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[Importance of bile acids for intra-hepatic cholestasis of pregnancy].

Intrahepatic cholestasis during pregnancy is a risk factor for prematurity, respiratory distress, fetal death in utero and exposure to meconium stained liquor. Treatment is based on ursodeoxycholic acid, which allows the pregnancy to continue until term. There is no consensus for labor induction criteria or for extraction of the fetus. We report a series of 10 patients who presented cholestasis during pregnancy and for whom we monitored the bile acid levels. These assays provided the means of confirming the diagnosis in patients suffering from pruritus. The threshold of 40 micromoles/L could b…

Bile acid therapy and markers of immune-mediated damage in primary biliary cirrhosis.

In primary biliary cirrhosis (PBC), liver damage results from both immune- and bile acid-mediated mechanisms. Therapies directed against the … Using this marker, evidence has been presented to suggest an additive effect of the combination of ursodeoxycholic acid (UDCA) with azathioprine and prednisone in a short-term trial in PBC.

Management of Intrahepatic Cholestasis of Pregnancy: Recommendations of the Working Group on Obstetrics and Prenatal Medicine - Section on Maternal Disorders.

Intrahepatic cholestasis of pregnancy (ICP) is the most common liver disease specific to pregnancy. The cardinal symptom of pruritus and a c … leads to stillbirth. Therefore, predictive fetal monitoring is not possible. While medication with ursodeoxycholic acid (UDCA) improves pruritus, it has not been shown to affect fetal outcome. The indication for induction of labour depends on bile acid levels and gestational age. There is a high risk of recurrence in subsequent pregnancies.

Comprehensive overview of progressive familial intrahepatic cholestasis type 3 and the importance of pruritus as a diagnostic clue.

This case report illustrates a rare case of progressive familial intrahepatic cholestasis type 3. Pruritus was the predominant symptom that led to the diagnosis of this condition in an otherwise healthy adolescent. The onset of symptoms can happen at any age, and the diagnosis may be delayed due to a lack of severe symptoms. Genetic testing confirms the diagnosis. Therapy with ursodeoxycholic acid is recommended, and other anti-pruritic medications can be adjuvant. Disease progression should be monitored.

Механизм защиты от гепатопатропии желчных солей в болезни печени.

Урзодоксихоловая кислота (UDCA) улучшает ферменты печени и во многих случаях гистология печени в случае заболеваний холестатической печени, таких, как первичный цирроз и первичный склерозный холангит. Помимо классических холестатических заболеваний, UDCA также улучшает биохимию печени в случае алкоголиков и хронического вирусного гепатита C. Однако главной целью лечения в рамках УДА является холестаз, и, следовательно, интерес представляют механизмы, способствующие благоприятному воздействию этих заболеваний, которые подробно рассматриваются в этой статье.

Совпадение в аннотации оригинала
Ursodeoxycholic acid (UDCA) improves liver enzymes and in many instances liver histology in cholestatic liver diseases such as primary biliary cirrhosis and primary sclerosing cholangitis. Besides classic cholestatic diseases, UDCA also improves liver biochemistry in alcoholic liver disease and in chronic viral hepatitis C. The main target of UDCA treatment,…

Case of primary biliary cirrhosis-autoimmune hepatitis overlap which manifested after delivery.

A 34-year-old woman showed liver dysfunction for the first time at 3 months after delivery. Two years later, she was referred to our department with continued liver dysfunction. She fulfilled the criteria for primary biliary cirrhosis (PBC)-autoimmune hepatitis (AIH) overlap. Liver dysfunction improved after administration of ursodeoxycholic acid and bezafibrate. To the best of our knowledge this represents the second report of PBC-AIH overlap after delivery and we discuss immunological changes during the perinatal period.

Quantitative Detection of 15 Serum Bile Acid Metabolic Products by LC/MS/MS in the Diagnosis of Primary Biliary Cholangitis.

