Quantitative Imaging for Diagnosis of Cystic Fibrosis-Associated Hepatobiliary, Pancreatic, and Splenic Disease
Study ID: STU-2022-0213
Summary
eligible patients include those persons with a diagnosis of cystic fibrosis who are either seen at uTSW and Children's Health or are an inpatient and registered with the Cystic Fibrosis Foundation Registry. Those who opt for enrollment in the study will have medical history and relevant pathology, lab, liver function, and imaging data retrospectively retrieved from their eMR. Prospectively, patients may undergo noninvasive quantitative imaging tests, which may include vibration controlled transient elastography (VCTe) of the liver/spleen and/or quantitative MRi (T1 relaxation value mapping (corrected for the effects of iron x cT1), proton density fat fraction, elastography, and/or quantitative magnetic resonance cholangiopancreatography). These techniques are FDa-cleared for clinical use and will be used within the scope of their respective FDa labeling.
The purpose of this study is to evaluate the feasibility of noninvasive and objective diagnosis and disease monitoring of cystic fibrosis associated hepatobiliary, pancreatic, and splenic disease using conventional diagnostic imaging and advanced quantitative imaging. We hope to determine the degree of association between conventional diagnostic imaging and quantitative imaging findings of disease presence and severity derived from ultrasound, vibration controlled transient elastography (VCTe), magnetic resonance imaging (MRi), and serum and tissue-based markers of cystic fibrosis associated hepatobiliary, pancreatic, and splenic disease and characterize the measurement variations of quantitative imaging techniques in patients with CF.
