scholarly journals Bile Acid Synthesis Disorders in Japan: Long-Term Outcome and Chenodeoxycholic Acid Treatment

2021 ◽  
Author(s):  
Akihiko Kimura ◽  
Tatsuki Mizuochi ◽  
Hajime Takei ◽  
Akira Ohtake ◽  
Jun Mori ◽  
...  
Keyword(s):  
Bile Acid ◽  
Chenodeoxycholic Acid ◽  
Acid Treatment ◽  
Acid Synthesis ◽  
Bile Acid Synthesis ◽  
Term Outcome ◽  
Long Term Outcome

Bile acid synthesis disorders in Japan: long-term outcome and chenodeoxycholic acid treatment

2020 ◽  
Author(s):  
Akihiko Kimura ◽  
Tatsuki Mizuochi ◽  
Hajime Takei ◽  
Akira Ohtake ◽  
Jun Mori ◽  
...  
Keyword(s):  
Bile Acid ◽  
Chenodeoxycholic Acid ◽  
Low Dose ◽  
Acid Synthesis ◽  
Bile Acid Synthesis ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Reductase Deficiency ◽  
Clinical Records

Abstract Background: We encountered 7 Japanese patients with bile acid synthesis disorders (BASD) over 21 years between 1996 and 2017. Diagnoses were made by bile acid and genetic analyses. We gave low-dose, long-term chenodeoxycholic acid (CDCA) treatment to 5 of the patients, who had 3β-hydroxy-Δ 5 -C 27 -steroid dehydrogenase/isomerase (3β-HSD) deficiency (n=3) or Δ 4 -3-oxosteroid 5β-reductase (5β-reductase) deficiency (n=2). Another patient with the latter diagnosis whose bile acid analyses had mitigating features was maintained on ursodeoxycholic acid according to parental preferences and now remains healthy after discontinuation of treatment. A patient with oxysterol 7α-hydroxylase deficiency developed liver failure and fully recovered after successful liver transplantation. We used clinical records to clarify long-term outcome and value of CDCA in the other patients. Efficacy of CDCA treatment was evaluated in the 5 patients given a low dose (5 to 10 mg/kg/day) for a long term. Results: Medians with ranges of current patient ages and duration of CDCA treatment are10 years (8 to 43) and 10 years (8 to 21), respectively. All 7 patients, who had homozygous or compound heterozygous mutations in the HSD3B7 , SRD5B1 , or CYP7B1 gene, are currently in good health without liver dysfunction. In the 5 patients with CDCA treatment, hepatic function gradually improved following initiation. No adverse effects were noted. Conclusions: We concluded that low-dose CDCA treatment is effective in 3β-HSD deficiency and 5β-reductase deficiency, as cholic acid has been in other countries. BASD carry a good prognosis following early diagnosis and initiation of long-term, low-dose CDCA treatment.

scholarly journals Effects of long-term plant sterol or stanol ester consumption on lipid and lipoprotein metabolism in subjects on statin treatment

2008 ◽  
Vol 100 (5) ◽  
pp. 937-941 ◽  
Author(s):  
Ariënne de Jong ◽  
Jogchum Plat ◽  
Dieter Lütjohann ◽  
Ronald P. Mensink
Keyword(s):  
Bile Acid ◽  
Plant Sterol ◽  
Ldl Cholesterol ◽  
Cholesterol Metabolism ◽  
Acid Synthesis ◽  
Statin Treatment ◽  
Bile Acid Synthesis ◽  
Control Group ◽  
Stanol Ester

Consumption of plant sterol- or stanol-enriched margarines by statin users results in an additional LDL-cholesterol reduction of approximately 10 %, which may be larger than the average decrease of 3–7 % achieved by doubling the statin dose. However, whether this effect persists in the long term is not known. Therefore, we examined in patients already on stable statin treatment the effects of 85 weeks of plant sterol and stanol ester consumption on the serum lipoprotein profile, cholesterol metabolism, and bile acid synthesis. For this, a double-blind randomised trial was designed in which fifty-four patients consumed a control margarine with no added plant sterols or stanols for 5 weeks (run-in period). For the next 85 weeks, seventeen subjects continued with the control margarine and the other two groups with either a plant sterol (n18) or plant stanol (n19) (2·5 g/d each) ester-enriched margarine. Blood was sampled at the end of the run-in period and every 20 weeks during the intervention period. Compared with the control group, plant sterol and stanol ester consumption reduced LDL-cholesterol by 0·28 mmol/l (or 8·7 %;P = 0·08) and 0·42 mmol/l (13·1 %;P = 0·006) respectively after 85 weeks. No effects were found on plasma concentrations of oxysterols or 7α-hydroxy-4-cholesten-3-one, a bile acid synthesis marker. We conclude that long-term consumption of both plant sterol and stanol esters effectively lowered LDL-cholesterol concentrations in statin users.

