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  • 1
    Online Resource
    Online Resource
    Descriptive Study of 123 Symptomatic Patients with IgA Deficiency, a Retrospective Case Series Study
    Knowledge E DMCC ; 2022
    In:  Immunology and Genetics Journal ( 2022-07-06)
    Details
    In: Immunology and Genetics Journal, Knowledge E DMCC, ( 2022-07-06)
    Abstract: Background: IgA deficiency (IgAD) is the most common primary immunodeficiency, which is caused by a defect in IgA antibody production. Most of the patients are asymptomatic. However, patients can present various manifestations. This study was designed to assess the clinical and laboratory manifestations of symptomatic patients with IgA deficiency. Method: A group of 123 patients with IgA deficiency referred from all over the country to the national immunodeficiency registration center were entered and followed in this study. The data including demographic characteristics, clinical manifestations and laboratory findings recorded at the registry and also the follow-up visits were extracted. Results: The mean age of studied patients was 17.1 years old. Regarding gender, 45 patients (36.5%) were female. The most common clinical presentations included upper respiratory tract infections in 22 (17.9%), enteropathy in 9 (7.9%), allergic rhinitis in 11 (8.9%), sepsis in 4 (3.3%) patients. Four cases of leukopenia with white blood cell (WBC) 〈 4,000/μl and 21 cases of leukocytosis with WBC 〉 10,000/μl were observed based on the laboratory results. Moreover, IgG2 and IgG4 in 2 and 11 patients were less than normal rate for their age, respectively. Conclusion: Although IgA deficient patients are almost always asymptomatic, clinical manifestations such as recurrent sinopulmonary infections, multiple autoimmune diseases, allergic respiratory and skin disorders, gastrointestinal diseases, and rarely severe life-threatening infections could occur.  
    Type of Medium: Online Resource
    ISSN: 2645-4831
    URL: Article
    DOI: 10.18502/igj.v4i2.9987
    Language: Unknown
    Publisher: Knowledge E DMCC
    Publication Date: 2022
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  • 2
    Online Resource
    Online Resource
    Atopy Patch Test in the Diagnosis of Food Allergens in Infants with Allergic Proctocolitis Compared with Elimination/Introduction Challenge
    Knowledge E DMCC ; 2021
    In:  Iranian Journal of Allergy, Asthma and Immunology ( 2021-10-12)
    Details
    In: Iranian Journal of Allergy, Asthma and Immunology, Knowledge E DMCC, ( 2021-10-12)
    Abstract: Allergic proctocolitis is a cell-dependent food allergy that is present in both breast and formula-fed infants. The presence of blood with different amounts in the stool is the main manifestation of the disease. Different results have been published on the accuracy and specificity of the atopic patch test (APT). The purpose of this study was to evaluate the results of the APT and compare them with those obtained in the food elimination/introduction (E/I) challenge, as the gold standard of confirming the allergy. Twenty-eight patients (18 boys, 10 girls, 〈 1 year) with allergic proctocolitis were recruited in this study. The mean age of the disease onset and enrolling the study were 2.23±1.7 and 5.25±2.19 months, respectively. After performing APT with fresh foods, an E/I challenge was done in a patient with positive tests, and results were analyzed. APT was positive in 14/28 (50%) individuals. The most common foods detected by APT in all of the individuals were: milk (10/28), rice (5/28), soy (4/28), and egg white (4/28), while in E/I challenge in the APT-positive individuals were: milk (8/10), rice (3/5), egg white (1/4), and soy (0/4). APT was positive in half of the infants 〈 1 year with allergic proctocolitis and there was no significant correlation between the APT results and the E/I challenge test for all foods. Comparing the results of APT and E/I challenge methods showed a convergence between the milk and rice sensitivity, thus we suppose APT to be a useful tool in identifying these two allergens in cell-mediated food allergies like allergic proctocolitis.
