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Objective: As a primary immunodeficiency X-linked agammaglobulinemia (XLA) that develops due to Bruton tyrosine kinase signal transduction protein deficiency which progresses with antibody deficiency was firstly described by an American pediatrician Ogden Bruton. In our study, we have aimed to evaluate the demographic, clinical, immunological, genetic characteristics and follow-up findings of patients diagnosed with XLA in our tertiary care Pediatric Immunology Clinic. Method: Twelve patients diagnosed with XLA between 2003-2022 in our pediatric immunology clinic we're included in our study. The patient's age, sex, age at symptom onset and diagnosis, family history, laboratory findings at the time of diagnosis, complications observed during clinical follow-up and treatment modalities used were evaluated retrospectively. Results: The median age of the patients at diagnosis was 36 [interquartile range (IQR) 10.2-69.0] months. While the median age of the patients without a family history at the time of diagnosis was 66 (IQR 41.2-66.0) months which was found to be significantly higher compared to the patients with a family history [11.5 (IQR 2.5-30.0) months] (p=0.004). Recurrent respiratory tract infections were the most common indications for admission. Agammaglobulinemia was detected in all patients except two cases. A significant decrease in B cells was detected by flow cytometry in all patients. The diagnoses were confirmed by genetic analysis for nine patients. Bronchiectasis was observed in four, arthritis in three, and inflammatory bowel disease in one case. In one patient, metaplasia was detected in the cytologic examination of the biopsy specimen obtained during endoscopy performed for the diagnosis of inflammatory bowel disease. Conclusion: Early diagnosis, treatment and regular follow-up convey critical importance in terms of preventing complications in patients with XLA.

Objective: Asthma is one of the most common chronic diseases of childhood. Many studies have shown that education positively affects asthma control and patients' quality of life. In this study, it was aimed to measure the awareness levels of children who have been under observation for a long time and who underwent Allergen Specific Immunotherapy (AIT). Methods: Patients with asthma, asthma & allergic rhinitis and/or rhinoconjunctivitis who received subcutaneous AIT between July 2019 and December 2019 were included in our prospective case-control study. A questionnaire was applied to each patient, in which both the levels of awareness related to their disease and AIT, as well as their knowledge of the allergens and prevention measures they were sensitive to were measured. The results were examined. Age, gender, type of allergic diseases, allergen type, AIT time were evaluated statistically. Results: 82 cases (48 boys and 34 girls) were included in the study. The number of patients who know the name of the disease correctly is 68 (82.9%); The number of patients who knew allergens to be sensitive was found to be 47 (57.3%). It was observed that 15%-72% of the cases had information about asthma disease. Age, gender and diseases of the patients were not statistically different. Conclusion: In our study, it was observed that our children were highly aware of especially about sports and environmental protection methods. It is essential to raise awareness of patients and parents for increase treatment success and control the asthma.