Long-term outcome of branched-chain amino acid treatment in patients with liver cirrhosis

2008 ◽  
Vol 38 ◽  
pp. S102-S106 ◽  
Author(s):  
Hisataka Moriwaki ◽  
Makoto Shiraki ◽  
Hideki Fukushima ◽  
Masahito Shimizu ◽  
Junpei Iwasa ◽  
...  
Keyword(s):  
Liver Cirrhosis ◽  
Amino Acid ◽  
Acid Treatment ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Branched Chain Amino Acid ◽  
Branched Chain

Oral Cholic Acid for Hereditary Defects of Primary Bile Acid Synthesis: A Safe and Effective Long-term Therapy

2009 ◽  
Vol 137 (4) ◽  
pp. 1310-1320.e3 ◽  
Author(s):  
Emmanuel Gonzales ◽  
Marie F. Gerhardt ◽  
Monique Fabre ◽  
Kenneth D.R. Setchell ◽  
Anne Davit–Spraul ◽  
...  
Keyword(s):  
Bile Acid ◽  
Cholic Acid ◽  
Acid Synthesis ◽  
Bile Acid Synthesis ◽  
Term Therapy ◽  
Long Term Therapy ◽  
Primary Bile Acid

Short-, medium- and long-term effects of ursodeoxycholic acid (UDCA) on cholesterol and bile acid synthesis in healthy humans

2000 ◽  
Vol 118 (4) ◽  
pp. A1439
Author(s):  
S. Fischer ◽  
K. Thiessen ◽  
G.H. Sauter ◽  
K. Parhofer ◽  
D. Juengst
Keyword(s):  
Bile Acid ◽  
Ursodeoxycholic Acid ◽  
Acid Synthesis ◽  
Bile Acid Synthesis ◽  
Long Term Effects ◽  
Healthy Humans

scholarly journals JNK-mediated disruption of bile acid homeostasis promotes intrahepatic cholangiocarcinoma

2020 ◽  
Vol 117 (28) ◽  
pp. 16492-16499
Author(s):  
Elisa Manieri ◽  
Cintia Folgueira ◽  
María Elena Rodríguez ◽  
Luis Leiva-Vega ◽  
Laura Esteban-Lafuente ◽  
...  
Keyword(s):  
Bile Acid ◽  
Intrahepatic Cholangiocarcinoma ◽  
Cholesterol Metabolism ◽  
Metabolic Stress ◽  
Signal Transduction Pathway ◽  
Acid Synthesis ◽  
Bile Acid Synthesis ◽  
Gene Ablation ◽  
Jnk Inhibitors

Metabolic stress causes activation of the cJun NH2-terminal kinase (JNK) signal transduction pathway. It is established that one consequence of JNK activation is the development of insulin resistance and hepatic steatosis through inhibition of the transcription factor PPARα. Indeed, JNK1/2 deficiency in hepatocytes protects against the development of steatosis, suggesting that JNK inhibition represents a possible treatment for this disease. However, the long-term consequences of JNK inhibition have not been evaluated. Here we demonstrate that hepatic JNK controls bile acid production. We found that hepatic JNK deficiency alters cholesterol metabolism and bile acid synthesis, conjugation, and transport, resulting in cholestasis, increased cholangiocyte proliferation, and intrahepatic cholangiocarcinoma. Gene ablation studies confirmed that PPARα mediated these effects of JNK in hepatocytes. This analysis highlights potential consequences of long-term use of JNK inhibitors for the treatment of metabolic syndrome.

scholarly journals Bile acid synthesis. Metabolism of 3 beta-hydroxy-5-cholenoic acid to chenodeoxycholic acid.

1986 ◽  
Vol 261 (27) ◽  
pp. 12486-12489 ◽  
Author(s):  
N B Javitt ◽  
E Kok ◽  
F Carubbi ◽  
T Blizzard ◽  
M Gut ◽  
...  
Keyword(s):  
Bile Acid ◽  
Chenodeoxycholic Acid ◽  
Acid Synthesis ◽  
Bile Acid Synthesis

Short-medium- and long-term effect of ursodeoxycholic acid on cholesterol an bile acid synthesis in healthy humans

2001 ◽  
Vol 120 (5) ◽  
pp. A387
Author(s):  
Kai Thiessen ◽  
Gerd Sauter ◽  
Klaus Georg Parhofer ◽  
Dieter Juengst ◽  
Sven Fischer
Keyword(s):  
Bile Acid ◽  
Ursodeoxycholic Acid ◽  
Acid Synthesis ◽  
Bile Acid Synthesis ◽  
Term Effect ◽  
Healthy Humans ◽  
Long Term Effect
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