Mediastinal Nontuberculous Mycobacterial Infection in Children: A Multidisciplinary Approach.

Background: Mediastinal infections due to nontuberculous mycobacteria remain an exceedingly rare entity. Most cases in the published literature do not include pediatric patients. Due to their clinical infrequency, poor response to antimicrobial therapy and often precarious anatomical location, the optimal management of these lesions can be challenging.

Comparing Cytology Brushes for Optimal Human Nasal Epithelial Cell Collection: Implications for Airway Disease Diagnosis and Research.

Primary nasal epithelial cells and culture models are used as important diagnostic, research and drug development tools for several airway diseases. Various instruments have been used for the collection of human nasal epithelial (HNE) cells but no global consensus yet exists regarding the optimal tool. This study compares the efficiency of two cytology brushes (Olympus (2 mm diameter) and Endoscan (8 mm diameter)) in collecting HNE cells.

Diet and the gut-lung axis in cystic fibrosis - direct & indirect links.

Cystic fibrosis (CF) is a multisystem, autosomal, recessive disease primarily affecting the lungs, pancreas, gastrointestinal tract, and liver. Whilst there is increasing evidence of a microbial 'gut-lung axis' in chronic respiratory conditions, there has been limited analysis of such a concept in CF. We performed a comprehensive dietary and microbiota analysis to explore the interactions between diet, gastrointestinal microbiota, respiratory microbiota, and clinical outcomes in children with CF.

Phage Therapy of Mycobacterium Infections: Compassionate Use of Phages in 20 Patients With Drug-Resistant Mycobacterial Disease.

Background: Nontuberculous Mycobacterium infections, particularly Mycobacterium abscessus, are increasingly common among patients with cystic fibrosis and chronic bronchiectatic lung diseases. Treatment is challenging due to intrinsic antibiotic resistance. Bacteriophage therapy represents a potentially novel approach.

Care recommendations for the respiratory complications of esophageal atresia-tracheoesophageal fistula.

Tracheoesophageal fistula (TEF) with esophageal atresia (EA) is a common congenital anomaly that is associated with significant respiratory morbidity throughout life. The objective of this document is to provide a framework for the diagnosis and management of the respiratory complications that are associated with the condition. As there are no randomized controlled studies on the subject, a group of experts used a modification of the Rand Appropriateness Method to describe the various aspects of the condition in terms of their relative importance, and to rate the available diagnostic methods and therapeutic interventions on the basis of their appropriateness and necessity.

Pepsin as a Marker of Reflux Aspiration in Children With Esophageal Atresia: A Pilot Study.

Reflux aspiration secondary to gastroesophageal reflux disease (GERD) is one of the causes of chronic gastrointestinal and respiratory morbidity in children with esophageal atresia (EA). Currently there are no simple, validated non-invasive tests for the diagnosis of reflux aspiration in children. The aim of this pilot study was to investigate pepsin detected in exhaled breath condensate (EBC) and saliva as a potential non-invasive marker of reflux aspiration in children with EA.

Distress during airway sampling in children with cystic fibrosis.

Background: Oropharyngeal suction and oropharyngeal swab are two methods of obtaining airway samples with similar diagnostic accuracy in children with cystic fibrosis (CF). The primary aim was comparing distress between suctioning and swabbing. A secondary aim was establishing the reliability of the Groningen Distress Rating Scale (GDRS).

Association of rhinovirus with exacerbations in young children affected by cystic fibrosis: Preliminary data.

Rhinovirus (RV) is a common respiratory viral infection linked to worsening of chronic respiratory diseases including cystic fibrosis (CF) and asthma. RV was tested by RT-PCR in samples (n = 465) collected from the upper (nasal swab, oropharyngeal suction, and sputum) and lower (bronchoalveolar washings) respiratory tract of 110 children with CF. Air samples (n = 52) collected from the operating theatres and outpatient clinics were tested for RV.

Prevalence of Malnutrition and Feeding Difficulties in Children With Esophageal Atresia.

Objectives: Growth and feeding problems have been described in children with esophageal atresia (EA). Ongoing gastrointestinal and respiratory complications such as Gastroesophageal reflux disease, esophageal dysmotility, strictures, and respiratory infections may contribute. The aim of the study was to document the prevalence of malnutrition and feeding difficulties and examine predictive factors, which may influence feeding and growth in children attending a multidisciplinary EA clinic in Sydney, Australia.

Diagnostic accuracy and distress associated with oropharyngeal suction in cystic fibrosis.

