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Cystic fibrosis in the 21st century: what every radiologist should know

  • Author Footnotes
    1 These authors contributed equally to this work.
    Oussama Hallouch
    Footnotes
    1 These authors contributed equally to this work.
    Affiliations
    Faculty of Medicine - Department of Radiology, Radiation Oncology and Nuclear Medicine, Université de Montréal, Montreal, Quebec, Canada
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  • Author Footnotes
    1 These authors contributed equally to this work.
    John Marinos
    Footnotes
    1 These authors contributed equally to this work.
    Affiliations
    Faculty of Medicine - Department of Radiology, Radiation Oncology and Nuclear Medicine, Université de Montréal, Montreal, Quebec, Canada
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  • Florence Thibault
    Affiliations
    Faculty of Medicine - Department of Radiology, Radiation Oncology and Nuclear Medicine, Université de Montréal, Montreal, Quebec, Canada
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  • Kim-Nhien Vu
    Affiliations
    Faculty of Medicine - Department of Radiology, Radiation Oncology and Nuclear Medicine, Université de Montréal, Montreal, Quebec, Canada
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  • Jean Chalaoui
    Affiliations
    Faculty of Medicine - Department of Radiology, Radiation Oncology and Nuclear Medicine, Université de Montréal, Montreal, Quebec, Canada
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  • Patrick Bourgouin
    Affiliations
    Faculty of Medicine - Department of Radiology, Radiation Oncology and Nuclear Medicine, Université de Montréal, Montreal, Quebec, Canada
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  • Laurence Péloquin
    Affiliations
    Faculty of Medicine - Department of Radiology, Radiation Oncology and Nuclear Medicine, Université de Montréal, Montreal, Quebec, Canada
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  • Véronique Freire
    Affiliations
    Faculty of Medicine - Department of Radiology, Radiation Oncology and Nuclear Medicine, Université de Montréal, Montreal, Quebec, Canada
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  • François Tremblay
    Affiliations
    Faculty of Medicine - Department of Medicine, Université de Montréal, Montreal, Quebec, Canada
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  • Carl Chartrand-Lefebvre
    Correspondence
    Corresponding author at: CHUM - Centre Hospitalier de l'Université de Montréal, Université de Montréal, Faculty of Medicine - Department of Radiology, Radiation Oncology and Nuclear Medicine, 1051 Rue Sanguinet, Montréal H2X 3E4, Quebec, Canada.
    Affiliations
    Faculty of Medicine - Department of Radiology, Radiation Oncology and Nuclear Medicine, Université de Montréal, Montreal, Quebec, Canada
    Search for articles by this author
  • Author Footnotes
    1 These authors contributed equally to this work.
Published:February 12, 2022DOI:https://doi.org/10.1016/j.clinimag.2022.02.004

      Highlights

      • Review the pathophysiology, genetics, demographics, diagnosis, and treatment of cystic fibrosis (CF)
      • Demonstrate the role of imaging (radiograph, CT, MR, ultrasound) in the diagnosis and management of patients with CF
      • Learn the appropriate imaging follow-up for these patients
      • Help improve radiology reports for patients with CF
      • Educate about recent advances in research

      Abstract

      Cystic Fibrosis (CF) is the most common lethal genetic disorder in Caucasian populations, affecting roughly 70,000 individuals worldwide. This autosomal recessive disorder causes a wide spectrum of multisystemic manifestations, most of which are either directly or indirectly related to defective epithelial chloride secretion. The current median life expectancy is 44 years; however, a significant proportion of the CF population now live into the 5th decade and beyond due to advances in treatment. As life expectancy of CF patients increases, there is a newly emerging adult CF population with unique radiological manifestations spanning multiple organ systems, which often require follow-up imaging. The goal of this article is to review the multiple systemic manifestations and complications of CF on different imaging modalities and explore the appropriate radiological follow up recommended.

      Keywords

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