A New Perspective on the Management of Primary Immunodeficiencies: Evaluation of Arrhythmia and Cardiac Diseases.

Introduction: Primary immunodeficiency diseases (PIDs), are a growing group of rarely seen diseases. Various clinical conditions like autoimmunity, lymphoproliferative/malignant diseases, chronic lung and gastrointestinal system diseases have been identified which accompanies PIDs besides recurrent infections. However, there is a lack of information about accompanying cardiovascular diseases.

A case of paradoxical vocal cord movement misdiagnosed as anaphylaxis.

Introduction: Anaphylaxis is a severe and life-threatening systemic hypersensitivity reaction. The most frequently encountered causes are foods, drugs, and bee venom, but anaphylaxis may also occur idiopathically. Paradoxical vocal cord movement (PVCM), is a cause of upper airway obstruction due to abnormal adduction of vocal cords during inspiration and, to some degree on expiration.

Adverse reactions in venom immunotherapy protocols: conventional versus ultra-rush.

Background: Venom immunotherapy (VIT) is an effective treatment in the patients at high risk of anaphylaxis or life-threatening systemic reactions due to Hymenoptera venom allergy. But, systemic and large local reactions can be observed, especially during the build-up phase of VIT. We evaluated the safety of conventional and ultra-rush build-up protocols.

Phenotypically defined subpopulations of circulating follicular helper T cells in common variable immunodeficiency.

Background: Common variable immunodeficiency (CVID) is characterized by low immunoglobulin G and IgA/IgM, decreased switched memory B cells, impaired response to vaccine, and an increased susceptibility to infections and autoimmunity. T cells play an important role in germinal center reaction where it supports isotype switching, somatic hypermutation, generation of memory B cells, and differentiation of B cells to plasma cells. The objective was to study the distribution of three subsets of T cells and their relationship with autoimmune diseases associated with CVID.

Haplotype analysis in a 16-year-old boy with vancomycin-induced DRESS syndrome.

Drug reaction with eosinophilia and systemic symptoms (DRESS) is a rare but life-threatening multisystem adverse reaction to a medication, with vancomycin being one of the most common cause reported. We present the HLA analysis of a pediatric patient who developed DRESS related to vancomycin and compared the results with the available literature. With further data, the use of pretreatment HLA analysis to prevent vancomycin related DRESS may be a valuable option in the near future.

Adults with common variable immunodeficiency: a single-center experience.

Background/aim: In this study, we aimed to assess the clinical and immunological findings of our patients with common variable immunodeficiency (CVID).

Materials And Methods: We analyzed the records of 31 adult patients with CVID (12 females, 19 males). The patients were classified into clinical and immunophenotypic subgroups for statistical comparisons.

Evaluation of chemerin and its receptors, ChemR23 and CCRL2, in gingival tissues with healthy and periodontitis.

Chemerin is a chemoattractant protein that directs inflammatory cells that express its receptor chemokine receptor-like 1 (ChemR23) towards sites of inflammation. C-C chemokine receptor-like 2 (CCRL2), is the other receptor of chemerin, improves the interaction between chemerin and ChemR23. The aim of this study was to evaluate the expression of chemerin and its receptors in gingival tissues with healthy and periodontitis.

Desensitization to Adalimumab: An Example of Repeated Desensitization to a Biological Agent.

Biological agents seem to have been more effective than classic immunosuppressive drugs; however, the adverse events including the hypersensitivity reactions are the main drawbacks of these drugs. We report a 35-year-old man who was treated with adalimumab for ankylosing spondylitis, had a local reaction on the injection site, and generalized itching with rash at the 62nd dose and repeated desensitizations to him with adalimumab. One month after the reaction, skin prick test was performed with a commercial preparation of adalimumab.

Increased visfatin expression is associated with nuclear factor-kappa B and phosphatidylinositol 3-kinase in periodontal inflammation.

Objective: Visfatin is an adipocytokine that plays a role in regulating periodontal inflammation by as yet identified mechanisms. It has been suggested that visfatin mediates inflammation via activation of the nuclear factor-kappa B (NF-κB) and phosphatidylinositol 3-kinase (PI3k) signaling pathways which play a role in the inhibition of neutrophil apoptosis. The aim of this study was to investigate the expression of visfatin, NF-κB (NF-κB and NF-κB), PI3k, tumor necrosis factor alpha (TNF-α), and interleukin-1 beta (IL-1β) in the tissue of healthy individuals and patients with periodontitis.

Killer-cell immunoglobulin-like receptor and human leukocyte antigen-C genes in common variable immunodeficiency.

Background: We aimed herein to investigate the killer-cell immunoglobulin-like receptor (KIR) genes and human leukocyte antigen (HLA)-C alleles in patients with common variable immunodeficiency (CVID), and to reveal their differences from those in healthy population.

Methods: In all, 18 patients who have been diagnosed with CVID and 15 living donors of kidney transplant recipients were enrolled in the study. Polymerase chain reaction-sequence-specific primer (PCR-SSP) typing method was used in molecular genetic analysis.

Ruptured sinus of Valsalva aneurysm associated with noncompaction of the ventricular myocardium.

Rupture of a sinus of Valsalva aneurysm is a rare, but life-threatening cardiac abnormality that requires surgical correction when diagnosed, and is frequently associated with other congenital defects, particularly with ventricular septal defect, aortic valve regurgitation, and bicuspid aortic valve. We present the case of a 21-year-old man who had a ruptured aneurysm of the noncoronary sinus into the right atrium, a ventricular septal defect, a persistent left superior vena cava and a noncompaction of the ventricular myocardium diagnosed by two-dimensional echocardiography. Surgical repair was carried out and the patient made an uneventful recovery.