The importance of aeroallergen sensitivity in children with cystic fibrosis.

BACKGROUND: Cystic fibrosis (CF) is an inherited autosomal recessive disorder that causes chronic airway disease. In addition to genetic factors, environmental factors may affect the clinical phenotype of CF. In this study, the presence of aeroallergen sensitivity in our patients with CF and its effects on clinical findings are evaluated. METHODS: In this study, patients included were diagnosed with CF and followed in the Pediatric Respiratory and Allergy Clinic of the Faculty of Medicine, Dokuz Eylul University, Izmir, Turkey. Demographic characteristics, clinical and laboratory findings, skin prick test (SPT) results, and modified Shwachman-Kulczycki (MSK) scores of the patients were evaluated. RESULTS: We evaluated 51 patients with CF with a median age of 10 (6-18) years. The mean MSK score of the patients was 72.54±11.50, and the mean predictive value of forced expiratory volume (FEV1) in the initial (1st) second was 80.43±19.50. According to SPT, aeroallergen sensitivity was detected in 17 (33.3%) patients. The prevalence of bacterial colonization and bronchiectasis was higher, and MSK scores were lower in Aspergillus fumigatus (AF)-sensitive patients (P ≤ 0.01). However, no similar difference was found in other allergen sensitivities. MSK scores (P = 0.001) and predictive FEV1 values (P = 0.005) of 25 (49%) patients with bacterial colonization were significantly lower than those without colonization. CONCLUSION: Aeroallergen sensitivity was detected in approximately one-third of CF patients. Although it has been emphasized in studies that environmental factors may have an impact on lung functions and clinical conditions in CF, the effect of allergens other than AF sensitivity may be less important compared to other environmental factors, such as the presence of bacterial colonization.

Autoimmune/autoinflammatory syndrome induced by adjuvants after multi-component meningococcal serogroup B vaccination in a 7-year-old girl: a case report.

BACKGROUND: Vaccines, which make it possible to be protected from many life-threatening infectious diseases, have been used safely and effectively for years. Most vaccines used today contain a variety of adjuvants and exogenous proteins. Severe reactions, in addition to transient and self-limiting mild reactions, mostly caused by these components, have been reported. The effects of vaccine adjuvants on the pathogenesis of immunemediated diseases are still under investigation. The syndrome called Autoimmune/inflammatory syndrome induced by adjuvants (ASIA) has been defined in the literature. CASE: We found a novel mutation of autoinflammatory diseases in the genetic analysis of our patient. The patient developed symptoms of prolonged fever, rash, arthritis and serositis after multicomponent serogroup B meningococcal (Bexsero®) vaccination, without a previously known rheumatic disease. In the presence of clinical findings in our patient, the diagnostic criteria of ASIA syndrome were met. CONCLUSION: To the best of our knowledge, this is the first case report of a patient diagnosed with the autoinflammatory disease with a novel mutation after Bexsero® vaccination. We consider that genetic examinations will be useful in patients with a systemic vaccine reaction in the presence of ASIA when diagnostic criteria are met.

Allergic bronchopulmonary Aspergillosis in children.

BACKGROUND: Allergic bronchopulmonary aspergillus (ABPA) is a lung disease caused by hypersensitivity from Aspergillus fumigatus. Diagnostic criteria, staging systems and treatment methods for ABPA disease have been reported in studies evaluating populations, the majority of which are adult patients. Our study aimed to discuss the use of ABPA diagnostic criteria in children, the success of other alternative regimens to oral corticosteroids in the treatment of ABPA, and the changes that occur during treatment, in the light of the literature. METHODS: Between January 2017 and 2020, patients diagnosed with ABPA at the Dokuz Eylül University Child Allergy and Immunology clinic were identified; demographic characteristics, clinical and laboratory findings, diagnostic scores and stages, and treatment protocols were analyzed retrospectively. RESULTS: The mean age of patients diagnosed with ABPA was 14.33 ± 1.96. At the time of ABPA diagnosis, the median total IgE level was 1033 IU/mL (1004-6129), and the median AF specific IgE was 10.64 (2.59-49.70) kU/L. Bronchiectasis was detected in HRCT of 5 cases. We detected significant improvement in spirometric analysis with omalizumab treatment in our patient with steroid-related complications. DISCUSSION: Today, although risk factors have been investigated for ABPA, it has not been revealed clearly. Both diagnostic criteria and treatment regimens have been described in research studies, mostly adults. In pediatric patients; clarification of diagnosis and treatment algorithms is necessary to prevent irreversible lung tissue damage and possible drug side effects.