Background: Early detection of bacterial pathogens in the lower airway is an important part of managing CF. This study aimed to assess the diagnostic accuracy of oropharyngeal suction (OPS) samples in obtaining airway bacterial cultures in young children with cystic fibrosis (CF), and the level of child distress caused by obtaining OPS samples.

Methods: Young children with CF undergoing broncho-alveolar lavage (BAL) as part of concurrent research or routine annual surveillance were studied.

Absence of back to school peaks in human rhinovirus detections and respiratory symptoms in a cohort of children with asthma.

Much of what is known about the seasonality of human rhinovirus (hRV) infections has been learned from the study of acute asthma exacerbations presenting to emergency care, including those among children at the start of the school term. Much less is known about the patterns of hRVs in the community. In this study, viruses and day-to-day symptoms of asthma and colds were monitored twice weekly in 67 children with asthma aged 5-12 years, over a 15 month period in Sydney, Australia.

Rhinoviruses significantly affect day-to-day respiratory symptoms of children with asthma.

Background: Viruses are frequently associated with acute exacerbations of asthma, but the extent to which they contribute to the level of day-to-day symptom control is less clear.

Objective: We sought to explore the relationship between viral infections, host and environmental factors, and respiratory symptoms in children.

Methods: Sixty-seven asthmatic children collected samples twice weekly for an average of 10 weeks.

Refractory otitis media: an unusual presentation of childhood granulomatosis with polyangiitis.

Childhood granulomatosis with polyangiitis (cGPA), previously known as Wegener's granulomatosis, is a rare, potentially fatal necrotizing vasculitis, the symptoms of which overlap with infection. We present a 16-year-old girl who, following 6 months of treatment for persistent middle ear effusion with progressive sensorineural hearing loss, developed rapidly progressing pneumonia, with pleural effusion, and multiple cavitatory lung lesions. Investigations demonstrated high titer c-ANCA and nasal septal biopsy confirmed the diagnosis of cGPA.

Single high-dose oral vitamin D3 (stoss) therapy--a solution to vitamin D deficiency in children with cystic fibrosis?

Objectives: To determine the safety and efficacy of stoss therapy on vitamin D levels over a 12 month period in children with cystic fibrosis and vitamin D deficiency (<75 nmol/L).

Study Design: Retrospective chart review of 142 paediatric CF patients from 2007 till 2011.

Results: Thirty eight children received stoss therapy and 37 children with vitamin D deficiency were not treated and served as a control group.

Expression of PPARγ and paraoxonase 2 correlated with Pseudomonas aeruginosa infection in cystic fibrosis.

The Pseudomonas aeruginosa quorum sensing signal molecule N-3-oxododecanoyl-l-homoserine lactone (3OC(12)HSL) can inhibit function of the mammalian anti-inflammatory transcription factor peroxisome proliferator activated receptor (PPAR)γ, and can be degraded by human paraoxonase (PON)2. Because 3OC(12)HSL is detected in lungs of cystic fibrosis (CF) patients infected with P. aeruginosa, we investigated the relationship between P.

Early cystic fibrosis lung disease detected by bronchoalveolar lavage and lung clearance index.

Rationale: Unrecognized airway infection and inflammation in young children with cystic fibrosis (CF) may lead to irreversible lung disease; therefore early detection and treatment is highly desirable.

Objectives: To determine whether the lung clearance index (LCI) is a sensitive and repeatable noninvasive measure of airway infection and inflammation in newborn-screened children with CF.

Methods: Forty-seven well children with CF (mean age, 1.

Acid and non-acid reflux during physiotherapy in young children with cystic fibrosis.

Background: Gastro-esophageal reflux (GOR) may contribute to lung disease in children with cystic fibrosis (CF). There is conflicting evidence regarding the effect of chest physiotherapy (CPT) in the head-down position on GOR. Furthermore, there is currently no evidence on the impact of physiotherapy on GOR as assessed by pH-multichannel intraluminal impedance (pH-MII).

Once daily insulin detemir in cystic fibrosis with insulin deficiency.

The aim of this study was to determine if once daily insulin detemir reverses decline in weight and lung function in patients with cystic fibrosis (CF). 12 patients with early insulin deficiency and six with CF related diabetes (aged 7.2-18.

Early glucose abnormalities in cystic fibrosis are preceded by poor weight gain.

Objective: Progressive beta-cell loss causes catabolism in cystic fibrosis. Existing diagnostic criteria for diabetes were based on microvascular complications rather than on cystic fibrosis-specific outcomes. We aimed to relate glycemic status in cystic fibrosis to weight and lung function changes.