Clinical utility of surveillance computed tomography scans in infants with cystic fibrosis

Newbegin, Katy; Pilkington, Katie; Shanthikumar, Shivanthan; Ranganathan, Sarath

doi:10.1002/ppul.24132

Cited by 11 publications

(15 citation statements)

References 12 publications

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“…As discussed above, CXR is relatively insensitive to mild focal disease (Figure 1). Cross‐sectional imaging via chest CT offers higher resolution and greater detail regarding the presence of regional anatomic abnormalities than CXR and is increasingly being used to monitor disease progression and make treatment decisions 5,23,24 . However, because of the great variability in scan protocols and acquisition timing, 5,25 there are still inconsistencies in the way data are interpreted.…”

Section: Computed Tomographymentioning

confidence: 99%

Novel imaging techniques for cystic fibrosis lung disease

Goralski

Stewart

Woods

2021

Pediatric Pulmonology

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With an increasing number of patients with cystic fibrosis (CF) receiving highly effective CFTR (cystic fibrosis transmembrane regulator protein) modulator therapy, particularly at a young age, there is an increasing need to identify imaging tools that can detect and regionally visualize mild CF lung disease and subtle changes in disease state. In this review, we discuss the latest developments in imaging modalities for both structural and functional imaging of the lung available to CF clinicians and researchers, from the widely available, clinically utilized imaging methods for assessing CF lung disease—chest radiography and computed tomography—to newer techniques poised to become the next phase of clinical tools—structural/functional proton and hyperpolarized gas magnetic resonance imaging (MRI). Finally, we provide a brief discussion of several newer lung imaging techniques that are currently available only in selected research settings, including chest tomosynthesis, and fluorinated gas MRI. We provide an update on the clinical and/or research status of each technique, with a focus on sensitivity, early disease detection, and possibilities for monitoring treatment efficacy.

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Section: Computed Tomographymentioning

confidence: 99%

Novel imaging techniques for cystic fibrosis lung disease

Goralski

Stewart

Woods

2021

Pediatric Pulmonology

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“…At present, the limitations of XV appear to be largely centered on the need to use ionizing radiation. Although the dose per scan is much lower than the CT scans currently used for monitoring structural lung disease progression in children with CF, 95 radiation effects are cumulative. XV analysis also currently requires a chest CT to determine the boundaries of the lung, however, a prior CT can be used to minimize the additional radiation exposure to the patient.…”

Section: X-ray Velocimetrymentioning

confidence: 99%

Will Airway Gene Therapy for Cystic Fibrosis Improve Lung Function? New Imaging Technologies Can Help Us Find Out

Parsons

Donnelley

2020

Human Gene Therapy

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The promise of genetic therapies has turned into reality in recent years, with new first-line treatments for fatal diseases now available to patients. The development and testing of genetic therapies for respiratory diseases such as cystic fibrosis (CF) has also progressed. The addition of gene editing to the genetic agent toolbox, and its early success in other organ systems, suggests we will see rapid expansion of gene correction options for CF in the future. Although substantial progress has been made in creating techniques and genetic agents that can be highly effective for CF correction in vitro, physiologically relevant functional in vivo changes have been largely prevented by poor delivery efficiency within the lungs. Somewhat hidden from view, however, is the absence of reliable, accurate, detailed, and noninvasive outcome measures that can detect subtle disease and treatment effects in the lungs of humans or animal models. The ability to measure the fundamental function of the lung-ventilation, the effective transport of air throughout the lung-has been constrained by the available measurement technologies. Without sensitive measurement methods, it is difficult to quantify the effectiveness of genetic therapies for CF. The mainstays of lung health assessment are spirometry, which cannot provide adequate disease localization and is not sensitive enough to detect small early changes in disease; and computed tomography, which provides structural rather than functional information. Magnetic resonance imaging using hyperpolarized gases is increasingly useful for lung ventilation assessment, and it removes the radiation risk that accompanies X-ray methods. A new lung imaging technique, X-ray velocimetry, can now offer highly detailed regional lung ventilation information well suited to the diagnosis, treatment, and monitoring needs of CF lung disease, particularly after the application of genetic therapies. In this review, we discuss the options now available for imaging-based lung function measurement in the generation and use of genetic and other therapies for treating CF lung disease.

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“…It has been established that radiographic evaluation of the lungs can demonstrate abnormalities before alterations in lung function. Evaluation of results of CT scan findings in infants were found to be abnormal in 75% of scans . Changes in care management were made based on CT scans in 36% at age 1 year of age and 26% at 3 years of age, with bronchiectasis as the most common reason for change in management.…”

Section: Infection Detection and Epidemiologymentioning

confidence: 99%

“…Evaluation of results of CT scan findings in infants were found to be abnormal in 75% of scans. 79 Changes in care management were made based on CT scans in 36% at age 1 year of age and 26% at 3 years of age, with bronchiectasis as the most common reason for change in management. While abnormalities may lead to clinical care changes, there continues to be concern regarding the safety of radiation, and more studies using magnetic resonance imaging of the lungs has emerged.…”

Section: Imagingmentioning

confidence: 99%