Search results for "Cholestasis"

Are the new genetic tools for diagnosis of Wilson disease helpful in clinical practice?

2020

Summary The diagnosis of Wilson disease is not always easy. For many patients, a combination of tests reflecting disturbed copper metabolism may be needed. Testing for ATP7B variants has become part of the routine diagnostic approach. The methods of genetic testing include analysis of the 21 coding exons and intronic flanking sequences, in which exons with recurrent variants would be prioritised depending on the mutation frequency in the local population. If sequencing the entire ATP7B gene cannot identify 2 variants and the suspicion for Wilson disease is high, after reviewing the clinical data, WES (whole-exome sequencing) or WGS (whole-genome sequencing) could be applied. A workflow base…

Double-balloon endoscopy for retrograde cholangiography in patients with choledochojejunostomy and Roux-en-Y reconstruction.

2009

BACKGROUND Choledochojejunal anastomoses with Roux-en-Y reconstruction excludes the biliary tract from conventional endoscopic retrograde cholangiography (ERC) with standard endoscopes due to the length of the interposed small bowel segment. Double-balloon enteroscopy (DBE) facilitates deep insertion into the small bowel and may be used to perform ERC in these patients. In the present case series we report our experience with diagnostic and therapeutic double-balloon ERC in patients with choledochojejunostomy to a long Roux-en-Y loop previously unavailable for standard length endoscopes. PATIENTS AND METHODS Between December 2004 and May 2008 15 patients (mean age: 60.2 years) with choledoc…

Intrahepatic cholestasis of pregnancy after ovarian hyperstimulation syndrome with wild-type ABCB4 gene: a peculiar case and literature review

2023

Background: Intrahepatic cholestasis of pregnancy (ICP) in the first trimester occurring after ovarian hyperstimulation syndrome (OHSS) is a rare condition and few cases are reported in the literature. Hyperestrogenism may explain this problem in genetically predisposed women. The objective of this article is to report one of these rare cases and offer an overview of the other published cases. Case presentation: We report a case of severe OHSS followed by ICP in the first trimester. The patient was admitted to the intensive care unit and was treated according to the guidelines for the management of OHSS. Moreover, the patient also received ursodeoxycholic acid for ICP, which brought to an i…

Quantitative Bestimmung des Gallenr�ckflusses in Blut und Leberlymphe von Ratten bei beginnender Gallenstauung

1970

1. Mit dem Ziel, die Anteile des bei einer Gallenstauung auftretenden Gallenruckflusses in Blut und Lymphe quantitativ zu erfassen, wurde ein Verfahren zur Gewinnung reiner Leberlymphe bei Ratten entwickelt. 2. Mit Hilfe dieser Methode konnte am Versuchstier mit normaler Korpertemperatur aus Messungen der Lymphstromstarke und des Eiweisgehaltes der Leberlymphe vor und wahrend einer Gallenstauung auf einen Ruckflus der fortlaufenden produzierten Galle in die Leberlymphe von ca. 7–10% geschlossen werden. 3. In Ruckflusversuchen an unterkuhlten Ratten mit Ringerlosung, die Evansblue enthielt, ergaben Analysen von Blut und Leberlymphe einen Anteil des Ruckflusses in diese Lymphe von ca. 13%.

Multicentre Survey of the Prevalence of Intrahepatic Cholestasis in 2520 Consecutive Patients with Newly Diagnosed Chronic Liver Disease

1992

In order to establish the prevalence of intrahepatic cholestasis (IHC) in chronic liver disease, we retrospectively evaluated the frequency of increased serum total bilirubin (STB), serum conjugated bilirubin (SCB) and serum alkaline phosphatase (SAP) levels in a cohort of 2520 patients with newly diagnosed chronic liver disease presenting over 2 years. 882 patients (35%) [mean age 52.2 years] had IHC involving abnormal levels of STB (mean 89.8 μmol/L), SCB (mean 52.5 μmol/L), and SAP (mean 5.7 μkat/L) together with a negative ultrasonic scan. Demographic data were not predictive of IHC. IHC was more frequent in patients with cirrhosis (43%), primary biliary cirrhosis (54%), and primary scl…

The ABCB4 p.T175A variant as potential modulator of hepatic fibrosis in patients with chronic liver diseases: Looking beyond the cholestatic realm

2017

TGF-β2 silencing to target biliary-derived liver diseases

2020

ObjectiveTGF-β2 (TGF-β, transforming growth factor beta), the less-investigated sibling of TGF-β1, is deregulated in rodent and human liver diseases. Former data from bile duct ligated and MDR2 knockout (KO) mouse models for human cholestatic liver disease suggested an involvement of TGF-β2 in biliary-derived liver diseases.DesignAs we also found upregulated TGFB2 in liver tissue of patients with primary sclerosing cholangitis (PSC) and primary biliary cholangitis (PBC), we now fathomed the positive prospects of targeting TGF-β2 in early stage biliary liver disease using the MDR2-KO mice. Specifically, the influence of TgfB2 silencing on the fibrotic and inflammatory niche was analysed on m…

A cholestatic pattern predicts major liver-related outcomes in patients with non-alcoholic fatty liver disease

2022

NAFLD patients usually have an increase in AST/ALT levels, but cholestasis can also be observed. We aimed to assess in subjects with NAFLD the impact of the (cholestatic) C pattern on the likelihood of developing major liver-related outcomes (MALO).

Deletion of organic cation transporter Oct3 promotes hepatic fibrosis via upregulation of TGFβ

2019

Organic cation transporters (OCT) are responsible for the intracellular uptake and detoxification of a broad spectrum of endogenous and exogenous substrates. OCTs are downregulated in cholestasis, fibrosis, and hepatocellular carcinoma, but the underlying molecular mechanisms and downstream effects of OCT deletion are unknown. Oct3-knockout ( Oct3−/−; FVB.Slc22a3tm10pb) and wild-type (WT; FVB) mice were subject to escalating doses of carbon tetrachloride (CCl4) or thioacetamide (TAA) for 6 wk to induce advanced parenchymal liver fibrosis. Secondary biliary fibrosis was generated by bile duct ligation. Liver fibrosis was assessed by hydroxyproline determination, quantitative Sirius red morp…

Both cholestatic and steatotic drugs trigger extensive alterations in the mRNA level of biliary transporters in rat hepatocytes: Application to devel…

2016

Disruption of the vectorial bile acid transport in the liver is a key feature of cholestatic drugs, although many causal and mechanistic aspects are still unknown. The aim of the present study was to explore if cholestatic drugs can repress or induce the expression of hepatic transporters. To this end, sandwich-cultured rat hepatocytes were treated with cholestatic and non-cholestatic (steatotic, non-hepatotoxic, etc.) drugs and the mRNA expression of 10 uptake and efflux biliary transporters was measured. Results evidenced that all cholestatic drugs cause extensive alterations in the mRNA expression of most biliary transporters. Surprisingly, nearly all steatotic drugs also affected the ex…