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Correlation of Transient Elastography With Severity of Cystic Fibrosis–related Liver Disease

Aqul, Amal*; Jonas, Maureen M.; Harney, Sarah; Raza, Roshan; Sawicki, Gregory S.§; Mitchell, Paul D.; Fawaz, Rima

Journal of Pediatric Gastroenterology and Nutrition: April 2017 - Volume 64 - Issue 4 - p 505–511
doi: 10.1097/MPG.0000000000001448
Original Articles: Hepatology

Objectives: The aim of the study was to evaluate whether liver stiffness measurement (LSM), determined by transient elastography, correlates with presence and severity of liver disease in children and young adults with cystic fibrosis (CF).

Methods: Subjects underwent LSM at routine CF visits. Presence and severity of cystic fibrosis liver disease (CFLD) was determined by clinical parameters. Subjects were classified as no CFLD, CFLD without portal hypertension (PHTN), and CFLD with PHTN. LSM was compared with aspartate aminotransferase/platelet ratio index (APRI) as a correlate to severity of CFLD.

Results: A total of 249 subjects (53% boys; mean age 14 ± 7 years; 7 [3%] <2 years and 74 [30%] 18–25 years) underwent LSM. Subjects were classified as 158 (64%) with no CFLD, 73 (29%) CFLD without PHTN, and 18 (7%) CFLD with PHTN. The median (interquartile range) LSM was different among the 3 groups: 4.4 (3.8–5.4), 5.1 (4.4–6.3), and 14.1 (8.8–24.8) kPa, respectively, with all pairwise comparisons different from one another (P < 0.0001). Similarly, median (interquartile range) APRI was different in groups 1 and 2 compared with CFLD with PHTN: 0.22 (0.17–0.27), 0.24 (0.17–0.33), and 0.53 (0.24–0.84), respectively (P < 0.01). Analysis of receiver operating characteristics for discriminating CFLD with PHTN from the other groups resulted in cut-points at 6.2 kPa (LSM) and 0.35 (APRI). LSM was superior to APRI in discriminating CFLD with PHTN from other groups, with areas under the curve 0.91 (LSM) versus 0.78 (APRI) (P = 0.05).

Conclusions: Liver stiffness, as determined by transient elastography, correlates with the presence and severity of CFLD. Although APRI provided some information regarding severity of liver disease, LSM performed better than APRI in this population.

*Division of Gastroenterology, Hepatology, and Nutrition, University of Texas Southwestern Medical Center, Dallas

Division of Gastroenterology, Hepatology, and Nutrition

Clinical Research Center

§Division of Respiratory Diseases, Cystic Fibrosis Center, Boston Children's Hospital, Boston, MA.

Address correspondence and reprint requests to Rima Fawaz, MD, Boston Children's Hospital, 300 Longwood Ave, Boston, MA 02115 (e-mail:

Received 3 September, 2015

Accepted 19 October, 2016

Echosens (Paris, France) provided the FibroScan machine, technical support, and training of investigators for the purpose of this study. Echosens had no role in study design, collection/analysis/interpretation of data, writing the manuscript, or the decision to submit the manuscript for publication. Intramural funding was provided by the Division of Gastroenterology, Hepatology and Nutrition and the Cystic Fibrosis Center at Boston Children's Hospital.

Portions of this study were presented at The Liver Meeting (the annual meeting of the American Association for the Study of Liver Diseases) on October 31, 2013 in Washington, DC.

The authors report no conflicts of interest.

© 2017 by European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology,