Long-term prospective study of the effect of ursodeoxycholic acid on cystic fibrosis-related liver disease

被引:41
作者
Nousia-Arvanitakis, S
Fotoulaki, M
Economou, H
Xefteri, M
Galli-Tsinopoulou, A
机构
[1] Aristotle Univ Thessaloniki, Dept Pediat 4, GR-54006 Thessaloniki, Greece
[2] Aristotle Univ Thessaloniki, Dept Radiol, GR-54006 Thessaloniki, Greece
关键词
cystic fibrosis-related liver disease; focal biliary cirrhosis; nodular biliary cirrhosis; ursodeoxycholic acid; cystic fibrosis;
D O I
10.1097/00004836-200104000-00009
中图分类号
R57 [消化系及腹部疾病];
学科分类号
摘要
Goals: To evaluate the efficacy of UDCA in arresting the progression of the early multifocal hepatic lesion to overt CF-related NBC. Background: Focal biliary cirrhosis is an early hepatic pathologic change related to the ion transport defect in cystic fibrosis. The factors involved in the progression of focal to nodular biliary cirrhosis are not clear. Ursodeoxycholic--a hydrophilic, nontoxic, choleretic, and hepatoprotective exogenous bile acid-has been reported to be effective in the management of cholestatic liver disease. Study: For 10 years at 6-month intervals, 70 individuals with cystic fibrosis (38 men and 32 women; age range, 2-29 years) were examined using hepatosplenomegaly, liver function tests, and ultrasound liver scan. Patients demonstrating evidence of liver involvement at the onset or during the study received ursodeoxycholic acid 20 mg/kg body weight. Results: After the administration of ursodeoxycholic acid, the progression of nodular biliary cirrhosis ultrasound changes was arrested, hepatic function was preserved, and no variceal bleeding was observed. No case of focal biliary cirrhosis progressed to nodular biliary cirrhosis. Furthermore, the multifocal, multilobular changes suggestive of focal biliary cirrhosis on ultrasound scan were reversed to normal. Conclusion: Ursodeoxycholic acid is effective in improving cholestasis and hepatic dysfunction in nodular biliary cirrhosis and, also, in reversing the early sonography findings suggestive of focal biliary cirrhosis. It is speculated that ursodeoxycholic acid may prove to affect the natural history of cystic fibrosis-related liver disease.
引用
收藏
页码:324 / 328
页数:5
相关论文
共 39 条
[21]   Ursodeoxycholic acid delays the onset of esophageal varices in primary biliary cirrhosis [J].
Lindor, KD ;
Jorgensen, RA ;
Therneau, TM ;
Malinchoc, M ;
Dickson, ER .
MAYO CLINIC PROCEEDINGS, 1997, 72 (12) :1137-1140
[22]   MULTIFOCAL UPTAKE OF TC-99M MEBROFENIN IN DILATED LIVER DUCTULES IN A PATIENT WITH CYSTIC-FIBROSIS [J].
MORRISON, RT ;
STEUART, RD ;
CHAUN, H .
CLINICAL NUCLEAR MEDICINE, 1995, 20 (01) :25-26
[23]   Hepatobiliary scintigraphy in children [J].
Nadel, HR .
SEMINARS IN NUCLEAR MEDICINE, 1996, 26 (01) :25-42
[24]   Effect of ursodeoxycholic acid therapy on hepatic function in children with intrahepatic cholestatic liver disease [J].
Narkewicz, MR ;
Smith, D ;
Gregory, C ;
Lear, JL ;
Osberg, I ;
Sokol, RJ .
JOURNAL OF PEDIATRIC GASTROENTEROLOGY AND NUTRITION, 1998, 26 (01) :49-55
[25]  
NobleJamieson G, 1996, J ROY SOC MED, V89, P31
[26]  
NOUSIAARVANITAK.S, 1988, HELL J GASTROENTEROL, V1, P323
[27]  
OConnor PJ, 1996, HEPATOLOGY, V23, P281
[28]   Ursodeoxycholic acid in the treatment of cholestasis of pregnancy: a randomized, double-blind study controlled with placebo [J].
Palma, J ;
Reyes, H ;
Ribalta, J ;
Hernandez, I ;
Sandoval, L ;
Almuna, R ;
Liepins, J ;
Lira, F ;
Sedano, M ;
Silva, O ;
Toha, D ;
Silva, JJ .
JOURNAL OF HEPATOLOGY, 1997, 27 (06) :1022-1028
[29]   Liver disease in children with cystic fibrosis: US-biochemical comparison in 195 patients [J].
Patriquin, H ;
Lenaerts, C ;
Smith, L ;
Perreault, G ;
Grignon, A ;
Filiatrault, D ;
Boisvert, J ;
Roy, CC ;
Rasquin-Weber, A .
RADIOLOGY, 1999, 211 (01) :229-232
[30]   Combined analysis of randomized controlled trials of ursodeoxycholic acid in primary biliary cirrhosis [J].
Poupon, RE ;
Lindor, KD ;
CauchDudek, K ;
Dickson, ER ;
Poupon, R ;
Heathcote, EJ .
GASTROENTEROLOGY, 1997, 113 (03) :884-890