[Congenital hepatic fibrosis. Five cases with late occurrence in adults].
Congenital hepatic fibrosis (CHF), is an autosomal recessive disease, presenting principally in childhood with portal hypertension and/or cholangitis, and often associated with renal malformations. The forms presenting later in adults are rare, and illustrated herein by 4 cases with dominant cholangitis, and one latent form. Biological tests and radiological imaging are often normal. The histopathologic diagnosis, sometimes difficult on liver needle biopsy is based on fibrous enlargement of portal areas, with numerous and tortuous bile ducts, lined by regular, cuboidal epithelium. Interportal fibrosis can mimic cirrhosis. In CHF, cholangitis are favoured by intrahepatic biliary dilatation, sometimes related to Caroli's disease, associated in 25% of cases. Suppurative complications, sometimes fatal explain the severity of cholangitis forms of CHF, contraindicating inopportune cholangiography and biliary surgery.
Mainguene
C
C
Service de Pathologie, Hôpital Princesse Grace, Monaco.
Clement
N
N
Cassagnau
E
E
Nomballais
F
F
Gavelli
A
A
Marmorale
A
A
Brunner
P
P
Huguet
C
C
D017668:Age of Onset; D000368:Aged; D000369:Aged, 80 and over; D001707:Biopsy, Needle; D005260:Female; D005808:Genes, Recessive; D006801:Humans; D008103:Liver Cirrhosis / Q000151:congenital* / Q000235:genetics / Q000473:pathology; D008297:Male; D008875:Middle Aged
Fibrose hépatique congénitale. Cinq cas à révélation tardive chez l'adulte.