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Objective Asthma is one of the most common diseases amongst children. Blood eosinophil count and neutrophil–lymphocyte ratio (NLR) are known as markers for phenotyping asthma. This study was performed to investigate blood eosinophil count and NLR as predictors of hospitalization in pediatric asthma exacerbations. Data sources and study selections In this cross-sectional study, children admitted to hospital ward for more severe asthma exacerbation were compared with non-hospitalized children with moderate to severe asthma exacerbation whose asthma exacerbation was managed in emergency department or outpatient clinic. We investigated patients’ characteristic and factors associated with hospitalization. Results A total of 211 children with moderate to severe asthma exacerbation (mean age $$5.76 \pm 3.28$$ 5.76 ± 3.28 years old) were enrolled in the study including 91 hospitalized patients and 120 non-hospitalized patients. For the prediction of hospitalization, an ROC Curve analysis was performed and revealed a cut-off of 298 cells/µL and 2.52 of blood eosinophil count and NLR, respectively. In multivariate analysis, not using an asthma action plan (OR 2.22, 95% CI 1.09–4.49; P = 0.027), a blood eosinophil count $$\ge$$ ≥ 298 (OR 8.79, 95% CI 4.44–17.4; P $$\ge$$ ≥ 2.52 (OR 2.13, 95% CI 1.09–4.14; P = 0.027) were associated with hospitalization. Conclusion Blood eosinophil count and NLR were found to be higher in hospitalized children with more severe asthma exacerbation compared to non-hospitalized patients. These markers can be indicators for asthma exacerbation severity.

Bronchiectasis is a clinical syndrome characterized by chronic cough, sputum production, recurrent respiratory infections, and permanent bronchial dilation. The association between the level of alpha-1 antitrypsin (AAT) and bronchiectasis is controversial. In this study, we aimed to investigate this association in children with idiopathic bronchiectasis. The study was conducted on 20 patients with idiopathic bronchiectasis as the case group (mean age 15.9±2.1) and 20 healthy individuals as the control group (mean age 14.9±2.6). Serum AAT level was measured using nephelometric analysis (g/L). Other criteria including sex, parent consanguinity, number of hospitalizations, age of the first symptom were evaluated in both groups related to AAT level. The mean serum level of AAT in the case and control groups were 1.3±0.29; 1.5±0.59, respectively, with statistical significance (P=0.001). There was a significant difference between the two groups in the AAT level distribution, according to AAT normal range (P=0.01). The case group had a more positive attitude toward consanguinity than the control group (66.7% versus 33.3%; P

Purpose This study aimed to assess the effect of COVID-19 on patients with IEIs, the potentially at-risk population, regarding the clinical course, complications, severity, and outcomes. Methods This two-phase study was conducted on patients from three referral immunodeficiency centers in Iran. At phase one, 98 IEI patients with COVID-19 infection were evaluated by telephone follow-up (TFU). At phase two, the demographic, clinical, and laboratory records of clinically confirmed 33 IEI patients with COVID-19 infection were collected and analyzed. Results At phase one, 16.3% represented COVID-19 infection without any report of pediatric intensive care unit (PICU) admission or death. During the second phase, combined immunodeficiency (CID) (42.4%) and predominantly antibody deficiencies (PADs) (33.3%) were the predominant immune defects. Atopy (27.3%) and lung disorders (27.3%) were the frequent pre-existing comorbidities. Organomegaly (p=0.030) and renal disorders (p=0.033) were significantly associated with the development of respiratory insufficiency. Cyanosis, tachypnea, intercostal retraction, and seizure were the chief complaints of patients who were more likely to progress respiratory insufficiency (p, being admitted to the PICU (p, and/or deceased (p. Laboratory evaluation revealed a marked positive correlation between D-Dimer (p=0.045), prothrombin time (p=0.045), C-reactive protein (p=0.041), proteinuria (p=0.013), ferritin (p=0.020), metabolic acidosis (p=0.003), and troponin (p=0.049) level with mortality. We detected a significant association between the chest X-ray pattern of COVID-19 infection with PICU admission (p=0.023) and death (p=0.046). Conclusion In the current study, patients with CID and PAD were introduced as patients at high risk of COVID-19 infection, who may need extra protective and therapeutic measurements.

Kawasaki disease is an acute systemic vasculitis which can cause cardiac involvement among other symptoms. In this study we aimed to assess the relationship between the echocardiographic findings of Kawasaki disease with the clinical and paraclinical findings of the patients. In this cross-sectional study, the symptoms of 307 Kawasaki patients were registered and the association of the symptoms with paraclinical findings and echocardiographic studies was assessed. 190 (61.9%) of the patients were male and 117 (38.1%) were female. 193 patients (62.9%) did not have any abnormalities in their echocardiography, while others showed coronary artery aneurysms, perivascular brightness, coronary artery dilatation, and trivial Mitral Regurgitation in their echocardiography. A significant inverse relationship was seen with echocardiographic findings and age. Thrombocytosis, conjunctivitis, and oral and/or pharyngeal erythema and/or strawberry tongue were associated with higher rates of echocardiographic abnormalities. Echocardiographic abnormalities are associated with younger age, higher platelets, and the existence of conjunctivitis and oral and/or pharyngeal erythema and/or strawberry tongue.

