title: Ductal plate malformation in patients with biliary atresia.
abstract: The presence of ductal plate malformation (DPM+) on liver histology in children with biliary atresia (BA) is a marker of early intrauterine disease onset and an indication of an unfavorable prognosis. We studied the prognostic value of DPM in infants with BA after hepatoportoenterostomy (HPE). We reviewed 28 BA patients who underwent HPE in a single medical center. We examined the time of jaundice onset after delivery (conjugated hyperbilirubinemia): early onset (fetal phenotype with no jaundice-free interval) vs. late onset (perinatal phenotype with jaundice-free interval) and the presence or absence of DPM (DPM+ or DPM-) histopathology. Primary outcome was jaundice clearance at 3 months after HPE and survival with native liver (SNL). Eight children had fetal and 20 had perinatal BA (8 DPM+, 12 DPM-). At 3 months after HPE, no patients with fetal BA had achieved jaundice clearance, while jaundice clearance was achieved in five patients with DPM+ perinatal disease and four patients with DPM- perinatal BA (P = 0.03, comparing all three groups; P = 0.36, comparing DPM+ vs. DPM- perinatal patients). Median SNL was 8.6 months for fetal BA patients, 148.2 months for DPM+ perinatal BA patients, and 93.2 months for DPM- perinatal BA patients (log-rank test, P < 0.001, comparing all three groups; P = 0.59, comparing DPM+ vs. DPM- perinatal patients). After adjusting for BA type, age older than 2 months at HPE was associated with worse SNL [P = 0.03; hazard ratio = 4.0 (95 % CI, 1.1-14.2)].
  
  
  CONCLUSIONS
  Early onset of jaundice, regardless of DPM histology, was the most ominous sign of poor outcome in infants with BA after HPE.
authors.affiliation: Department of Pediatrics, University of Zagreb Medical School, University Hospital Centre Zagreb, Zagreb, Croatia.
authors.forename: Jurica
authors.initials: J
authors.lastname: Vuković
authors.forename: Ruža
authors.initials: R
authors.lastname: Grizelj
authors.forename: Katarina
authors.initials: K
authors.lastname: Bojanić
authors.forename: Marijana
authors.initials: M
authors.lastname: Corić
authors.forename: Tomislav
authors.initials: T
authors.lastname: Luetić
authors.forename: Stipe
authors.initials: S
authors.lastname: Batinica
authors.forename: Mirjana
authors.initials: M
authors.lastname: Kujundžić-Tiljak
authors.forename: Darrell R
authors.initials: DR
authors.lastname: Schroeder
authors.forename: Juraj
authors.initials: J
authors.lastname: Sprung
mesh_terms: D000465:Algorithms; D001652:Bile Ducts / Q000002:abnormalities*; D001653:Bile Ducts, Intrahepatic / Q000002:abnormalities; D001656:Biliary Atresia / Q000150:complications / Q000473:pathology / Q000601:surgery*; D015331:Cohort Studies; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D008297:Male; D016017:Odds Ratio; D011171:Portoenterostomy, Hepatic / Q000379:methods; D012189:Retrospective Studies; D016896:Treatment Outcome