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.

Associations between income level and health outcomes in people with cystic fibrosis in Turkey.

Background: Our study aimed to identify the social domains that pose the greatest barriers to managing and supporting pwCF, particularly in relation to income levels.

Methods: To identify associations between income and health outcomes in pwCF in our center the shorter form of the survey "Your Current Life Situation" (YCLS) was used in face-to-face interviews. Participants were also asked to complete the validated Turkish versions of the 9-item Patient Health Questionnaire (PHQ-9) and the 7-item Generalized Anxiety Disorder scale (GAD-7) to assess depression and anxiety, respectively.

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.

Long-term outcomes of standardized training for caregivers of children with tracheostomies: The IStanbul PAediatric Tracheostomy (ISPAT) project.

Unlabelled: BACKGROUND AND OBJECTIVES: Children with tracheostomies are at increased risk of tracheostomy-related complications and require extra care. Standardized training programs for caregivers can improve tracheostomy care and reduce complications. In this study, we compared caregiver knowledge and skill scores after a standardized theoretical and practical training program on tracheostomy care (IStanbul PAediatric Tracheostomy (ISPAT) project) immediately and 1 year post-training and evaluated how this training affected the children's clinical outcomes.

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).

Medical Treatment of Obstructive Sleep Apnea in Children.

Obstructive sleep apnea (OSA) is characterized by recurrent complete or partial obstruction of the upper airway. The prevalence is 1-4% in children aged between 2 and 8 years and rising due to the increase in obesity rates in children. Although persistent OSA following adenotonsillectomy is usually associated with obesity and underlying complex disorders, it can also affect otherwise healthy children.

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.

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%).

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 safety and sustainability of bottle-pep therapy in pediatric patients with cystic fibrosis.

Introduction: Airway clearance techniques, which include positive expiratory pressure (PEP) devices, are essential in the pulmonary rehabilitation of cystic fibrosis (CF). Bottle-PEP is a low-cost but an effective alternative.

Objective: The aim of this study is to document the sustainability and safety of Bottle-PEP therapy as a home rehabilitation aid.

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.

Geographical barriers to timely diagnosis of cystic fibrosis and anxiety level of parents during newborn screening in Turkey.

Background: Despite the availability of cystic fibrosis (CF) screening countrywide, diagnostic delay is still a crucial issue. The objectives of this study were to explore the stages of the NBS process, determine the risk factors associated with diagnostic delay and evaluate parent anxiety and experience throughout the process.

Methods: This is a multicenter cross-sectional study.

Pediatric flexible bronchoscopy in the intensive care unit: A multicenter study.

Introduction: Flexible bronchoscopy (FB) is frequently used for assessment and treatment of patients with respiratory diseases. Our aim was to investigate the contribution of FB to diagnosis and therapy in children admitted to the intensive care units (ICU) and to evaluate the safety of FB in this vulnerable population.

Methods: Children less than 18 years of age who underwent FB in the five neonatal and pediatric ICUs in Istanbul between July 1st, 2015 and July 1st, 2020 were included to the study.

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.

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.

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.

An Adolescent Presented With Hemoptysis: Pulmonary Artery Aneurysm in Pediatric Behçet's Disease.

Behçet's disease (BD) is a rare autoimmune and chronic inflammatory vasculitis, characterized by relapsing episodes of oral aphthous and genital ulcers, skin lesions, ocular lesions and vascular involvement. Pulmonary artery involvement is rare in BD but it carries a high mortality risk. In this article, we report a 15-year-old male patient presented with a two-month history of hemoptysis, cough, fewer and weight loss.

Novel mutations and deletions in cystic fibrosis in a tertiary cystic fibrosis center in Istanbul.

Background: Cystic fibrosis (CF) genotyping has garnered increased attention since the discovery of the cystic fibrosis transmembrane conductance regulator (CFTR) gene in 1989 led to the identification of over 1700 mutations on chromosome 7. Yet, little is known about the genetic profile of CF patients in Turkey. This study sought to determine the mutation distribution among CF patients seeking care at Marmara University.