To determine 15 bile acid metabolic products in human serum by liquid chromatography-tandem mass spectrometry (LC/MS/MS) and value their dia … eened out: Deoxycholic acid (DCA), Glycine deoxycholic acid (GDCA), Lithocholic acid (LCA), Glycine ursodeoxycholic acid (GUDCA), Taurolithocholic acid (TLCA), Tauroursodeoxycholic acid (TUDCA), Taurodeoxycholic acid (TDCA), Glycine chenodeoxycholic acid (GCDCA). The performance of the biomarkers was evaluated by the AUC, specificity and sensitivity. In conclusion, DC …

Hepatic real-world outcomes with obeticholic acid in primary biliary cholangitis (HEROES): A trial emulation study design.

BACKGROUND AND AIMS: Primary biliary cholangitis is a rare, progressive liver disease. Obeticholic acid (OCA) received accelerated approval for treating patients with primary biliary cholangitis in whom ursodeoxycholic acid failed, based on a surrogate endpoint of reduction in ALP. Analysis of the long-term safety extension with 2 external control groups demonstrated a significant increase in event-free survival in OCA-treated patients. This fully real-world evidence study assessed the effect of OCA treatment on clinical outcomes. APPROACH AND RESULTS: This trial emulation used data from the …

Second-Line Treatment for Patients With Primary Biliary Cholangitis: A Systematic Review With Network Meta-Analysis.

BACKGROUND & AIMS: Approximately 40% of patients with Primary Biliary Cholangitis (PBC) show incomplete response to ursodeoxycholic acid, thus needing second-line treatment to prevent disease progression. As no head-to-head comparison study is available, we used a network meta-analysis (NMA) to compare efficacy and safety of available second-line therapies. METHODS: We performed a systematic literature review including randomised, placebo-controlled trials of patients with PBC and incomplete response, or intolerance, to ursodeoxycholic acid, and compared relative risks (RRs) for primary (bioc…

Clinical grading of intrahepatic cholestasis pregnancy.

Intrahepatic cholestasis of pregnancy (ICP) is the pregnancy induced liver disorder causing intense itching of palm, sole or even whole body … us in uterus is unsafe if mother's bile acid exceeds normal value. ICP is successfully treated with Ursodeoxycholic acid (UDCA), S-adenosyl methionine (SAME), Dexamethasone and vitamin K.

Tauroursodeoxycholic acid for treatment of primary biliary cirrhosis. A dose-response study.

Tauroursodeoxycholic acid, a highly hydrophilic bile acid, may be of therapeutic value for chronic cholestatic liver diseases. We performed a dose-response study on 24 patients with primary biliary cirrhosis who were randomly assigned to receive 500, 1000, or 1500 mg daily of tauroursodeoxycholic acid for six months. Biliary enrichment with ursodeoxycholic acid ranged from 15% to 48% and was not related with the dose. Serum liver enzyme levels decreased significantly after the first month of treatment with all the three doses. No significant difference among the three doses was found, although…

[Bile acids in liver diseases--current indications].

Ursodeoxycholic acid (UDCA) has beneficial effects in cholestatic diseases such as primary biliary cirrhosis, primary sclerosing cholangitis and cholestasis of pregnancy. In chronic hepatitis and alcoholic hepatitis, an effect of UDCA is uncertain. After organ transplantation (liver, bone, heart), favorable effects of UDCA which await confirmation were observed. UDCA very likely has a beneficial effect in children with Byler's and Alagille's syndrome, extrahepatic biliary atresia after the Kasai procedure and in cholestasis of cystic fibrosis. Unclear is the effect of UDCA in benign intermitte…

Laparoscopic aided cholecystostomy as a treatment of inspissated bile syndrome.

We report a case of a newborn girl with inspissated bile syndrome (IBS) that did not respond to treatment with oral ursodeoxycholic acid (Ursofalk). A solution was found using laparoscopic aided cholecystostomy with an indwelling catheter for local Ursofalk flushing in the gallbladder and the choledochus. This is the first report of a laparoscopic aided management of IBS without cholecystectomy or exploration of the bile ducts. This minimal invasive approach showed a clear advantage for the patient. There were no complications. The method is recommended in the treatment of IBS.

[Development of vibration-induced intrahepatic cholestasis in pilots and new ways of correcting these disorders].

