mohammad nabavi
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Little is known about the quality of life of patients with anaphylaxis to Hymenoptera venom. The Vespid Allergy Quality of Life Questionnaire (VQLQ) is commonly used to assess the psychological burden of this condition. This study aimed to evaluate the validity and reliability of the Persian version of VQLQ. In this cross-sectional study, VQLQ was translated into Persian according to expert recommendations. The final translated version of VQLQ was then administered to 115 patients with Hymenoptera venom allergy at an asthma and allergy clinic in Iran. More than half of the participants were between 20 and 40 years of age, and 60% were male. Fear, anxiety, and outdoor activities had the most significant impact on the quality of life of patients with Hymenoptera venom allergy. Additionally, quality of life was more affected in women than in men, while no correlation was found with age. Furthermore, the quality of life was affected by a history of acute anaphylactic shock due to Hymenoptera venom. The Persian version of VQLQ enables the measurement of quality of life in patients with Hymenoptera venom allergy in the Iranian population. The inclusion of VQLQ in the initial evaluation of these patients may potentially guide allergist in providing support for venom-specific immunotherapy.
Keywords: Allergy quality of life, Anaphylaxis, Hymenoptera, Hymenoptera venom, Quality of life -
Background
NSAID-exacerbated respiratory disease (N-ERD) is a highly heterogeneous disorder with various clinical symptoms. The aspirin challenge test is a gold standard method for its diagnosis, and there are still no reliable in vitro diagnostic biomarkers yet. Oral challenge tests are time-consuming and may be associated with a risk of severe systemic reactions. This study aimed to evaluate whether patients with poor responses to medical management are more susceptible to being aspirin-sensitive.
MethodsIn this cohort study, after CT scanning of all patients and subject selection, conventional medical treatment was started as follows and continued for three consecutive months: at first, saline nose wash twice per day, intranasal beclomethasone spray one puff in each nostril twice per day, montelukast 10 mg tablet once daily, a ten-day course of oral prednisolone starting with the dose of 25 mg per day and taper and discontinued thereafter. Sinonasal outcome test 22 (SNOT22) was used for the evaluation of symptom severity. Statistical analyses were performed with SPSS version 23, and data were analyzed using an independent samples T-test, paired T-test, and Receiver operating curve analysis
Results25 males and 53 females were enrolled in this study, with an average age of 41.56 ± 11.74 years old (18-36). Aspirin challenge test results were positive in 29 (37.2%) patients. The average SNOT22 scores before the treatment were 52.97 ± 17.73 and 47.04 ± 18.30 in aspirin-sensitive and aspirin-tolerant patients, respectively, and decreased to 27.41 ± 16.61 and 24.88 ± 16.72 in aspirin-sensitive and aspirin-tolerant patients after the treatment, respectively. There was no significant difference in SNOT22 scores between the groups.
ConclusionThe severity of symptoms before treatment and clinical improvement after treatment are not good predictors of N-ERD.
Keywords: N-ERD, Aspirin-sensitive, Aspirin-tolerant, Chronic rhinosinusitis, Nasal polyposis, Asthma, SNOT22 -
Chronic granulomatous disease (CGD) is a rare primary immunodeficiency disorder more common in autosomal recessive (AR) than X-linked in Iran. This study aimed to assess whether having a child with AR-CGD would increase the likelihood of the next child being affected by CGD. Ninety-one families with at least one child affected by AR-CGD entered this study. Out of the 270 children, 128 were affected by AR-CGD. We used a cross tab for the odds ratio (OR) calculation, in which exposure to a previously affected child and the next child’s status were evaluated. This study illustrated that the chances of having another child afflicted with AR-CGD are significantly increased if the previous child had AR-CGD (OR=2.77, 95% CI=1.35-5.69).Althoug h AR disorders affect 25% of each pregnancy, we showed that the chance that the next child would be affected by CGD, given that the previous child was affected, is 2.77 times greater than in families with a normal child. It is recommended to warn families with one or more affected children to evaluate the risk of CGD in their subsequent pregnancies with prenatal diagnosis.
Keywords: Autosomal recessive, Chronic granulomatous disease, Parental consanguinity, Siblings -
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.
Keywords: Chromosomal Instability, Immunodeficiency-Centromeric Instability-Facial Anomalies Syn-drome, Mental Retardation -
Background
Post-acute COVID-19syndrome involves the persistence of the patient’s symptoms due tothe residual inflammation of the acute phase.
