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Abstract Background: Allergic rhinitis is a common disease which is present in 20% of general population. Major symptoms include sneezing, rhinorrhea, nasal congestion, and nasal pruritus. It seems that tricyclic antidepressants blocking histamine receptors may be used as an effective treatment in allergic rhinitis. Materials and Methods: In this clinical trial study, 84 patients with allergic rhinitis entered the study and randomly divided into two groups. Patients of 1st group received cetirizine and 2nd group received doxepin for 2 weeks. After 2 weeks patients were evaluated about sneezing, rhinorrhea, nasal congestion, and nasal pruritus. Results: The results of our study showed that clinical score of patients after 2 weeks was not different in both groups (p=0.261). The only symptom that was affected by the kind of treatment and was significantly different between groups was sneezing (p=0.005). Conclusion: It seems that there is not much difference in using cetirizine and doxepin in treatment of seasonal allergic rhinitis sympotoms. It is recommended that future studies use TCAs with more potent blocking anti-histamine receptors feature and on more samples.

"n 800x600 Normal 0 false false false EN-US X-NONE AR-SA MicrosoftInternetExplorer4 /* Style Definitions */ table.MsoNormalTable {mso-style-name:"Table Normal"; mso-tstyle-rowband-size:0; mso-tstyle-colband-size:0; mso-style-noshow:yes; mso-style-priority:99; mso-style-parent:""; mso-padding-alt:0in 5.4pt 0in 5.4pt; mso-para-margin:0in; mso-para-margin-bottom:.0001pt; mso-pagination:widow-orphan; font-size:10.0pt; font-family:"Times New Roman","serif";} Background: Common Variable Immunodeficiency (CVID) is a primary immunodeficiency disease, characterized by hypogammaglobulinemia and heterogeneous clinical manifestations. This study was performed to evaluate the clinical and immunological features of pediatric patients with CVID. "n"nMethods: We reviewed the records of 69 children diagnosed under age of 16 years with CVID (35 males and 34 females)."n"nResults: By the year 2008, 15 patients (21%) had died. The total follow-up period was 333 patient-years. The mean diagnostic time between onset and diagnosis in our patient group was 4.40 years. The overall rate of consanguineous marriages was 58%. 10 patients had a positive family history of immunodeficiency. At the time of diagnosis, the mean levels of serum immunoglobulin G (IgG), IgM, and IgA levels were 286.86, 39.92, and 18.39 mg/dl, respectively which were below the normal levels for age. All of the patients presented with infectious diseases at the time of onset, the most common of which were pneumonia, diarrhea and sinusitis. Acute and recurrent infections were also found in almost all of the patients, particularly involving respiratory and gastrointestinal systems. The most common infections during follow-up period were pneumonia (31.9%), acute diarrhea (18.8%), acute sinusitis (18.8%), and otitis media (14.5%). Post-diagnosis survival was estimated to be 79% during the first five years. The survival rate was not shown to be influenced by delayed diagnosis, serum levels of IgG and B-lymphocyte count at the time of diagnosis."n"nConclusions: Any child with a history of recurrent infections, decreased levels of serum immunoglobulin isotypes and consanguineous parents should be considered as a CVID patient.

Introduction: There has been substantial increase in food allergies in recent decades. The management of severe food allergy often includes strict avoidance and medical therapies. However, oral immunotherapy (OIT) is a promising treatment option for these patients, which is still being investigated., Methods: The study recruited children from 2 years onward with a history of wheat anaphylaxis who had been referred to the Mofid Children Hospital. Wheat allergy was confirmed by a double-blind placebo-controlled food challenge. OIT was started to reach 5.28 g of wheat protein supplied in 60 g of bread. Besides immunologic measurements, a second and third oral food challenge (OFC) was performed after 3 months and 1 year of maintenance therapy to evaluate the long-term efficacy of wheat OIT (WOIT)., Results: Seventeen patients completed the 3-month maintenance phase; 8 of them demonstrated negative OFCs. All of the 9 with positive OFCs were asked to continue the daily consumption of 60 g of bread for another year. Three patients with positive OFCs were followed for 1 more year and were asked to continue eating 60 g of bread every other day. The serum level of wheat sIgE was significantly increased at the end of the buildup phase (p = 0.026) and dramatically dropped at the end of the maintenance phase (p = 0.022)., Conclusion: To conclude, WOIT is an effective and safe modality of treatment if it is administered under strict supervision., (© 2021 S. Karger AG, Basel.)

