Paediatric Thoracic Imaging in Cystic Fibrosis in the Era of Cystic Fibrosis Transmembrane Conductance Regulator Modulation
Cystic fibrosis (CF) is one of the most common progressive life-shortening genetic conditions worldwide. Ground-breaking translational research has generated therapies that target the primary cystic fibrosis transmembrane conductance regulator (CFTR) defect, known as CFTR modulators. A crucial aspec...
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| Format: | Article |
| Language: | English |
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MDPI AG
2024-02-01
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| Series: | Children |
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| Online Access: | https://www.mdpi.com/2227-9067/11/2/256 |
| _version_ | 1797298583904452608 |
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| author | Patrick W. O’Regan Niamh E. Stevens Niamh Logan David J. Ryan Michael M. Maher |
| author_facet | Patrick W. O’Regan Niamh E. Stevens Niamh Logan David J. Ryan Michael M. Maher |
| author_sort | Patrick W. O’Regan |
| collection | DOAJ |
| description | Cystic fibrosis (CF) is one of the most common progressive life-shortening genetic conditions worldwide. Ground-breaking translational research has generated therapies that target the primary cystic fibrosis transmembrane conductance regulator (CFTR) defect, known as CFTR modulators. A crucial aspect of paediatric CF disease is the development and progression of irreversible respiratory disease in the absence of clinical symptoms. Accurate thoracic diagnostics have an important role to play in this regard. Chest radiographs are non-specific and insensitive in the context of subtle changes in early CF disease, with computed tomography (CT) providing increased sensitivity. Recent advancements in imaging hardware and software have allowed thoracic CTs to be acquired in paediatric patients at radiation doses approaching that of a chest radiograph. CFTR modulators slow the progression of CF, reduce the frequency of exacerbations and extend life expectancy. In conjunction with advances in CT imaging techniques, low-dose thorax CT will establish a central position in the routine care of children with CF. International guidelines regarding the choice of modality and timing of thoracic imaging in children with CF are lagging behind these rapid technological advances. The continued progress of personalised medicine in the form of CFTR modulators will promote the emergence of personalised radiological diagnostics. |
| first_indexed | 2024-03-07T22:38:02Z |
| format | Article |
| id | doaj.art-cfb3149dc2e54fed9e8f6fac80faeb5d |
| institution | Directory Open Access Journal |
| issn | 2227-9067 |
| language | English |
| last_indexed | 2024-03-07T22:38:02Z |
| publishDate | 2024-02-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Children |
| spelling | doaj.art-cfb3149dc2e54fed9e8f6fac80faeb5d2024-02-23T15:12:31ZengMDPI AGChildren2227-90672024-02-0111225610.3390/children11020256Paediatric Thoracic Imaging in Cystic Fibrosis in the Era of Cystic Fibrosis Transmembrane Conductance Regulator ModulationPatrick W. O’Regan0Niamh E. Stevens1Niamh Logan2David J. Ryan3Michael M. Maher4Department of Radiology, Cork University Hospital, T12 DC4A Cork, IrelandDepartment of Surgery, Mercy University Hospital, T12 WE28 Cork, IrelandDepartment of Medicine, Mercy University Hospital, T12 WE28 Cork, IrelandDepartment of Radiology, Cork University Hospital, T12 DC4A Cork, IrelandDepartment of Radiology, Cork University Hospital, T12 DC4A Cork, IrelandCystic fibrosis (CF) is one of the most common progressive life-shortening genetic conditions worldwide. Ground-breaking translational research has generated therapies that target the primary cystic fibrosis transmembrane conductance regulator (CFTR) defect, known as CFTR modulators. A crucial aspect of paediatric CF disease is the development and progression of irreversible respiratory disease in the absence of clinical symptoms. Accurate thoracic diagnostics have an important role to play in this regard. Chest radiographs are non-specific and insensitive in the context of subtle changes in early CF disease, with computed tomography (CT) providing increased sensitivity. Recent advancements in imaging hardware and software have allowed thoracic CTs to be acquired in paediatric patients at radiation doses approaching that of a chest radiograph. CFTR modulators slow the progression of CF, reduce the frequency of exacerbations and extend life expectancy. In conjunction with advances in CT imaging techniques, low-dose thorax CT will establish a central position in the routine care of children with CF. International guidelines regarding the choice of modality and timing of thoracic imaging in children with CF are lagging behind these rapid technological advances. The continued progress of personalised medicine in the form of CFTR modulators will promote the emergence of personalised radiological diagnostics.https://www.mdpi.com/2227-9067/11/2/256cystic fibrosisradiographycomputed tomographymagnetic resonance imagingpaediatricscystic fibrosis transmembrane conductance regulator modulator |
| spellingShingle | Patrick W. O’Regan Niamh E. Stevens Niamh Logan David J. Ryan Michael M. Maher Paediatric Thoracic Imaging in Cystic Fibrosis in the Era of Cystic Fibrosis Transmembrane Conductance Regulator Modulation Children cystic fibrosis radiography computed tomography magnetic resonance imaging paediatrics cystic fibrosis transmembrane conductance regulator modulator |
| title | Paediatric Thoracic Imaging in Cystic Fibrosis in the Era of Cystic Fibrosis Transmembrane Conductance Regulator Modulation |
| title_full | Paediatric Thoracic Imaging in Cystic Fibrosis in the Era of Cystic Fibrosis Transmembrane Conductance Regulator Modulation |
| title_fullStr | Paediatric Thoracic Imaging in Cystic Fibrosis in the Era of Cystic Fibrosis Transmembrane Conductance Regulator Modulation |
| title_full_unstemmed | Paediatric Thoracic Imaging in Cystic Fibrosis in the Era of Cystic Fibrosis Transmembrane Conductance Regulator Modulation |
| title_short | Paediatric Thoracic Imaging in Cystic Fibrosis in the Era of Cystic Fibrosis Transmembrane Conductance Regulator Modulation |
| title_sort | paediatric thoracic imaging in cystic fibrosis in the era of cystic fibrosis transmembrane conductance regulator modulation |
| topic | cystic fibrosis radiography computed tomography magnetic resonance imaging paediatrics cystic fibrosis transmembrane conductance regulator modulator |
| url | https://www.mdpi.com/2227-9067/11/2/256 |
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