Evaluation of Classic Wiskott Aldrich Syndrome with Mild Symptoms in Two Cousins: A Case Report

Introduction: Wiskott–Aldrich syndrome (WAS) is characterized by microthrombocytopenia, eczema, recurrent infections, and an increased incidence of autoimmunity. Commonly, classicWAS is presented with severe clinical symptoms. Case Presentation:We report a new phenotype of classic Wiskott–Aldrich syndrome with mild symptoms in two cousins who were 7 years old. They had not severe infections or hemorrhage, in spite of having genetic mutation in WAS gene. The symptoms and infections of the patients responded to treatment with IVIG and antibiotics. Conclusions: This report is presenting a novel clinical phenotype of classicWAS with milder symptoms

Association Between Allergic Diseases and Food Allergens Based on Skin Prick Test in Bushehr Province

Background: The Frequency of allergic diseases is growing in recent years. Identification of frequency of food allergens in different areas play an important role in diagnosis and treatment of these diseases. The aim of this study was to determine frequency and association of common food allergens in patients with allergic diseases based on Skin Prick Test in Bushehr province. Material and Methods: In this descriptive cross-sectional study, 1100 patients were enrolled with allergic diseases which had a sensitivity to at least one allergen. This test was carried out with 21 common food allergens extract. Results: In all patients, association between the severity of the reaction prick allergy test and severity of allergic diseases with shrimp, cow's Milk and peanuts were (P= 0.01), (P= 0.02) and (P=0.04) respectively. In this study, the frequency of allergic rhinitis, asthma, chronic and acute urticaris and atopic eczema were 54.2%, 23%, 12.4%, 4.1% and 12%, respectively. While the the most common food allergens were peanuts (46.6%), egg yolk (43.1%) and shrimp (42%) respectively. Conclusion: This study indicated that food allergens such as shrimp, cow's Milk and peanuts have a greater role in severity of allergic diseases and this food allergens showed the highest frequency in patients

Autoimmune versus Non-autoimmune Cutaneous Features in Monogenic Patients with Inborn Errors of Immunity

Cutaneous manifestations are one of the most common presentations among patients with inborn errors of immunity (IEI). These skin manifestations are often among the first presenting features in the majority of patients preceding the IEI diagnosis. We studied 521 available monogenic patients with IEI listed in the Iranian IEI registry up to November 2022. We extracted each patient's demographic information, detailed clinical history of cutaneous manifestations, and immunologic evaluations. The patients were then categorized and compared based on their phenotypical classifications provided by the International Union of Immunological Societies. Most patients were categorized into syndromic combined immunodeficiency (25.1%), non-syndromic combined immunodeficiency (24.4%), predominantly antibody deficiency (20.7%), and diseases of immune dysregulation (20.5%). In total, 227 patients developed skin manifestations at a median (IQR) age of 2.0 (0.5-5.2) years; a total of 66 (40.7%) of these patients initially presented with these manifestations. Patients with cutaneous involvement were generally older at the time of diagnosis [5.0 (1.6-8.0) vs. 3.0 (1.0-7.0) years; p = 0.022]. Consanguinity was more common among patients who developed skin disorders (81.4% vs. 65.2%, p < 0.001). The overall skin infection rate and the type of dominant pathogens were significantly different among the IEI patients in different phenotypical classifications (p < 0.001). Atopic presentation, including urticaria, was highly prevalent among patients with congenital defects of phagocytes (p = 0.020). The frequency of eczema was also significantly higher among cases with both syndromic and non-syndromic combined immunodeficiency (p = 0.009). In contrast, autoimmune cutaneous manifestations, including alopecia and psoriasis, were most common in patients with immune dysregulation (p = 0.001) and defects in intrinsic or innate immunity (p = 0.031), respectively. The presence of autoimmune cutaneous complications significantly improved the survival rate of IEI patients (p = 0.21). In conclusion, cutaneous manifestations were observed in nearly 44% of Iranian patients with monogenic IEI. A considerable number of patients with cutaneous involvements developed these disorders as their first manifestation of the disease, which was particularly noticeable in patients with non-syndromic combined immunodeficiency and phagocytic defects. The neglected skin disorders in IEI patients might delay diagnosis, which is generally established within a 3-year interval from the development of skin-related problems. Cutaneous disorders, especially autoimmune features, might indicate a mild prognosis in IEI patients

Does treatment of gastro-esophageal reflux disease with omeprazole decrease allergic rhinitis symptoms?

