title: Biliary atresia: which factors predict the success of a Kasai operation? An analysis of 36 patients.
abstract: Biliary atresia (BA) is one of the most frequent causes of neonatal cholestasis. Portoenterostomy is one therapeutic option in these patients with a success rate of 30-40%. To answer the question of therapy liver transplantation or Kasai operation - we analyzed 36 consecutive patients being followed in our center during the past 7 years. Two groups were formed: group I : patients developing cirrhosis within the first 2 years of life with the need for liver transplantation (n = 21). Group II: patients without need for transplantation within the first 2 years of life (n = 15). The two groups were compared regarding birth weight, age at diagnosis, age at Kasai-procedure, liver histology. The following biochemical parameters were analyzed at the time of diagnosis, 1 week and 5 weeks after Kasai: AST, ALT, gammaGT, and bilirubin. -
  
  
  RESULTS
  Clinical characteristics were similar in both groups. However BA was diagnosed in group I 8.2 weeks after birth compared to 5.6 wk in group II. gammaGT, ALT, AST, and bilirubin were similar in both groups at the time of diagnosis and 1 wk after Kasai. However 5 wk after Kasai gammaGT was 276 U/l in group I compared to 72 U/l in group II (p <0.001), bilirubin was 6.3mg/dl in group I compared to 2. 3mg/dl in group II (p <0.001). -
  
  
  CONCLUSIONS
  Kasai operation before the 7th wk of life increases the success rate of this technique significantly. Children with cirrhosis at the time of diagnosis should be evaluated for primary liver transplantation. gammaGT and bilirubin 5 weeks after Kasai operation may be useful markers for the success of this procedure. Patients with a gammaGT > 100 U/l and a bilirubin level >5mg/dl should be followed closely and should be evaluated for liver transplantation early.
authors.affiliation: Pediatric Gastroenterology and Hepatology, Childrens University Hospital, Dr. v. Haunersches Kinderspital, Lindwurmstrasse 4, D-80337 Munich, Germany. thomas.lang@kk-i.med.uni-muenchen.de.
authors.forename: T
authors.initials: T
authors.lastname: Lang
authors.forename: M
authors.initials: M
authors.lastname: Kappler
authors.forename: H
authors.initials: H
authors.lastname: Dietz
authors.forename: H K
authors.initials: HK
authors.lastname: Harms
authors.forename: R
authors.initials: R
authors.lastname: Bertele-Harms
mesh_terms: D001656:Biliary Atresia / Q000601:surgery*; D001663:Bilirubin / Q000097:blood; D001724:Birth Weight; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D016031:Liver Transplantation; D008297:Male; D011171:Portoenterostomy, Hepatic; D012189:Retrospective Studies; D013997:Time Factors; D016896:Treatment Outcome
chemical_list: D001663:Bilirubin