title: Biliary atresia due to delayed maturation of the gut hormones' system?--Introducing a new treatment modality.
abstract: BACKGROUND
  Congenital biliary atresia is suspected to originate from prenatal biliary duct inflammation of unknown etiology.
  
  
  OBJECTIVE
  Based on clinical grounds, we aimed to establish a hypothesis on the primary cause of inflammation, and to suggest a causal treatment modality.
  
  
  METHODS
  History. A 28 years old Turkish woman had lost her first child aged two years from congenital biliary atresia (parents second degree cousins). After a miscarriage, in her otherwise uneventful third pregnancy sonography at 34 wks revealed echogenic material in the fetal gallbladder. Nine days later the gallbladder was completely filled with sludge. Chemical inflammation was suspected, and birth was induced at 36+3 weeks in order to allow for surgical flushing of the bile duct. Neonatal clinical chemistry was insuspicious. There was no spontaneous resolution of the sludge within the first 24 hours of life. A trial of medical treatment with intermittent i.v. secretin (0.03 CU/kg/h) and i.v. coeruletid (60 ng/kg/h) was started. Within 24 hours, sludge had resolved.
  
  
  CONCLUSIONS
  We hypothesize that dysmaturation may lead to insufficient induction/production/activity of intrinsic gut hormones resulting in prenatally impaired bile flow, or even inspissated bile. Familial occurrence suggests a genetic defect. Exogenous hormone therapy might be an appropriate treatment modality.
authors.affiliation: Department of Pediatrics, Neukölln General Hospital, Berlin, Germany. Erik.Michel@t-online.de
authors.forename: Erik
authors.initials: E
authors.lastname: Michel
authors.forename: Omer
authors.initials: O
authors.lastname: Kilavuz
authors.forename: Ralph
authors.initials: R
authors.lastname: Jäger
authors.forename: Rohangiz
authors.initials: R
authors.lastname: Nasir
mesh_terms: D000328:Adult; D001656:Biliary Atresia / Q000175:diagnosis* / Q000000981:diagnostic imaging / Q000196:embryology / Q000235:genetics / Q000628:therapy; D003937:Diagnosis, Differential; D005260:Female; D020022:Genetic Predisposition to Disease; D006801:Humans; D007231:Infant, Newborn; D008297:Male; D011247:Pregnancy; D016216:Ultrasonography, Prenatal