Successful Desensitization With ELX/TEZ/IVA.

Elexacaftor/tezacaftor/ivacaftor (ELX/TEZ/IVA) was given US Food and Drug Administration approval based on its therapeutic benefits to treat patients with cystic fibrosis (CF) who had at least 1 allele of the CF transmembrane conductance regulator (CFTR) with phenylalanine deleted at position 508 (F508del). The increase in genotyping studies has increased the frequency of use of CFTR modulators; however, severe allergic reactions to CFTR modulators have also been described. It is critical to avoid the offending medication and select alternative treatments while dealing with drug allergies.

Is It Possible to Use Inflammatory Markers as Potential Biomarkers for Chronic Urticaria?

This study aimed to determine whether the neutrophil-lymphocyte ratio (NLR), platelet-lymphocyte ratio (PLR), and systemic immune-inflammation index (SII) at admission affect the transition of pediatric patients diagnosed with acute spontaneous urticaria to chronic urticaria. This study included 390 patients who presented to the Department of Pediatrics at Akdeniz University Hospital with acute spontaneous urticaria between January 2020 and December 2022. A statistical comparison was made between the hematological parameters of patients who developed chronic urticaria and those who did not.

Therapeutic modalities and clinical outcomes in a large cohort with LRBA deficiency and CTLA4 insufficiency.

Background: LPS-responsive beige-like anchor (LRBA) deficiency (LRBA) and cytotoxic T-lymphocyte-associated antigen-4 (CTLA4) insufficiency (CTLA4) are mechanistically overlapped diseases presenting with recurrent infections and autoimmunity. The effectiveness of different treatment regimens remains unknown.

Objective: Our aim was to determine the comparative efficacy and long-term outcome of therapy with immunosuppressants, CTLA4-immunoglobulin (abatacept), and hematopoietic stem cell transplantation (HSCT) in a single-country multicenter cohort of 98 patients with a 5-year median follow-up.

Incidental pulmonary nodules in children: characteristic features and clinical course.

Background: There exists insufficient information about the natural course of incidental pulmonary nodules (IPN) determined on tomography in children. The aim was to determine the characteristic features and factors affecting the course of IPN.

Methods: This retrospective study included patients who presented at the Pediatric Pulmonology, Allergy & Immunology Section of Akdeniz University Hospital between January 2014-2020, and were determined with pulmonary nodules on high-resolution computed tomography (HRCT).

Specific Granule Deficiency Due To Novel Homozygote Variant.

Specific granule deficiency (SGD) is a rare immunodeficiency associated with gene variants. It can cause severe recurrent infections and is lethal without successful stem cell transplantation. Few cases with SGD of both type 1 and type 2 have been described in the literature.

Psychological State of Parents of Children with Primary Immunodeficiencies During the COVID-19 Pandemic.

COVID-19 has affected humanity not only physically but also mentally. It was expected to have impact on high-risk groups such as the immunocompromised patients and parents/caregivers of them. Our study was aimed to investigate the COVID-19 related anxiety, post-traumatic stress levels, and sleep-related parameters of the parents of children with primary immunodeficiency.

Association Between Cystic Fibrosis Severity Markers and CFTR Genotypes in Turkish Children.

Objective: To compare class I/II cystic fibrosis transmembrane conductance regulator (CFTR) mutations to class III-V mutations with regards to cystic fibrosis disease severity markers in children.

Material And Methods: This study was designed as a cross-sectional study in Antalya province, located on the south coast of Turkey. The study included 38 cystic fibrosis patients aged between 0.

Clinical phenotypes of childhood food allergies based on immune mechanisms: A multicenter study.

Food allergies (FA) are an important public health concern that place a major burden on the lives of children and their families. The complex pathogenesis of FAs results in multisystemic and heterogenous clinical presentations. To evaluate, according to immune mechanisms, the characteristics and risk factors of childhood FA in Turkey.

Hematopoietic stem cell transplantation in CD40 ligand deficiency: A single-center experience.

Deficiency of the CD40L, expressed on the surface of T lymphocytes, is caused by mutations in the glycoprotein CD40L (CD154) gene. Resulting defective humoral and cellular responses cause a clinical presentation that includes recurrent sinopulmonary bacterial infections, opportunistic infections, sclerosing cholangitis, neutropenia, and autoimmune manifestations. HSCT represents the only curative treatment modality.

An evaluation of banana allergy in children living in the Mediterranean region.

Background/aim: We aimed to apply a double-blind placebo-controlled food challenge (DBPCFC) test to patients who reported a reaction to banana and showed positivity for banana-specific IgE in Turkey. Materials and methods: Medical data of patients who had been analyzed for banana allergy were reviewed, focusing on banana-specific IgE positivity at the Department of Pediatric Allergy-Immunology at the Akdeniz University Faculty of Medicine between the years 2004 and 2012. Patients were called to the clinic for a DBPCFC test.