometry.
    Results
    The results of this study showed that the percentage of Th1 cells was significantly decreased in patients compared to the control group (P
    Conclusion
    The reduction in Th1 lymphocytes may play an important role in the pathogenesis of autosomal recessive hyper-IgE syndrome and their increased susceptibility to bacterial and fungal infections.
    Keywords: Autosomal recessive, hyper-IgE Syndrome, IFN-γ, Th1
  • زهرا کرمی، مهرناز مصداقی *، زهرا چاووش زاده، پروانه کریم زاده، محبوبه منصوری، رضا شکرریز
    سابقه و هدف
    مصرف فنی توئین می تواند همراه با واکنش های دارویی مختلفی از جمله ازدیاد حساسیت دارویی باشد.تست تحریک لنفوسیتی ممکن است روشی مفید جهت تشخیص داروی ایجاد کننده ی واکنش افزایش حساسیت باشد. لذا به منظور بررسی نتایج تست تحریک لنفوسیتی در مبتلایان به واکنش های ازدیاد حساسیت به دنبال مصرف فنی توئین و گروه شاهد این مطالعه انجام گردید.
    مواد و روش ها
    این مطالعه به روش مورد- شاهدی انجام گرفت. گروه مورد شامل 4 بیمار مبتلا به واکنش های ازدیاد حساسیت به دنبال مصرف فنی توئین و گروه شاهد، شامل 10 نفر (افرادی که فنی توئین مصرف کرده ولی عوارض حساسیت دارویی نداشتند) بودند. سلولهای تک هسته ای خون محیطی بیماران به وسیله ی گرادیان غلظتی توسط ماده فایکول جدا شد و با فنی توئین، Phytohaemagglutininبه عنوان میتوژن و کاندیدا به عنوان آنتی ژن تحریک شدند. میزان تکثیر سلولی با کیت5-bromo-2''- deoxyuridineارزیابی شد و نتایج به صورت اندکس تحریک پذیری بیان گردید. اندکس تحریک پذیری بیشتر از 2 مثبت در نظر گرفته شد و دو گروه با آزمون دقیق فیشر مورد مقایسه قرار گرفتند.
    نتایج
    از 4 نفر بیمار مصرف کننده ی فنی توئین در گروه آزمون 3 نفر نتیجه تست مثبت و 1 نفر نتیجه منفی داشت و در گروه کنترل نتایج همه بیماران منفی بودP<0.01)) میانگین فاصله ی بین زمان واکنش دارویی و انجام تست تحریک لنفوسیتی در گروه آزمون در کسانی که نتیجه تست LTT مثبت داشتند9/6± 16 ماه و درکسانی که نتیجه تست منفی داشتند 38 ماه بود که این اختلاف به لحاظ آماری معنی دار نبود(174/0p=).
    نتیجه گیری
    به نظر می رسد نتایج تست تحریک لنفوسیتی می تواند در تشخیص عامل ایجاد کننده واکنشهای افزایش حساسیت دارویی کمک کننده باشد.
    کلید واژگان: تست تحریکی لنفوسیتی, داروهای ضد تشنج, ازدیاد حساسیت دارویی, فنی توئین
    Zahra Karami, Mehrnaz Mesdaghi *, Zahra Chavoshzadeh, Parvaneh Karimzadeh, Mahboubeh Mansouri, Reza Shekarriz
    Background
    Administration of phenytoin can been associated with severe adverse reactions such as hypersensitivity reactions. Lymphocyte transformation test (LTT) can be a useful method for determination of the drug, which has caused hypersensitivity reaction. This study was done to evaluate the results of lymphocyte transformation test in patients with delayed hypersensitivity reactions following phenytoin administration and control group.
    Methods
    In a case-control study, four patients with hypersensitivity reactions following administration of phenytoin and ten patients, who had used phenytoin without hypersensitivity reactions, were included. Peripheral blood mononuclear cells were isolated. The cells were stimulated with Phenytoin, PHA ( Phytohaemagglutinin ) as a mitogen and candida as an antigen. Lymphocyte proliferation was measured using Brdu proliferation assay kit (Roche, Germany). The stimulation index was calculated by dividing OD of stimulated to unstimulated cells. Stimulation index more than 2 were considered as positive. The results in case and control groups were compared, using Fisher's exact test.
    Results
    Of 4 patients in the test group,3 had positive LTT results and one had negative test result. Among patients in control group, none of them had positive LTT result. This difference was statistically significant (p=0.002). The mean time between development of drug reactions and perform the LTT was 16±6.9 months in the test group with positive result and 38 months in test group with negative test result. This difference was not statistically significant (p=0.174).
    Discussion
    .LTT can be a helpful method in diagnosis of the drug that has caused hypersensitivity reaction.
