Evaluation of Expression of LRBA and CTLA-4 Proteins in Common Variable Immunodeficiency Patients.

Common variable immunodeficiency (CVID) is a primary immunodeficiency disease with a heterogeneous genetic background. Lipopolysaccharide-responsive beige-like anchor (LRBA), as well as cytotoxic T-lymphocyte-associated protein 4 (CTLA-4), have important regulatory roles in the immune responses. Here, we have investigated the expression of LRBA and CTLA-4 proteins in CVID patients with at least one presentation of early-onset occurrence, autoimmunity, or enteropathy.

Anti-Inflammatory Effect of KW-2449 on Autoimmune Encephalomyelitis: An Experimental Study on Mice.

Background: The KW-2449 is a novel multikinase inhibitor that inhibits FLT3, ABL, ABL-T315I, and Aurora A. FLT3 and Aurora A kinases play an important role in the pathogenesis of multiple sclerosis (MS). KW-2449 could modulate immune cells, but the immunomodulatory effects of KW-2449 on experimental autoimmune encephalomyelitis (EAE) have not been investigated yet.

Biosimilarity Assessment of 2 Filgrastim Therapeutics in Healthy Volunteers.

This study aimed to compare the pharmacokinetic, pharmacodynamic, and safety profiles of a proposed biosimilar and innovator filgrastim therapeutics in healthy volunteers. In a crossover design, 23 subjects received a single subcutaneous injection of 300-µg filgrastim, followed by a 7-day washout period. Assessed pharmacokinetic parameters were the maximum observed filgrastim serum concentration (C ), time to reach C (t ), the area under the concentration-time curve (AUC), and elimination half-life.

Evaluation of Radiation Sensitivity in Patients with Hyper IgM Syndrome.

Background: HIGM syndrome is a rare form of primary immunodeficiencies characterized by normal/increased amounts of serum IgM and decreased serum levels of other switched immunoglobulin classes. Since the affected patients are continuously infected with various types of pathogens and are susceptible for cancers, diagnostic and therapeutic tests including imaging techniques are recommended for the diagnosis and treatment of these patients, which predispose them to higher accumulated doses of radiation. Given the evidence of class switching recombination machinery defect and its association with an increased rate of DNA repair, we aimed to evaluate radiation sensitivity among a group of patients diagnosed with HIGM syndrome.

Triceps-sparing Posterior Approach for Supracondylar Humeral Fracture in Children.

Background: The most common pediatric elbow fracture is supracondylar humeral fracture which accounts for 60% of elbow fractures in children. The aim of this study was to evaluate the results of open reduction and internal fixation of type III supracondylar humeral fractures using a Triceps sparing posterior approach.

Methods: In total, 98 patients were evaluated from June 2007 to 2014.

Modified Tension Band Wiring in Adult Distal Humeral Fracture Types A2 and C1.

Background: Distal humeral fractures accounts for approximately 2% of all fractures and nearly one-third of humeral fractures in adults. In this regard, Modified Tension Bind Wiring (MTBW) technique was used for the fixation of the distal humeral fractures type A2 and C1 (AO) to evaluate the early movement and complications of the patients.

Methods: This study was conducted on 25 patients, who were subjected to open reduction and internal fixation using MTBW techniques, to evaluate the incidence of complications.

Correlation Between HMGB1 and TLR4 Expression in Sinonasal Mucosa in Patients With Chronic Rhinosinusitis.

Objectives: Chronic rhinosinusitis (CRS) is one of the most common inflammations in the upper airway. Despite the wide prevalence of CRS, the pathogenesis of this disease is poorly understood. Several components of the innate immune system may play a significant role in CRS, including Toll-like receptor 4 (TLR4), TLR9, and high-mobility group box 1 protein (HMGB1).

The Heterogeneous Pathogenesis of Selective Immunoglobulin A Deficiency.

Selective immunoglobulin A deficiency (SIgAD) is the most prevalent type of primary immunodeficiency disorder. The phenotypic feature of SIgAD is related to a defect in B lymphocyte differentiation into plasma cell-producing immunoglobulin A (IgA). In this review, we summarize the recent advances in this regard.

Comparison of clinical and immunological features and mortality in common variable immunodeficiency and agammaglobulinemia patients.

Common Variable Immunodeficiency (CVID) and agammaglobulinemia are two of the main types of symptomatic primary antibody deficiencies. The pathogenic origins of these two diseases are different; agammaglobulinemia is a group of inherited disorders that usually are caused by mutations in the gene encoding Bruton Tyrosine Kinase (BTK) protein while CVID is a heterogeneous disorder mainly without monogenic cause. However, both diseases share a characteristic of frequent bacterial infections, a decline in serum immunoglobulin levels, and abnormality in antibody responses.

