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Pediatric Radiology

Published in:

01-07-2010 | Original Article

Transient elastography in patients with cystic fibrosis

Authors: Renaud Menten, Anissa Leonard, Philippe Clapuyt, Pierre Vincke, Alexandra-Cristina Nicolae, Patrick Lebecque

Published in: Pediatric Radiology | Issue 7/2010

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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Title: Transient elastography in patients with cystic fibrosis
Authors: Renaud Menten
Anissa Leonard
Philippe Clapuyt
Pierre Vincke
Alexandra-Cristina Nicolae
Patrick Lebecque
Publication date: 01-07-2010
Publisher: Springer-Verlag
Published in: Pediatric Radiology / Issue 7/2010
Print ISSN: 0301-0449
Electronic ISSN: 1432-1998
DOI: https://doi.org/10.1007/s00247-009-1531-z

At a glance: The STEP trials

Springer Medicine

Transient elastography in patients with cystic fibrosis

Abstract

Background

Objectives

Materials and methods

Results

Conclusion

At a glance: The STEP trials

Springer Medicine

Abstract

Background

Objectives

Materials and methods

Results

Conclusion

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