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.

Background: In this study, we aimed to evaluate the losses in the gains of children who had to discontinue their modulator therapies due to drug delivery procedures. Methods: Demographic, clinical, microbiologic, radiologic, and pulmonary function test parameters of twelve CF children, were evaluated. Parameters were divided into three groups as ‘before treatment’ (BT), ‘during treatment’ (DT) and ‘after interruption of treatment’ (AT) to show differences between. Results: There was a significant increase in forced expiratory volume in 1 s (FEV1) z-score, body mass index (BMI) z-score and Cystic Fibrosis Questionnaire-Revised respiratory domain score (CFQR-RS) of DT compared with the values BT (p=0.001, p=0.012, p<0.001; respectively). It was found that FEV1 z-score, BMI z-score and CFQR-RS of DT decreased significantly compared with the values AT (p=0.003, p=0.01, and p<0.001, respectively). When post and pre-treatment levels were compared, there was no significant difference between FEV1 z-score (p=0.07), BMI z-score (p=0.56), CFQR-RS (p=0.7). Half of patients had percent-predicted forced expiratory volume levels with a drop of more than 20%. It was also detected that Pseudomonas aeruginosa colonization was a significant factor in degradation of FEV1 z score to the lower pre-treatment levels. Conclusion: This is the first retrospective detailed study about discontinuation of modulatory therapies in children. Our study shows the importance of treatment continuation as well as the patients access to these drugs. We hope that this study will raise awareness about the regular long-term use of modulator therapies. To make these therapies available worldwide, immediate action is required.

Background: Cystic fibrosis (CF) is reported to be a risk factor for drug hypersensitivity. However, there is conflicting data about true prevalence of drug allergy in children with CF. Methods: The suspicious drug hypersensitivity reactions (DHR) of children with CF were enquired by European Network for Drug Allergy (ENDA) questionnaire and skin tests and/or drug provocation tests were performed according to established guidelines. Results: Two hundred and nineteen children (48.9% boys; median [IQR] age, 8.4 years [4.8-12.4 years]) with cystic fibrosis were included in the study, from whom 22 patients with 24 suspected DHRs were evaluated. Most of the suspected DHRs were non-immediate (n=16, 66.6%) type and the offending drugs were amoxicillin clavulanic acid (n=7), macrolides (n=4), trimethoprim sulfamethoxazole (TMP/SMX) (n=2), piperacillin tazobactam (n=1), pancrelipase (n=1) and ursodeoxycholic acid (n=1). Eight (33.3%) of the DHRs were classified as immediate [ceftriaxone (n=2), ceftazidim (n=2), meropenem (n=1), ambisome (n=2), vancomycin (n=1)]. The main presenting clinical presentations were maculopapular eruption (41.6%) and urticaria (37.5%), accompanied by angioedema (8.3%), flushing (12.5%) and vomiting (8.3%). Nine skin tests (with beta-lactam protocol in 6 patients) and 24 DPTs were performed and none of the skin tests revealed a positive result, however 2 DPTs with TMP/SMX were positive. Conclusion: Actual drug allergy was demonstrated in 2 of 219 patients (0.9%) with nonbeta-lactam antibiotics. These results conflict with previous researches that showed higher drug allergy rates but were consistent with some recent studies. Numerous and long-term use of multiple drugs during management of cystic fibrosis may contribute to tolerance development.