The profile of IL-4, IL-5, IL-10 and GATA3 in patients with LRBA deficiency and CVID with no known monogenic disease: Association with disease severity.

Background: Common variable immunodeficiency (CVID) is the most common symptomatic form of primary immunodeficiency (PID). LPS-responsive beige-like anchor protein (LRBA) deficiency is an autosomal recessive disease characterized by a CVID-like phenotype. T cell abnormality was reported in patients with CVID and LRBA deficiency.

Ethnicity Influences Corpus Callosum Dimensions.

Background And Objective: Corpus callosum (CC), the main white matter cable which connects two hemispheres of brain, is important in special procedures such as stereotaxic surgeries vary in size, in different populations. Determination of possible size differences in ethnical groups has special values.

Patients And Methods: The size of the CC on midsagittal view was determined in 76 normal male subjects using MRI of brain hemispheres in northern Iran.

Building cancer registries in a lower resource setting: The 10-year experience of Golestan, Northern Iran.

Introduction: The Golestan population-based cancer registry (GPCR) was established in Golestan province, Northern Iran, within the Asian belt with predominance of upper-gastrointestinal cancers. We aimed to present the experiences of the registry in a resource-limited setting over the 10 years since its inception (2004-2013).

Methods: The GPCR was established as a research project to enable sustainable funding.

gene amplification in gastric adenocarcinoma and its relationship with clinical and pathological findings.

Background: Amplification of the oncogene influences the progression of gastric cancer, its prognosis, and therapy. A precise examination of -amplified tumor tissue is essential for managing disease and prescribing the appropriate treatment. This study aimed to investigate the status of gene in the gastric cancer samples and its relationship with clinical and pathological information.

Comparison of the Effect of Dexamethasone and Tranexamic Acid, Separately or in Combination on Post-Rhinoplasty Edema and Ecchymosis.

Background: Dexamethasone and tranexamic acid are used to decrease post-rhinoplasty periorbital edema and ecchymosis. We compared the impact of each medication separately or in combination in this regard.

Methods: A prospective, randomized triple-blinded study was undertaken on 60 patients who underwent primary open rhinoplasty.

The effects of probiotic supplement on hemoglobin in chronic renal failure patients under hemodialysis: A randomized clinical trial.

Background: Chronic inflammation is one of the causes of anemia in chronic renal failure patients under hemodialysis. Probiotics probably establish a balance between pro- and anti-inflammatory cytokines. The study was conducted to determine the effects of probiotic supplementation on hemoglobin (Hb) in hemodialysis patients.

Neglected foreign body aspiration mimicking bronchial carcinoma.

Foreign body aspiration can occur in any age group, but it is more commonly seen in children. In adults, there is usually a predisposing condition that poses a risk of aspiration. If aspiration occurs, prompt diagnosis and extraction of the foreign body is needed to prevent early and late complications.

Massive malignant pleural effusion due to lung adenocarcinoma in 13-year-old boy.

A 13-year-old boy with no risk factors for lung cancer presented with a massive left-sided pleural effusion and a mediastinal shift on chest radiography and computed tomography. A chest tube drained bloody pleural fluid with an exudative pattern. A pleural biopsy and wedge biopsy of the left lower lobe revealed mucinous adenocarcinoma in the left lower lobe wedge biopsy and metastatic adenocarcinoma in the pleural biopsy.

Ellis van Creveld Syndrome (EVCS) in an Iranian, Three-Year-old Girl: A Case Report.

Ellis van Creveld syndrome (EVCS) is mainly characterized by dysplatic changes in skeletal structures along with variable inter- and intra-familial patterns. Shortening of bony structures such as ribs and limbs, postaxial polydactyly, and dysplastic teeth and nails are among the clinical presentation of this autosomal recessive disease.

Relationship between Serum Level of Interleukin-2 in Patients with Systemic Lupus Erythematosus and Disease Activity in Comparison with Control Group.

Background: Despite the large number of surveys, there are not any validated biomarkers for SLE disease activity till now. This study aimed to evaluate the relationship between serum level of IL-2 in patients with SLE and disease activity in comparison with control group.

Materials And Methods: In this case-control study, 73 patients with lupus and 73 healthy subjects referred to the rheumatology clinic of 5 Azar Hospital in Gorgan (North of Iran).

Measurement of the corpus callosum using magnetic resonance imaging in the north of iran.

Background: Morphometric measurements of the corpus callosum (CC) are important to have normative values according to sex, age and race/ethnicity.

Objectives: This study was done to measure the size of CC and to identify its gender- and age-related differences in the North of Iran.

Patients And Methods: The size of CC on midsagittal section was measured in 100 (45 males, 55 females) normal subjects using magnetic resonance imaging (MRI) admitted to the Kowsar MRI center in Gorgan-Northern Iran.