title: Delayed intestinal visualization at hepatobiliary scintigraphy is associated with response to long-term treatment with ursodeoxycholic acid in patients with cystic fibrosis-associated liver disease.
abstract: OBJECTIVE
  Abnormalities of biliary drainage have been documented at hepatobiliary scintigraphy in many but not all patients studied with cystic fibrosis-associated liver disease. Ursodeoxycholic acid was shown to be beneficial in this disease, mainly by improving biliary secretion. Therefore, patients with impaired biliary drainage are expected to obtain the greatest benefit from this treatment.
  
  
  METHODS
  We evaluated the effects of long-term treatment with ursodeoxycholic acid in 36 patients with cystic fibrosis-associated liver disease, and compared the response in patients presenting a normal (n=18) or delayed time of intestinal visualization (n=18) at baseline hepatobiliary scintigraphy.
  
  
  RESULTS
  The mean treatment duration was 58+/-26 (S.D.) months and 63+/-29 months in the groups with normal or delayed time of intestinal visualization, respectively. The time of intestinal visualization decreased (57+/-23%, p<0.001) from baseline in patients with initially abnormal values and became normal in four (22%). Treatment failure, i.e. lack of sustained normalization of serum liver enzymes or the occurrence of a clinically relevant adverse event, was more frequently observed in patients with a normal time of intestinal visualization at baseline (OR, 5.50; 95% CI, 1.32-22.7). When only clinically relevant adverse events were considered, they occurred in six of the latter patients (liver transplantation in one case, development of ultrasographic or endoscopic signs of portal hypertension in six cases), but in only one patient (development of portal hypertension) in the group with delayed time of intestinal visualization (OR, 10.82; 95% CI, 1.17-100.4).
  
  
  CONCLUSIONS
  Delayed intestinal visualization at hepatobiliary scintigraphy in patients with cystic fibrosis-associated liver disease seems to predict a better response to ursodeoxycholic acid.
authors.affiliation: Department of Pediatrics, University of Milan, Italy.
authors.forename: C
authors.initials: C
authors.lastname: Colombo
authors.forename: A
authors.initials: A
authors.lastname: Crosignani
authors.forename: P M
authors.initials: PM
authors.lastname: Battezzati
authors.forename: M R
authors.initials: MR
authors.lastname: Castellani
authors.forename: S
authors.initials: S
authors.lastname: Comi
authors.forename: M L
authors.initials: ML
authors.lastname: Melzi
authors.forename: A
authors.initials: A
authors.lastname: Giunta
mesh_terms: D000293:Adolescent; D000328:Adult; D001652:Bile Ducts / Q000000981:diagnostic imaging; D002648:Child; D002675:Child, Preschool; D002756:Cholagogues and Choleretics / Q000009:adverse effects / Q000627:therapeutic use*; D003550:Cystic Fibrosis / Q000150:complications*; D005260:Female; D006801:Humans; D007422:Intestines / Q000000981:diagnostic imaging*; D008099:Liver / Q000000981:diagnostic imaging; D008107:Liver Diseases / Q000000981:diagnostic imaging / Q000188:drug therapy* / Q000209:etiology*; D008297:Male; D011877:Radionuclide Imaging; D013997:Time Factors; D017211:Treatment Failure; D014463:Ultrasonography; D014580:Ursodeoxycholic Acid / Q000009:adverse effects / Q000627:therapeutic use*
chemical_list: D002756:Cholagogues and Choleretics; D014580:Ursodeoxycholic Acid