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At least in the first 2 years of life, lung function is not a good end-point for RCTs; routine bronchoalveolar lavage and HRCT cannot be justified. Newborn screening has greatly improved outcomes, but we need better point-of-care biomarkers.
At least in the first 2 years of life, lung function is not a good end-point for RCTs; routine bronchoalveolar lavage and HRCT cannot be justified. Newborn screening has greatly improved outcomes, but we need better point-of-care biomarkers.
Regular physical activity and exercise have become important components of cystic fibrosis care. This review summarizes the recent evidence in favour of regular exercise in cystic fibrosis that has accumulated over the past years.
Several recently published small randomized-controlled trials and observational studies have added to our knowledge on positive effects of exercise training on pulmonary function and aerobic fitness in cystic fibrosis. Relevant outcomes, such as body posture, health-related quality of life and rate of hospitalization, are increasingly studied. Findings from these studies suggest that exercise might also be beneficial for these outcomes. So far, many important questions such as the best way of integrating exercise in cystic fibrosis care and the determination of the optimal strategies for training and motivation remain mostly unanswered.
Over the past years, evidence for the beneficial effects of regular exercise on lung health and aerobic exercise capacity is strengthening. Despite the fact that most of the knowledge is based on small studies, the observed effects are encouraging and there is no reason why exercise should not be implemented in all patients' care.
Over the past years, evidence for the beneficial effects of regular exercise on lung health and aerobic exercise capacity is strengthening. Despite the fact that most of the knowledge is based on small studies, the observed effects are encouraging and there is no reason why exercise should not be implemented in all patients' care.
MRI has emerged as radiation-free imaging modality for assessment of cystic fibrosis lung disease. Here, we review the current status and new developments of MRI in cystic fibrosis.
MRI is sensitive to detect abnormalities in lung structure and perfusion, even in preschool children with cystic fibrosis with early lung disease. Further, MRI is sensitive to detect changes associated with pulmonary exacerbations and response to antibiotic therapy in this challenging age group.
MRI is sensitive to detect hallmarks of cystic fibrosis lung disease such as bronchial wall thickening, bronchiectasis, mucus plugging and abnormal lung perfusion. A morpho-functional MRI score has been established for semiquantitative assessment of these characteristic abnormalities over a broad range of disease severity. Recent studies demonstrated that MRI is sensitive to detect changes in mucus plugging and lung perfusion in response to antibiotic therapy for pulmonary exacerbations. These results suggest that MRI may be suitableurier decomposition imaging are expected to enhance morphological and functional MRI of cystic fibrosis lung disease without the need of contrast medium in the near future.
The purpose of this review was to evaluate the consequence of obstructive sleep apnea (OSA) in pulmonary hypertension by reviewing the current literature and understanding potential pathophysiological mechanisms.
Small studies have suggested a high prevalence of comorbid OSA in those with known pulmonary hypertension. Pathophysiological mechanisms are highly suggestive of potential deleterious effect of OSA on pulmonary hemodynamics.
Clearly, current research work on comorbid OSA and pulmonary hypertension is still in its infancy and the field is ripe for future investigation. The significance of OSA in this population has yet to be fully determined.
Clearly, current research work on comorbid OSA and pulmonary hypertension is still in its infancy and the field is ripe for future investigation. The significance of OSA in this population has yet to be fully determined.
The current paradigm shift in the diagnosis of sleep apnea in adults has further emphasized the urgent need for the development and validation of less inconvenient and laborious approaches than the in-laboratory nocturnal polysomnography for evaluation of children.
These efforts have been primarily centered around the following first, refinements and validation of questionnaires; second, single-channel recordings such as oximetry, airflow, or ECG; third, home-based polysomnography and polygraphy; and fourth, biomarkers. The major overall findings emanating from such studies indicate that none of the approaches provides an ideal substitute to in-laboratory nocturnal polysomnography. Conversely, many of the proposed approaches enable effective screening in a cost-effective manner, and may be particularly suitable when access to pediatric sleep medicine facilities is limited or unavailable.
The overall improvements in technologies and in our understanding of pediatric sleep-disordered breathing should enable population-tailored effective home-based diagnostic approaches that reduce the overall burden to the family, while achieving high levels of diagnostic accuracy. Newer algorithms will have to be developed and validated to allow for effective implementation of such approaches.
