Multiple Right and Left Pulmonary Arteries and Subdivisions of Inferior Mesenteric Artery Aneurysms in Behcet’s Disease Case: A Rare Clinical Presentation

Behcet’s disease is a multi-systemic inflammatory disorder with cutaneous acneiform eruptions, orogenital aphthae, uveitis, arthritis and systemic vascular inflammation. One of the rare vascular manifestations is thoraco-abdominal aortic and pulmonary aneurysm that is associated with high risk of morbidity and mortality. We report a 36-year-old man with chronic cough, hemoptysis, significant weight loss, and orogenital ulcers from one year before referral. Initial assessments revealed multiple parahillar nodules in chest X-ray, chronic inflammatory anemia, erythrocyte sedimentation rate more than 100, and positive Human Leukocyte Antigen B5 and B51. Evaluation for infection and malignancies was unremarkable. Open exploratory lung study showed multiple pulsatile nodules in both lungs. AMIGO computed tomogram confirmed multiple right and left pulmonary artery aneurysms and impending to rupture aneurysm at subdivision of inferior mesenteric artery. After beginning of three methylprednisolone and cyclophosphamide pulse doses, the clinical aspect of the patient dramatically improved. Although pulmonary aneurysm is a rare manifestation of Behcet’s disease and it is more infrequent in the distal branches, it can be seen in patients presenting with inflammatory disease and respiratory manifestations and with Behcet’s disease diagnosis. Corticosteroid pulse-therapy could be considered as the first line of medical treatment in these patient

Evaluation of effectiveness of specific subcutaneous immunotherapy for patients with allergic rhinitis and asthma

Background: Allergen immunotherapy involves the administration of gradually increasing quantities of specific allergens to patients with IgE-mediated conditions until a dose is reached that is effective in reducing disease severity from natural exposure. This study evaluated the clinical efficacy of immunotherapy with extracts of common aeroallergens North-East of Iran in asthma and allergic rhinitis. Material and Methods: In this prospective study 156 cases were chosen randomley. The mean age of patients was 37 years (range 5-65 years). The patients with mild to moderate asthma and allergic rhinitis and history of atopy were selected for immunotherapy when they showed no effective response to medical treatment.Immunotherapy materials were made from common aeroallergens in north-eastern region of Iran by Dome Hollister US company. Immunotherapy schedule for injection of the extract with vial dilution of 1:10000pg was one injection every week for ten weeks and one injection with dilution of 1:1000pg every other week for the other ten weeks and one injection monthly from dilution of 1:100pg for two years. Results: One hundred twenty (77%) of cases had allergic rhinitis 29(18.5%) cases had allergic asthma and 7(4.5%) cases were mixed. Mean age of patients were 37 years old. 48(30.8%) cases were male. Analysis of efficacy of treatment showed that immunotherapy significantlyimproved the signs and symptoms of all the groups. In allergic rhinitis group 84(70%) cases completely improved, 22(18.4%) patients moderately responded and no response to immunotherapy was observed in 14(11.6%) patients. In allergic asthma group, 22(75%) cases completely improved 4(13.6%) cases moderately responded and no response to immunotherapy was detected in 3(11.4%) cases. In mixed group, 3(42.8%) cases completely improved, 3(42.8%) cases moderately responded and no response was observed in 1(14.4%) case. Conclusion: Specific allergen immunotherapy for patients with allergic persistent mild to moderate asthma and moderate to severe allergic rhinitis without good response to medical treatment is highly recommended. It is recommended as effective treatment in such patients

Hydroxyl-Functionalized 1‑(2-Hydroxyethyl)-3-methyl Imidazolium Ionic Liquids: Thermodynamic and Structural Properties using Molecular Dynamics Simulations and ab Initio Calculations

The influences of hydroxyl functional group (−OH) on the thermodynamic and structural properties of ionic liquids (ILs) composed of 1-(2-Hydroxyethyl)-3-methyl imidazolium ([C₂OHmim]⁺) cation and the six different conventional anions, including [Cl]⁻, [NO₃]⁻, [BF₄]⁻, [PF₆]⁻, [TfO]⁻, and [Tf₂N]⁻ have been extensively investigated using classical molecular dynamics (MD) simulations combined with ab initio calculations over a wide range of temperature (298–550 K). The volumetric thermodynamic properties, enthalpy of vaporization, cohesive energy density, Hildebrand solubility parameter, and heat capacity at constant pressure were estimated at desired temperature. The simulated densities were in good agreement with the experimental data with a slight overestimation. The interionic interaction of selected ILs was also computed using both the MD simulations and ab initio calculations. It was found that the highest association of cation and anion is attributed to [C₂OHmim][Cl] followed by [C₂OHmim][NO₃], and [C₂OHmim][Tf₂N] with the bulkiest anion has the weakest interionic interaction among chosen ILs. The similar trend of interactions energies was nearly observed from cohesive energy density results. Additional structural details were comprehensively yielded by calculating radial distribution functions (RDFs) and spatial distribution function (SDFs) at 358 K. The most stable configurations of isolated and dimer ion pairs of these ILs were in excellent consistency with RDFs and SDFs results. Significant changes in arrangement of anions around the [C₂OHmim]⁺ cation in comparison with conventional imidazolium-based ILs can be inferred from the MD simulations and ab initio results. Also, microscopic structural properties disclosed that the most strong cation–cation interaction is ascribed to the hydroxyl-functionalized ILs composed of bulkier anions, whereas ILs incorporating [Cl]⁻ and [NO₃]⁻ anions are mainly involved in cation–anion interactions. The formation of the intramolecular hydrogen bonding in the [C₂OHmim]⁺ cation is another interesting result of the present study

Comparison of Common Monogenic Defects in a Large Predominantly Antibody Deficiency Cohort

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

Comparison of Common Monogenic Defects in a Large Predominantly Antibody Deficiency Cohort

Predominantly antibody deficiencies (PADs) are the most common primary immunodeficiencies, characterized by hypogammaglobulinemia and inability to generate effective antibody responses