Chronic urticaria is in some cases very difficult to treat, we aimed to evaluate the effect of colchicine on chronic urticaria management. Fifty-five euthyroid patients with chronic urticaria were examined based on UQL and UAS7 questionnaires. The overall UQL-Q score after three months has not any statistical difference between groups. The overall UQL-Q score changes between groups was not statistically different. However, the UQL-Q score significantly reduces during study period in each group. In conclusion, colchicine can manage the sign and symptoms of chronic urticaria which improve the patients' quality of life. This article is protected by copyright. All rights reserved.

Background Chronic urticaria is a common distressing allergic skin disorder. Immune dysregulation, histamine release and mast cell degranulation are suggested as its underlying mechanisms. Objective Add-on therapy of vitamin D was evaluated in patients with chronic spontaneous urticaria to determine the quality of life and urticaria severity score. Methods In a prospective, double-blinded study, 80 participants with chronic spontaneous urticaria were randomized to low (4200 IU/week, group 1) and high (28,000 IU/week, group 2) vitamin D3 supplementation groups for 12 weeks. Demographic data; quality of life, urticaria severity and medication scores; 25-hydroxyvitamin D and anti-thyroid peroxidase antibody levels; and autologous serum skin test data were collected. Results Both groups showed significantly reduced total urticaria severity score; decrement in group 2 score was significant compared to group 1 at week 6 (P = 0.010). Quality of life score was also significantly reduced; decrement in group 2 score was significant compared to group 1 at both weeks 6 (P = 0.005) and 12 (P = 0.007). 25-hydroxyvitamin D levels were elevated significantly over the course of 12 weeks in both groups; however, the elevation in group 2 was significantly higher than group 1 at week 12 (P = 0.002). Medication score was significantly reduced, with no significant difference between groups. No association was observed between positive autologous serum skin test, angioedema and high level of Anti thyroperoxidase antibody with positive response to vitamin D. Conclusions Add-on therapy with vitamin D (28,000 IU/week) can be considered as a safe and potentially beneficial treatment in patients with chronic spontaneous urticaria.

Whey protein is a popular dietary product that has numerous health benefits such as immune modulation. In this study, we assessed efficacy of whey protein in management of patients with contact dermatitis (CD) through a double blind, randomized controlled clinical trial in Emam Reza clinic, Shiraz, Iran. Twenty-five patients in each group were randomly assigned to receive whey protein or placebo powder, 30 g in 200 mL warm water at fasting time for 4 weeks. Outcome measures were Dermatology Life Quality Index (DLQI) questionnaire and the Eczema Area and Severity Index (EASI) score. Furthermore, subjective total improvement (assessed by visual analogue scale) was set as the other primary outcome measure. A significant decrease was observed in the scores of EASI, DLQI, and subjective total improvement in the drug group after the intervention compared with the baseline. However, no changes were seen in the placebo group. Moreover, there was a significant reduction in the scores of EASI (P = .002) and subjective total improvement scores (P = .039) over the study period in the drug group compared with the placebo group. According to the results of this study, it seems that oral supplementation of whey protein could improve the symptoms of CD compared with placebo.

Background: Common variable immunodeficiency (CVID) is the most frequent primary immunodeficiency disorder mainly characterized by recurrent bacterial infections besides other immunological defects including loss of or dysfunction of B cells and decreased immunoglobulin levels. In this study, our aim is to evaluate clinical, immunological, and molecular data of patients with a primary clinical diagnosis of CVID and autoimmune phenotype with a confirmed genetic diagnosis. Methods: Among 297 patients with CVID, who were registered in the Iranian Primary Immunodeficiency Registry at Children’s Medical Center Hospital in Iran, 83 patients have been genetically examined and 27 patients with autoimmunity and confirmed genetic mutations were selected for analysis. Whole-exome sequencing and confirmatory Sanger sequencing methods were used for the study population. A questionnaire was retrospectively filled for all patients to evaluate demographic, laboratory, clinical, and genetic data. Results: In the 27 studied patients, 11 different genetic defects were identified, and the most common mutated gene was LRBA, reported in 17 (63.0%) patients. Two patients (7.7%) showed autoimmune complications as the first presentation of immunodeficiency. Eleven patients (40.7%) developed one type of autoimmunity, and 16 patients (59.3%) progressed to poly-autoimmunity. Most of the patients with mono-autoimmunity (n = 9, 90.0%) primarily developed infectious complications, while in patients with poly-autoimmunity, the most common first presentation was enteropathy (n = 6, 37.6%). In 13 patients (61.9%), the diagnosis of autoimmune disorders preceded the diagnosis of primary immunodeficiency. The most frequent autoimmune manifestations were hematologic (40.7%), gastrointestinal (48.1%), rheumatologic (25.9%), and dermatologic (22.2%) disorders. Patients with poly-autoimmunity had lower regulatory T cells than patients with mono-autoimmunity. Conclusion: In our cohort, the diagnosis of autoimmune disorders preceded the diagnosis of primary immunodeficiency in most patients. This association highlights the fact that patients referring with autoimmune manifestations should be evaluated for humoral immunity.

