[Byler's disease (familial fibrogenic cholestasis in children). Apropos of 7 cases].
Seven cases presenting with the features of Byler's disease (BD) are reported. The diagnosis of BD can only be made after exclusion of other causes of cholestasis. It seems likely that BD is a syndrome and that more accurate biological and histological investigations will allow to differentiate several entities within this affection. At the present, liver transplantation appears to be the only rational treatment.
Service de pédiatrie et génétique médicale, Hôpital Claude-Huriez, CHRU, Lille, France.
M
M
Razemon-Pinta
M
M
Lecomte-Houcke
J P
JP
Mary
G A
GA
Loreille
D002648:Child; D002675:Child, Preschool; D002780:Cholestasis, Intrahepatic / Q000150:complications / Q000235:genetics* / Q000473:pathology; D005260:Female; D006801:Humans; D007223:Infant; D008099:Liver / Q000473:pathology* / Q000648:ultrastructure; D008103:Liver Cirrhosis / Q000209:etiology* / Q000473:pathology; D008297:Male
La maladie de Byler (cholestase familiale fibrogène de l'enfant). A propos de 7 cas.