- Biliary papillomatosis. [Journal Article]
- DEDig Endosc 2018 Nov 15
- A 74-year-old asymptomatic man was referred to the outpatient clinic by his general practitioner because of abnormal liver enzymes. He had no important medical history. Blood analysis showed mild cho...
A 74-year-old asymptomatic man was referred to the outpatient clinic by his general practitioner because of abnormal liver enzymes. He had no important medical history. Blood analysis showed mild cholestasis. Abdominal computed tomography showed bile duct dilatation with contrast capturing mural nodules in the common bile duct with protrusion into the dilated lumen (Figure 1a). Magnetic resonance cholangiopancreaticography revealed bile duct dilatation with multiple irregular filling defects (Figure 1b). This article is protected by copyright. All rights reserved.
- Functional analysis of the correlation between ABCB11 gene mutation and primary intrahepatic stone. [Journal Article]
- MMMol Med Rep 2018 Nov 15
- The adenosine 5'‑triphosphate binding cassette subfamily B member (ABCB)11 gene is involved in bile transport, and mutations in this gene are associated with cholestasis and cholelithiasis. Therefore...
The adenosine 5'‑triphosphate binding cassette subfamily B member (ABCB)11 gene is involved in bile transport, and mutations in this gene are associated with cholestasis and cholelithiasis. Therefore, the aim of the present study was to investigate the association between ABCB11 gene mutation and primary intrahepatic stone (PIS)s and to investigate the mechanism through which ABCB11 gene mutations affect the expression of the corresponding protein. Mutations of the ABCB11 gene in 443 PIS patients and 560 healthy participants were detected by exon sequencing. The expression levels of ABCB11 mRNA and bile salt export pump (BSEP) protein in the liver tissues of patients with PISs were measured by quantitative polymerase chain reaction and western blot analysis. The mutant plasmids constructed by site‑directed mutagenesis of the human BSEP gene were transfected into human embryonic kidney 293 (293) cells and Madin‑Darby canine kidney (MDCK) cells, and the expression and distribution of rs118109635 of BSEP was measured. There were two significant mutations in the ABCB11 gene of the PIS patients compared with the healthy population; a missense mutation, rs118109635 (P=0.025), and a synonymous mutation, rs497692 (P=0.006). The two mutations were associated with the occurrence of preoperative jaundice (P=0.026, and P=0.011, respectively). The expression levels of BSEP in PIS patients with the missense mutation rs118109635 was decreased, whereas its mRNA expression levels remained unchanged. In PIS patients with the synonymous mutation rs497692, the expression levels of ABCB11 were decreased at both the mRNA and protein level. It was also found that mutation A865V reduced the expression levels of BSEP in 293 cells at the cellular level; its distribution in MDCK cell membranes was decreased, whereas its mRNA levels remained unchanged. The mutated loci at rs118109635 and rs497692 of the ABCB11 gene were correlated with PISs, causing a decreased expression of BSEP and reduced distribution of the protein in the cell membrane. Therefore, mutations at rs118109635 and rs497692 of the ABCB11 gene may be risk factors for PISs.
- [Radiologic diagnosis of the gallbladder and bile ducts - part 2 : Acute and chronic cholecystitis, primary sclerosing cholangitis (PSC), benign and malignant masses of the biliary system]. [Journal Article]
- RRadiologe 2018 Nov 14
- Upper abdominal pain, icterus and cholestasis are the symptoms leading to evaluation of the biliary tract. Together with its complications biliary stone disease is the main reason for inflammation of...
Upper abdominal pain, icterus and cholestasis are the symptoms leading to evaluation of the biliary tract. Together with its complications biliary stone disease is the main reason for inflammation of the biliary system. A distinction is made between acute and chronic variants. In chronic bile duct inflammation primary sclerosing cholangitis (PSC), primary biliary cirrhosis (PBC) and more recently IgG4-associated sclerosing cholangitis are of particular importance. Besides benign and tumor-like-lesions, malignant entities as gallbladder carcinoma and cholangiocarcinoma (CCC) in its three locations have to be mentioned. Despite all recent improvements, specificity of bile-tract imaging still remains limited, especially regarding malignant masses. Therefore the final diagnosis is made in many cases by histological analysis.
