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Multicenter Study
. 2011 Sep;10(5):338-42.
doi: 10.1016/j.jcf.2011.04.004. Epub 2011 May 7.

Transient elastography and portal hypertension in pediatric patients with cystic fibrosis Transient elastography and cystic fibrosis

A C Malbrunot-Wagner  1 , L Bridoux, J B Nousbaum, C Riou, A Dirou, J L Ginies, C Maurage, B Cagnard, C Pelatan, A Dabadie
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Free article
Multicenter Study

Transient elastography and portal hypertension in pediatric patients with cystic fibrosis Transient elastography and cystic fibrosis

A C Malbrunot-Wagner et al. J Cyst Fibros. 2011 Sep.
Free article

Abstract

Background: Esophageal varices (EV) are a complication of cystic fibrosis-associated liver disease. Esophagogastroduodenoscopy (EGD) is currently used to diagnose varices but is invasive for pediatric patients. The goal of this study was to explore the relationship between transient elastography (FibroScan®) and the presence of EV in patients with liver disease defined by clinical, laboratory, sonographic, and/or endoscopic criteria.

Methods: 18 patients with cystic fibrosis underwent EGD and transient elastography. 12 patients had EV.

Results: Patients with EV had higher FibroScan values than those without varices with median values of 22.4 kPa (14.4-30.4 kPa) vs. 7.9 kPa (4.4-13.7 kPa) (p=0.01). Using a threshold of 12 kPa, four of six patients without EV would not have needed EGD.

Conclusions: Elastography should be recommended for all cystic fibrosis patients with liver disease to follow its progression. A prospective study is needed to define an elastography threshold value that predicts the presence of EV.

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