not-yet-known not-yet-known not-yet-known unknown Background: Allergic bronchopulmonary aspergillosis (ABPA), which is one of the respiratory complications of cystic fibrosis, is a hypersensitivity reaction in the lung against the antigens of the fungus Aspergillus fumigatus ( A.fumigatus). If left untreated, it may cause irreversible deteriorations in lung functions. The aim of this study is to determine the incidence of ABPA, according to years and to determine the risk factors leading to the development of ABPA by using the CF Registry System of Turkey data. Methods: The study was designed as a retrospective cohort study. Using the CF Registry System of Turkey data, the incidence of ABPA was determined and the data of 44 patients newly diagnosed with ABPA in 2022 and 132 patients with similar mutation weight and age range without ABPA diagnosis in that year were compared, and the risk factors affecting the development of ABPA were determined. Results: Low pulmonary function test values, having had at least one pulmonary exacerbation in that year and receiving intravenous antibiotic treatment, using inhaled antibiotics, having a high number of pulmonary exacerbations, having Pseudomonas aeroginosa (P.aeroginosa) colonization, having a low body mass index (BMI) and having cystic fibrosis related diabetes mellitus (CFRD) were found to be among the risk factors for the development of ABPA. Conclusions: Early recognition and treatment of ABPA is essential to prevent further damage to the lungs. Patients with frequent pulmonary exacerbations, low BMI and low pulmonary function test values, chronic colonization should definitely be considered for ABPA.

Objectives: To evaluate the risk factors of recurrent pulmonary exacerbation and poor prognosis in children with idiopathic pulmonary hemosiderosis (IPH).  Methods: In this multicenter study, 54 patinets with diagnosis of IPH included. Medical records were retrospectively reviewed from three tertiary care hospitals between 1979 and 2019. Also, current information and the long-term progress of patients was determined by contacting the families by telephone. Results: A total of 54 children were included. The median age of onset of symptoms was 4.5 ± 3.8 years. The median time from onset to diagnosis was 0.9 years ± 2.2. The mean number of recurrent episodes per child in the recurrence-positive group was 3.55 (1-15). Univariate analysis demonstrated that patients presenting with hypoxia or requiring transfusion at the time of presentation had significantly more recurrence episodes (P=0.002). Multivariate analysis showed that the presence of hypoxia at the time of initial presentation was a significant independent predictor of recurrent episodes (P=0.027). The median follow-up was 3.3 ± 4.8 years (0.75 months-27 years). There was a significant relationship between the presence of hypoxia, transfusion history, ANA positivity, and elevated transaminases at the time of initial evaluation and treatment response. Conclusions: The present study provides important information on the clinical course and outcome of pediatric IPH, and substantial information regarding factors that affect recurrent exacerbations and prognosis. Demonstrating of hypoxia as an independent risk factor in recurrence episodes could be guide physicians in the planning of treatment strategies.