Background: Predominantly antibody deficiencies (PADs) are the most common primary immunodeficiencies, characterized by hypogammaglobulinemia and inability to generate effective antibody responses., Objective: We intended to report most common monogenic PADs and to investigate how patients with PAD who were primarily diagnosed as suffering from agammaglobulinemia, hyper-IgM (HIgM) syndrome, and common variable immunodeficiency (CVID) have different clinical and immunological findings., Methods: Stepwise next-generation sequencing and Sanger sequencing were performed for confirmation of the mutations in the patients clinically diagnosed as suffering from agammaglobulinemia, HIgM syndrome, and CVID., Results: Among 550 registered patients, the predominant genetic defects associated with agammaglobulinemia (48 Bruton's tyrosine kinase [BTK] and 6 μ heavy chain deficiencies), HIgM syndrome (21 CD40 ligand and 7 activation-induced cytidine deaminase deficiencies), and CVID (17 lipopolysaccharides-responsive beige-like anchor deficiency and 12 atypical Immunodeficiency, Centromeric instability, and Facial dysmorphism syndromes) were identified. Clinical disease severity was significantly higher in patients with μ heavy chain and CD40 ligand mutations compared with patients with BTK (P = .003) and activation-induced cytidine deaminase (P = .009) mutations. Paralysis following live polio vaccination was considerably higher in patients with μ heavy chain deficiency compared with BTK deficiency (P < .001). We found a genotype-phenotype correlation among patients with BTK mutations regarding clinical manifestation of meningitis and chronic diarrhea. Surprisingly, we noticed that first presentations in most patients with Immunodeficiency, Centromeric instability, and Facial dysmorphism were respiratory complications (P = .008), whereas first presentations in patients with lipopolysaccharides-responsive beige-like anchor deficiency were nonrespiratory complications (P = .008)., Conclusions: This study highlights similarities and differences in the clinical and genetic spectrum of the most common PAD-associated gene defects. This comprehensive comparison will facilitate clinical decision making, and improve prognosis and targeted treatment., (Copyright © 2018 American Academy of Allergy, Asthma & Immunology. Published by Elsevier Inc. All rights reserved.)

Background: The number of inherited diseases and the spectrum of clinical manifestations of primary immunodeficiency disorders (PIDs) are ever-expanding. Molecular diagnosis using genomic approaches should be performed for all PID patients since it provides a resource to improve the management and to estimate the prognosis of patients with these rare immune disorders., Method: The current update of Iranian PID registry (IPIDR) contains the clinical phenotype of newly registered patients during last 5 years (2013-2018) and the result of molecular diagnosis in patients enrolled for targeted and next-generation sequencing., Results: Considering the newly diagnosed patients (n = 1395), the total number of registered PID patients reached 3056 (1852 male and 1204 female) from 31 medical centers. The predominantly antibody deficiency was the most common subcategory of PID (29.5%). The putative causative genetic defect was identified in 1014 patients (33.1%) and an autosomal recessive pattern was found in 79.3% of these patients. Among the genetically different categories of PID patients, the diagnostic rate was highest in defects in immune dysregulation and lowest in predominantly antibody deficiencies and mutations in the MEFV gene were the most frequent genetic disorder in our cohort., Conclusions: During a 20-year registration of Iranian PID patients, significant changes have been observed by increasing the awareness of the medical community, national PID network establishment, improving therapeutic facilities, and recently by inclusion of the molecular diagnosis. The current collective study of PID phenotypes and genotypes provides a major source for ethnic surveillance, newborn screening, and genetic consultation for prenatal and preimplantation genetic diagnosis.

