[Chronic therapy with ursodeoxycholic acid in a child with Alagille syndrome].
The effectiveness of oral bile acid therapy with ursodeoxycholic acid (10 mg/kg/day) was investigated in a 10 year old boy affected by Alagille's Syndrome, a chronic cholestatic disorder due to congenital hypoplasia of the intrahepatic biliary ducts. Cholestatic and hepatonecrotic indices were measured before and during ursodeoxycholic acid therapy and 1 month after stopping and 36 months after restarting the treatment. Ursodeoxycholic acid led to a marked improvement in the cholestatic and hepatonecrotic parameters which was maintained during all the treatment phase. Pruritus and steatorrhea disappeared during the treatment with ursodeoxycholic acid. Histological examination of the liver biopsy after the treatment revealed a disappearance of the biliary plugs but without increasing he intrahepatic bile ducts. The results suggest that ursodeoxycholic acid may improve the condition of the children affected by Alagille's Syndrome, specially when the liver transplantation is required, and indicate a need for long term studies in a larger number of patients.
Clerici
C
C
Istituto di Gastroenterologia ed Endoscopia Digestiva, Università degli Studi di Perugia, Italia.
Gentili
G
G
Dozzini
G
G
Distrutti
E
E
Solinas
A
A
Vedovelli
A
A
Pelli
M A
MA
Bragetti
P
P
Setchell
K
K
Morelli
A
A
D001653:Bile Ducts, Intrahepatic / Q000002:abnormalities*; D002648:Child; D002779:Cholestasis / Q000188:drug therapy*; D002908:Chronic Disease; D006801:Humans; D008297:Male; D013577:Syndrome; D014580:Ursodeoxycholic Acid / Q000627:therapeutic use*
D014580:Ursodeoxycholic Acid
Terapia cronica con acido ursodesossicolico in un bambino affetto da Sindrome di Alagille.