[Congenital disorders of the biliary ducts].
Congenital disorders of the bile ducts manifesting as neonatal cholestasis, are: biliary atresia, Alagille syndrome and neonatal sclerosing cholangitis. Biliary atresia must be considered as a neonatal surgical emergency: diagnosis and therapeutic intervention should be completed before one month of age. Survival at 10 years is about 70%, 14% without and 56% with liver transplantation. The cause of the disease is unknown. Alagille syndrome is defined by the association of bile duct paucity, pulmonary stenosis, butterfly-like vertebrae, peculiar facies and posterior embryotoxon. JAGGEDI gene mutations have been identified allowing prenatal diagnosis. The diagnosis of neonatal sclerosing cholangitis can only be performed by cholangiography.
Gauthier
F
F
Département de pédiatrie et INSERM U347 Hôpital de Bicêtre 94275 Le Kremlin-Bicêtre. hadchoue@kb.inserm.fr
Hadchouel
M
M
D000328:Adult; D016738:Alagille Syndrome / Q000175:diagnosis / Q000235:genetics / Q000473:pathology*; D001656:Biliary Atresia / Q000175:diagnosis / Q000473:pathology*; D002758:Cholangiography; D015209:Cholangitis, Sclerosing / Q000151:congenital* / Q000175:diagnosis / Q000473:pathology; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D007231:Infant, Newborn; D016031:Liver Transplantation; D008297:Male; D011247:Pregnancy; D011296:Prenatal Diagnosis; D011379:Prognosis
Maladies congénitales des voies biliaires.