Transepithelial nasal potential difference (NPD) measurements assess ion conductance in the upper respiratory epithelium. NPD is useful in assisting in the diagnosis of classical and atypical cystic fibrosis (CF) and of cystic fibrosis transmembrane regulator (CFTR)-related disorders, as well as for monitoring the effect of pharmacological agents and gene transfer approaches to correct the abnormalities of ion transport in CF. The article summarizes the objectives and the principle of NPD measurements, describes a hands-on protocol of the procedure and provides quality control measures, practical hints and troubleshooting.
This is the first report quantifying the important impact of obstructive ventilatory disorders on lung function in very preterm infants at term. Besides its important role in preterm lung function consecutive overinflation could furthermore be shown to mask reduction of lung volume in these infants. Thus, body plethysmographic measurements seem to be an important diagnostic tool in preterm infants at term before hospital discharge in order to quantify ventilation disorders and to define therapeutic strategies.
Wegener's granulomatosis is together with Churg-Strauss syndrome and microscopic polyangiitis a very rare cause of hemoptysis in childhood. We report on a 14 years old girl who developed arthralgia, purpura, glomerulonephritis and a diffuse alveolar haemorrhage with hemoptysis after a short time period of unspecific symptoms.
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