Assessment of Liver Disease Progression in Cystic Fibrosis Using Transient Elastography

Abstract

The present study shows that the slope of worsening of liver stiffness is greater in patients who will develop CFLD, suggesting that annual transient elastography may be useful to detect risk of severe liver disease at an earlier stage.

Accuracy of Transient Elastography Data Combined With APRI in Detection and Staging of Liver Disease in Pediatric Patients With Cystic Fibrosis

“…Our model-generated cut-points require validation studies in a larger patient cohort, although liver biopsy in children with CF is not widely adopted. Lastly, this cross-sectional study does not address the utility of serial TE examinations even though this is an advantage of Fibroscan ® TE as highlighted by recent publications that demonstrated the clinical utility of serial LSM in predicting progression of CFLD 26,27 . The noninvasive nature of TE makes it an ideal tool to monitor progression of CFLD, however the failure of a significant number of patients progressing from CFnoLD to CFLD, or from mild CFLD to advanced fibrosis during the study period limited the potential to assess the predictive value of LSM and APRI.…”

Association Between Transient Elastography and Controlled Attenuated Parameter and Liver Ultrasound in Children With Cystic Fibrosis

“…(8) VCTE has been studied in patients with CF, but its interpretation is confounded by nonuniform definitions of CFLD as well as a wide spectrum of proposed cutoff values. (9)(10)(11)(12)(13)(14)(15) ELASTIC (Longitudinal Assessment of Transient Elastography in Cystic Fibrosis; clinicaltrials.gov: NCT03001388) is a nested study within the previously described Prediction by Ultrasound of the Risk of Hepatic Cirrhosis in Cystic Fibrosis (PUSH; clinicaltrials.gov: NCT01144507) study conducted by the CFLD Network (CFLD-NET). (16) Ultimately, the ELASTIC study will determine whether VCTE, when combined with US pattern characterization, can improve the identification of children with, and who will progress to, advanced CFLD.…”

“…( 8 ) VCTE has been studied in patients with CF, but its interpretation is confounded by nonuniform definitions of CFLD as well as a wide spectrum of proposed cutoff values. ( 9 , 10 , 11 , 12 , 13 , 14 , 15 )…”

Non-invasive assessment of paediatric cystic fibrosis liver disease and the role of microRNAs in disease mechanism

“…Fibroscan has been successfully used to demonstrate increase liver stiffness in several diseases including CFLD, biliary atresia or non-alcoholic fatty liver disease among others (105)(106)(107)(108)(109).…”

“…Liver stiffness progression was evaluated using SSWE in children with CF with more than one LSM taken in no less than a 12 month period. Following a similar strategy as performed using Fibroscan®-based transient elastography (105)…”