    Keywords: lymphocyte transformation test, hypersensitivity reaction, anticonvulsant drugs, phenytoin
  • Mahboubeh Mansouri
    Drug Rash with Eosinophilia and Systemic Symptoms (DRESS) is a potentially life-threatening, complex, and multifaceted disease which may imitate other grave conditions. It presents with cutaneous drug eruptions, fever, hematologic abnormalities (an eosinophil count of 1500/mm3 or atypical lymphocytosis), and systemic involvement including hematologic, renal, pulmonary, hepatic, cardiac, gastrointestinal, neurologic, and endocrine abnormalities. Anticonvulsant therapies (mainly carbamazepine) are among the most important causative drugs.Case report: Herein we present a10-year-old girl who developed skin rash, systemic symptoms, marked eosinophilia, and kidney involvement following anticonvulsive treatment with phenobarbital and sodium valproate. She experienced multiple hospitalizations due to an improper diagnosis and management.
    Conclusion
    Drug Induced Hypersensitivity Syndrome (DIHS) is a severe life-threatening disorder which mostly occurs due to aromatic anticonvulsive drugs. The disease may mimic other serious conditions and delay in the diagnosis and improper treatment may cause organ involvement and more severe outcomes.
    Keywords: Drug Hypersensitivity Syndrome, DRESS Syndrome, Drug Reaction with Eosinophilia, Systemic Symptoms, Drug Eruptions, Interstitial Nephritis
  • Atena Ramezanali Yakhchali, Zahra Chavoshzadeh, Mehrnaz Mesdaghi, Mahboubeh Mansouri, Reza Shiari *
    Introduction
    X-linked Agammaglobulinemia (XLA) is one of the primary humoral immunodeficiencies. It usually presents symptoms of recurrent infections, but in some unusual cases it may present rheumatologic manifestations..
    Case Presentation
    The current paper presents the cases of two boys with arthritis treated for juvenile rheumatoid arthritis (JRA) without proper responses. Addition of some recurrent infections in the course of their disease led to work-up them for immunodeficiencies..
    Conclusions
    According to the results of these work-ups, XLA was diagnosed for the cases..
    Keywords: Agammaglobulinemia, Arthritis, Agammaglobulinemia
  • Mahboubeh Mansouri *
    Context: Food allergy is a growing health problem. Avoidance of the food allergen is the only accepted treatment. Because the major food allergens are among the most commonly used foods including cow’s milk, egg, nuts, wheat, soya, fish, and seafood, avoiding them is difficult and might negatively affect the patients’ and their families’ health..Evidence Acquisition: This brief review concerns the prevalence, importance, definition, types, clinical symptoms, diagnosis, and management of food allergy. The information were retrieved searching a wide range of published data, especially in PubMed, from January 2000 through July 2014..
    Results
    Food allergies are mainly classified into IgE mediated and non-IgE mediated; the latter is classified into cell-mediated, and mixed IgE-non-IgE-mediated food allergy. Medical history can provide detailed information essential to make the diagnosis. The current approach to the management of food allergy substantially relies on allergen avoidance and prescriptions to treat allergic reactions..
    Conclusions
    The characteristic features of IgE-mediated food allergy are abrupt onset of clinical symptoms, which may result in a life-threatening events, and positive results of the majority of the paraclinical tests that mainly trace the specific IgE to foods. Moreover, non–IgE-mediated food allergies present as chronic diseases and due to lack of proper diagnostic tests the similarity of the clinical presentation with other chronic clinical conditions, the exact prevalence may remain underestimated..
    Keywords: Food Hypersensitivity, Hypersensitivity, Hypersensitivity, Food
  • Mahboubeh Mansouri, Azadeh Rakhshan, Mohammad Shahidi-Dadras, Abdollah Karimi, Samin Alavi
    Pyoderma vegetans (PV) is a rare inflammatory disorder characterized by vegetating pustules and plaques affecting the skin and mucosal membranes. It is believed that this entity is mostly associated with inflammatory bowel disease (IBD), chronic malnutrition, human immunodeficiency virus (HIV), malignancies, and other immunocompromised states. Pyoderma vegetans occurs more commonly in young and middle-aged adults. There is no sex predilection for this entity. The lesions could heal spontaneously, but usually recur and become chronic.Our patient was an 11-year-old girl suspected to have primary combined immunodeficiency complicated by chronic recurrent vegetating pustular lesions on the face and postauricular area since one year of age. The histological features of the lesions were consistent with pyoderma vegetans. This is the first case of PV beginning from early infancy in the setting of primary immunodeficiency and in an unusual location.
  • Mahboubeh Mansouri
    Allergic diseases have increased in prevalence during recent years. Although they impose numerous health and financial burden on patients and their family and also the society, they are to some extent preventable and manageable. While reviewing the literature to find any relationship between allergic disease as the familiar and preventable conditions and the two unfamiliar entities, named Eosinophilic Cystitis (EC) and Interstitial cystitis(IC); a significant number of reports were found about the etiological roll of atopy and allergy in the development of these conditions. Nevertheless this relationship is stronger concerning IC than EC. Referring to allergy as a contributing factor makes it more understandable and controllable. Although in order to reach a proper conclusion more thorough studies are required.