    Type of Medium: Online Resource
    ISSN: 1735-5249 , 1735-1502
    URL: Article
    DOI: 10.18502/ijaai.v20i5.7402
    Language: Unknown
    Publisher: Knowledge E DMCC
    Publication Date: 2021
    detail.hit.zdb_id: 2388260-8
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  • 3
    Online Resource
    Online Resource
    Comparison of Diagnostic Tests with Oral Food Challenge in a Clinical Trial for Adult Patients with Sesame Anaphylaxis
    Knowledge E DMCC ; 2020
    In:  Iranian Journal of Allergy, Asthma and Immunology ( 2020-02-22)
    Details
    In: Iranian Journal of Allergy, Asthma and Immunology, Knowledge E DMCC, ( 2020-02-22)
    Abstract: Sesame food allergy (SFA); especially anaphylaxis, is a life-threatening condition. The accurate diagnosis of SFA is done by skin prick test (SPT), skin prick to prick (SPP) or specific IgE (sIgE) and is confirmed by oral food challenge (OFC). Since there are few studies evaluating and comparing the utility of these methods for diagnosis of sesame anaphylaxis in adult patients, we aimed to compare OFC with diagnostic tests, including SPT, SPP, and sesames IgE; using ImmunoCAP considering the sensitivity and specificity issues in patients with sesame anaphylaxis. Twenty patients with sesame anaphylaxis were diagnosed based on OFC. Then SPT, SPP, and sIgE were evaluated. Sixteen patients had positive OFC; while 4 patients had negative results. Out of 16 OFC+ patients, 7 patients were SPT+, 15 patients were SPP+, and 2 patients had detectable sIgE. A positive SPT indicated 44% sensitivity and 50% specificity. A positive SPP showed 87.5% sensitivity and 75% specificity. A positive ImmunoCAP test demonstrated 12.5% sensitivity and 75% specificity. The AUC of SPP was significant for the diagnosis of sesame anaphylaxis (p=0.038). In conclusion, when the OFC is not possible, the SPP test with natural sesame seed may be applicable in patients with a convincing history instead of the artificial or commercial extracts of sesame used for SPT. Positive SPP is a good alternative diagnostic method for patients with sesame anaphylaxis. Also, the poor sensitivity of SPT and sIgE may indicate the poor discriminative capability of these tests.
    Type of Medium: Online Resource
    ISSN: 1735-5249 , 1735-1502
    URL: Article
    DOI: 10.18502/ijaai.v19i1.2415
    Language: Unknown
    Publisher: Knowledge E DMCC
    Publication Date: 2020
    detail.hit.zdb_id: 2388260-8
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  • 4
    Online Resource
    Online Resource
    Immunodeficiency, Centromeric Region Instability, and Facial Anomalies Syndrome (ICF) in a Boy with Variable Clinical and Immunological Presentations
    Knowledge E DMCC ; 2021
    In:  Iranian Journal of Allergy, Asthma and Immunology ( 2021-04-19)
    Details
    In: Iranian Journal of Allergy, Asthma and Immunology, Knowledge E DMCC, ( 2021-04-19)
    Abstract: Immunodeficiency, centromeric instability, and facial anomalies (ICF) syndrome is a rare primary immunodeficiency disorder characterized by recurrent infections and low immunoglobulin levels due to variable combined immunodeficiency, and centromeric region instability, and facial dysmorphism. We describe a 12-year-old boy with recurrent respiratory tract infections, facial anomalies, scoliosis, and psychomotor retardation. He had recurrent pneumonia with low serum IgG and IgM levels during infancy and preschool age. Later at the age of 10, he developed recurrent ear infections. An IgA and IgM deficiency was found accompanied by a normal B-cell and T-cell count as well as an impaired candida-induced T-cell proliferation. Further evaluations revealed a missense mutation in the DNMT3B gene on chromosome 20. Chromosomal analysis showed a sunburst multi-radial feature on chromosome 1, which is a hallmark of ICF syndrome. The genetic mutation and chromosomal abnormality along with clinical findings are compatible with the diagnosis of ICF syndrome. To the best of our knowledge, this is the first time that scoliosis is observed in an ICF patient. The additional variable clinical symptoms in the case were the presence of spastic gait as well as hypogammaglobulinemia with immunoglobulin isotype switch at different ages.
