Cystic Fibrosis

Cystic Fibrosis

 

    Cystic fibrosis (CF) is an autosomal recessive disorder caused by mutations encoding the cystic fibrosis transmembrane conductance regulator (CFTR) protein, affecting approximately 70,000 people worldwide. CF leads to progressive obstructive lung disease, which is the primary cause of disease morbidity and mortality in this patient population.  Tools to monitor CF lung disease and therapeutic responses are currently limited in young patients with particularly sensitivity to subclinical or regional disease.  With ultrashort echo time (UTE) MRI, we were able to identify CF-specific lung structural abnormalities caused by bronchiectasis, bronchial wall thickening, mucus plugging, ground glass opacity, and consolidation.  UTE MRI involves no ionizing radiation and its continuing development places this technique as a potential longitudinal imaging surrogate for CT.

 

Through the combination of UTE MRI and hyperpolarized 129Xe MRI we are able to identify structure-function relationships within cystic fibrosis patients.

 

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