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Objective: Physicians’ awareness about pediatric health problems is very important in health system. This has not been investigated in Iran as yet. Therefore this study was conducted to characterize the knowledge of the Iranian physicians which has direct association with health status of children. Methods: One hundred and four physicians, mainly pediatric specialists (58.6%) working in the state hospitals (45.1%) were enrolled. They filled a valid and reliable questionnaire, containing 26 questions about basic and important pediatric issues before and after an educational pediatric program (EPP). Findings: Thirty nine (37.5%) physicians answered correctly more than 2/3 of all questions (passed the examination) before EPP, which increased to 42.3% after EEP. Subgroup analysis showed that the total scores of general practitioners (P=0.007) was significantly increased after the EPP. Moreover, physicians with shorter practicing time (P=0.006) and those with shorter time past graduation (P=0.01) had a significant improvement in their total scores after the program. The best scores of educational issues were documented in growth and development (16.0%; P=0.04), followed by dermatology (9.2%, P=0.04), urology (9.1%; P=0.04), and asthma and allergy (9.0%, P=0.04). Conclusion: This study revealed that there are gaps in the knowledge of professionals about the pediatric issues. [ABSTRACT FROM AUTHOR]

Objective: Common variable immunodeficiency (CVID) is a heterogeneous disorder characterized by reduced serum level of IgG, IgA or IgM and recurrent bacterial infections. Class switch recombination (CSR) as a critical process in immunoglobulin production is defective in a group of CVID patients. Activation-induced cytidine deaminase (AID) protein is an important molecule involving CSR process. The aim of this study was to investigate the AID gene mRNA production in a group of CVID patients indicating possible role of this molecule in this disorder. Methods: Peripheral blood mononuclear cells (PBMC) of 29 CVID patients and 21 healthy controls were isolated and stimulated by CD40L and IL-4 to induce AID gene expression. After 5 days AID gene mRNA production was investigated by real time polymerase chain reaction. Findings: AID gene was expressed in all of the studied patients. However the mean density of extracted AID mRNA showed higher level in CVID patients (230.95±103.04 ng/ml) rather than controls (210.00±44.72 ng/ml; P=0.5). CVID cases with lower level of AID had decreased total level of IgE (P=0.04) and stimulated IgE production (P=0.02); while cases with increased level of AID presented higher level of IgA (P=0.04) and numbers of B cells (P=0.02) and autoimmune disease (P=0.02). Conclusion: Different levels of AID gene expression may have important roles in dysregulation of immune system and final clinical presentation in CVID patients. Therefore investigating the expression of AID gene can help in classifying CVID patients. [ABSTRACT FROM AUTHOR]

Objective: There are some controversial studies on effects of anti-epileptic drugs (AEDs) on serum IgG subclasses; however, the role of these medications is still unclear. The aim of this study was evaluation the effects of anti-epileptic drugs on serum concentration of IgG and its subclasses. Methods: Serum IgG and IgG subclasses of 61 newly diagnosed epileptic patients were measured at the beginning of monotherapy with carbamazepine, sodium valproate, and Phenobarbital, and 6 months later. Measurement of IgG and its subclasses was performed using nephlometry and ELISA techniques, respectively. Findings: Reduction of at least one IgG subclass was found in 6 patients 6 months after treatment with AEDs. Among 27 patients receiving carbamazepine, decrease in at least one serum IgG subclass level was found in 5 patients. Among 20 patients using sodium valproate, only one patient showed decrease in IgG2 subclass. None of the 14 patients using phenobarbital revealed significant decrease in IgG subclasses. No infection was seen in the patients with reduction of subclasses. Conclusion: Although in our study, children with selective IgG subclass deficiency were asymptomatic, assessment of serum immunoglobulin levels could be recommended at starting the administration of AEDs and in serial intervals afterward in epileptic patients. [ABSTRACT FROM AUTHOR]