    Keywords: Interstitial Cystitis, Hypersensitivity, Atopic Hypersensitivity, Eosinophilia, Human, Urinalysis.
  • Mahboubeh Mansouri, Mehrnaz Mesdaghi, Zahra Chavoshzadeh, Marzieh Heidarzadeh, Fatemeh Abdollah Gorji
    Background
    Adverse drug reactions (ADRs) occur in 10-20% of hospitalized patients and approximately 7% of general population. Drug-induced allergic reactions can affect numerous organ systems and manifest in a variety of reactions..
    Objectives
    This study was designed to describe the frequency of different types of allergic drug reactions and uncover culprit drugs..Patients and
    Methods
    All patients who had been admitted to Mofid Children’s Hospital, Tehran, Iran due to drug reactions during April 2009 to April 2010 were included in this study. Patients who fulfilled the criteria for an allergic drug reaction according to Gell and Coombs classification, were enrolled in the study and patients with ADRs whose symptoms were not compatible with allergic reactions, were excluded from the study. An immunologist and allergist diagnosed drug allergy. A complete questionnaire was filled out for each patient..
    Results
    A total number of 117 patients were evaluated for adverse drug reactions, among them, 97 (82.9%) were considered to have immunological drug reactions. The most common symptoms of allergic drug reactions were morbilliform eruptions, serum sickness, DRESS (drug rash with eosinophilia and systemic symptoms), and toxic epidermal necrolysis. In 62 patients, anticonvulsant drugs had the prominent role and the most important anticonvulsants were phenobarbital, lamotrigin, and valproic acid. In 52 patients, antibiotics were found culprit and the most common antibiotics were cefixime, co-trimoxazole, and furazolidone..
    Conclusions
    We found that delayed types of allergic drug reactions, as well as morbilliform skin eruptions, were the most frequent presentations among our patients. Anticonvulsants were identified as the first cause in the majority of drug reactions. These medications should only be prescribed when necessary and the patients should be informed about adverse reactions. This study provides a background for more extensive studies in this regard..
    Keywords: Drug Hypersensitivities, Allergy, Immunology, Drug
  • Shahnaz Armin, Koosha Ramezani, Zahra Chavoshzadeh, Mahboubeh Mansouri
    Introduction
    Drug Rash with Eosinophilia and Systemic Symptoms (DRESS) syndrome is a severe form of adverse drug reaction which describes a potentially life threatening condition, associated with a high mortality rate. This syndrome is rarely seen in childhood even though a large number of children receive anticonvulsant treatment..
    Case Presentation
    We report here two infants under two months of age, whose findings were consistent with DRESS syndrome due to phenobarbital usage. Considering their age, these two cases appeared to be unique to our knowledge. Good responses were observed in both cases after stopping the culprit drug and administration of systemic corticosteroid..
    Discussion
    Early recognition of DRESS syndrome is of a particular importance. Although rare, in newborn infants with the presence of skin rash, liver involvement, hyper-eosinophilia and lymphadenopathy, DRESS diagnosis should be highly suspected and prompt intervention including withdrawal of causative drug is required to prevent potentially fatal outcomes..
    Keywords: Drug Hypersensitivity, Phenobarbital, Eosinophilia
  • Mahboubeh Mansouri, Zahra Pourpak, Habibeh Mozafari, Fatemeh Abdollah Gorji, Raheleh Shokouhi Shoormasti
    Allergy to wheat is a common food allergy. In spite of this fact, there is not enough literature regarding the features and outgrowing of this allergy. The objective of this study was to evaluate the manifestations of this allergy and to follow the patients to evaluate whether outgrowing allergy happens again and when it occurs.Eight wheat allergic patients diagnosed between 2000 and 2001 were re-evaluated together with 13 other new cases of wheat allergy referred to the Immunology and Allergy Pediatric Department from June 2004 to March 2006. For all cases, the demographic data along with a complete history regarding allergy to wheat and other types of allergy were collected in questionnaires. The specific IgE measurements (in vivo and in vitro) and oral food challenge (in the absence of a relevant history related to allergy to wheat) were performed. Severe anaphylaxis was seen after wheat ingestion in more than 90% of the patients. Oral tolerance to wheat developed in three patients (37.5%) out of 8 known previous cases who had been followed for eight years, the mean age of oral tolerance to wheat was 68±6.36 (range; 36 months to 108 months).Clinical reactions in our wheat-allergic patients were more severe than those reported before. These patients were at risk for developing chronic allergic symptoms such as asthma. We also found that oral tolerance to wheat was happening in a minority of our patients.
    Keywords: Anaphylaxis, IgE mediated hypersensitivity
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