    Type of Medium: Online Resource
    ISSN: 1735-5249 , 1735-1502
    URL: Article
    DOI: 10.18502/ijaai.v20i2.6058
    Language: Unknown
    Publisher: Knowledge E DMCC
    Publication Date: 2021
    detail.hit.zdb_id: 2388260-8
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  • 5
    Online Resource
    Online Resource
    Aspirin Sensitivity in Patients with Moderate to Severe Asthma
    Knowledge E DMCC ; 2020
    In:  Iranian Journal of Allergy, Asthma and Immunology ( 2020-09-02)
    Details
    In: Iranian Journal of Allergy, Asthma and Immunology, Knowledge E DMCC, ( 2020-09-02)
    Abstract: Asthma induced by ingestion of aspirin occurs when symptoms arise within 30 minutes to three hours after aspirin consumption. Previous data indicate that sensitivity to aspirin may be associated with poorly controlled asthma. This study aims to evaluate the frequency of aspirin sensitivity in patients with moderate to severe asthma receiving conventional asthma therapy. This clinical trial was conducted on 65 patients aged 18 to 65 years with moderate to severe asthma from February 2015 to February 2016 at the Allergy Department, Hazrat-e-Rasoul Hospital, Iran University of Medical Sciences, Tehran. To assess treatment responses in patients, forced expiratory volume in the first second (FEV1) and asthma control test (ACT) scores were measured at baseline and after 3 months. The results of the oral aspirin challenge revealed a prevalence of 35.38% for sensitivity to aspirin. Hypersensitivity reactions to aspirin were detected in 60.9% of the patients with moderate asthma and 39.1% of the patients with severe asthma. All patients with positive aspirin challenge tests suffered from rhinosinusitis and in 56.5% of cases, history of previous hypersensitivity reactions to nonsteroidal anti-inflammatory drugs (NSAIDs) was detected. No meaningful differences were found between those patients with aspirin sensitivity and those with aspirin tolerance neither in mean prebronchodilator FEV1 nor in ACT scores pre- and post-treatment. To conclude, aspirin sensitivity was not found to have an association with an unfavorable response to conventional treatment in patients with uncontrolled asthma.
    Type of Medium: Online Resource
    ISSN: 1735-5249 , 1735-1502
    URL: Article
    DOI: 10.18502/ijaai.v19i4.4120
    Language: Unknown
    Publisher: Knowledge E DMCC
    Publication Date: 2020
    detail.hit.zdb_id: 2388260-8
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  • 6
    Online Resource
    Online Resource
    A Case of Linear IgA Bullous Dermatosis Induced by Aspirin Therapy
    Knowledge E DMCC ; 2020
    In:  Iranian Journal of Allergy, Asthma and Immunology ( 2020-10-27)
    Details
    In: Iranian Journal of Allergy, Asthma and Immunology, Knowledge E DMCC, ( 2020-10-27)
    Abstract: Linear IgA bullous dermatosis (LABD) is a rare autoimmune blistering disease that may be triggered by some diseases and medications. For the latter one, non-steroidal anti-inflammatory drugs (NSAIDs) have been identified as one of the potential causative agents to develop LABD. Here, a rare case of drug-induced LABD is introduced. A 13-month-old Iranian boy presented with a history of generalized blisters, displaying the classic “string of pearls” sign who was eventually diagnosed as a case of LABD. In his admission, he was diagnosed whit Mucocutaneous lymph node syndrome and treated with aspirin.  Some features like appearing the characteristic lesions one week following the administration of aspirin, rapid clearance of lesions after the withdrawal of the drug, and reappearance of new lesions after readministration of aspirin were highly suggestive of aspirin-induced LABD. To establish the diagnosis, we used the “Naranjo probability score” which determined the probable causative role of aspirin. The diagnosis was confirmed by showing the positive IgA deposition in the basement membrane zone in a direct immunofluorescence study of the skin biopsy. The child was treated with dapsone with dramatical response to the drug.
    Type of Medium: Online Resource
    ISSN: 1735-5249 , 1735-1502
    URL: Article
    DOI: 10.18502/ijaai.v19i5.4473
    Language: Unknown
    Publisher: Knowledge E DMCC
    Publication Date: 2020
    detail.hit.zdb_id: 2388260-8
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  • 7
    Online Resource
    Online Resource
    Eosinophilic Fasciitis/Generalized Morphea Overlap”: A Rare Manifestation in a Patient with X-Linked Agammaglobulinemia
    Knowledge E DMCC ; 2022
    In:  Immunology and Genetics Journal ( 2022-07-06)
    Details
    In: Immunology and Genetics Journal, Knowledge E DMCC, ( 2022-07-06)
    Abstract: X-Linked Agammaglobulinemia (XLA) is a prototype of humoral immunodeficiency disorders manifested by recurrent sinopulmonary infections and characterized with low to absence of immunoglobulin production due to absence of B lymphocytes. There are many reports of unusual complications of this genetic disease such as Pneumocystis carinii pneumonia, enteroviral infections with diverse manifestations, neutropenia during severe infections and also uncommon reports of some autoimmunities. Moreover, Rheumatological diseases are reported as a manifestation of XLA among which dermatomyositis is a known and expected condition. Other connective tissue diseases are rarely reported. In this report, the researchers described a known case of XLA disease with progressive body pain, muscle ache, tender and tense skinand finally confirmed as a rare occurrence of Eosinophilic Fasciitis / Morphea Overlap.
