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Necrotizing pneumonia is characterized by destruction and liquefaction of the lung tissue and loss of the normal pulmonary parenchymal architecture. During the course of resolution areas of hyperlucency are formed, sometimes with the development of giant lung cysts that can be a field with fluid resembling lung abscess. There is no consensus on the management of these abnormalities.
Severe asthma is a relatively uncommon condition in children but one which causes morbidity, occasionally mortality, and is a challenging condition to manage. There are several definitions of severe asthma, which have a common theme of poor control despite high dose inhaled corticosteroid treatment. Depending on the definition chosen, the prevalence of severe childhood asthma may be up to 5% within populations with asthma. Collectively, there is some evidence that the treatments used in severe asthma are be...
The outlook for people with cystic fibrosis (CF) has improved considerably as a result of conventional therapies including aerosolized agents for airway clearance. These will continue to play a significant role in maintaining well-being and improving survival, even as newer agents emerge that correct the underlying CF defect. In this review, we explore the evidence supporting the use of dornase alfa, hypertonic saline, and mannitol in improving mucus clearance in patients with CF from different age groups w...
Decreased lung function is common in preterm-born survivors. Increased fractional exhaled nitric oxide (FeNO) appears to be a reliable test for eosinophillic airway inflammation especially in asthma. We, systematically, reviewed the literature to compare FeNO levels in preterm-born children and adults who did or did not have chronic lung disease of prematurity (CLD) in infancy with term-born controls.
Bronchoalveolar lavage (BAL) is a standardized method to obtain specimen samples from the airway lumen of the respiratory system. BAL is used to diagnose lung infection and infection markers in neonates.
Cystic Fibrosis Transmembrane Regulator (CFTR) dysfunction is associated with epithelial cell vulnerability and with dysregulation of the local inflammatory responses resulting in excessive airway neutrophilic inflammation and pathogen growth. In combination with impaired mucociliary clearance, and dysregulation of defense function, bacterial infection follows with eventual airway damage and remodeling. Because of these inherent vulnerabilities, viral infections are also more severe and prolonged and appear...
Although many children with asthma do not experience persistence into adulthood, recent studies have suggested that poorly controlled asthma in childhood may be associated with significant airflow obstruction in adulthood. However, data regarding disease progression are lacking, and clinicians are not yet able to predict the course of a child's asthma. The goal of this article was to assess the current understanding of childhood asthma treatment and progression and to highlight gaps in information that rema...
In the national newborn screening programme for CF in Switzerland, we compared the performance of two sweat test methods, by investigating the feasibility and diagnostic performance of the Macroduct collection method (with chloride mesurement) and Nanoduct test (measuring conductivity) for diagnosing CF.
The objective of our study was to examine whether outpatient respiratory morbidities in infants with bronchopulmonary dysplasia (BPD) are influenced by the human milk consumption.
To identify the determinants of nocturnal hypoxemia in children with CF using clinical parameters, polysomnography (PSG), and lung function.
Most respiratory bacterial carriage studies in children are based on cross-sectional samples or longitudinal studies with infrequent sampling points. The prospective Observational Research in Childhood Infectious Diseases birth cohort study intensively evaluated the community-based epidemiology of respiratory viruses and bacteria during the first 2-years of life. Here we report the bacteriologic findings.
Diffuse panbronchiolitis (DPB) is a rare progressive and eventually fatal pulmonary disease first identified in Japan and initially seen predominantly in Southeast Asia. Macrolide antibiotics rapidly reverse symptoms and pathology, and their use increased the 5 and 10-year survival from 50 and 30 percent, respectively, to over 90%. Review of 181 case reports from previous publications found patients with DPB commonly had their pulmonary symptoms preceded by rhinosinusitis, frequently by many years. Long del...
Exhaled breath temperature (EBT) has been suggested as a non-invasive marker of airway inflammation in asthma. There have been no studies examining longitudinal changes in EBT following asthma attacks.
Asthma is a chronic inflammatory disorder; type 2 inflammation is the most common asthma endotype in childhood. T regulatory cells are functionally and allergen-specifically defective in allergic patients. Vitamin D3 exerts many functions on immune system, mainly concerning T regulatory function. Asthma control is the goal of the asthma treatment. We aimed to stratified a group of 76 consecutive children (50 males, 26 females, mean age 10.4 + 2.2 years) with allergic asthma and visited for the first tim...
The transition from a fee-for-service payment system to value-based payment system gained momentum in the US in 2010 with the passage of the Affordable Care Act and continues to progress rapidly. Market research estimates that value-based payment models will surpass fee-for-service by 2020. This change offers both great opportunity and great risk to the medical care of the heterogeneous populations of children with chronic respiratory disease. The fee-for-service model has driven the emergence of a healthca...
Non-tuberculous mycobacteria (NTM) have emerged as an important pathogen in cystic fibrosis (CF). Early detection and treatment of NTM can preserve lung function and maintain good lung health. Many children with CF are not regular sputum producers and cough swabs cannot routinely be used to diagnose NTM. We aimed to test the hypothesis that performing sputum induction at routine annual review results in earlier identification of NTM in non-sputum producing children with CF.
Primary ciliary dyskinesia (PCD) is a rare genetic condition characterized by respiratory tract infections, situs inversus or heterotaxy, and male infertility. Chronic respiratory infections begin in childhood and result in complications such as bronchiectasis. As hypoxemia is often attributed to bronchiectasis, other etiologies for desaturation in this setting are not routinely evaluated. The development of pulmonary arteriovenous malformations (PAVMs) in PCD is not an established association. PAVMs as the...
CFTR mutations permitting residual function (RF) of the CFTR protein are disease-causing. These mutations are associated with a pneumopathy that is delayed in onset and is slower in progression than are more common forms of cystic fibrosis (CF), although the disease may become severe in some patients. RF mutations are among the most frequent in Italy, thus encouraging investigation of their prevalence and associated phenotypes.
To determine the association of timing of steroid therapy for bronchopulmonary dysplasia (BPD) and outcomes.
Cystic fibrosis (CF) is an autosomal recessive genetic disease caused by variants in the gene encoding the cystic fibrosis transmembrane conduction regulator (CFTR) protein. Loss of CFTR function disrupts chloride, bicarbonate and regulation of sodium transport, producing a cascade of mucus obstruction, inflammation, pulmonary infection, and ultimately damage in numerous organs. Established CF therapies treat the downstream consequences of CFTR dysfunction and have led to steady improvements in patient surv...