Paediatric Thoracic Imaging in Cystic Fibrosis in the Era of Cystic Fibrosis Transmembrane Conductance Regulator Modulation.

Autor:	O'Regan PW; Department of Radiology, Cork University Hospital, T12 DC4A Cork, Ireland.; Department of Radiology, School of Medicine, University College Cork, T12 AK54 Cork, Ireland., Stevens NE; Department of Surgery, Mercy University Hospital, T12 WE28 Cork, Ireland., Logan N; Department of Medicine, Mercy University Hospital, T12 WE28 Cork, Ireland., Ryan DJ; Department of Radiology, Cork University Hospital, T12 DC4A Cork, Ireland.; Department of Radiology, School of Medicine, University College Cork, T12 AK54 Cork, Ireland., Maher MM; Department of Radiology, Cork University Hospital, T12 DC4A Cork, Ireland.; Department of Radiology, School of Medicine, University College Cork, T12 AK54 Cork, Ireland.
Jazyk:	angličtina
Zdroj:	Children (Basel, Switzerland) [Children (Basel)] 2024 Feb 16; Vol. 11 (2). Date of Electronic Publication: 2024 Feb 16.
DOI:	10.3390/children11020256
Abstrakt:	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.
Databáze:	MEDLINE
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