ObjectivesIn the current study, we aimed to evaluate medication intervention to accelerate the improvement of prolonged respiratory symptoms in this phase.
MethodsThirty-four patients, aged 20-50 years, in the recovery phase of COVID-19, were enrolled, who still suffered from respiratory problems even two weeks after being discharged from Rasool Akram Hospital, Tehran, Iran. They were divided into three groups based on the type of treatment for eliminatingthe remaining symptoms: hydroxychloroquine (HCQ, 200 mg twice daily for four weeks), clarithromycin (500 mg twice daily for four weeks), and control (receiving a placebo similar to the last two groups). At the beginning and end of the treatment, patients’dyspnea and cough were assessed using Medical Research Council and visual analog scale (VAS), respectively, their laboratory tests were checked, and they took a 6-min walk test.
ResultsAt the end of the treatment,the VAS of cough was 0.74 in the HCQ group, which was higher than that in the clarithromycin group. In addition, dyspnea decreased in theHCQ and clarithromycin groups by 64% and 40%, respectively,compared to the control group. Furthermore, there was a significant relationship between residualdyspnea at the end of the treatment and the severity of initial lung involvement in the acute phase.
ConclusionBased on these findings, it can be concluded that HCQ was more effective in reducing dyspnea, compared to clarithromycin, in the recovery phase, especially in patients with milder lung involvement in the acute phase. Additionally, clarithromycin was found to be more effective in improving coughs.
Keywords: Clarithromycin, Cough, COVID-19, Hydroxychloroquine, Post-acute COVID-19 -
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.
Keywords: Common variable immunodeficiency, COVID-19, COVID-19 Vaccines, Primary immunodeficiency -
Background
IL-17 producing T cells are a distinct subset of CD4+ T cells, which are recognized to have an essential role in protection against certain fungi and extracellular pathogens.
ObjectivesThis study aims to evaluate the number of IL-17 producing T cells as a predictor of primary immunodeficiency disorders in patients with Candida infections and low numbers of IL-17 producing T cells.
MethodsSeven newly diagnosed patients with documented Candida infections aged between 4 and 35 years were included in this study. Before establishing the diagnosis of chronic mucocutaneous candidiasis, a thorough immunodeficiency workup, including complete blood count, serum Ig levels and antibody responses, flow-cytometry evaluation, and LTT, was completed for all the patients, and other immunodeficiency disorders, including combined and phagocytic deficiencies, were ruled out. Then, IL-17 producing T cells were detected using antiCD3 and anti-IL-17 antibodies through flow-cytometry evaluation.
ResultsThe IL-17 producing T cells significantly decreased in the peripheral blood of patients with PID and candida infections. A P-value of less than 0.05 was considered statistically significant.
ConclusionsTo conclude, in cases of recurrent candida infections, initial assessment of IL-17 producing T cells may act as a predictor of an underlying primary immunodeficiency. In patients with low counts of IL-17 producing T cells, selecting a targeted panel of genetic tests may become more helpful in the detection of certain immunodeficiency disorders than performing whole-exome sequence analysis.
Keywords: Candida, Primary Immunodeficiency, IL-17 Producing T Cells -
Type 2 Griscelli syndrome (Type2 GS) is a primary inborn error of the immune system, classified in the immune dysregulation group.1,2 There are three different types of the disease, with different genetic causes responsible for the autosomal recessive inheritance pattern. Although hypopigmentation is common in all variants, neurological involvement or immunodeficiency with varying severity is seen in different types. Molecular motor protein myosin 5 an (MYo5A) [Type1GS], guanosine Triphosphate (GTP) binding protein (RAB27A) [Type2GS], and mutation in human melanophilin (MLPH) [Type 3GS] which is limited to hypopigmentation are reported as the known genetic defects in GS.3 Severe, ineffective, and uncontrolled inflammatory reactions are referred to as the pathogenesis of Hemophagocytic lymphohistiocytosis (HLH). HLH is a life-threatening condition that can be defined as either primary or secondary. Secondary causes happen in the context of autoimmunity, malignancy, spontaneous, or infections.4 Prenatal infections play an important role in causing long-term complications in the fetus. Some of them include toxoplasmosis, rubella, cytomegalovirus, herpes simplex, and other organisms including syphilis, parvovirus, and Varicella zoster, known as TORCH syndrome (5).TORCH has been well described for a long time but there are limited reports of developing HLH in the context of prenatal infections. We described a type 2GS syndrome with neonatal-onset HLH triggered by a prenatal infection.