Background: Combined immunodeficiencies (CIDs) are diseases of defective adaptive immunity with diverse clinical phenotypes. Although CIDs are more prevalent in the Middle East than Western countries, the resources for genetic diagnosis are limited., Objectives: This study aims to characterize the categories of patients with CIDs in Iran clinically and genetically., Methods: Clinical and laboratory data were obtained from 696 patients with CIDs. Patients were subdivided into those with syndromic (344 patients) and nonsyndromic (352 patients) CIDs. Targeted DNA sequencing was performed on 243 (34.9%) patients., Results: The overall diagnostic yield of the 243 sequenced patients was 77.8% (189 patients). The clinical diagnosis of hyper-IgE syndrome (P < .001), onset of disease at greater than 5 years (P = .02), and absence of multiple affected family members (P = .04) were significantly more frequent in the patients without a genetic diagnosis. An autosomal recessive disease was found in 62.9% of patients, reflecting the high rate of consanguinity in this cohort. Mutations impairing VDJ recombination and DNA repair were the most common underlying causes of CIDs. However, in patients with syndromic CIDs, autosomal recessive mutations in ataxia-telangiectasia mutated (ATM), autosomal dominant mutations in signal transducer and activator of transcription 3 (STAT3), and microdeletions in 22q11.21 were the most commonly affected genomic loci. Patients with syndromic CIDs had a significantly lower 5-year survival rate rather than those with nonsyndromic CIDs., Conclusions: This study provides proof of principle for the application of targeted next-generation sequencing panels in countries with limited diagnostic resources. The effect of genetic diagnosis on clinical care requires continued improvements in therapeutic resources for these patients., (Copyright © 2017 American Academy of Allergy, Asthma & Immunology. All rights reserved.)

Background: Asthma is the first cause of children hospitalization and need for emergency and impose high economic burden on the families and governments. We aimed to investigate the economic burden of pediatric asthma and its contribution to family health budget in Iran., Methods: Overall, 283 pediatric asthmatic patients, who referred to two tertiary pediatric referral centers in Tehran capital of Iran, included from 2010-2012. Direct and indirect asthma-related costs were recorded during one-year period. Data were statistically analyzed for finding association between the costs and factors that affect this cost (demographic variables, tobacco smoke exposure, control status of asthma and asthma concomitant diseases)., Results: Ninety-two (32.5%) females and 191(67.5%) males with the age range of 1-16 yr old were included. We found the annual total pediatrics asthma related costs were 367.97±23.06 USD. The highest cost belonged to the medications (69%) and the lowest one to the emergency (2%). We noticed a significant increasing in boys' total costs ( P =0.011), and 7-11 yr old age group ( P =0.018). In addition, we found significant association between total asthma costs and asthma control status ( P =0.011)., Conclusion: The presence of an asthmatic child can consume nearly half of the health budget of a family. Our results emphasis on improving asthma management programs, which leads to successful control status of the disease and reduction in economic burden of pediatric asthma., Competing Interests: Conflict of Interests The authors declare that there is no conflict of interests.

Zinc deficiency, which is common in patients with cystic fibrosis (CF), can lead to several complications that may increase the number of hospital admissions in this group of patients. As supplementary zinc can prevent such complications, this study was performed to evaluate the effect of supplementary zinc on body mass index (BMI), forced expiratory volume in one second (FEV1) and number of hospitalizations in CF patients. In this study, 30 children with CF, who were referred to the Digestive Diseases Clinic of the Children's Medical Center in Tehran, were enrolled. Supplementary zinc of 2 mg/kg per day was administered to all patients. Serum level of zinc, alkaline phosphatase, and albumin as well as BMI, FEV1, and number of hospitalizations were compared before and after zinc administration. Height (p<0.001), weight (p<0.001) and BMI (p=0.001) were significantly increased after zinc, while the number of hospitalizations was significantly decreased (p=0.023). In contrast to patients with normal pulmonary function tests who received supplement therapy, BMI was not increased in those with abnormal pulmonary function after supplementary zinc. Supplementary zinc can increase BMI in CF patients, mostly in those with normal pulmonary function. While supplementary zinc may decrease the number of hospitalizations, other factors can also influence the hospitalization number.

