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

Patrick W O'Regan1,2, Niamh E Stevens3, Niamh Logan4

  • 1Department of Radiology, Cork University Hospital, T12 DC4A Cork, Ireland.

Children (Basel, Switzerland)
|February 24, 2024
PubMed

Insights

New therapies called CFTR modulators slow cystic fibrosis progression. Low-dose CT scans offer sensitive thoracic diagnostics for children with CF, improving early detection and personalized care.

Area of Science:

  • Medical Imaging
  • Pulmonology
  • Genetics

Background:

  • Cystic fibrosis (CF) is a common, progressive, life-shortening genetic disorder.
  • Early respiratory disease in children with CF can develop before symptoms appear.
  • Traditional chest X-rays lack sensitivity for early CF lung changes.

Purpose of the Study:

  • To highlight the role of advanced thoracic imaging in diagnosing early cystic fibrosis lung disease in children.
  • To discuss the impact of novel CFTR modulator therapies on CF management and the need for tailored diagnostics.
  • To emphasize the potential of low-dose computed tomography (CT) in pediatric CF care.

Main Methods:

  • Review of current literature on cystic fibrosis diagnostics and therapeutics.
  • Analysis of the advancements in computed tomography (CT) technology for pediatric imaging.
  • Comparison of sensitivity and radiation dose between chest radiography and low-dose CT.

Main Results:

  • Computed tomography (CT) offers superior sensitivity for detecting subtle early lung changes in pediatric CF compared to chest radiographs.
  • Recent technological advancements enable low-dose thoracic CT scans in children with radiation doses comparable to standard chest X-rays.
  • CFTR modulators are improving patient outcomes, necessitating personalized diagnostic approaches.

Conclusions:

  • Low-dose thoracic CT is poised to become a cornerstone in the routine management of pediatric cystic fibrosis.
  • Personalized radiological diagnostics are emerging in parallel with personalized medicine (CFTR modulators).
  • International guidelines need updating to reflect rapid advancements in imaging technology and CF treatment.

Keywords:
computed tomographycystic fibrosiscystic fibrosis transmembrane conductance regulator modulatormagnetic resonance imagingpaediatricsradiography

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