Pediatric immunology came into sight in the second half of 20th century, when pediatricians and basic immunologists began to give attention to diagnosis and treatment of children with primary immunodeficiency diseases (PIDs). Understanding the genetic and mechanistic basis of PIDs provides unique insight into the functioning of the immune system. By progress in basic and clinical immunology, many infrastructural organizations and academic centers have been established in many countries worldwide to focus on training and research on the immune system and related disorders. Along with progress in basic and clinical immunology in the world, pediatric immunology had a good progress in Iran during the last 33-year period. Now, patients with PIDs can benefit from multidisciplinary comprehensive care, which is provided by clinical immunologists in collaboration with other specialists. Patients with history of recurrent and/or chronic infections suggestive of PIDs are evaluated by standard and research-based testing and receive appropriate treatment. The progress in PIDs can be described in three periods. Development of training program for clinical fellowship in allergy and immunology, multidisciplinary and international collaborative projects, primary immunodeficiency diseases textbooks, meetings on immunodeficiency disorders, improvement in diagnosis and treatment, and construction of Iranian primary immunodeficiency association, Students' research group for immunodeficiencies, Iranian primary immunodeficiency registry, and the immunological societies and centers were the main activities on PIDs during these years. In this article, we review the growth of modern pediatric immunology and PIDs status in Iran. [ABSTRACT FROM AUTHOR]

The article presents a case study of a 7-year old boy with clinical presentation of fever and nonproductive cough and also had hepatomegaly and digital clubbing. It mentions that the patient was later on diagnosed with Lymphocytic Interstitial Pneumonitis. It describes the presentation of the rare primary immunodeficiency disease of XHIGM syndrome presented with refractory pneumonia.

Objective: Common variable immunodeficiency (CVID) is a heterogeneous group of disorders, characterized by hypogammaglobulinemia, defective specific antibody responses to pathogens and increased susceptibility to recurrent bacterial infections. Delay in diagnosis and inadequate treatment can lead to irreversible complications and mortality. In order to determine infectious complications among undiagnosed CVID patients, 47 patients diagnosed in the Children's Medical Center Hospital during a period of 25 years (1984-2009) were enrolled in this study. Methods: Patients were divided into two groups including Group 1 (G1) with long diagnostic delay of more than 6 years (24 patients) and Group 2 (G2) with early diagnosis (23 patients). The clinical manifestations were recorded in a period prior to diagnosis in G1 and duration follow up in G2. The number of infections, non infectious complications, hospitalizations, and mortality rate was compared between the two groups. Findings: The patients in G1 group had 500 episodes of infections before diagnosis in 256 patientyears (0.08 per patient per year) and 203 times of hospitalization (0.03 per patient per year), which were significantly higher than in G2 patients, who had 75 episodes of infections (0.015 per patient per year) and 88 hospital admissions (0.018 per patient per year) during 207 patient follow-up years. Frequency of enteropathies and liver diseases in G1 were also significantly higher than in G2. Lack of awareness about nature of disease, especially among rural and suburban physicians, single organ involvement as a site of clinical presenting, and predomination of non infectious presentation in G1 were the major factors of delayed diagnosis. Conclusion: Diagnostic delay is a major concern in CVID patients, which could result in irreversible complications and mortality, while early diagnosis and proper initial treatment leads to better outcomes and quality of life. [ABSTRACT FROM AUTHOR]

Objective: Juvenile idiopathic arthritis (JIA) is the most common rheumatic disease in children. The exact causes of disease are still poorly understood. It seems that B cells have several functions in JIA, including production of autoantibodies, antigen presentation, production of cytokines, and activation of T cells. Here, we aimed to evaluate B-cell lineage and its precursors in the bone marrow of patients with JIA. Methods: Twenty consecutive patients with JIA were enrolled in this study. JIA is subdivided into three groups of Pauciarticular, Polyarticular, and Systemic JIA. Bone marrow mononuclear cells were separated. Then we analyzed the immunophenotype of the JIA patients by flow cytometry. After separation, the mononuclear cells were stained specific for B cell lineage [CD10, CD19 and CD20], T cell lineage [CD3] and non specific lineage (CD34, HLA-DR and TdTJ. Findings: Flow cytometric study of bone marrow showed that JIA patients had low level of CD10, CD19, and CD20. Polyarticular patients had lower level of DiG, CD19, and CD20 than pauciarticular JIA patients and systemic onset JIA patients had lower levels than both of them. Conclusion: Decreasing of B cell precursor in bone marrow is one of mechanisms for pathogenesis of JIA and the more decreased B cell precursors in bone marrow are, the worst severity of the disease is. Significant differences in CD10 content of bone marrow were detected between the polyarticular and pauciarticular groups. So, it seems that polyarticular JIA patients had lower percentage of pre B cell stage. [ABSTRACT FROM AUTHOR]

The article presents a case study of an 11-year-old boy with hyper immunoglobulin M (IgM) syndrome. The patient was diagnosed with recurrent gallbladder hydrops and sclerosing cholangitis, which was associated with associated with Cryptosporidium infection. His clinical and paraclinical symptoms were normalized following Paramomycin and Azitromycin treatment.