    Type of Medium: Online Resource
    ISSN: 2645-4831
    URL: Article
    DOI: 10.18502/igj.v4i2.9988
    Language: Unknown
    Publisher: Knowledge E DMCC
    Publication Date: 2022
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  • 8
    Online Resource
    Online Resource
    The Novel ZBTB24 Mutation Identified in an Iranian Patient with Type 2 ICF Syndrome
    Knowledge E DMCC ; 2023
    In:  Immunology and Genetics Journal ( 2023-03-07)
    Details
    In: Immunology and Genetics Journal, Knowledge E DMCC, ( 2023-03-07)
    Abstract: Autosomal-recessive immunodeficiency, centromeric instability, and facial anomalies (ICF) syndrome is mainly determined by recurrent tract respiratory and gastrointestinal infections in early childhood due to agammaglobulinemia. Most patients with ICF syndrome die of infection at a young age, usually in the first or second decade of life. The leading cause of ICF disorders is mutations in genes whose products play a role in DNA methylation. ICF syndrome is classified into two groups: type 1 (ICF1) patients have mutations in the DNMT3B gene, and about half of type 2 (ICF2) patients have mutations in the ZBTB24 gene. In this study, we report the case of a 34-year-old female of Iranian consanguineous parents, who was diagnosed at one year of age with ICF-2 syndrome with recurrent infections, mental retardation, and a homozygous novel mutation in the ZBTB24 gene.
    Type of Medium: Online Resource
    ISSN: 2645-4831
    URL: Article
    DOI: 10.18502/igj.v4i3.12117
    Language: Unknown
    Publisher: Knowledge E DMCC
    Publication Date: 2023
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  • 9
    Online Resource
    Online Resource
    A Rare Case with Quail Egg Allergy without Allergic Reactions to Oral Food Challenge with Hen’s Egg White
    Knowledge E DMCC ; 2020
    In:  Iranian Journal of Allergy, Asthma and Immunology ( 2020-06-29)
    Details
    In: Iranian Journal of Allergy, Asthma and Immunology, Knowledge E DMCC, ( 2020-06-29)
    Abstract: Hen’s egg, as one of the most common reasons for IgE-mediated food hypersensitivity, affects both children and adults. Taking precautionary measures is suggested for the consumption of other birds’ eggs for patients with allergy to hen’s egg. This paper describes a rare patient with quail egg allergy, which manifested no allergic reactions after oral food challenge with hen’s egg white
    Type of Medium: Online Resource
    ISSN: 1735-5249 , 1735-1502
    URL: Article
    DOI: 10.18502/ijaai.v19i3.3459
    Language: Unknown
    Publisher: Knowledge E DMCC
    Publication Date: 2020
    detail.hit.zdb_id: 2388260-8
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  • 10
    Online Resource
    Online Resource
    The Uncomplicated Course of COVID-19 in Primary Immunodeficiency Patients: A Report of 14 Common Variable Immunodeficiency Patients
    Knowledge E DMCC ; 2022
    In:  Iranian Journal of Allergy, Asthma and Immunology ( 2022-10-30)
    Details
    In: Iranian Journal of Allergy, Asthma and Immunology, Knowledge E DMCC, ( 2022-10-30)
    Abstract: Coronavirus disease 2019 (COVID-19) affects millions of people worldwide. Clinical manifestations range from asymptomatic to severe viral pneumonia. CVID patients with COVID-19 infection are not adequately studied. In some studies, CVID patients had higher mortality rates, although other studies showed that CVID patients might have an uncomplicated COVID-19 infection. We describe 14 cases of COVID-19 infection in Iranian CVID patients in this study, including clinical manifestations, laboratory findings, and treatment strategies. There were 29% of patients with mild disease, 43% with moderate disease, and 29% with severe disease in this study. A critical case and a death occurred in none of our patients. There were six cases of infection more than two weeks after receiving the second dose of Sinopharm BIBP COVID-19 vaccine; all had mild to moderate disease. Among these patients, Remdesivir was the most frequently prescribed medication. According to this study, most of our patients presented with an uncomplicated disease course.
    Type of Medium: Online Resource
    ISSN: 1735-5249 , 1735-1502
    URL: Article
    DOI: 10.18502/ijaai.v21i5.11046
    Language: Unknown
    Publisher: Knowledge E DMCC
    Publication Date: 2022
    detail.hit.zdb_id: 2388260-8
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