Experience with flexible bronchoscopy for noncoronavirus disease of 2019 indications in pediatric patients during the coronavirus disease of 2019 pandemic.

Background And Aim: Flexible bronchoscopy (FB) poses a risk of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) transmission due to aerosol generation. This study aimed to assess the utilization, indications, outcomes, and safety of FB in pediatric patients for noncoronavirus disease of 2019 (COVID-19) reasons during the pandemic.

Materials And Methods: We retrospectively analyzed pediatric patients who underwent FB for non-COVID-19 indications at a tertiary children's hospital's pulmonary clinic during the COVID-19 pandemic.

Macrophage activation syndrome induced by in a pediatric patient with cystic fibrosis and familial Mediterranean fever.

is the most frequently isolated member of the coagulase-negative staphylococci, which colonizes the skin and mucous membranes of the human body. Despite being a component of the normal flora, can act as an opportunistic pathogen and is frequently implicated in both bacterial and nosocomial infections. Macrophage activation syndrome (MAS) is a rare but potentially fatal disease that occurs as a result of increased activation and proliferation of T lymphocytes and macrophages in response to infections and immune mediated diseases.

Clinical characteristics of patients requiring lung transplantation referral in national cystic fibrosis registry data.

Background: We aimed to determine the number of cystic fibrosis (CF) patients recorded in the Cystic Fibrosis Registry of Türkiye (CFRT) who were in need of lung transplantation (LT) referral and examine clinical differences between patients who were LT candidates due to rapid forced expiratory volume in one second (FEV₁) decline and LT candidates without rapid FEV₁ decline in the last year to identify a preventable cause in patients with such rapid FEV₁ decline.

Methods: All CF patients recorded in the CFRT in 2018 were evaluated in terms of LT. Patients were divided into those with FEV₁ below 50% and in need of LT due to a decrease of 20% or more in the previous year (Group 1) and those who did not have FEV₁ decline of more than 20% in the previous year but had other indications for LT (Group 2).

Cardiac involvement in a case of juvenile dermatomyositis with positive anti-melanoma differentiation associated protein 5 antibody.

Juvenile dermatomyositis (JDM) is an autoimmune disease characterized by muscle weakness and specific skin lesions, as well as non-muscular involvement such as interstitial lung disease (ILD), cardiac involvement and arthritis. Anti-melanoma differentiation-associated protein 5 (anti-MDA5)-positive JDM patients are typically characterized by the presence of skin ulcers and rapidly progressing ILD (RP-ILD). Although cardiac involvement is not an expected involvement in anti-MDA5-positive JDM cases, it is significant because it can be fatal.

Evaluation of ciliary functions and ciliary beat frequency via cell culture method in patients with primary ciliary dyskinesia.

Background: Cell culture increases both diagnostic specificity and sensitivity of primary ciliary dyskinesia (PCD) and the most important reason to use cell culture for definitive diagnosis in PCD is to exclude secondary ciliary defects. Here we aimed to evaluate the cilia functions and cilia ultrastructural abnormalities after ciliogenesis of cell culture in patients with definitive diagnosis of PCD. We also aimed to compare high speed videomicroscopy (HSVM) results of patients before and after ciliogenesis and to compare them with electron microscopy, genetic and immunofluorescence results in patients with positive diagnosis of PCD.

Surgery versus conservative management in congenital lobar emphysema: follow up and indicators for surgery.

Introduction: Congenital lobar emphysema (CLE), a rare developmental lung malformation, involves the hyperaeration of one or more lung lobes caused by 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 by surgical or non-surgical (conservative) approaches at our center and compared our results with those in the literature.

Methods: We examined the clinical, radiological, and bronchoscopy findings of 20 patients with CLE aged 0-18 years at our center between 2013 and 2020.

The success of the Cystic Fibrosis Registry of Turkey for improvement of patient care.

Background: Cystic fibrosis (CF) registries play an essential role in improving disease outcomes of people with CF. This study aimed to evaluate the association of newly established CF registry system in Turkey on follow-up, clinical, growth, treatment, and complications of people with this disease.

Methods: Age at diagnosis, current age, sex, z-scores of weight, height and body mass index (BMI), neonatal screening results, pulmonary function tests, history of meconium ileus, medications, presence of microorganisms, and follow-up were evaluated and compared to data of people with CF represented in both 2017 and 2019 registry data.

Increased Plasma YKL-40 Level and Chitotriosidase Activity in Cystic Fibrosis Patients.

We investigated plasma YKL-40 levels and chitotriosidase (CHIT1) activity in patients with cystic fibrosis (CF) lung disease and evaluated clinically relevant factors that may affect their levels. Plasma samples were obtained from pediatric (n = 19) and adult patients (n = 15) during exacerbation, discharge, and stable period of the disease. YKL-40 levels and chitotriosidase activity were measured by enzyme-linked immunosorbent assay and fluorometric assay, respectively.

Obstructive sleep apnea in children with Down syndrome: is it possible to predict severe apnea?

The objectives are to explore the demographic and polysomnographic features of children with Down syndrome and to determine the predictive factors associated with severe sleep apnea. A total of 81 children with Down syndrome referred for full-night polysomnography were analyzed. In addition, parental interviews were performed for each child.

Evaluation of pulmonary complications and affecting factors in children for repaired esophageal atresia and tracheoesophageal fistula.

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-esophageal 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.

Takayasu arteritis presenting with spontaneous pneumothorax.

Background: Takayasu arteritis (TA) is an idiopathic chronic inflammatory arteritis that affects the large blood vessels. Pulmonary involvement was considered an uncommon manifestation of the disease and spontaneous pneumothorax has not been previously described in association with TA.

Case: We report a 13-year-old female who had TA complicated by spontaneous pneumothorax during treatment.

Diverse clinical characteristics of Aspergillus growth in patients with cystic fibrosis.

Background And Objectives: Patients with cystic fibrosis (CF) have a varying spectrum of clinically significant Aspergillus disease in addition to allergic bronchopulmonary aspergillosis (ABPA). Here we aimed to review the different clinical phenotypes related with Aspergillus growth on the airway culture of patients with CF, we also aimed to investigate the effect of Aspergillus growth on lung function tests.

Method: The medical records of 100 patients with CF who had Aspergillus growth on airway culture within the period of April 2001 and June 2016 were retrospectively analyzed.

Plasma Ceramides and Sphingomyelins of Pediatric Patients Increase in Primary Ciliary Dyskinesia but Decrease in Cystic Fibrosis.

We investigated plasma sphingomyelin (CerPCho) and ceramide (Cer) levels in pediatric patients with cystic fibrosis (CF) and primary ciliary dyskinesia (PCD). Plasma samples were obtained from CF (n = 19) and PCD (n = 7) patients at exacerbation, discharge, and stable periods. Healthy children (n = 17) of similar age served as control.

Impact of mannose-binding lectin 2 gene polymorphisms on disease severity in noncystic fibrosis bronchiectasis in children.

Background: Mannose-binding lectin (MBL) is a complement protein involved in the innate immune system, and is associated with some chronic respiratory diseases including noncystic fibrosis (non-CF) bronchiectasis in adults. The aim of this study was to investigate the frequency of MBL2 gene polymorphisms in children with non-CF bronchiectasis, and the effect of MBL deficiency on disease severity.

Methods: Fifty children with non-CF bronchiectasis (bronchiectasis group) and 50 healthy controls (control group) were included.