Changes in classic and alternative pathways of bile acid synthesis in chronic liver disease

2007 ◽  
Vol 382 (1-2) ◽  
pp. 82-88 ◽  
Author(s):  
Andrea Crosignani ◽  
Marina Del Puppo ◽  
Matteo Longo ◽  
Emma De Fabiani ◽  
Donatella Caruso ◽  
...  
Keyword(s):  
Liver Disease ◽  
Bile Acid ◽  
Chronic Liver Disease ◽  
Acid Synthesis ◽  
Bile Acid Synthesis ◽  
Chronic Liver ◽  
Alternative Pathways

scholarly journals Serum Metabonomics Analysis of Liver Failure Treated by Nonbioartificial Liver Support Systems

2018 ◽  
Vol 2018 ◽  
pp. 1-10 ◽  
Author(s):  
Chunyan Wu ◽  
Ying Zhu ◽  
Mengqian Yu
Keyword(s):  
Bile Acid ◽  
Liver Failure ◽  
Hepatic Failure ◽  
Metabolic Pathways ◽  
Acid Metabolism ◽  
Acid Synthesis ◽  
Bile Acid Synthesis ◽  
Chronic Liver ◽  
Artificial Liver ◽  
Chronic Liver Failure

Objective. To analyze the small molecular metabolic compounds of nonbioartificial liver for treatment of hepatic failure and make further efforts to study the clinical efficacy, mechanism of action, and pathogenesis of hepatic failure. Methods. 52 patients who met the standard of artificial liver treatment for liver failure were enrolled; these patients included 6 cases of acute liver failure (11.54%), 3 cases of subacute liver failure (5.77%), acute-on-chronic liver failure in 10 cases (19.23%), and 33 cases of chronic liver failure (63.46%). Treatment modes included plasma exchange in 34 patients (65.38%), bilirubin adsorption in 9 patients (17.31%), and hemofiltration in 9 patients (17.31%). The clinical efficacy of artificial liver was assessed by monitoring the effects in the near future. Significant changes in metabolic compounds of liver failure in the treatment before and after artificial liver were screened by using Ultra-Performance Liquid Chromatography-Mass Spectrometry (UPLC-MS). Related metabolic pathways were analyzed by MetaboAnalyst. Results. After artificial liver treatment, the liver function and coagulation function of liver failure patients were significantly improved (P < 0.01), the Meld score was lower than that before treatment, and the difference was statistically significant (P < 0.05). Serum metabolomics identified 29 small metabolic compounds and 12 metabolic pathways with variable projection importance (VIP) greater than 1 before and after artificial liver treatment. There were 11 metabolic compounds of VIP over 1 and 7 metabolic pathways in the different modes of artificial liver treatment for chronic liver failure. Among them, bile acid metabolism, fatty acid metabolism, and amino acid metabolism are the main sources. Conclusion. Artificial liver treatment can effectively improve liver function and blood coagulation function and Meld score, clinical symptoms and signs in patients with liver failure; the curative effect of artificial liver was verified, which reflected the clinical value of artificial liver in the treatment of liver failure. Artificial liver treatment of liver failure on fatty acids and primary bile acid synthesis pathway was the most significant. The difference of fatty acid, primary bile acid synthesis pathway, and phenylalanine metabolic pathway in different artificial liver patterns of chronic liver failure was the most significant. This provides a new basis for understanding the mechanism of hepatic failure and the mechanism of liver failure by artificial liver treatment.

scholarly journals Total bile acid in chronic liver disease.

Kanzo ◽  
1985 ◽  
Vol 26 (7) ◽  
pp. 883-890
Author(s):  
Kenichi FUKUOKA
Keyword(s):  
Liver Disease ◽  
Bile Acid ◽  
Chronic Liver Disease ◽  
Total Bile Acid ◽  
Chronic Liver

scholarly journals Rifampicin, not vitamin E, suppresses parenteral nutrition-associated liver disease development through the pregnane X receptor pathway in piglets

2020 ◽  
Vol 318 (1) ◽  
pp. G41-G52 ◽  
Author(s):  
Gregory Guthrie ◽  
Barbara Stoll ◽  
Shaji Chacko ◽  
Charlotte Lauridsen ◽  
Jogchum Plat ◽  
...  
Keyword(s):  
Liver Disease ◽  
Bile Acid ◽  
Vitamin E ◽  
Parenteral Nutrition ◽  
Pregnane X Receptor ◽  
Acid Synthesis ◽  
Bile Acid Synthesis ◽  
Bile Acid Metabolism ◽  
Neonatal Piglets ◽  
Hyocholic Acid