Keywords: Griscelli syndrome type 2, Hemophagocytic lymphohistiocytosis, Primary immunodeficiencydiseases -
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.
Keywords: X-Linked Agammaglobulinemia, Morphea, Eosinophilic Fasciitis, Rheumatologic Manifesta-tion, Sensorineural Hearing Loss -
Descriptive Study of 123 Symptomatic Patients with IgA Deficiency: A Retrospective Case Series StudyBackground
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.
MethodA 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.
ResultsThe 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.
ConclusionAlthough 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.
Keywords: IgA Deficient Patients, Primary Immunodeficiency Disorder, Clinical Manifestations, Laboratory Results -
Ligustrum vulgare (Privet) pollen proteins are responsible for allergies in susceptible individuals in many regions of the world. This study investigated the immunochemical characterization of Privet pollen extract and the occurrence of skin prick test reactivity to Privet and other allergenic pollen grains in allergic rhinitis patients. All subjects experienced a skin prick test with twenty-two allergen extracts. sodium dodecyl sulfate-polyacrylamide gel electrophoresis (SDS-PAGE) separated Privet pollen extract, IgE-immunoblotting, and specific ELISA procedures determined the allergenic profile on forty-five Privet allergic patients. A positive allergic reaction to L. vulgare pollen extract was observed in forty-five (31.4%) out of 145 patients. Ten resolved protein fractions were found on SDS-PAGE, ranging from 10 to 80 kDa. IgE-specific antibodies interacted with several allergenic protein bands from Privet-allergic patients in the immunoblotting assay. The most significant interaction was observed in proteins with molecular weights of approximately 15, 18, 43, and 66 kDa. Privet pollen is regarded as a potent allergen composed of IgE-binding constituents. Considering the high allergenicity of Privet pollen grains and since many countries are rich in this plant, identification and production of recombinant forms of common allergens in this species can be used for developing more efficient diagnostic, therapeutic, and preventive approaches.
Keywords: Allergens, Iran, Ligustrum, Prevalence, Pollen -
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.
Keywords: Food hypersensitivity, Patch tests, Proctocolitis -
Immunoglobulin G4-Related Disease (IgG4-RD) is a systemic fibro-inflammatory disease that has been proposed as a separate entity since the beginning of this century. The disease is often manifested by increased serum IgG4 levels and certain histopathological manifestations. The patient mentioned in this article is a 29-year-old man from Tajikistan, who has had a chronic cough since the beginning of 2018 without a previous history of the disease. At first, he was diagnosed with pneumonia for a long time and then underwent a lung biopsy due to exacerbation of symptoms and the spread of lung lesions in radiology but no abnormalities were found in these evaluations. The patient traveled to Iran to continue his treatment. He was re-evaluated and then the previous samples taken from the patient's lung tissue were re-examined. Here are the key findings in favor of diagnosing IgG4 RD. Evaluations did not confirm the involvement of other organs. He was first treated with steroids and due to recurrence of symptoms, he was treated with rituximab once which was significantly effective in improving the patient's clinical symptoms. In general, it can be concluded that the diagnosis of IgG4-RD is very challenging and if it has not diagnosed and treated in time, it can lead to irreversible fibrosis and permanentloss of function of the involved organ.
Keywords: fibro-inflammatory disease, Immunoglobulin G4-Related Disease, Rituximab -
Asthmatic patients may have aspirin-exacerbated respiratory disease and experience acute dyspnea and nasal symptoms within 3 hours after the ingestion of aspirin. This study aimed to evaluate the effect and outcome of daily low-dose aspirin in the treatment of moderate to severe asthma in patients with concomitant aspirin hypersensitivity and chronic rhinosinusitis with nasal polyposis (CRSwNP). This clinical trial was conducted from February 2014 to February 2015 on 46 adult patients with moderate to severe asthma accompanied by CRSwNP. Patients with a positive aspirin challenge were blindly randomized in three groups receiving placebo/day (A); aspirin 100 mg/day (B); and aspirin 325mg/day (C), respectively. Clinical findings, FEV1 and ACT scores were recorded and compared before, during, and after treatment for 6 months. Of 46 participants at baseline, 30 patients completed this 6-month trial study. The level of asthma control was significant; based on Asthma Control Test (ACT) when comparing the results in groups A and C and also groups B and C, but it was not significant when comparing ACT scores between groups A and B. FEV1 before and after treatment was significant when comparing groups A and B, groups A and C, and groups B and C. To conclude, aspirin desensitization with a daily dose of 325 mg aspirin resulted in the improvement of long-term control of asthma. A daily aspirin dose of 100 mg was not associated with such an increase in ACT score.
