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Transient elastography in patients with cystic fibrosis

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Abstract

Background

Hepatic involvement is frequent in patients with cystic fibrosis (CF), with focal biliary cirrhosis being the pathognomonic hepatic manifestation. In around one-quarter of CF patients, it results in CF-associated liver disease (CFLD). This occurs as a relatively early complication with the majority of patients presenting in childhood or their early teens. However, a normal US does not preclude significant liver fibrosis and liver biopsy is an invasive procedure that is hampered by potential sampling errors. Transient elastography (TE) (Fibroscan) is a non-invasive, user-friendly and quick technique that provides an objective and reproducible measure of liver stiffness. This is accomplished with a device using an US probe mounted in the axis of a vibrator. Vibrations are transmitted by the transducer, inducing an electronic shear wave that propagates through the underlying tissue.

Objectives

We aimed to prospectively compare TE and transabdominal US scanning in children and adults attending a CF clinic.

Materials and methods

A total of 134 consecutive patients with documented CF were prospectively studied. In each case, transient elastography measurement was performed immediately after the routine annual US evaluation of the liver. Sonographic appearance of the liver was classified from 1 to 5. Ten validated TE measurements were performed in each patient with the result expressed in kilopascals (kPa). The median value was considered representative of the elastic modulus of the liver.

Results

Measurements were performed in 59 CF adults, 75 CF children and 31 control children. There was no relationship between age and liver stiffness in either the control group or CF patients. Elasticity values of controls, CF pancreatic sufficient (PS) patients and pancreatic insufficient (PI) CF patients with a US score <3 were comparable and significantly lower than in CF patients with a US score ≥ 3 (all PI) (P < 0.002). Median elasticity in CF patients was significantly higher in males (4.7 kPa) than in females (3.9 kPa) (P = 0.0013).

Conclusion

Considering the limitations of US and the low risk-benefit rate of liver biopsy in most CF patients, this preliminary study suggests that TE is an attractive non-invasive way to assess and follow-up liver disease in these patients.

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References

  1. Stewart L (2005) The role of abdominal ultrasound in the diagnosis, staging and management of cystic fibrosis liver disease. J R Soc Med 98(Suppl 45):17–27

    PubMed  Google Scholar 

  2. Colombo C, Russo MC, Zazzeron L et al (2006) Liver disease in cystic fibrosis. J Pediatr Gastroenterol Nutr 43(Suppl 1):S49–55

    PubMed  Google Scholar 

  3. Dondos V, Westaby D (2007) Liver, biliary and pancreatic disease. In: Hodson M, Geddes D, Bush A (eds) Cystic fibrosis. Hodder Arnold, London, pp 225–240

    Google Scholar 

  4. Lindblad A, Glaumann H, Strandvik B (1999) Natural history of liver disease in cystic fibrosis. Hepatology 30:1151–1158

    Article  CAS  PubMed  Google Scholar 

  5. Colombo C, Battezzati PM, Crosignani A et al (2002) Liver disease in cystic fibrosis: A prospective study on incidence, risk factors, and outcome. Hepatology 36:1374–1382

    PubMed  Google Scholar 

  6. Bravo AA, Sheth SG, Chopra S (2001) Liver biopsy. N Engl J Med 344:495–500

    Article  CAS  PubMed  Google Scholar 

  7. Ling SC, Wilkinson JD, Hollman AS et al (1999) The evolution of liver disease in cystic fibrosis. Arch Dis Child 81:129–132

    Article  CAS  PubMed  Google Scholar 

  8. Lenaerts C, Lapierre C, Patriquin H et al (2003) Surveillance for cystic fibrosis-associated hepatobiliary disease: early ultrasound changes and predisposing factors. J Pediatr 143:343–350

    Article  PubMed  Google Scholar 

  9. Mueller-Abt PR, Frawley KJ, Greer RM et al (2007) Comparison of ultrasound and biopsy findings in children with cystic fibrosis related liver disease. J Cyst Fibros 7:215–221

    Article  PubMed  Google Scholar 

  10. Sandrin L, Fourquet B, Hasquenoph JM et al (2003) Transient elastography: a new non-invasive method for assessment of hepatic fibrosis. Ultrasound Med Biol 29:1705–1713

