title: Plasma endothelin-1 level as a marker reflecting the severity of portal hypertension in biliary atresia.
abstract: OBJECTIVE
  The aim of this study was to examine if the plasma endothelin-1 (ET-1), a potent vasoconstrictor, level may reflect the severity of portal hypertension associated with liver cirrhosis in biliary atresia (BA).
  
  
  METHODS
  Forty-eight postoperative BA patients aged 6 months to 20 years were studied. Plasma ET-1 was measured by a sandwich method of enzyme immunoassay. ET-1 was compared with Child's score and laboratory data. ET-1 levels were compared among groups of patients with various degrees of histologic fibrosis and portal hypertension.
  
  
  RESULTS
  Plasma ET-1 was 5.3 +/- 3.5 pg/mL in BA, higher than in controls (3.1 +/- 0.8, n = 27; P <.05). ET-1 correlated with Child's score, serum total bilirubin, direct bilirubin, aspartate aminotransferase, albumin, prothrombin time, hepaplastin test, fibrinogen, cholinesterase, total cholesterol, Fischer's molar ratio, prealubumin, and hyaluronic acid, respectively (P <.05). ET-1 also correlated with the severity of histologic fibrosis, gastroesophageal varices, the presence of splenomegaly, ascites, venous dilatation on the abdominal wall, or pulmonary vascular abnormalities. In 4 patients undergoing liver transplantation (LTx), ET-1 after LTx was lower than that before LTx (P <.05).
  
  
  CONCLUSIONS
  Plasma ET-1 level may be a useful index reflecting the severity of cirrhosis and portal hypertension in BA.
authors.affiliation: Department of Pediatric Surgery, Osaka University Medical School, 2-2 Yamadaoka, Suita City, Osaka, 565-0871 Japan.
authors.forename: T
authors.initials: T
authors.lastname: Hasegawa
authors.forename: T
authors.initials: T
authors.lastname: Kimura
authors.forename: T
authors.initials: T
authors.lastname: Sasaki
authors.forename: A
authors.initials: A
authors.lastname: Okada
mesh_terms: D000293:Adolescent; D000328:Adult; D001656:Biliary Atresia / Q000097:blood* / Q000150:complications / Q000601:surgery; D015415:Biomarkers / Q000097:blood; D002648:Child; D002675:Child, Preschool; D019332:Endothelin-1 / Q000097:blood*; D006801:Humans; D006975:Hypertension, Portal / Q000097:blood* / Q000150:complications / Q000601:surgery; D007223:Infant; D008103:Liver Cirrhosis / Q000097:blood* / Q000150:complications / Q000601:surgery; D011171:Portoenterostomy, Hepatic; D012720:Severity of Illness Index
chemical_list: D015415:Biomarkers; D019332:Endothelin-1