Keywords: Aspirin, Asthma, Nasal polyps -
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.
Keywords: Chromosomal instability, DNA methyltransferase 3B, Immunodeficiency, Scoliosis -
Hereditary angioedema (HAE) is characterized by recurrent attacks of skin and mucosal swelling in any part of the body including the digestive and respiratory tract which generally improve spontaneously within 12-72 hours. The underlying mechanism in HAE is related to bradykinin dysregulation which causes these attacks not to respond to common treatment strategies including epinephrine or corticosteroid. There are several types of HAE with different etiology but with the same clinical picture. Type 1 is due to the deficiency of C1 Inhibitor (C1-INH) protein and type 2 is related to dysfunctional C1-INH protein. The third type of HAE which comprises the minority of cases is associated with the normal amount and function of C1-INH protein. The presented case in this report was a 15-years old girl with a history of spontaneous angioedema attacks from the age of 14. The frequency of attacks was initially every two months but consequently increased to every two weeks after using some hormonal medications for ovarian cyst. Each episode has lasted around 10 days without any symptoms in between. Complement studies including C4, C1q, and C1-INH protein, both quantitative and qualitative, were reported as normal. A genetic assessment revealed a mutation in the exon 9 on the gene related to factor XII, hence the diagnosis of HAE type 3 was confirmed. This was a rare type of angioedema with normal amount and function of C1-INH protein which is predominantly seen in women during periods of imbalanced estrogen increments like pregnancy, lactation, and menopause, and hence it is responsive to hormonal manipulation strategies such as the use of progesterone containing medications.
Keywords: Bradykinin, Complement C1 inhibitor protein, Factor XII, Hereditary angioedema -
Chronic eosinophilic pneumonia (CEP) is a rare idiopathic interstitial lung disease, predominantly observed in females. Eosinophilia is present in most cases, and alveolar eosinophilia is a diagnostic criterion in more than 40% of bronchoalveolar lavage (BAL) samples. The current study reported a 27-year-old male patient, non-smoker, with a history of uncontrolled asthma, presented to the emergency room with a complaint of cough, fever, and moderate dyspnea. A 30% eosinophilia was reported in his peripheral blood sample. A chest-X ray examination showed an upper and middle lobe consolidation, especially in the left lung. Broad-spectrum antibiotics were then started with a presumptive diagnosis of pneumonia, but no improvements were evident. The chest computed tomography scan showed air space opacities with septal thickening and predominant involvement of upper and middle lobes. Flexible bronchoscopy was performed, and the BAL sample analysis showed eosinophil infiltration, while negative culture. No parasites were identified. Transbronchial biopsies demonstrated eosinophil accumulation in alveoli and interstitium
ConclusionEarly recognition, diagnosis, and prompt treatment with corticosteroids are the main therapeutic approaches to CEP.
Keywords: Chronic Eosinophilic Pneumonia, Eosinophilia, pneumonia, Interstitial lung disease -
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.
Keywords: Aspirin, Dapsone, Linear IgA bullous dermatosis, Mucocutaneous lymphnode syndrome -
Background
Rhinosinusitis is a nasal and paranasal sinus infection. Chronic infection and allergies are two major causes of nasal polyp formation.
ObjectivesIn this study, we examined the relationship between NO and aeroallergens to determine the relationship between exhaled nitric oxide level and sensitivity to aeroallergens in chronic rhinosinusitis with nasal polyps (CRSwNP) patients referring to Hazrat Rasool Hospital in Tehran from December 2016 to May 2017.
MethodsA cross-sectional study was conducted on 72 patients. The prick test and exhaled NO measurements were done to determine allergens in CRSwNP patients. Statistical analysis was done to determine the relationship between the allergy and sensitivity to indoor aeroallergens in patients with CRSwNP.
ResultsThe correlation coefficient of. 0.014 and 0.001 were evaluated for Mosquito and Cockroach respectfully, which were significantly correlated with nitric oxide. The less correlation with nitric oxide was 0.003, which was evaluated for MUCOR.
