Published ahead of print on January 7, 2005, doi:10.1164/rccm.200408-1116OC Am. J. Respir. Crit. Care Med., Volume 171, Number 9, May 2005, 1015-1019 A more recent version of this article appeared on May 1, 2005
Submitted on August 31, 2004 Potential Difference Measurements in the Lower Airway of Children with and without Cystic FibrosisJane C Davies1*,1 Department of Gene Therapy, National Heart and Lung Institute, Imperial College London, London, United Kingdom; Department of Paediatric Respiratory Medicine, Royal Brompton Hospital, London, United Kingdom; UK Cystic Fibrosis Gene Therapy Consortium, London, United Kingdom, 2 Department of Gene Therapy, National Heart and Lung Institute, Imperial College London, London, United Kingdom; Department of Thoracic Medicine, Royal Brompton Hospital, London, United Kingdom, 3 Department of Gene Therapy, National Heart and Lung Institute, Imperial College London, London, United Kingdom; Department of Paediatric Respiratory Medicine, Royal Brompton Hospital, London, United Kingdom, 4 Department of Gene Therapy, National Heart and Lung Institute, Imperial College London, London, United Kingdom; UK Cystic Fibrosis Gene Therapy Consortium, London, United Kingdom, 5 Department of Gene Therapy, National Heart and Lung Institute, Imperial College London, London, United Kingdom, 6 Department of Paediatric Respiratory Medicine, Royal Brompton Hospital, London, United Kingdom, 7 Department of Anaesthesia, Royal Brompton Hospital, London, United Kingdom, 8 Department of Cardiothoracic Surgery, Royal Brompton Hospital, London, United Kingdom, 9 Department of Thoracic Medicine, Royal Brompton Hospital, London, United Kingdom; UK Cystic Fibrosis Gene Therapy Consortium, London, United Kingdom, 10 Department of Paediatric Respiratory Medicine, Royal Brompton Hospital, London, United Kingdom; UK Cystic Fibrosis Gene Therapy Consortium, London, United Kingdom * To whom correspondence should be addressed. E-mail: j.c.davies{at}ic.ac.uk.
Nasal potential difference measurements are valuable end-point assays in clinical studies of novel treatments for cystic fibrosis. Similar measurements made on the lower airway via the bronchoscope have been successful in adults, but have not been reported in children, the group most likely to benefit from such therapies. Here we report the design and validation of a small, single lumen catheter technique allowing baseline potential difference and chloride secretion to be assessed in the distal airways of children as young as a year of age. Tracheal baseline values were significantly higher in CF than non-CF children, although this was not the case more distally. In airways between the third and seventh generation, perfusion with a zero chloride solution containing isoprenaline led to a significant change in PD in children without CF, whereas no change was seen in those with CF. This measure provided a reliable distinguishing test between the two disease groups. We confirm that invasive bronchoscopic techniques can be performed safely and reliably in small children. Potential difference measurements could form a useful functional end-point assay for future studies of either CFTR gene or protein-based therapies in future trials in the paediatric age group. Key words: ion transport, airway, child
This article has been cited by other articles:
|
|
||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
