The European respiratory journal : official journal of the European Society for Clinical Respiratory Physiology
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Comparative Study
Protocols for in vivo measurement of the ion transport defects in cystic fibrosis nasal epithelium.
New treatments for cystic fibrosis (CF), including gene therapy, are currently being assessed. These aim to correct the basic defects of increased sodium absorption and decreased chloride secretion in airway epithelia. Assessment of these bioelectric parameters, particularly in the nasal epithelium, is likely to be used as a measure of treatment efficacy. ⋯ The responses both to terbutaline (delta PD: non-CF: -0.8 (SEM 0.7) mV; CF: -3.6 (0.5) mV) and isoprenaline (non-CF: 1.5 (0.6) mV; CF: -2.9 (0.6) mV) differentiated the two groups of subjects, but there was considerable overlap of values. Perfusion with a low chloride solution (non-CF: 12.6 (1.2) mV; CF: 0.6 (0.4) mV), as well as subsequent perfusion with isoprenaline (non-CF: 10.0 (1.1) mV; CF: -1.4 (0.4) mV) allowed clear separation of the two groups, with no overlap of values. Some CF subjects showed a transient hyperpolarization to these stimuli, which could clearly be differentiated from the sustained responses seen in non-cystic fibrosis subjects.(ABSTRACT TRUNCATED AT 250 WORDS)
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A patient with platypnoea after right pneumonectomy and radiotherapy is described. On transoesophageal contrast echocardiography and cardiac catheterization, an atrial septal aneurysm with interatrial right-to-left shunting was detected. Symptoms disappeared after surgical correction. To the best of our knowledge, this is the first report of a patient with a septal aneurysm and severe platypnoea.