Data of analysis of the role of vibration in the development of hepatobiliary pathology in helicopter pilots are reported. Vibration was found to drastically deteriorate colloid-osmotic qualities of the bile and increase the lithogenesis risk. Exposure to vibration over 10 and more years of the flying career may instigate cholelithiasis. Dynamic USI with functional testing for early diagnostics and correction with ursodeoxycholic acid (ursosan) of disorders in the colloid-osmotic properties of the bile and can be proposed as one of the methods to prevent cholelithiasis.

[Medical treatment of primary biliary cirrhosis].

The medical treatment of primary biliary cirrhosis can be divided into two parts: the treatment of the complications (cholestasis and portal hypertension) and the treatment of the underlying disease. As regards the treatment of the disease itself, there is to date no drug which has been shown to ameliorate the pathological process and to prolong survival: cyclosporine and ursodeoxycholic acid, recently used have shown promising responses in liver biochemistry but long-term controlled studies are required to assess their effectiveness on the disease process and on survival.

Potential Antihypertensive Mechanisms of the Egg White-Derived Peptide QIGLF in Spontaneously Hypertensive Rats Revealed Using Untargeted Serum Metabolomics.

The angiotensin-converting enzyme (ACE) inhibitory peptide QIGLF derived from egg white was shown to have significant in vivo antihypertensi … oups. The results suggested that eight potential biomarkers were identified, that is, adrenic acid, ursodeoxycholic acid, glycocholic acid, taurocholic acid, tryptophan, acetylindoxyl, tyrosine, and 2-phenylethanol, which were mainly involved in aromatic amino acid biosynthesis and metabolism, biosynthesis of bile acid, and biosynthesis of unsaturated fatty acids. QIG …

Differential response of the liver to bile acid treatment in a mouse model of Niemann-Pick disease type C.

Niemann-Pick disease type C (NPC) disease is a neurodegenerative lysosomal storage disease caused by mutations in the NPC1 or NPC2 genes. Liver disease is also a common feature of NPC that can present as cholestatic jaundice in the neonatal period. Liver enzymes can remain elevated above the normal range in some patients as they age. We recently reported suppression of the P450 detoxification system in a mouse model of NPC disease and in post-mortem liver from NPC patients. As bile acids regulate the P450 system, we tested bile acid treatment using ursodeoxycholic acid (UDCA; 3α, 7β-dihydroxy-…

Effective treatment of autoimmune hepatitis-primary biliary cholangitis overlap syndrome with obeticholic acid.

There is no consensus treatment for patients with autoimmune hepatitis (AIH) - primary biliary cholangitis (PBC) overlap syndrome who are not responding to conventional therapy. We present a case of a 43-yr-old woman with AIH-PBC overlap syndrome treated with obeticholic acid (OCA). The patient showed a reduction in liver enzymes and no fibrosis progression during 15 months of follow up.

Therapeutic role of bile acids and nuclear receptor agonists in fibrosing cholangiopathies.

Chronic inflammatory bile duct diseases such as primary biliary cirrhosis (PBC) and primary sclerosing cholangitis (PSC) result in progressive fibrosis of the biliary tract and ultimately cirrhosis of the liver. Since the etiology and pathogenesis of these fibrosing cholangiopathies are still poorly understood, therapeutic options are rather limited at present. Ursodeoxycholic acid (UDCA) is the paradigm therapeutic bile acid and established standard treatment for PBC, but its role for medical therapy of PSC is still under debate. Promising novel bile acid-based therapeutic options include 24-…

[Chronic cholestatic liver diseases - Primary biliary cholangitis and Primary sclerosing cholangitis].

Cholestasis is defined as hepatocyte and cholangiocyte bile excretion failure or failure of bile transport to the duodenum. Primary biliary … cal histological patern. Most patients are asymptomatic in time of diagnosis. First line therapy is ursodeoxycholic acid. In case of first line therapy failure, the prognosis is unfavourable. In this case, second line therapy must be considered. In case of PSC the diagnosis is based on MRCP finding mainly, laboratory test and liver biopsy in some cases. Progressive in …