Long-term Home Mechanical Ventilation of Children in İstanbul.

Objective: The aims of this multi-center study were to describe the characteristics of children receiving long-term home mechanical ventilation (HMV) in İstanbul and to compare the patients receiving non-invasive and invasive ventilation.

Material And Methods: This cross-sectional multicenter study included all children receiving long-term HMV followed by admission to six tertiary hospitals. The data were collected between May 2020 and May 2021.

Implementation of Cystic Fibrosis Responsibility, Independence, Self-Care, Education Program Enhances Cystic Fibrosis Knowledge in Limited Resource Country: Results From a Randomized Controlled Trial.

Background: CF R.I.S.

Microbial Contamination of Nebulizers in Patients With Cystic Fibrosis.

Objective: Nebulizer contamination has potential harmful effects on the respiratory system. The aim was to investigate the contamination profile of the nebulizers in cystic fibrosis patients and evaluate the relationship between hygiene practices and microbial contamination. Materials and Methods: Microbiological swab samples were taken from 3 different locations of the nebulizers of 102 patients.

Can Sniff Nasal Inspiratory Pressure be a guide in detecting of sleep-disordered breathing in children with Duchenne Muscular Dystrophy?

Purpose: Duchenne muscular dystrophy (DMD) is a severe, progressive condition characterized by muscle degeneration and weakness, significantly affecting respiratory function. This study aimed to evaluate the presence of sleep-disordered breathing (SDB) in children with DMD and investigate the relationships between sleep and respiratory function using spirometry, sniff nasal inspiratory pressure (SNIP), and polysomnography (PSG) along with capnography.

Research Question: Can low SNIP be a guide for detecting respiratory muscle involvement early and determining the right time to perform early PSG and capnography in DMD?

Study Design: Prospective, observational, cross-sectional study.

Central Sleep Apnea in Children-10 Years Experience at a Tertiary Sleep Laboratory.

Objective: Central sleep apnea (CSA) is a rare condition in children; however, it can cause significant morbidity if not diagnosed early. We aimed to increase the knowledge about CSA in children by describing the clinical characteristics of children diagnosed with CSA at our sleep center.

Material And Methods: We retrospectively reviewed 1263 polysomnographies (PSG) performed between 2012 and 2023 at our tertiary sleep center and evaluated the clinical characteristics of the patients with CSA.

The Association between Aminoglycoside Exposure and Ototoxicity in Children with Cystic Fibrosis.

Introduction: Pulmonary exacerbations increase the requirement of aminoglycoside (AG) antibiotics in people with cystic fibrosis (pwCF). Several studies have shown that AGs have a cumulative effect on ototoxicity. We aimed to investigate the relationship between AG exposure and ototoxicity by using 3 different methods in patients with CF.

Assessment of patients' baseline cystic fibrosis knowledge levels following translation and adaptation of the CF R.I.S.E translation program into Turkish.

Background: Cystic fibrosis (CF) patients have a limited life expectancy, but significant medical advances now highlight the need for successful transition programs from pediatric to adult care. The goal of this project was to introduce the transition program CF R.I.

Electronic home monitoring of children with cystic fibrosis to detect and treat acute pulmonary exacerbations and its effect on 1-year FEV.

Background: We aimed to investigate the effect of the use of electronic home spirometry in children with cystic fibrosis (CF) on 1-year FEV (% predicted, pp) change.

Methods: This is a randomised, one-year prospective study including children with CF between 6 and 18 years of age. Subjects were randomised into home spirometry group (HSG) and usual care group (UCG).

Implementation of standardized cystic fibrosis care algorithm to improve the center data-quality improvement project international collaboration.

Background: A collaboration between the University of Michigan (U of M) Cystic Fibrosis Center (CFC) and Marmara University (MU) CFC was initiated to improve the health status of people with cystic fibrosis (pwCF) at MU through implementing Quality Improvement (QI) initiatives. The main aim was to improve lung function in children with FEV1pp <80. The secondary aim was to assess the changes in health related quality of life.

Night-to-night variability of polygraphy in children with sleep disordered breathing symptoms.

Introduction: Polygraphy (PG) can be used as an alternative test for the diagnosis of obstructive sleep apnea syndrome (OSAS) in children. Night-to-night variability of PG in children is not known. Our aim was to determine whether a single night PG was reliable for OSAS diagnosis in children with symptoms of sleep-disordered breathing (SDB).

Depression, anxiety, and sleep quality of caregivers of children with spinal muscular atrophy.

Background: The aim of this study was to evaluate the prevalence of anxiety, depression, sleep, and associated factors in caregivers of children with spinal muscular atrophy (SMA).

Materials And Methods: Beck Depression Inventory (BDI), the State-Trait Anxiety Inventory-State (STAI-S), the State-Trait Anxiety Inventory-Trait (STAI-T), and Pittsburgh Sleep Quality Index (PSQI) were used to assess the anxiety, depression, and sleep quality of the caregivers of children with SMA. Higher scores indicated worse outcome for all three questionnaires.

Improvements in body mass index of children with cystic fibrosis following implementation of a standardized nutritional algorithm: A quality improvement project.