- Liver Cholestasis Secondary to Syphilis in an Immunocompetent Patient. [Journal Article]
- CRCase Reports Hepatol 2018; 2018:8645068
- Liver involvement is a known feature of secondary syphilis. The prevalence of hepatitis in secondary syphilis ranges broadly from 1 to 50%. We report a case of a 37-year-old man with type 1 diabetes ...
Liver involvement is a known feature of secondary syphilis. The prevalence of hepatitis in secondary syphilis ranges broadly from 1 to 50%. We report a case of a 37-year-old man with type 1 diabetes mellitus and sickle cell trait presenting with jaundice and acute liver cholestasis. Abdominal ultrasound revealed mild hepatic fatty infiltration. RPR and Treponema pallidum IgG results were positive with a reflex titer of 1:64. Liver biopsy revealed chronic hepatitis with normal hepatic architecture, Kupffer cell hyperplasia, hepatic cholestasis, and ductal proliferation suggestive of syphilitic hepatitis.
- Neonatal Cholestasis: A Primer of Selected Etiologies. [Journal Article]
- PAPediatr Ann 2018 Nov 01; 47(11):e433-e439
- Cholestasis refers to impairment in formation or excretion of bile. This can be due to defects in intrahepatic production of bile, defects in the transmembrane transport of bile, or mechanical obstru...
Cholestasis refers to impairment in formation or excretion of bile. This can be due to defects in intrahepatic production of bile, defects in the transmembrane transport of bile, or mechanical obstruction to bile flow. Clinical features of cholestasis reflect the retention of components of bile (bilirubin, bile acids, cholesterol) in the body. In the neonatal period, hyperbilirubinemia can be categorized as either unconjugated (and often benign) hyperbilirubinemia, or conjugated hyperbilirubinemia due to cholestasis. It is for this reason that the first laboratory evaluation in a patient with jaundice, dark urine, and/or acholic stool is a fractionated bilirubin. This article serves as a practical primer for pediatric and neonatology trainees and covers common causes of neonatal cholestasis, as well as the diagnostic work-up and treatment. Causes that are discussed include biliary atresia, idiopathic neonatal hepatitis, gestational alloimmune liver disease, metabolic and genetic diseases, total parenteral nutrition cholestasis, and congenital infection. [Pediatr Ann. 2018;47(11):e433-e439.].
- Alpha1-Antitrypsin Deficiency: Transition of Care for the Child With AAT Deficiency into Adulthood. [Journal Article]
- CPCurr Pediatr Rev 2018 Nov 12
- CONCLUSIONS: AND RELEVANCE There should be a greater vigilance for AAT deficiency testing among pediatricians. Diagnosis should prompt assessment of liver involvement. Children with AAT-deficiency-associated liver disease should be referred to a liver specialist and monitored throughout their lifetimes for the symptoms of AAT-deficiency-related pulmonary involvement.
- Adaptive homeostasis of the vitamin D-vitamin D nuclear receptor axis in 8-methoxypsoralen-induced hepatotoxicity. [Journal Article]
- TAToxicol Appl Pharmacol 2018 Nov 09
- 8-methoxypsoralen (8-MOP) with ultraviolet A radiation therapy (PUVA) is the standard therapy for patients with psoriasis, despite the reported potential risks of 8-MOP-induced cholestatic liver inju...
8-methoxypsoralen (8-MOP) with ultraviolet A radiation therapy (PUVA) is the standard therapy for patients with psoriasis, despite the reported potential risks of 8-MOP-induced cholestatic liver injury in both humans and animals. Usually, patients with chronic cholestasis exhibit lower serum 25-hydroxy vitamin D (25(OH)D) levels. But those patients receiving PUVA for psoriasis showed an increase in serum 25(OH)D levels, probably highlighting that the vitamin D-vitamin D nuclear receptor (VD-VDR) axis play a protective role in 8-MOP-induced hepatotoxicity. The present study confirmed 8-MOP could increase serum 25(OH)D levels in conventional lighting and diet (CLD) and vitamin D deficient (VDD) Sprague-Dawley rats. Potential liver risks were also found in CLD and VDD rats after 8-MOP treatment. We proved that 8-MOP could be a potent ligand for VDR using molecular docking and luciferase report assay. Effect of 8-MOP on VDR subcellular distribution was determined using human liver cell line L02. We found 8-MOP could increase VDR protein expression in the nuclear and cytosol extracts and also total cell extracts in L02. siRNAs for VDR were used to determine the role of VDR in protecting 8-MOP-induced cholestasis and potential cellular mechanisms. The results showed 8-MOP could affect the CYP7A1, SHP and MRP3 expression via VDR, and such effects could be reversed by knockdown of VDR expression, suggesting a vital role of VDR involved in 8-MOP-regulated bile acid synthesis and transportation. In conclusion, these results revealed activation of VD-VDR axis may play a beneficial role in 8-MOP-mediated regulation of bile acid synthesis and transportation.