ConclusionsThis study showed that most people with CRSwNP had a sensitivity to at least one indoor aeroallergen, and cockroaches were the most common allergen in patients with CRSwNP with a 17.9% prevalence. This study showed that the correlation of FeNO with the sensitivity of these patients to home aeroallergens can be used, and this correlation for mosquito and cockroach aeroallergens is very valuable. The NO level and prick testing of aeroallergens can be used to estimate the relapse or lack of appropriate response to medical treatment in patients with CRSwNP.
Keywords: Allergy, Asthma, Allergic Rhinitis -
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 non-steroidal 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 pre-bronchodilator 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.
Keywords: Asthma, Aspirin, Hypersensitivity -
Increased susceptibility to autoimmunity, malignancy, and allergy in addition to recurrent infections are the main characteristics suggesting for the primary immunodeficiency diseases (PID). CTLA-4 is predominantly expressed on activated and regulatory T-cells, which can bind to CD80/CD86 molecules on antigen-presenting cells as a negative regulator. Here, we describe a 24-year-old male born from consanguineous parents with heterozygous CTLA-4 mutation who presented with multiple autoimmune diseases. His past clinical history revealed alopecia areata atfour years old and subsequently, he developed Evans syndrome, type 1 diabetes mellitus, hypothyroidism, and chronic diarrhea while chronic rhinosinusitis and cytomegalovirus (CMV) colitis were the only infectious manifestations. Immunologic investigations revealed: low B cell count, abnormal Lymphocyte transformation test (LTT) to phytohemagglutinin (PHA), and hypogammaglobulinemia. Although all available treatments such as Intravenous Immunoglobulin (IVIG) therapy, immunosuppressive drugs, and antibiotic therapy were applied, diarrhea was not controlled due to colitis, which remained challenging. Whole exome sequencing was performed and the result showed heterozygous variant CHR2.204,735,635 G>A in the CTLA-4 gene, which was confirmed by the Sanger method. CTLA4 haploinsufficiency leads to autoimmune disorders, recurrent respiratory infections, hypogammaglobulinemia, lymphoproliferation with organ infiltration, and lymphocytic interstitial lung disease.
Keywords: Abatacept, CTLA-4 Deficiency, Haploinsufficiency, immunodeficiency, Multiple Autoimmunities -
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.
Keywords: Egg allergy, Oral food challenge, Quail egg allergy -
Serum Concentration of Selenium and GPX Enzyme Activity in Iranian Children with AsthmaBackground
Asthma is a disease with chronic airway inflammation, whereby the airway hyper-reactivity increases. Oxidative stress
balance plays a critical role in asthma.ObjectivesThe present study aimed to evaluate serum glutathione peroxidase (GPX) enzyme activity and selenium levels in patients with asthma.
MethodsThis case-control study was conducted on 64 children aged 1 - 5 years, 32 subjects as controls, and 32 subjects with asthma
who were selected randomly. The serum GPX activity and selenium levels of the studied subjects were determined spectrophotometrically.ResultsThe present study showed that serum GPX activity and Selenium (Se) levels of patients with asthma were significantly lower
than those of controls (P < 0.001, P < 0.05, respectively).ConclusionsReduction of serum GPX activity and Se concentration in patients could have a potential role in the pathogenesis and
modulation of asthma. Micronutrient supplementation of Se may be beneficial in asthma.Keywords: Glutathione Peroxidase, Selenium, IgG, Oxidative Stress, Asthma -
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.
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Severe Combined Immunodeficiency (SCID), characterized by a profound decrease in both the number and function of T cells, is related to more than 20 different mutations. Recombination-activating gene (RAG) 1 and 2 seem to be two of the most common forms presenting with various manifestations, including typical SCID, Omenn syndrome (OS), atypical SCID (AS), or delayed onset combined immunodeficiency with granulomas. One interesting manifestation in RAG mutation is the change in the immunophenotype over time, even after hematopoietic stem cell transplantation (HSCT). As bone marrow transplantation (BMT) is the only curative treatment of SCID, it is necessary to differentiate between SCID and OS due to the different conditioning regimens (CR). We present a novel case of atypical SCID (SCID manifestations with more than 300 CD3+T cells) caused by RAG 2 gene mutation whose immunophenotype changed to atypical Omenn syndrome (all Omenn syndrome manifestations except rash, eosinophilia, and elevated IgE) over time.Differentiation of leaky SCID, SCID and Omenn syndrome in RAG mutation genes and overlap manifestations is important in treatment plan and prognosis.
Keywords: Atypical Omen Syndrome_Atypical SCID_RAG 2 Mutation_Severe Combined Immunodeficiency
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