    Article  PubMed  Google Scholar 

  11. Cobbold JF JFL, Morin S, Taylor-Robinson D (2007) Transient elastography for the assessment of chronic liver disease: Ready for the clinic? World J Gastroenterol 13:4791–4797

    PubMed  Google Scholar 

  12. Castera L, Forns X, Alberti A (2008) Non-invasive evaluation of liver fibrosis using transient elastography. J Hepatol 48:835–847

    Article  PubMed  Google Scholar 

  13. Yeshua H, Oren R (2008) Non-invasive assessment of liver fibrosis. Ann Transplant 13:5–11

    PubMed  Google Scholar 

  14. Rockey DC (2008) Non-invasive assessment of liver fibrosis and portal hypertension with transient elastography. Gastroenterology 134:8–14

    Article  PubMed  Google Scholar 

  15. Fraquelli M, Rigamonti C, Casazza G et al (2007) Reproducibility of transient elastography in the evaluation of liver fibrosis in patients with chronic liver disease. Gut 56:968–973

    Article  PubMed  Google Scholar 

  16. Corpechot C, El Naggar A, Poujol-Robert A et al (2006) Assessment of biliary fibrosis by transient elastography in patients with PBC and PSC. Hepatology 43:1118–1124

    Article  PubMed  Google Scholar 

  17. Gómez-Dominguez E, Mendoza J, García-Buey L et al (2008) Transient elastography to assess hepatic fibrosis in primary biliary cirrhosis. Aliment Pharmacol Ther 27:441–447

    Article  PubMed  Google Scholar 

  18. Castéra L, Vergniol J, Foucher J et al (2005) Prospective comparison of transient elastography, Fibrotest, APRI, and liver biopsy for the assessment of fibrosis in chronic hepatitis C. Gastroenterology 128:343–350

    Article  PubMed  Google Scholar 

  19. Ziol M, Handra-Luca A, Kettaneh A et al (2005) Non-invasive assessment of liver fibrosis by measurement of stiffness in patients with chronic hepatitis C. Hepatology 41:48–54

    Article  PubMed  Google Scholar 

  20. Ganne-Carrié N, Ziol M, de Ledinghen V et al (2006) Accuracy of liver stiffness measurement for the diagnosis of cirrhosis in patients with chronic liver diseases. Hepatology 44:1511–1517

    Article  PubMed  Google Scholar 

  21. Foucher J, Chanteloup E, Vergniol J et al (2006) Diagnosis of cirrhosis by transient elastography (FibroScan): a prospective study. Gut 55:403–408

    Article  CAS  PubMed  Google Scholar 

  22. Lupşor M, Badea R, Stefănescu H et al (2008) Analysis of histopathological changes that influence liver stiffness in chronic hepatitis C. Results from a cohort of 324 patients. J Gastrointestin Liver Dis 17:155–163

    PubMed  Google Scholar 

  23. Rigamonti C, Donato MF, Fraquelli M et al (2008) Transient elastography predicts fibrosis progression in patients with recurrent hepatitis C after liver transplantation. Gut 57:821–827

    Article  CAS  PubMed  Google Scholar 

  24. Arena U, Vizzutti F, Abraldes GJ et al (2008) Reliability of transient elastography for the diagnosis of advanced fibrosis in chronic hepatitis C. Gut 57:1288–1293

    Article  CAS  PubMed  Google Scholar 

  25. de Lédinghen V, Le Bail B, Rebouissoux L et al (2007) Liver stiffness measurement in children using FibroScan: feasibility study and comparison with Fibrotest, aspartate transaminase to platelets ratio index, and liver biopsy. J Pediatr Gastroenterol Nutr 45:443–450

    Article  PubMed  Google Scholar 

  26. Nobili V, Vizzutti F, Arena U et al (2008) Accuracy and reproducibility of transient elastography for the diagnosis of fibrosis in pediatric nonalcoholic steatohepatitis. Hepatology 48:442–448

    Article  CAS  PubMed  Google Scholar 

  27. Lucidarme D, Foucher J, Le Bail B et al (2007) The ratio interquartile range/median value of liver stiffness measurements is a key factor of accuracy of transient elastography (FibroScan) for the diagnosis of liver fibrosis (abstract). Hepatology 46:318A