Background/aims: Inflammatory bowel disease (IBD) is a chronic disease of the gastrointestinal tract, whose etiologies are still unknown. This study was performed to evaluate the humoral immune response in terms of B cell functions in selected IBD patients., Methods: Eighteen pediatric patients with IBD, including 12 cases of ulcerative colitis (UC) and six with Crohn disease (CD), were enrolled in this study. The pneumococcal vaccine was injected in all patients, and the IgG antibody level to the polysaccharide antigen was measured before and 4 weeks after injection. The B cell switch-recombination process was evaluated., Results: Five patients with IBD (three CD and two UC) had defects in B cell switching, which was significantly higher than in controls (p=0.05). Ten patients had a specific antibody deficiency and exhibited a higher frequency of bacterial infection than the healthy group. The mean increased level of IgG after vaccination was lower in IBD patients (82.9±32.5 µg/mL vs 219.8±59.0 µg/mL; p=0.001). Among the patients who had an insufficient response, no significant difference in the number of switched memory B-cell was observed., Conclusions: A defect in B lymphocyte switching was observed in pediatric IBD patients, and especially in those patients with CD. Owing to an increased risk of bacterial infections in those patients with antibody production defects, pneumococcal vaccination could be recommended. However, not all patients can benefit from the vaccination, and several may require other prophylactic methods.

Background: Selective IgA deficiency (SIgAD) is the most common primary immunodeficiency disorder, which is characterized by significantly decreased serum levels of IgA. Abnormalities of CD4+CD25(high)forkhead box P3 (FoxP3)+ regulatory T cells (T(reg)) have been shown in association with autoimmune and inflammatory disorders., Methods: In order to evaluate the relationship between autoimmunity and T(reg) in SIgAD, we studied 26 IgA-deficient patients (aged 4-17 years) with serum IgA levels <7 mg/dl, 26 age- and sex-matched healthy controls and 26 age- and sex matched idiopathic thrombocytopenic purpura cases with normal immune system. T(reg) were determined by flow cytometry using T(reg) markers, including CD4, CD25 and FoxP3., Results: The mean percentage of CD4, CD25+FoxP3+ T(reg) from all CD4+ cells was 4.08 ± 0.86 in healthy controls, which was significantly higher than in SIgAD patients (2.93 ± 1.3; p = 0.003). We set a cutoff point (2.36%) for T(reg), which was two standard deviations lower than the mean of normal controls. According to this cutoff point and in order to assess the role of T(reg) in clinical SIgAD manifestation, we classified patients into two groups: 16 patients in G1 with T(reg) <2.36% and 10 patients in G2 with T(reg) >2.36%. Autoimmunity was recorded in 9 patients (53.3%) of G1 and only 1 patient of G2, respectively (p = 0.034). Although a defect in class switching recombination was observed in 40% of the patients in G1, none of the G2 patients had such a defect (p = 0.028)., Conclusion: This study showed decreased proportions of T(reg) in SIgAD patients, particularly in those with signs of chronic inflammation., (Copyright © 2012 S. Karger AG, Basel.)

Considering the 50% mortality rate of neonatal septicemia associated with neutropenia and increasing resistance to antibiotics, simultaneous antibiotic therapy strategies are becoming more important. However, few studies have been performed to evaluate effectiveness of RhG-CSF in the treatment of neutropenia in neonates. This randomized clinical trial was performed on 40 neutropenic neonates with septicemia who were hospitalized in Vali-e-Asr and Mirza Koochak Khan Hospitals (Tehran, Iran). The neonates were randomly divided into two equal groups RhG-CSF was administered as a subcutaneous single dose of 10 μg/kg/s.c. to neonates in group A and as 10 μg/kg/s.c./day once daily for 3 days to neonates in group B. CBC and differential count was checked 6, 24 and 48 hours after the last dose. There was no significant difference in mean birth weight, gender, age, and risk factors between two groups. Neutropenia was improved 48 hours after the last dose, whilst there was no significant statistical difference between two groups (P>0.05). The final outcome including death, duration of hospitalization and duration of antibiotics therapy after RhG-CSF administration did not differ between two groups (P>0.05). The results of this study showed that administration of a single dose of RhG-CSF (10 μg/kg) was effective in treating neonatal septicemic neutropenia.