Correlation of Transient Elastography With Severity of Cystic Fibrosis Related Liver Disease

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

Research output: Contribution to journalArticle

21 Scopus citations


OBJECTIVE:: To evaluate whether liver stiffness measurement (LSM), determined by transient elastography (TE), correlates with presence and severity of liver disease (LD) in children and young adults with cystic fibrosis (CF). METHODS:: Subjects underwent LSM at routine CF visits. Presence and severity of CFLD was determined by clinical parameters. Subjects were classified as no CFLD, CFLD without portal hypertension (PHTN), and CFLD with PHTN. LSM was compared to AST/platelet ratio index (APRI) as a correlate to severity of CFLD. RESULTS:: 249 subjects [53%male; mean age 14?±?7y; 7(3%)?<?2y and 74(30%) 18–25y] 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; IQR) LSM was different among the three 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 (IQR) APRI was different in groups 1 and 2 compared to CFLD with portal hypertension: 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) vs. 0.78 (APRI) (P?=?0.05). CONCLUSIONS:: Liver stiffness, as determined by TE, 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.

Original languageEnglish (US)
JournalJournal of Pediatric Gastroenterology and Nutrition
StateAccepted/In press - Oct 25 2016

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Gastroenterology

Fingerprint Dive into the research topics of 'Correlation of Transient Elastography With Severity of Cystic Fibrosis Related Liver Disease'. Together they form a unique fingerprint.

  • Cite this