Allergic bronchopulmonary aspergillosis (ABPA) is the most common immunologic reaction following fungal allergen exposure in asthmatic patients. A less frequent syndrome in response to other fungal species like candida is allergic bronchopulmonary mycosis (ABPM). This reaction is mostly associated with asthma exacerbation, changes in Immunoglobulin E levels, and nonspecific findings in high resolution computed tomography (HRCT). This study presents a 9-year-old girl, a known case of childhood asthma, resolved 4 years ago as a novel case of ABPM due to Candida albicans manifested by severe emphysema, bronchiectasis, and pneumothorax which consequently required long-term treatment to get relieved.

Background: Aspergillus sensitization (AS) and allergic bronchopulmonary aspergillosis (ABPA) can occur as a cause of permanent lung damage in patients with cystic fibrosis (CF) and non-CF bronchiectasis. Objective: The aim of this study was to determine the frequency of AS and ABPA in patients with CF and non- CF bronchiectasis in southwestern Iran. Methods: This cross-sectional study was conducted on 33 patients with CF and 27 patients with non-CF bronchiectasis from southwestern Iran who were referred to Namazi Hospital affiliated to Shiraz University of Medical Sciences from July 2015 to February 2016. Skin prick test to Aspergillus fumigatus, peripheral blood eosinophil count, total serum IgE, specific IgE and IgG against Aspergillus fumigatus as well as radiologic chest studies were done for each patient. Statistical analysis was done by Mann-Whitney U test, Fisher Exact test, and Kappa weighted in SPSS software version 18. Level of significance was set at p

BACKGROUND/AIMS Celiac disease (CD) is a common enteropathy triggered by the ingestion of gluten in genetically susceptible individuals. It is frequently found in conjunction with other autoimmune diseases. The purpose of this study was to investigate the prevalence of CD in patients with juvenile idiopathic arthritis (JIA) and those with rheumatoid arthritis (RA) in southwestern Iran. MATERIALS AND METHODS A total of 53 children with JIA and 55 adults with RA were enrolled. Anti-tissue transglutaminase (tTG) immunoglobulin (Ig) A serum levels were measured by performing an enzyme-linked immunosorbent assay. Patients with anti-tTG IgA serum levels of >15 U/mL were considered seropositive and subjected to upper gastrointestinal endoscopy. Duodenal biopsies were histopathologically evaluated based on the modified Marsh classification. RESULTS One child with JIA (1.8%) and six adults with RA (11.3%) were positive for the anti-tTG IgA antibody, but the histopathological evaluations of the duodenal biopsies in these patients revealed no evidence of CD-related enteropathy. CONCLUSION Although the investigation of anti-tTG antibodies is widely used as a noninvasive serologic test for the initial diagnosis of CD, because of the high positive predictive value, the clinical utility of this test alone for making a diagnosis is doubtful. We found no cases of CD among our patients with JIA and RA. The periodic screening of rheumatologic patients with positive anti-tTG IgA for CD can be helpful in making an early diagnosis of CD in these patients.

Atopic Dermatitis (AD) or eczema is a chronic and relapsing, itchy skin inflammatory condition in infancy and childhood. The diagnosis is based on pruritus and an eczematous dermatitis with typical presentation. A five-month-old infant who was suffering from itches and eczema since three months, was diagnosed with AD. After clinical diagnosis of AD, the condition seemed to be superimposed by a bacterial infection, an empirical treatment of antibiotic (oral cephalexin) was started. However, the patient showed no improvement. As per the hospital protocol, he received clindamycin, to which the lesions responded and totally resolved. The AD in our case was reported due to severe facial impetigo with an unusual feature that it resembled a primary immunodeficiency. However, in our case, the patient had an intact immune system and did not have any transient hypogammaglobulinemia.

©2014 Turkish League Against Rheumatism. All rights reserved. Systemic lupus erythematosus (SLE) is an autoimmune disease with multi-organ involvement with a higher prevalence among young adult women.1,2 Even though SLE is usually rare in individuals less than five years of age, its prevalence is 10 to 15% during childhood.3 Children with SLE present with constitutional symptoms such as fever, fatigue, anorexia and lymphadenopathy. The disease affects several organs; however, the most commonly involved organs are the skin, joints, kidney, lung, central nervous system, blood vessels and blood cells.4 Of note, kidney involvement is present in up to 80% of children and is more severe than that seen in adults.5,6

Lung involvement is one of known complications of thalassemia. The aim of this study was to determine predominant type of pulmonary dysfunction and its relationship to iron overload in β-thalassemia children. Fifty thalassemia major children with treatment of regular blood transfusion and desferrioxamine participated in the study. Thirty-three boys and 17 girls (median age 12.5 years) with β-thalassemia enrolled in the study. Other information including body mass index, hematocrit, and the number of years of blood transfusion were recorded. Serum ferritin level and hematocrit were 3346 ± 1667 mg/dL and 27.7 ± 2, respectively. Pulmonary function tests were performed in all subjects for detecting pulmonary dysfunction. Thirty-five patients (70%) with thalassemia had abnormal result of spirometry. Obstructive airway disease based on reduced forced expiratory volume in 1 second (FEV(1)) and FEV(1)/forced vital capacity (FVC) ratio80% was detected in 4 patients (8%). Six patients (12%) showed restrictive pattern, as defined by a reduction FVC80% and FEV(1)/FVC ratio ≥80%. In this study, small airway involvement based on presence of forced expiratory flow (FEF(25%-75%))60%, FEV(1)/FVC ratio70%, and FVC80% was detected in 25 subjects (50%). Decreased values of peak expiratory flow rate (PEF) were detected in 23 (46%) and low FEV(1) in 10 (20%) subjects. There was no significant correlation between abnormal pulmonary function test and serum ferritin level in children with thalassemia. This study showed small airway disease was predominant abnormality in thalassemia patients, although additional larger studies are needed to evaluate underlying mechanisms and validate these findings.

Asthma is the most common chronic respiratory disease in childhood. The clinical presentation of asthma may worsen after food allergen ingestion in sensitized patients. To avoid nonspecific dietary restrictions in children with asthma, laboratory-based advice about foods is potentially helpful. The purpose of this study was to determine food sensitization in children with asthma.Seventy-nine children with mild to moderate persistent asthma were included in this study. Commercial food allergens including cow's milk, egg white, almond, potato, and soybean were used in skin prick tests. Specific IgE to 20 common food allergens was also measured in serum.Twelve (15.2%) of the patients had a positive skin prick test to at least one of the five food extracts. Sensitization was detected by skin prick tests to cow's milk and egg white (each 6.3%), almond (3.8%), potato (2.5%) and soybean (1.3%). Specific IgE levels ≥0.35 kAU/L were detected in the serum of 47% of the children with asthma. The most common food allergens were cow's milk (26.6%), hazelnuts (25.3%), wheat flour (15.2%) and egg white (12.6%). Patients with a history of at least one hospital admission due to asthma attack had a higher rate of sensitization to egg.In our study, food sensitization was frequent in Iranian children with asthma. Although clinical food allergy could not be evaluated because food challenge tests were not used in our study, skin prick tests and serum-specific IgE to common food allergens might be helpful in identifying children with food sensitization.

Myocarditis is a well-recognized component of Kawasaki disease, with left ventricular dysfunction occurring in more than half of patients during the acute phase. The purpose of this study was to evaluate myocardial function in patients with Kawasaki disease using pulsed tissue Doppler imaging (TDI). Twenty-five patients with the diagnosis of acute Kawasaki disease were enrolled in the study. All patients underwent echocardiographic studies at the time of diagnosis of the disease, in its acute phase, prior to treatment, and then 4 weeks later. For an aged-matched control group with fever and no cardiac disease, the same echocardiographic evaluations were performed. Peak velocities of systolic (Sa), early diastolic (Ea), and late diastolic (Aa) motion of the annulus were obtained at the lateral and septal sides in apical four-chamber view, and TDI-derived myocardial performance index (TDI-MPI) was also calculated. Peak Ea velocity of lateral mitral annulus was decreased significantly during the acute phase of illness (14 +/- 4.40 vs. 17.67 +/- 4.41; P = 0.028). In seven patients with carditis, changes in Ea-to-Aa ratio of septum (1.28 +/- 0.278 vs. 1.78 +/- 0.49; P = 0.018) and lateral mitral annulus (1.23 +/- 0.496 vs. 2.11 +/- 0.822; P = 0.014) were statistically significant but TDI-MPI showed no statistically significant changes. This study showed that peak mitral annular Ea velocities obtained by TDI were significantly altered in the acute phase of Kawasaki disease. TDI- MPI does not add an incremental benefit to other indexes of myocardial performance for comprehensive myocardial function in the acute phase of Kawasaki disease.