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: 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.

Introduction:Congenital lobar emphysema (CLE) is a rare developmental lung malformation that involves the hyperaeration of one or more lung lobes due to partial obstruction and occurs at a rate of 1/20,000–30,000 live births.Here,we aimed to retrospectively examine the clinical, radiological, and bronchoscopy findings of patients with CLE who were diagnosed and treated with surgical or conservative approaches in our clinic to compare our results with those in the literature. Method:We examined the clinical, and radiological data and FB findings of the patients with CLE aged 0–18 years at our center between 2013 and 2020.We also examined the symptoms and findings recorded during the patients’ follow-up. Results:.The median age of 20 patients with CLE at diagnosis was 3.2 years (range, 1 day–17 years).Respiratory distress and mediastinal shift were more prominent in the patients who underwent surgery than the patients who were followed up conservatively and diagnosed at an early age (p = 0.001, p = 0.049, p = 0.001, respectively). Discussion: In line with studies in the literature, the pulmonary symptoms and CLE-related imaging findings in our study regressed during the conservative follow-up.We recommend clinicians consider performing a detailed anamnesis for patients with unresolved respiratory symptoms and unilateral or bilateral increased ventilation,along with appropriate imaging tests and examinations, and should consider CLE in the diagnosis.

Introduction:Community-acquired pneumonia(CAP) in children has tended to decrease in recent years with effective vaccination and treatments.However,the frequency of complicated community-acquired pneumonia (CCAP) is gradually increasing.By determining risk factors for the development of CCAP,new approaches for early diagnosis and effective treatment can be determined. Method: Records of 113 patients who were hospitalized due to CAP and CCAP between January 2017 and December 2017 were examined.Demographic characteristics of the patients,comorbid diseases,admission symptoms,physical examination findings,laboratory and imaging results, treatments, hospital stay and treatment response were recorded. Pleural effusion,if empyema developed in patients with CCAP,thoracentesis and/or chest tube insertion,fibrinolytic therapy,and the need for additional surgical intervention were investigated. Results:Our patients had a mean age of 3.6 ± 2.2, 60(53%) were female and 53(47%) were male.93 (82.3%) of the patients were diagnosed with CAP and 18(15.9%) were diagnosed with CCAP.Detection of pleural effusion at the admission of patients in multivariate logistic regression[(OR (95% CI),4.24 (1.77-10.16), p <0.001)] respiratory distress(tachypnea and retraction)[(OR (95% CI) 3.04 (1.30-7.130) ),p <0.001)] and hypoxia (92% and less saO2 measured by pulse oximeter) at admission [(OR (95% CI),: 5.247 (1.58-9.46),p <0.001)] were identified as independent risk factors for diagnosis of CCAP. Discussion:Hypoxia,respiratory distress and imaging pleural effusion are important distinguishing findings for development of complications in patients admitted for CAP. Determining the etiology of CCAPs and early diagnosis and treatment approaches can be established, and protection measures can be taken. With future prospective studies, the causes of CCAP can be determined and preventive measures and new treatment approaches can be developed

INTRODUCTION: Recurrent pulmonary infections, wheezing and stridor due to swallowing dysfunction, esophageal dysmotility, gastroesophageal reflux, tracheomalacia and bronchomalacia are frequently seen complications after esophageal atresia and tracheo-oesophageal fistula (EA-TEF) surgeries. This study aimed to investigate the frequency and causes of respiratory problems and to evaluate the factors that affect respiratory morbidity in patients who had undergone EA-TEF repair in a tertiary referral center. METHODS: Preoperative and postoperative records of patients with EA, TEF+EA and isolated EA were examined retrospectively. Accompanied diseases and swallowing dysfunction symptoms were questioned. Bronchoalveolar lavage results were investigated if the patient had flexible bronchoscopy. RESULTS: A total of 71 children with EA were included in the study, and seven patients who did not have follow-up after surgery were excluded. 46 of the 64 patients continue regular follow-up visits in our department. Male sex, primary EA repair in another center, EA type C, accompanying genetic anomalies, severe tracheomalacia, late per oral feeding (1 year after surgery), and severe GER were found to cause significantly higher incidence of coughing, recurrent wheezing, recurrent pneumonia, and bronchiectasis despite surgical and medical treatments (p = 0.048, p = 0.045, p = 0.009, p = 0.029, p = 0.025) CONCLUSİON: Even if anatomical anomalies are corrected by surgery in patients who underwent EA repair, precautions can be taken for GERD, laryngotracheomalacia, and swallowing dysfunction, and effective pulmonary rehabilitation can be initiated with early multidisciplinary approach before the development of respiratory tract symptoms.

A twenty-five days old girl, admitted to pediatric pulmonology clinic, with collapse on the anterior chest wall while breathing. Physical examination revealed total retraction of the chest wall especially on the upper part, paradoxical chest wall movements with respiration, and a raphe extending from the umbilicus to the lower end of the sternum. Hypoplasia of the inferior and non-development of the superior segment of the sternum was shown on 3-dimensional(3D) thorax CT. At the age of fifty-six days, she had been operated. Due to the large defect, primary closure couldn’t be performed and the sternum was reconstructed with the cartilage obtained from the tissue around the ribs. The sternal cleft is a rare malformation; complete assessment, based primarily on thoracic imaging is required for diagnosis. Also cardiac, abdominal and cranial imaging for concominant malformations should be performed; our patient had none. Immediate surgical management is essential to avoid possible complications.