Background: Allergic rhinitis is the most common type of allergic disease among population. Its accurate treatment is very important for cutting of allergic march. On the other hand, gasteroesophageal reflux disease (GERD) is one of the most common gastrointestinal problems among allergic patients mainly asthmatic cases. It might conflict treatment. Despite of asthma, a few studies have been conducted on the impact of GERD treatment on allergic rhinitis symptoms. In this study, we assessed GERD treatment and its effects on improving of allergic rhinitis patients with GERD. Materials and Methods: In a prospective cross-sectional study, March - September 2012, 103 consecutive patients with persistent moderate to severe seasonal allergic rhinitis enrolled. For allergic rhinitis patients with GERD 20 mg omeperazole once daily for 6 weeks prescribed, empirically. Conventional allergy treatment continued and finally the allergic rhinitis symptoms were assessed clinically and recorded before, 5th, 10th and 30th days of omeprazole treatment period. Results: Our study included 103 patients with seasonal allergic rhinitis who were divided into GERD (n=33, 38%) and non-GERD (n=70, 68%) groups with the mean age 28 and 25.7 years, respectively. The first group developed significant improvement for GERD symptoms on days 5, 10 and 30 after beginning of therapy (P=0.03). No association was found between GERD treatment and relief of allergic symptoms or TNSS improvement (P>0.05). Data analyzed by Epi info (ver 7) and SPSS software (ver 11.5), and by Chi squeare test and paired T test. P lower than 0.05 was considered as significant. Conclusion: This study showed no significant association between empirical treatment of GERD and improvement of allergic symptoms in patients with allergic rhinitis. However, further studies with a larger sample size might be needed

Association Between Aeroallergens and Allergic Diseases Based on Skin Prick Test in Bushehr Province

Background: The prevalence of allergic diseases is growing in the world and Iran. Allergens play an important role in creating these diseases. Since inhalation allergens are an important part of these allergens the aim of this study was to assess the prevalence of aeroallergens and their association with allergic diseases based on skin prick test (SPT). Material and Methods: In this study, the frequency of these allergens were examined by using 22 aeroallergens and based on Skin Prick Test in 1100 patients with allergic diseases who referred to Shohadaye- Khalije- Fars university hospital. Results: The reaction severity of SPT and the severity of disease associated with inhaled allergens with Russian thistle (21.1%, P꞊0.001), Chenopodium album pollen (14%, P꞊0.001) and Dermatophagoid Pteronyssinus (9%, P꞊0.001) respectively. Also, in these patients, the frequency of inhalation allergens were House Dust Mite (HDM) (69%), feather (60.8%), Russian this tle (59.9%) pollen and Alternaria mold (59.6%) respectively. Conclusion: This study indicated that outdoors inhaled allergens &nbsp;such as Russian thistle and Chenopodium album pollen have the most allergenicity in patients with allergic and asthma in Bushehr province, Meanwhile, the prevalence of indoor aeroallergens such as HDM, feather and mold were high in these patients

Study of Prevalence and Risk Factors of Asthma and Allergic Diseases Among School Children (6-7 and 13-14 years) Based on ISAAC Protocol in Jam City, Bushehr Province in 2014

Background: Asthma and allergic diseases are raised as a major health problem in the world. The prevalence of these diseases is on the rise specifically in the world and Iran. Accordingly, the aim of this study was to assess the prevalence of these diseases in Jam City, from environs of Bushehr Province. Material and Methods: this study was performed on 516 schoolchildren aged 6-7 years and 569 schoolchildren aged 13-14 years in schools of this city based on a standard questionnaire of allergic conditions and asthma (ISAAC) phase 1 and 3, which mention to the prevalence and risk factors for these diseases in 2014. &nbsp;&nbsp;&nbsp; Results: in prevalence of atopic eczema, allergic rhinitis and asthma among 6-7year old schoolchildren were 13.8 %, 20% and 3.9%, respectively, While, the prevalence of these diseases among 13-14 year old schoolchildren were 19.5%, 19.2% and 6.7%, respectively. There were a significant association in each group, between affliction rate to asthmaand its association with rhinitis and eczema (P<0.05). Also, there were a significant association between the prevalence of these diseases and the presence of risk factors such as exposure to cigarette smoke, pet care and use of gas as a fuel at home. (P<0.05). Conclusion: the prevalence of asthma and allergic diseases was high in Jam city and this increase is associated to risk factors such as exposure to cigarette smoke, pet care and use of gas as a fuel at home

Prevalence of Asthma and Allergic Diseases and Its Risk Factors in School Children Aged (6-7 and 13-14 Years) in Assalouyeh City, Bushehr Province Based on III ISAAC Protocol Phase I, in 2014

