Role of Transient Elastography and APRI in the Assessment of Pediatric Cystic Fibrosis Liver Disease

Overview

Journal Can Liver J

Specialty Gastroenterology

Date 2022 Aug 22

PMID 35991474

Authors

Jessica P Woolfson

Richard A Schreiber

Shraavan Raveendran

Mark Chilvers

Collin Barker

Orlee R Guttman

Affiliations

Soon will be listed here.

Abstract

Background: Diagnosis and monitoring of cystic fibrosis liver disease (CFLD) is challenging. Transient elastography (TE) is a rapid, non-invasive method for assessing liver fibrosis. Its role in detecting fibrosis in CFLD has only begun to be explored. The aspartate aminotransferase to platelet ratio index (APRI) has been validated as a predictor of hepatic fibrosis in other chronic liver diseases. The purpose of this study was to assess the utility of APRI and TE in identifying liver fibrosis in pediatric CF patients.

Methods: Patients aged 2-18 years were recruited from the British Columbia Children's Hospital CF clinic. Patients were determined to have CFLD using standard criteria. Charts were reviewed, and each patient underwent TE.

Results: Of the 55 patients included in the study (50.9% male, mean age 11.6 y), 22 (40%) had CFLD. All mean liver enzymes were higher in the CFLD group, notably alanine transaminase ( = 0.031). Mean liver stiffness (LS) and APRI were also higher in the CFLD group (LS: 5.9 versus 4.5 kPa, = 0.015; APRI: 0.40 versus 0.32, = 0.119). Linear regression showed a mild positive association between the two ( = 0.386).

Conclusions: TE values were higher among CFLD patients and correlated with APRI values, suggesting that these tools may have clinical applications for identifying and following this population. Further research is needed on a larger scale to determine the relative value and clinical utility of TE and APRI among patients with CFLD.

References

Lindblad A, Glaumann H, Strandvik B . Natural history of liver disease in cystic fibrosis. Hepatology. 1999; 30(5):1151-8. DOI: 10.1002/hep.510300527. View

Herrmann U, Dockter G, Lammert F . Cystic fibrosis-associated liver disease. Best Pract Res Clin Gastroenterol. 2010; 24(5):585-92. DOI: 10.1016/j.bpg.2010.08.003. View

Corbett K, Kelleher S, Rowland M, Daly L, Drumm B, Canny G . Cystic fibrosis-associated liver disease: a population-based study. J Pediatr. 2004; 145(3):327-32. DOI: 10.1016/j.jpeds.2004.05.051. View

Rath T, Hage L, Kugler M, Menendez Menendez K, Zachoval R, Naehrlich L . Serum proteome profiling identifies novel and powerful markers of cystic fibrosis liver disease. PLoS One. 2013; 8(3):e58955. PMC: 3597583. DOI: 10.1371/journal.pone.0058955. View

Leung D, Khan M, Minard C, Guffey D, Ramm L, Clouston A . Aspartate aminotransferase to platelet ratio and fibrosis-4 as biomarkers in biopsy-validated pediatric cystic fibrosis liver disease. Hepatology. 2015; 62(5):1576-83. DOI: 10.1002/hep.28016. View

Polineni D, Piccorelli A, Hannah W, Dalrymple S, Pace R, Durie P . Analysis of a large cohort of cystic fibrosis patients with severe liver disease indicates lung function decline does not significantly differ from that of the general cystic fibrosis population. PLoS One. 2018; 13(10):e0205257. PMC: 6181334. DOI: 10.1371/journal.pone.0205257. View

Chou R, Wasson N . Blood tests to diagnose fibrosis or cirrhosis in patients with chronic hepatitis C virus infection: a systematic review. Ann Intern Med. 2013; 158(11):807-20. DOI: 10.7326/0003-4819-158-11-201306040-00005. View

Mueller-Abt P, Frawley K, Greer R, Lewindon P . Comparison of ultrasound and biopsy findings in children with cystic fibrosis related liver disease. J Cyst Fibros. 2007; 7(3):215-21. DOI: 10.1016/j.jcf.2007.08.001. View

Flass T, Narkewicz M . Cirrhosis and other liver disease in cystic fibrosis. J Cyst Fibros. 2012; 12(2):116-24. PMC: 3883947. DOI: 10.1016/j.jcf.2012.11.010. View

10.