- Steps Forward in the Management of Familial Cholestasis. [Journal Article]
- JPJ Pediatr Gastroenterol Nutr 2018 Nov 08
- Hepatoprotective Activity of the Ethanolic Extract of Polygonum multiflorum Thunb. against Oxidative Stress-Induced Liver Injury. [Journal Article]
- EBEvid Based Complement Alternat Med 2018; 2018:4130307
- Oxidative stress is an important pathological mechanism in various liver diseases. Polygonum multiflorum Thunb. (PM) can be used for the treatment of diseases associated with aging, hyperlipidemia, a...
Oxidative stress is an important pathological mechanism in various liver diseases. Polygonum multiflorum Thunb. (PM) can be used for the treatment of diseases associated with aging, hyperlipidemia, and oxidative stress in traditional Chinese medicine. In this study, we examined the hepatoprotective effects of the ethanolic extract of PM (PME) in in vitro and in vivo models. The PME induced expression of antioxidant-response-element- (ARE-) related genes in HepG2 cells showed a dose-dependent manner. Pretreatment of HepG2 cell with PME suppressed H2O2- and acetaminophen- (APAP-) induced cellular reactive oxygen species (ROS) generation and cytotoxicity. In APAP-induced mouse liver injury, pretreatment with PME also showed ability to increase the survival rate and reduce the severity of liver injury. Treatment with PME attenuated bile duct ligation-induced extrahepatic cholestatic liver injury and further increased multidrug resistance protein 4 (MRP4) and reduced organic anion-transporting polypeptide (OATP) expression. Furthermore, increased nuclear translocation of the nuclear factor erythroid 2-related factor 2 (Nrf2) was observed after treatment with PME in both in vivo models. In conclusion, the current study showed the hepatoprotective activity of PME by regulating the redox state in liver injury through Nrf2 activation and controlling hepatic bile acid homeostasis in obstructive cholestasis, through bile acid transporter expression modulation.
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- Ductopenia and cirrhosis in a 32-year-old woman with progressive familial intrahepatic cholestasis type 3: A case report and review of the literature. [Journal Article]
- WJWorld J Gastroenterol 2018 Nov 07; 24(41):4716-4720
- Progressive familial intrahepatic cholestasis type 3 is caused by a mutation in the ATP-binding cassette, subfamily B, member 4 (ABCB4) gene encoding multidrug resistance protein 3. A 32-year-old wom...
Progressive familial intrahepatic cholestasis type 3 is caused by a mutation in the ATP-binding cassette, subfamily B, member 4 (ABCB4) gene encoding multidrug resistance protein 3. A 32-year-old woman with a history of acute hepatitis at age 9 years was found to have jaundice during pregnancy in 2008, and was diagnosed as having intrahepatic cholestasis of pregnancy. In 2009, she underwent cholecystectomy for gallstones and chronic cholecystitis. However, itching and jaundice did not resolve postoperatively. She was admitted to our hospital with fatigue, jaundice, and a recently elevated γ-glutamyl transpeptidase level. Liver biopsy led to the diagnosis of biliary cirrhosis with ductopenia. Genetic testing revealed a pathogenic heterozygous mutation, ex13 c.1531G > A (p.A511T), in the ABCB4 gene. Her father did not carry the mutation, but her mother's brother carried the heterozygous mutation. We made a definitive diagnosis of familial intrahepatic cholestasis type 3. Her symptoms and liver function improved after 3 mo of treatment with ursodeoxycholic acid.