Background: Asthma and allergic diseases are raised as a major health &nbsp;problem. The prevalence of these diseases are increasing in Iran and &nbsp;all over the world. Based on this, the present study assessed to prevalence of these diseases in Assalouyeh region, Bushehr Province. Material and Methods: This study was performed on 190 school children aged 6-7 years and 223 girl student aged 13-14 years in Assalouyeh city in 2014 based on ISAAC standard questionnaires Phase I and III which examined prevalence and risk factors for these diseases. Results: The prevalence of atopic eczema, allergic rhinitis and asthma among &nbsp;6-7 year-old students were 11.6%, 13.7% and 5.8%, respectively. While, the prevalence of these diseases among 13-14 year-old students were 14.3%, 21.5% and 15.2%, respectively. Also, there were a significant association between risk factors such as exposure to tobacco smoke, pet keeping, consumption of fast and sea foods with prevalence of these diseases (P<0.05). Conclusion: Asthma and allergic diseases were high among school children in Assalouyeh and it seems that this increasing prevalenc can be associated with risk factors such as exposure to tobacco smoke, pet keeping, consumption of fast and sea foods

Multiple Right and Left Pulmonary Arteries and Subdivisions of Inferior Mesenteric Artery Aneurysms in Behcet’s Disease Case: A Rare Clinical Presentation

Behcet’s disease is a multi-systemic inflammatory disorder with cutaneous acneiform eruptions, orogenital aphthae, uveitis, arthritis and systemic vascular inflammation. One of the rare vascular manifestations is thoraco-abdominal aortic and pulmonary aneurysm that is associated with high risk of morbidity and mortality. We report a 36-year-old man with chronic cough, hemoptysis, significant weight loss, and orogenital ulcers from one year before referral. Initial assessments revealed multiple parahillar nodules in chest X-ray, chronic inflammatory anemia, erythrocyte sedimentation rate more than 100, and positive Human Leukocyte Antigen B5 and B51. Evaluation for infection and malignancies was unremarkable. Open exploratory lung study showed multiple pulsatile nodules in both lungs. AMIGO computed tomogram confirmed multiple right and left pulmonary artery aneurysms and impending to rupture aneurysm at subdivision of inferior mesenteric artery. After beginning of three methylprednisolone and cyclophosphamide pulse doses, the clinical aspect of the patient dramatically improved. Although pulmonary aneurysm is a rare manifestation of Behcet’s disease and it is more infrequent in the distal branches, it can be seen in patients presenting with inflammatory disease and respiratory manifestations and with Behcet’s disease diagnosis. Corticosteroid pulse-therapy could be considered as the first line of medical treatment in these patient

Evaluation of effectiveness of specific subcutaneous immunotherapy for patients with allergic rhinitis and asthma

Background: Allergen immunotherapy involves the administration of gradually increasing quantities of specific allergens to patients with IgE-mediated conditions until a dose is reached that is effective in reducing disease severity from natural exposure. This study evaluated the clinical efficacy of immunotherapy with extracts of common aeroallergens North-East of Iran in asthma and allergic rhinitis. Material and Methods: In this prospective study 156 cases were chosen randomley. The mean age of patients was 37 years (range 5-65 years). The patients with mild to moderate asthma and allergic rhinitis and history of atopy were selected for immunotherapy when they showed no effective response to medical treatment.Immunotherapy materials were made from common aeroallergens in north-eastern region of Iran by Dome Hollister US company. Immunotherapy schedule for injection of the extract with vial dilution of 1:10000pg was one injection every week for ten weeks and one injection with dilution of 1:1000pg every other week for the other ten weeks and one injection monthly from dilution of 1:100pg for two years. Results: One hundred twenty (77%) of cases had allergic rhinitis 29(18.5%) cases had allergic asthma and 7(4.5%) cases were mixed. Mean age of patients were 37 years old. 48(30.8%) cases were male. Analysis of efficacy of treatment showed that immunotherapy significantlyimproved the signs and symptoms of all the groups. In allergic rhinitis group 84(70%) cases completely improved, 22(18.4%) patients moderately responded and no response to immunotherapy was observed in 14(11.6%) patients. In allergic asthma group, 22(75%) cases completely improved 4(13.6%) cases moderately responded and no response to immunotherapy was detected in 3(11.4%) cases. In mixed group, 3(42.8%) cases completely improved, 3(42.8%) cases moderately responded and no response was observed in 1(14.4%) case. Conclusion: Specific allergen immunotherapy for patients with allergic persistent mild to moderate asthma and moderate to severe allergic rhinitis without good response to medical treatment is highly recommended. It is recommended as effective treatment in such patients