    Google Scholar 

  28. Colombo C, Apostolo MG, Ferrari M et al (1994) Analysis of risk factors for the development of liver disease associated with cystic fibrosis. J Pediatr 124:393–396

    Article  CAS  PubMed  Google Scholar 

  29. Wilschanski M, Rivlin J, Cohen S et al (1999) Clinical and genetic risk factors for cystic fibrosis-related liver disease. Pediatrics 103:52–57

    Article  CAS  PubMed  Google Scholar 

  30. Scott-Jupp R, Lama M, Tanner MS (1991) Prevalence of liver disease in cystic fibrosis. Arch Dis Child 66:698–701

    Article  CAS  PubMed  Google Scholar 

  31. Feigelson J, Anagnostopoulos C, Poquet M et al (1993) Liver cirrhosis in cystic fibrosis. Therapeutic implications and long-term follow-up. Arch Dis Child 68:653–657

    CAS  Google Scholar 

  32. Debray D, Lykavieris P, Gauthier F et al (1999) Outcome of cystic fibrosis-associated liver cirrhosis: management of portal hypertension. J Hepatol 31:77–83

    Article  CAS  PubMed  Google Scholar 

  33. Roulot D, Czernichow S, Le Clesiau H et al (2008) Liver stiffness values in apparently healthy subjects: Influence of gender and metabolic syndrome. J Hepatol 48:606–613

    Article  PubMed  Google Scholar 

  34. Colombo C, Apostolo MG, Ferrari M et al (1994) Analysis of risk factors for the development of liver disease associated with cystic fibrosis. J Pediatr 124:393–399

    Article  CAS  PubMed  Google Scholar 

  35. Fuchs JR, Langer JC (1998) Long-term outcome after neonatal meconium obstruction. Pediatrics 101:E7

    Article  CAS  PubMed  Google Scholar 

  36. Potter CJ, Fishbein M, Hammond S et al (1997) Can the histologic changes of cystic fibrosis-associated hepatobiliary disease be predicted by clinical criteria? J Pediatr Gastroenterol Nutr 25:32–36

    Article  CAS  PubMed  Google Scholar 

  37. Coco B, Oliveri F, Maina AM et al (2007) Transient elastography:a new surrogate marker of liver fibrosis influenced by major changes of transaminases. J Viral Hepat 14:360–369

    Article  CAS  PubMed  Google Scholar 

  38. Arena U, Vizzutti F, Corti G et al (2008) Acute viral hepatitis increases liver stiffness values measured by transient elastography. Hepatology 47:380–384

    Article  CAS  PubMed  Google Scholar 

  39. Sagir A, Erhardt A, Schmitt M et al (2007) Transient elastography is unreliable for detection of cirrhosis in patients with acute liver damage. Hepatology 47:592–595

    Article  CAS  Google Scholar 

  40. Talwalkar JA, Kurtz DM, Schoenleber SJ et al (2007) Ultrasound-based transient elastography for the detection of hepatic fibrosis: systematic review and meta-analysis. Clin Gastroenterol Hepatol 5:1214–1220

    Article  PubMed  Google Scholar 

  41. Friedrich-Rust M, Ong MF, Martens S et al (2008) Performance of transient elastography for the staging of liver fibrosis: a meta-analysis. Gastroenterology 134:960–974

    Article  PubMed  Google Scholar 

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Authors and Affiliations

  1. Radiology, Cliniques Universitaires St Luc, Avenue Hippocrate 10, Brussels, 1200, Belgium

    Renaud Menten, Philippe Clapuyt, Pierre Vincke & Alexandra-Cristina Nicolae

  2. Pediatrics, Cliniques Universitaires St Luc, Brussels, Belgium

    Anissa Leonard & Patrick Lebecque

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  1. Renaud Menten
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  2. Anissa Leonard
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  3. Philippe Clapuyt
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  4. Pierre Vincke
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Correspondence to Renaud Menten.

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Menten, R., Leonard, A., Clapuyt, P. et al. Transient elastography in patients with cystic fibrosis. Pediatr Radiol 40, 1231–1235 (2010). https://doi.org/10.1007/s00247-009-1531-z

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  • DOI: https://doi.org/10.1007/s00247-009-1531-z

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