Background: A collaboration between the University of Michigan (UM) Cystic Fibrosis Center (CFC) and Marmara University (MU) CFC was initiated in MU through conducting Quality Improvement projects (QIP). The global aim was to improve nutritional status of children with CF (cwCF), with a specific aim to increase the mean BMI percentile (BMIp) for cwCF by 10 percentile points in 12 months.

Methods: Body mass index (BMI) percentiles of cwCF were categorized as: nutritionally adequate (BMIp ≥ 50%); at risk (BMIp 25%-49%); urgently at risk (BMIp 10%-25%); critically at risk (BMIp < 10%).

The utility of risk assessment tools for acute pulmonary embolism in children.

Unlabelled: BACKGROUND AND AIM: Pulmonary embolism (PE) is a potentially life-threatening disease in children. The objective of the study is to evaluate the utility of adult-based pulmonary embolism rule-out criteria (PERC), Pediatric PE Model, and D-dimer in the diagnosis of PE in children.

Material And Methods: The study consisted of patients under 18 years of age who were consulted to the Pediatric Pulmonology Clinic for the evaluation of PE.

Respiratory outcome of spinal muscular atrophy type 1 patients treated with nusinersen.

Background: Respiratory failure is the leading cause of mortality in spinal muscular atrophy type 1 (SMA1) children. The current study aims to evaluate the effect of nusinersen treatment on respiratory outcome of the patients with SMA1.

Methods: In this retrospective, single-center study, 52 SMA1 patients treated with nusinersen were included in the analysis.

The effect of coronavirus disease 2019 on anxiety levels of children with cystic fibrosis and healthy peers.

Background: Cystic fibrosis (CF) is a chronic disease causing recurrent respiratory tract infections. Viral respiratory tract infections are more severe in CF. The first case of coronavirus disease 2019 (COVID-19) was seen in Turkey on March 11, 2020, and nationwide school closure and lockdown were implemented.

The ISPAT project: Implementation of a standardized training program for caregivers of children with tracheostomy.

Background: Tracheostomy-related morbidity and mortality mainly occur due to decannulation, misplacement, or obstruction of the tube. A standardized training can improve the skills and confidence of the caregivers in tracheostomy care (TC).

Objective: Our primary aim was to evaluate the efficiency of standardized training program on the knowledge and skills (changing-suctioning the tracheostomy tube) of the participants regarding TC.

Comparison of intravenous and non-intravenous antibiotic regimens in eradication of P. aeruginosa and MRSA in cystic fibrosis.

Background: Chronic pulmonary infection is the leading cause of mortality and morbidity in patients with cystic fibrosis (CF). The most common pathogens isolated in CF are Staphylococcus aureus (SA) and Pseudomonas aeruginosa (PA). Chronic infection of PA and methicillin-resistant S.

The effects of nebulizer hygiene training on the practices of cystic fibrosis patients and caregivers.

Background: Nebulizers can be contaminated with microorganisms and may be a source of infection in the lower airways in patients with cystic fibrosis (CF).

Objective: Primary aim of this study was to determine the level of knowledge regarding nebulizer hygiene and adherence to CF foundation infection prevention and control (IPC) measures of CF patients in our center. We also evaluated the effect of a standardized training program on nebulizer cleaning and disinfection practises with pre and posttest.

Screening of depression and anxiety in adolescents with cystic fibrosis and caregivers in Turkey by PHQ-9 and GAD-7 questionnaires.

Background: Depression and anxiety symptoms in patients with cystic fibrosis (CF) and their caregivers are 2-3 times higher than in the normal population. This study aims to evaluate the frequency and severity of depression and anxiety symptoms and to determine possible risk factors in CF patients and their mother and/or fathers at Marmara University CF center.

Methods: The study included 132 CF patients who were followed up at our CF center.

Depression and anxiety in mothers of home ventilated children before and during COVID-19 pandemic.

Background: Due to advances in technology, home ventilation in children has increased in recent years. The provision of proper care for a home-ventilated (HV) child can have a strong impact on the lifestyle of caregivers. The aim of this study was to evaluate the depression and anxiety levels of the mothers of HV children during the current COVID-19 pandemic and compare them to those of mothers of healthy peers.

Comparison of conventional chest physiotherapy and oscillatory positive expiratory pressure therapy in primary ciliary dyskinesia.

Background: Chest physiotherapy (CP) is a recommended treatment modality in primary ciliary dyskinesia (PCD).

Objective: Primary aim was to compare the efficacy and safety of the conventional chest physiotherapy (CCP) and oscillatory positive expiratory pressure therapy (OPEPT). Secondary aims were to compare the exacerbation rate, time until the first exacerbation, patient compliance and comfort between the two CP methods.

Long-term respiratory outcomes of post-op congenital lung malformations.

Background: Congenital lung malformations (CLM) are rare disorders and surgical intervention is the definitive treatment. Our aim is to evaluate the long-term lung function of patients with CLM after surgery compared to healthy children.

Methods: Sixteen children with CLM (M/F: 9/7) and 30 age-matched, healthy controls (M/F: 13/17) were included in the study.