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Cryptococcosis is an invasive, opportunistic, fungal infection that predominantly effects the respiratory tract and central nervous system in immunocompromised patients. It is classically associated with defects in cellular immunity such as acquired immunodeficiency syndrome. Here we describe a case of life-threatening laryngitis, endobronchitis and pneumonia due to Cryptococcus neoformans in a teenager with hypogammaglobulinaemia. To the best of our knowledge, no previous cases of laryngeal cryptococcosis have been reported in the paediatric population.

Introduction: While bronchiolitis is regarded as a viral lower respiratory tract infection, there is some evidence for the perturbation of the airway microbiota in infants with bronchiolitis. Little is known about the lower airway microbiota in children with bronchiolitis due to difficulty obtaining samples. Aim: To describe the lower airway microbiota in comparison with upper airway microbiota in infants with severe bronchiolitis needing intubation and ventilation. Methods: Infants intubated with bronchiolitis in the paediatric intensive care unit were recruited and nasal swabs and non-bronchoscopic lavage were performed. Bacterial DNA was extracted and the V3-V4 region of the 16S rRNA gene was amplified and sequenced using Illumina MiSeq. The relative abundance of taxa and alpha diversity were calculated. Results: Ten infants (median age of 3 months) were sampled. Five children had RSV bronchiolitis and five had Rhinovirus bronchiolitis. Proteobacteria were the most abundant taxa in both upper and lower airway samples. In the lower airway samples, Haemophilus was the most abundant genus (mean relative abundance 67.7%), followed by Streptococcus (15.1%) and Moraxella (5.1%). This was very similar to the upper airway samples (mean relative abundance Haemophilus 42.2% followed by Streptococcus 18.1%, Prevotella 10.5% and Moraxella 6.7%). Conclusion: In infants with severe bronchiolitis, the lower and upper airway microbiota are largely similar in bacterial composition with dominance of Proteobacteria at the Phylum level and Haemophilus at the genus level.

Young children diagnosed with bronchiectasis have a lower airway microbiota that is highly diverse and very similar to healthy controls. Haemophilus, Moraxella, Neisseria and Streptococcus are the most abundant operational taxonomic units identified.http://bit.ly/2SkqRrq

Patients with severe cystic lung disease are considered to be at risk for cyst rupture during air travel because of the possibility of increase in cyst size and impaired equilibration of pressure between the cysts and other parts of the lung. This may have clinically devastating consequences for the patient but may also result in significant costs for emergency alteration of flight schedule. We report the use of a hypobaric chamber to simulate cabin pressure changes encountered on a commercial flight to assess the safety to fly of a child with severe cystic lung disease secondary to Langerhans cell histiocytosis. The test did not result in an air leak, and the child subsequently undertook air travel without mishap. This is the first reported use of a hypobaric chamber test in a child with severe cystic lung disease. This test has the potential to be used as a fitness to fly test in children at risk for air leak syndromes who are being considered for air travel.

There is emerging evidence that cystic fibrosis lung disease begins early in infancy. Newborn screening allows early detection and surveillance of pulmonary disease and the possibility of early intervention in this life-shortening condition. We report two children with cystic fibrosis who underwent a comprehensive assessment from diagnosis that included measurement of lung function, limited-slice high-resolution CT and BAL performed annually. Early aggressive surveillance enabled significant lung disease and bronchiectasis to be detected during the first few years of life and led to a change in management, highlighting a clinical role for CT scanning during the preschool years in children with cystic fibrosis.

Many countries have introduced newborn screening for cystic fibrosis to facilitate diagnosis prior to the development of lung disease. Although most infants with cystic fibrosis are asymptomatic from a respiratory point of view at diagnosis, structural lung disease has been detected by computed tomography. We present a case of an asymptomatic infant with cystic fibrosis diagnosed following newborn screening who had endobronchial infection with Pseudomonas aeruginosa and radiological evidence of bronchiectasis at 3 months of age.

Better understanding of evolution of lung function in infants with cystic fibrosis (CF) and its association with pulmonary inflammation and infection is crucial in informing both early intervention studies aimed at limiting lung damage and the role of lung function as outcomes in such studies.To describe longitudinal change in lung function in infants with CF and its association with pulmonary infection and inflammation.Infants diagnosed after newborn screening or clinical presentation were recruited prospectively. FVC, forced expiratory volume in 0.5 seconds (FEV(0.5)), and forced expiratory flows at 75% of exhaled vital capacity (FEF(75)) were measured using the raised-volume technique, and z-scores were calculated from published reference equations. Pulmonary infection and inflammation were measured in bronchoalveolar lavage within 48 hours of lung function testing.Thirty-seven infants had at least two successful repeat lung function measurements. Mean (SD) z-scores for FVC were -0.8 (1.0), -0.9 (1.1), and -1.7 (1.2) when measured at the first visit, 1-year visit, or 2-year visit, respectively. Mean (SD) z-scores for FEV(0.5) were -1.4 (1.2), -2.4 (1.1), and -4.3 (1.6), respectively. In those infants in whom free neutrophil elastase was detected, FVC z-scores were 0.81 lower (P=0.003), and FEV(0.5) z-scores 0.96 lower (P=0.001), respectively. Significantly greater decline in FEV(0.5) z-scores occurred in those infected with Staphylococcus aureus (P=0.018) or Pseudomonas aeruginosa (P=0.021).In infants with CF, pulmonary inflammation is associated with lower lung function, whereas pulmonary infection is associated with a greater rate of decline in lung function. Strategies targeting pulmonary inflammation and infection are required to prevent early decline in lung function in infants with CF.

There is emerging evidence that cystic fibrosis lung disease begins early in infancy. Newborn screening allows early detection and surveillance of pulmonary disease and the possibility of early intervention in this life-shortening condition. We report two children with cystic fibrosis who underwent a comprehensive assessment from diagnosis that included measurement of lung function, limited-slice high-resolution CT and BAL performed annually. Early aggressive surveillance enabled significant lung disease and bronchiectasis to be detected during the first few years of life and led to a change in management, highlighting a clinical role for CT scanning during the preschool years in children with cystic fibrosis.

We aimed to determine whether myeloperoxidase (MPO) is the main peroxidase present in the airways of children with cystic fibrosis (CF) and to assess which oxidants it produces and whether they are associated with clinical features of CF. Children with CF (n=54) and without CF (n=16) underwent bronchoscopy and bronchoalveolar lavage (BAL) for assessment of pulmonary infection and inflammation. BAL fluid was analyzed for MPO, halogenated tyrosines as markers of hypohalous acids, thiocyanate, and protein carbonyls. MPO was the only peroxidase detected in BAL samples from children with CF and its concentration was markedly higher than in controls. Levels of 3-chlorotyrosine and 3-bromotyrosine in proteins were higher in the CF group. They correlated with neutrophils and MPO. The concentration of thiocyanate in BAL samples was below 1μM. Protein carbonyl levels correlated with MPO and halogenated tyrosines in patients with CF. Levels of MPO and halogenated tyrosines were higher in children with infections, especially Pseudomonas aeruginosa, and in the presence of respiratory symptoms. They also correlated with the Kanga clinical score. Our findings suggest that MPO produces hypobromous acid as well as hypochlorous acid in the airways of children with CF and that these oxidants are involved in the early pathogenesis of CF.