title: Transient elastography and portal hypertension in pediatric patients with cystic fibrosis Transient elastography and cystic fibrosis.
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.
authors.affiliation: Center for Cystic Fibrosis Resources and Expertise, Medical Center, boulevard de l'hôpital 44600 Saint Nazaire, France. ac.wagner@ch-saintnazaire.fr
authors.forename: A C
authors.initials: AC
authors.lastname: Malbrunot-Wagner
authors.forename: L
authors.initials: L
authors.lastname: Bridoux
authors.forename: J B
authors.initials: JB
authors.lastname: Nousbaum
authors.forename: C
authors.initials: C
authors.lastname: Riou
authors.forename: A
authors.initials: A
authors.lastname: Dirou
authors.forename: J L
authors.initials: JL
authors.lastname: Ginies
authors.forename: C
authors.initials: C
authors.lastname: Maurage
authors.forename: B
authors.initials: B
authors.lastname: Cagnard
authors.forename: C
authors.initials: C
authors.lastname: Pelatan
authors.forename: A
authors.initials: A
authors.lastname: Dabadie
mesh_terms: D000293:Adolescent; D002648:Child; D003550:Cystic Fibrosis / Q000150:complications*; D018450:Disease Progression; D054459:Elasticity Imaging Techniques / Q000379:methods*; D004932:Esophageal and Gastric Varices / Q000175:diagnosis* / Q000209:etiology*; D005260:Female; D006801:Humans; D006975:Hypertension, Portal / Q000175:diagnosis* / Q000209:etiology*; D008297:Male; D010976:Platelet Count; D011237:Predictive Value of Tests; D012189:Retrospective Studies; D012720:Severity of Illness Index