Infants receiving long-term parenteral nutrition (PN) develop PN-associated liver disease (PNALD). We previously (Ng K et al. JPEN J Parenter Enteral Nutr 40: 656–671, 2016. doi: 10.1177/0148607114567900 .) showed that PN containing soy-based lipid supplemented with vitamin E (α-tocopherol) prevents the development of PNALD. We hypothesize that this occurs via vitamin E activation of pregnane X receptor (PXR)-mediated pathways involved in bile acid metabolism. Neonatal piglets received PN for 14 days containing Intralipid (IL; soy-based lipid emulsion), IL supplemented with 12.6 mg·kg−1·day−1 vitamin E (VITE), or IL with 10 mg·kg−1·day−1 Rifadin IV (RIF), a PXR agonist. Pigs treated with IL and VITE, but not RIF, developed cholestasis and hyperbilirubinemia, markers of liver disease. The hepatic PXR target genes CYP3A29 and UGT1A6 increased during RIF treatment. RIF also modestly increased metabolism of chenodeoxycholic acid to the more hydrophilic bile acid hyocholic acid. Serum fibroblast growth factor (FGF)-19, a key regulator in suppressing hepatic bile acid synthesis, significantly increased in the RIF group. We conclude rifampicin modified markers of PNALD development by increased metabolism of bile acids and potentially suppressed bile acid synthesis. Vitamin E was ineffective at high lipid doses in preventing PNALD. NEW & NOTEWORTHY Intravenous vitamin E and rifampicin were administered to neonatal piglets receiving parenteral nutrition to determine their efficacy in reducing the progression of parenteral nutrition-associated liver disease (PNALD). Rifampicin increased serum FGF-19 concentrations and synthesis of the bile acid hyocholic acid which led to a reduction of PNALD parameters at 2 wk of administration. This result has potential clinical implications for the use of rifampicin as a safe and inexpensive treatment for short-term development of PNALD.

scholarly journals Fasting serum bile acid level in chronic liver disease

Kanzo ◽  
1975 ◽  
Vol 16 (12) ◽  
pp. 851-858 ◽  
Author(s):  
Chisato HIRAYAMA ◽  
Hironaka KAWASAKI ◽  
Shunichi KOGA ◽  
Toshitake IRISA ◽  
Katsuhiko ARIMURA ◽  
...  
Keyword(s):  
Liver Disease ◽  
Bile Acid ◽  
Chronic Liver Disease ◽  
Acid Level ◽  
Chronic Liver ◽  
Serum Bile Acid ◽  
Fasting Serum ◽  
Bile Acid Level

Familial Cholestatic Cirrhosis Associated with Kayser-Fleischer Rings

PEDIATRICS ◽  
1980 ◽  
Vol 65 (4) ◽  
pp. 782-788
Author(s):  
Chaim Kaplinsky ◽  
Irmin Sternlieb ◽  
Norman Javitt ◽  
Yaacov Rotem
Keyword(s):  
Liver Disease ◽  
Bile Acid ◽  
Clinical Picture ◽  
Hepatic Cirrhosis ◽  
Wilson’S Disease ◽  
Acid Synthesis ◽  
Bile Acid Synthesis ◽  
Wilson's Disease ◽  
Acid Transport ◽  
Loading Tests

A brother and sister who suffered from pruritus since infancy developed hepatic cirrhosis early in life. Although this clinical picture has never been seen in Wilson's disease, Kayser-Fleischer rings in the boy made further studies necessary. Oral radiocopper loading tests administered to both children and to their parents served to exclude Wilson's disease conclusively. Determinations of the concentrations and patterns of bile acids in the serum indicated that the abnormalities observed in these children are not related to errors in bile acid synthesis. Although a defect in bile acid transport is present, it appears to have occurred as a consequence of the liver disease.

Cholestyramine loading test to assess hepatic reserve for bile acid synthesis in patients with chronic liver diseases

1997 ◽  
Vol 112 (4) ◽  
pp. 1277-1283 ◽  
Author(s):  
S Kuroki ◽  
T Naito ◽  
S Okamoto ◽  
H Sakai ◽  
H Yamashita ◽  
...  
Keyword(s):  
Bile Acid ◽  
Liver Diseases ◽  
Acid Synthesis ◽  
Bile Acid Synthesis ◽  
Chronic Liver ◽  
Chronic Liver Diseases ◽  
Loading Test ◽  
Hepatic Reserve
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