The overall improvements in technologies and in our understanding of pediatric sleep-disordered breathing should enable population-tailored effective home-based diagnostic approaches that reduce the overall burden to the family, while achieving high levels of diagnostic accuracy. Newer algorithms will have to be developed and validated to allow for effective implementation of such approaches.This study established the between-days reliability and sensitivity of a countermovement jump (CMJ), plyometric push-up, well-being questionnaire, and whole-blood creatine kinase concentration ([CK]) in elite male youth rugby union players. The study also established the between-days reliability of 1, 2, or 3 CMJs and plyometric-push-up attempts. Twenty-five players completed tests on 2 occasions separated by 5 d (of rest). Between-days typical error, coefficient of variation (CV), and smallest worthwhile change (SWC) were calculated for the well-being questionnaire, [CK], and CMJ and plyometric-push-up metrics (peak/mean power, peak/mean force, height, flight time, and flight-time to contraction-time ratio) for 1 maximal effort or taking the highest score from 2 or 3 maximal efforts. The results suggest that CMJ mean power (2 or 3 attempts), peak force, or mean force and plyometric-push-up mean force (from 2 or 3 attempts) should be used for assessing lower- and upper-body neuromuscular function, respectively, due to both their acceptable reliability (CV SWC). The findings from this study can be used when interpreting fatigue markers to make an objective decision about a player's readiness to train or compete.Proteins and synthetic polymers that undergo aqueous phase transitions mediate self-assembly in nature and in man-made material systems. Yet little is known about how the phase behaviour of a protein is encoded in its amino acid sequence. Here, by synthesizing intrinsically disordered, repeat proteins to test motifs that we hypothesized would encode phase behaviour, we show that the proteins can be designed to exhibit tunable lower or upper critical solution temperature (LCST and UCST, respectively) transitions in physiological solutions. We also show that mutation of key residues at the repeat level abolishes phase behaviour or encodes an orthogonal transition. Furthermore, we provide heuristics to identify, at the proteome level, proteins that might exhibit phase behaviour and to design novel protein polymers consisting of biologically active peptide repeats that exhibit LCST or UCST transitions. These findings set the foundation for the prediction and encoding of phase behaviour at the sequence level.Natural composites are often heterogeneous to fulfil functional demands. Manufacturing analogous materials remains difficult, however, owing to the lack of adequate and easily accessible processing tools. https://www.selleckchem.com/products/abt-199.html Here, we report an additive manufacturing platform able to fabricate complex-shaped parts exhibiting bioinspired heterogeneous microstructures with locally tunable texture, composition and properties, as well as unprecedentedly high volume fractions of inorganic phase (up to 100%). The technology combines an aqueous-based slip-casting process with magnetically directed particle assembly to create programmed microstructural designs using anisotropic stiff platelets in a ceramic, metal or polymer functional matrix. Using quantitative tools to control the casting kinetics and the temporal pattern of the applied magnetic fields, we demonstrate that this approach is robust and can be exploited to design and fabricate heterogeneous composites with thus far inaccessible microstructures. Proof-of-concept examples include bulk composites with periodic patterns of microreinforcement orientation, and tooth-like bilayer parts with intricate shapes exhibiting site-specific composition and texture.Crystallography, the primary method for determining the 3D atomic positions in crystals, has been fundamental to the development of many fields of science. However, the atomic positions obtained from crystallography represent a global average of many unit cells in a crystal. Here, we report, for the first time, the determination of the 3D coordinates of thousands of individual atoms and a point defect in a material by electron tomography with a precision of ∼19 pm, where the crystallinity of the material is not assumed. From the coordinates of these individual atoms, we measure the atomic displacement field and the full strain tensor with a 3D resolution of ∼1 nm(3) and a precision of ∼10(-3), which are further verified by density functional theory calculations and molecular dynamics simulations. The ability to precisely localize the 3D coordinates of individual atoms in materials without assuming crystallinity is expected to find important applications in materials science, nanoscience, physics, chemistry and biology.Topological defects in liquid crystals (LCs) have been widely used to organize colloidal dispersions and template polymerization, leading to a range of assemblies, elastomers and gels. However, little is understood about molecular-level assembly processes within defects. Here, we report that nanoscopic environments defined by LC topological defects can selectively trigger processes of molecular self-assembly. By using fluorescence microscopy, cryogenic transmission electron microscopy and super-resolution optical microscopy, we observed signatures of molecular self-assembly of amphiphilic molecules in topological defects, including cooperativity, reversibility and controlled growth. We also show that nanoscopic o-rings synthesized from Saturn-ring disclinations and other molecular assemblies templated by defects can be preserved by using photocrosslinkable amphiphiles. Our results reveal that, in analogy to other classes of macromolecular templates such as polymer-surfactant complexes, topological defects in LCs are a versatile class of three-dimensional, dynamic and reconfigurable templates that can direct processes of molecular self-assembly.
