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Imaging Lung Function Abnormalities in Primary Ciliary Dyskinesia Using Hyperpolarized Gas Ventilation MRI

by West, Noreen , Hughes, David , Moya, Eduardo , Schofield, Lynne , Stewart, Neil J. , Marshall, Helen , Rao, Madhwesha , Norquay, Graham , Wild, Jim M. , Smith, Laurie J. , Capener, David J. , Chan, Ho-Fung , Chetcuti, Phil , Hughes, Paul J. C. , Bray, Jody , Collier, Guilhem J. , Johns, Christopher S.

in Child / Ciliary Motility Disorders - diagnostic imaging / Ciliary Motility Disorders - physiopathology / Cystic fibrosis / Cysts / Defects / Dyskinesia / Female / Forced Expiratory Volume / Helium / Humans / Isotopes / Letters / Lung - diagnostic imaging / Lung - physiopathology / Lung diseases / Magnetic Resonance Imaging - methods / Male / Medical imaging / Morphology / NMR / Nuclear magnetic resonance / Pediatrics / Respiration, Artificial / Retrospective Studies / Spirometry / Thorax

2018

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Imaging Lung Function Abnormalities in Primary Ciliary Dyskinesia Using Hyperpolarized Gas Ventilation MRI

2018

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Imaging Lung Function Abnormalities in Primary Ciliary Dyskinesia Using Hyperpolarized Gas Ventilation MRI

2018

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Journal Article

Imaging Lung Function Abnormalities in Primary Ciliary Dyskinesia Using Hyperpolarized Gas Ventilation MRI

West, Noreen,

Hughes, David,

Moya, Eduardo,

Schofield, Lynne,

Stewart, Neil J.,

Marshall, Helen,

Rao, Madhwesha,

Norquay, Graham,

Wild, Jim M.,

Smith, Laurie J.,

Capener, David J.,

Chan, Ho-Fung,

Chetcuti, Phil,

Hughes, Paul J. C.,

Bray, Jody,

Collier, Guilhem J.,

Johns, Christopher S.

2018

Overview

Assessment of lung function in PCD may have a significant clinical overlap with cystic fibrosis (CF) (3), the aim being to identify small but clinically significant airways obstruction within the lung. Hyperpolarized gas ventilation magnetic resonance imaging (MRI) has been proven to be highly sensitive to early lung disease (7, 8), response to treatment (9), and the deterioration of lung function (10) in CF, and is well tolerated by children as young as 5 years of age (11). From these images two indices were calculated: 1) ventilation defect percentage (VDP), which quantifies the percentage of the lung volume that is not ventilated; and 2) the mean coefficient of variance of ventilated image signal intensity (CV), a metric of regional ventilation heterogeneity. 1H steady-state free precession magnetic resonance images were separately acquired for assessment of lung morphology and mucus (15). [...]ventilation defects are present in children with PCD even in the presence of normal LCI and FEV1.

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Publisher

Oxford University Press,American Thoracic Society

Subject

Child

/ Ciliary Motility Disorders - diagnostic imaging

/ Ciliary Motility Disorders - physiopathology

/ Cystic fibrosis

/ Cysts

/ Defects

/ Dyskinesia