BACKGROUND: Physical exercise training may form an important part of regular care for people with cystic fibrosis. This is an update of a previously published review. OBJECTIVES: To assess the effects of physical exercise training on exercise capacity by peak oxygen consumption, pulmonary function by forced expiratory volume in one second, health-related quality of life and further important patient-relevant outcomes in people with cystic fibrosis. SEARCH METHODS: We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Trials Register which comprises references identified from comprehensive electronic database searches and handsearches of relevant journals and abstract books of conference proceedings.Date of the most recent search: 04 May 2017.We searched ongoing trials registers (clinicaltrials.gov and the WHO ICTRP). Date of most recent search: 10 August 2017. SELECTION CRITERIA: All randomised and quasi-randomised controlled clinical trials comparing exercise training of any type and a minimum duration of two weeks with conventional care (no training) in people with cystic fibrosis. DATA COLLECTION AND ANALYSIS: Two authors independently selected studies for inclusion, assessed methodological quality and extracted data. The quality of the evidence was assessed using the GRADE system. MAIN RESULTS: Of the 83 studies identified, 15 studies which included 487 participants, met the inclusion criteria. The numbers in each study ranged from nine up to 72 participants; two studies were in adults, seven were in children and adolescents and six studies included all age ranges. Four studies of hospitalised participants lasted less than one month and 11 studies were outpatient-based, lasting between two months and three years. The studies included participants with a wide range of disease severity and employed differing levels of supervision with a mixture of types of training. There was also wide variation in the quality of the included studies.This systematic review shows very low-to low-quality evidence from both short-and long-term studies that in people with cystic fibrosis aerobic or anaerobic physical exercise training (or a combination of both) has a positive effect on aerobic exercise capacity, pulmonary function and health-related quality of life. No study reported on mortality; two studies reported on adverse events (moderate-quality evidence); one of each study reported on pulmonary exacerbations (low-quality evidence) and diabetic control (very low-quality evidence). Although improvements were not consistent between studies and ranged from no effects to clearly positive effects, the most consistent effects of the heterogeneous exercise training modalities and durations were found for maximal aerobic exercise capacity (in four out of seven studies) with unclear effects on forced expiratory volume in one second (in two out of 11 studies) and health-related quality of life (in two out of seven studies). AUTHORS' CONCLUSIONS: Evidence about the efficacy of physical exercise training in cystic fibros...

Three-dimensional respiratory tissue models have been generated using, for example, human primary airway epithelial cells (hAEC) or respective cell lines. To investigate ciliopathies, such as primary ciliary dyskinesia, the presence of functional kinocilia in vitro is an essential prerequisite. Since access to hAEC of healthy donors is limited, we aimed to identify a respiratory epithelial cell line that is capable to display functional kinocilia on at least 60% of the apical surface. Thus, we cultured four different human respiratory cell lines with human primary airway fibroblasts under airlift conditions, characterized the morphology, and analyzed ciliary function. Only one of the tested cell lines showed beating kinocilia; however, <10% of the whole surface was covered and ciliary beating was undirected. Positive control tissue models using hAEC and fibroblasts displayed expected directed ciliary beating pattern around 11 Hz. Our data show that the available cell lines are not suitable for basic and applied research questions whenever functional kinocilia are required and that, rather, hAEC-or human induced pluripotent stem cell-derived tissue models need to be generated.To study ciliopathies or Bordetella pertussis infection in vitro, three-dimensional respiratory tissue models with functional kinocilia covering at least 60% of the model's surface are mandatory. We cultured four respiratory cell lines on a fibroblastloaded biological scaffold and showed that none of them met this requirement. In contrast, primary airway cell-derived models sufficiently reflected the mucociliary phenotype. To further search for an alternative to primary respiratory cells, investigations on other cell lines should be conducted or even new cell lines have to be generated. IntroductionT hree-dimensional (3D) cell cultures of the respiratory epithelium/mucosa afford research on-for examplethe impact of airborne pollutants, host-pathogen interactions, and drug permeation. 1-5 As source for respiratory epithelial cells, primary cells, induced pluripotent stem cells (iPSC), or cell lines can be used. Both primary cells and iPSC provide the opportunity to generate personalized tissue models, for example, to study individual drug responses or drug efficacy. Moreover, they show a high in vitro-in vivo correlation. These models feature a pseudostratified epithelial morphology, barrier properties, basal cells, mucus-producing goblet cells, and ciliated cells facilitating mucociliary clearance. [6][7][8][9]

Necrotizing sarcoid granulomatosis (NSG) is characterized by pulmonary nodular infiltrates, a typical histology, and a benign clinical course. The etiology and pathogenesis of the disease are still unknown. In childhood, it is extremely rare, with only three reported cases so far. Here we report on an 8-year-old girl, who to our knowledge is the youngest reported patient with NSG. The girl presented with shortness of breath and a sore throat. Chest X-ray and computed tomography (CT) scan revealed multiple nodular opacities of the lung. The symptoms and radiological findings disappeared within 6 months without any treatment. The diagnosis was based on the typical signs and symptoms of NSG and on the exclusion of other diseases. As abnormal immunological findings such as the lack of specific diphtheria antibodies in spite of vaccination against diphtheria were present, we suggest that immunologic mechanisms could play an etiologic role in the pathogenesis of NSG. In addition, the ratio of CD4+/CD8+ T-cells in the peripheral blood was significantly reduced, whereas the CD4+/CD8+ T-cell ratio in the immunohistochemical staining of the lung tissue was elevated. Since this compartmentalization is a typical finding in sarcoidosis, it supports the theory that NSG may represent a variant of sarcoidosis. However, because some characteristics of NSG are uncommon in typical sarcoidosis, NSG may also be an entity in its own right.

Background: Persistent tachypnea of infancy (PTI) is a rare pediatric lung disease of unknown origin. The diagnosis can be made by clinical presentation and chest high resolution computed tomography after exclusion of other causes. Clinical courses beyond infancy have rarely been assessed. Methods: Patients included in the Kids Lung Register diagnosed with PTI as infants and now older than 5 years were identified. Initial presentation, extrapulmonary comorbidities, spirometry and clinical outcome were analyzed. Results: Thirty-five children older than 5 years with PTI diagnosed as infants were analyzed. At the age of 5 years, 74% of the patients were reported as asymptomatic and did not develope new symptoms during the observational period at school-age (mean, 3.9 years; range, 0.3-6.3). At the age of about 10 years, none of the symptomatic children had abnormal oxygen saturation during sleep or exercise anymore.