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The natural history of MECP2 duplication syndrome (MDS), a rare X-linked neurodevelopmental disorder with an estimated birth prevalence of 1/150,000 live births, is poorly understood due to a lack of clinical data collected for research. Such information is critical to the understanding of disease progression, therapeutic endpoints and outcome measures for clinical trials, as well as the development of therapies and orphan products.
Young people who are overweight/obese are more likely to display expFL during submaximal exercise compared with children of healthy weight.
To evaluate current ILF testing practices and to survey users regarding the indications, limitations and perceived clinical benefits of ILF testing.
Inert gas washout tests, performed using the single- or multiple-breath washout technique, were first described over 60 years ago.
Measurements of maximal voluntary inspiratory (Pi max) and expiratory (Pe max) pressures are used in the management of respiratory muscle disease...
Previous studies of pulmonary diffusing capacity in children differed greatly in methodologies; numbers of subjects evaluated, and were performed prior to...
We compared the ability of Ars, to standard oscillatory outcomes, to determine respiratory disease and differentiate responses to inhaled bronchial challenges.
Mosaic attenuation on expiratory chest computed tomography (CT) is common in early life cystic fibrosis (CF) and often referred to as "air trapping"
This is the largest collection of normative T LCO data, and the first global reference values available for T LCO.
The lung clearance index may be a useful surveillance tool for monitoring the presence and extent of lower airway inflammation and infection