Lewindon P, Puertolas-Lopez M, Ramm L, Noble C, Pereira T, Wixey J . Accuracy of Transient Elastography Data Combined With APRI in Detection and Staging of Liver Disease in Pediatric Patients With Cystic Fibrosis. Clin Gastroenterol Hepatol. 2019; 17(12):2561-2569.e5. DOI: 10.1016/j.cgh.2019.03.015. View

11.

Colombo C, Battezzati P, Crosignani A, Morabito A, Costantini D, Padoan R . Liver disease in cystic fibrosis: A prospective study on incidence, risk factors, and outcome. Hepatology. 2002; 36(6):1374-82. DOI: 10.1053/jhep.2002.37136. View

12.

Sandrin L, Fourquet B, Hasquenoph J, Yon S, Fournier C, Mal F . Transient elastography: a new noninvasive method for assessment of hepatic fibrosis. Ultrasound Med Biol. 2003; 29(12):1705-13. DOI: 10.1016/j.ultrasmedbio.2003.07.001. View

13.

Potter C, Fishbein M, Hammond S, McCoy K, Qualman S . Can the histologic changes of cystic fibrosis-associated hepatobiliary disease be predicted by clinical criteria?. J Pediatr Gastroenterol Nutr. 1997; 25(1):32-6. DOI: 10.1097/00005176-199707000-00005. View

14.

Hillaire S, Cazals-Hatem D, Bruno O, de Miranda S, Grenet D, Pote N . Liver transplantation in adult cystic fibrosis: Clinical, imaging, and pathological evidence of obliterative portal venopathy. Liver Transpl. 2017; 23(10):1342-1347. DOI: 10.1002/lt.24842. View

15.

Van Biervliet S, Verdievel H, Velde S, De Bruyne R, De Looze D, Verhelst X . Longitudinal Transient Elastography Measurements Used in Follow-up for Patients with Cystic Fibrosis. Ultrasound Med Biol. 2016; 42(4):848-54. DOI: 10.1016/j.ultrasmedbio.2015.11.011. View

16.

Engelmann G, Gebhardt C, Wenning D, Wuhl E, Hoffmann G, Selmi B . Feasibility study and control values of transient elastography in healthy children. Eur J Pediatr. 2011; 171(2):353-60. DOI: 10.1007/s00431-011-1558-7. View

17.

Lewindon P, Balouch F, Pereira T, Puertolas-Lopez M, Noble C, Wixey J . Transient liver elastography in unsedated control children: Impact of age and intercurrent illness. J Paediatr Child Health. 2016; 52(6):637-42. DOI: 10.1111/jpc.13151. View

18.

McGoogan K, Smith P, Choi S, Berman W, Jhaveri R . Performance of the AST-to-platelet ratio index as a noninvasive marker of fibrosis in pediatric patients with chronic viral hepatitis. J Pediatr Gastroenterol Nutr. 2010; 50(3):344-6. PMC: 3314885. DOI: 10.1097/MPG.0b013e3181aed725. View

19.

Fitzpatrick E, Quaglia A, Vimalesvaran S, Basso M, Dhawan A . Transient elastography is a useful noninvasive tool for the evaluation of fibrosis in paediatric chronic liver disease. J Pediatr Gastroenterol Nutr. 2012; 56(1):72-6. DOI: 10.1097/MPG.0b013e31826f2760. View

20.

Fraquelli M, Rigamonti C, Casazza G, Conte D, Donato M, Ronchi G . Reproducibility of transient elastography in the evaluation of liver fibrosis in patients with chronic liver disease. Gut. 2007; 56(7):968-73. PMC: 1994385. DOI: 10.1136/gut.2006.111302. View