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Intrahepatic Cholestasis

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https://www.readbyqxmd.com/read/28437442/relationship-between-early-onset-severe-intrahepatic-cholestasis-of-pregnancy-and-higher-risk-of-meconium-stained-fluid
#1
Maria C Estiú, Maria A Frailuna, Carla Otero, Marcela Dericco, Catherine Williamson, Jose J G Marin, Rocio I R Macias
BACKGROUND: Intrahepatic cholestasis of pregnancy (ICP) is the commonest gestational liver disease. The risk of adverse fetal outcome has been associated with the severity of maternal hypercholanemia after diagnosis. OBJECTIVE: To investigate whether there is a relationship between the severity and timing of onset of hypercholanemia and the risk of meconium-stained amniotic fluid (MSAF) and adverse neonatal events. STUDY DESIGN: The study included 382 pregnancies complicated by ICP managed at a referral hospital in Buenos Aires (Argentina) between June 2009 and December 2013...
2017: PloS One
https://www.readbyqxmd.com/read/28425419/a-rare-bsep-mutation-associated-with-a-mild-form-of-progressive-familial-intrahepatic-cholestasis-type-2
#2
Orith Waisbourd-Zinman, Lea F Surrey, Anna E Schwartz, Pierre A Russo, Jessica Wen
Progressive Familial Intrahepatic Cholestasis type 2 (PFIC2) is a rare cholestatic disorder diagnosed in infancy or childhood that can lead to severe hepatic fibrosis and liver failure. Mutations in the ABCB11 gene result in a deficiency of the bile salt export protein (BSEP) and accumulation of bile inside the hepatocytes. Hepatocellular carcinoma is another condition associated with severe forms of deletion mutations in the ABCB11 gene. Treatment options including ursodeoxycholic acid biliary diversion have mixed outcomes and some patients require liver transplantation...
May 2017: Annals of Hepatology
https://www.readbyqxmd.com/read/28425250/retrospective-analysis-of-maternal-fetal-and-neonatal-outcomesof-intrahepatic-cholestasis-of-pregnancy-at-gazi-university
#3
Berrin Günaydin, Merih Bayram, Melis Altuğ, Semra Cevher, Nuray Bozkurt
BACKGROUND/AIM: Maternal, fetal, and neonatal outcomes in parturients with intrahepatic cholestasis of pregnancy (ICP) have been retrospectively documented. We aimed to present pregnancy outcomes of parturients with ICP who underwent delivery. The study was conducted during a 1-year period. MATERIALS AND METHODS: After ethics committee approval, data from 1 January to 31 December 2015 were collected to identify parturients with ICP. RESULTS: Ten out of 37 patients underwent normal spontaneous vaginal delivery (NSVD), and the remaining 27 parturients underwent cesarean section (CS)...
April 18, 2017: Turkish Journal of Medical Sciences
https://www.readbyqxmd.com/read/28416007/disseminated-alveolar-echinococcosis-resembling-metastatic-malignancy-a-case-report
#4
Laura Caire Nail, Ezequiel Rodríguez Reimundes, Christelle Weibel Galluzzo, Dan Lebowitz, Yasmine Lucile Ibrahim, Johannes Alexander Lobrinus, François Chappuis
BACKGROUND: Alveolar echinococcosis is a potentially lethal zoonosis caused by larval forms of the tapeworm Echinococcus multilocularis. Humans are aberrant intermediate hosts who become infected by ingestion of egg-contaminated food or water or via physical contact with domestic or wild animals that carry the parasite in their small intestine. In humans, the disease usually affects the liver and can spread to other organs causing metastatic infiltration. In this report, we describe an advanced presentation of human alveolar echinococcosis mimicking metastatic malignancy...
April 18, 2017: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/28385542/model-systems-for-studying-the-role-of-canalicular-efflux-transporters-in-drug-induced-cholestatic-liver-disease
#5
REVIEW
Bruno Stieger, Zainab M Mahdi
Bile formation is a key function of the liver. Disturbance of bile flow may lead to liver disease and is called cholestasis. Cholestasis may be inherited such as for example in progressive familial intrahepatic cholestasis or acquired, such as for example by drug-mediated inhibition of bile salt export from hepatocytes into the canaliculi. The key transport system for exporting bile salts into the canaliculi is the bile salt export pump. Inhibition of the bile salt export pump by drugs is a well-established cause of drug-induced cholestasis...
April 3, 2017: Journal of Pharmaceutical Sciences
https://www.readbyqxmd.com/read/28379597/post-liver-transplant-intrahepatic-cholestasis-etiology-clinical-presentation-therapy
#6
F R Ponziani, S Bhoori, M Pompili, M A Zocco, M Biolato, G Marrone, A Gasbarrini, V Mazzaferro, A Grieco
Post-liver transplant intrahepatic cholestasis is consequent to the impairment of bile flow or formation. It may develop in the early (within 6 months) or in the late (more than 6 months) post-liver transplant period and different causes may be recognized according to the time elapsed from a liver transplant. The raise at various degrees of serum bilirubin, alkaline phosphatase, and gamma-glutamyl transpeptidase, with or without increased transaminases levels, are common hematochemical findings. Liver histology is helpful for diagnostic assessment, and sometimes crucial to differentiate among possible causes of cholestasis...
March 2017: European Review for Medical and Pharmacological Sciences
https://www.readbyqxmd.com/read/28376740/maternal-27-hydroxycholesterol-concentrations-during-the-course-of-pregnancy-and-in-pregnancy-pathologies
#7
Brigitte Sophia Winkler, Ulrich Pecks, Laila Najjari, Nicola Kleine-Eggebrecht, Nicolai Maass, Markus Mohaupt, Geneviève Escher
BACKGROUND: The oxysterol 27-hydroxycholesterol (27-OHC) plays an important role in the regulation of cholesterol homeostasis. Pregnancy pathologies like preeclampsia (PE), HELLP-syndrome (HELLP), intrauterine growth restriction (IUGR) and intrahepatic cholestasis in pregnancy (ICP) are linked to disturbances in lipid metabolism. In the present study, we hypothesized a specific gestational regulation of 27-OHC and compromised 27-OHC levels due to placental and hepatic diseases in pregnancy resulting in a dysregulation of lipid metabolism...
April 4, 2017: BMC Pregnancy and Childbirth
https://www.readbyqxmd.com/read/28337145/dioscin-protects-anit-induced-intrahepatic-cholestasis-through-regulating-transporters-apoptosis-and-oxidative-stress
#8
Hong Yao, Youwei Xu, Lianhong Yin, Xufeng Tao, Lina Xu, Yan Qi, Xu Han, Pengyuan Sun, Kexin Liu, Jinyong Peng
Intrahepatic cholestasis, a clinical syndrome, is caused by excessive accumulation of bile acids in body and liver. Proper regulation of bile acids in liver cells is critical for liver injury. We previously reported the effects of dioscin against α-naphthylisothio- cyanate (ANIT)-induced cholestasis in rats. However, the pharmacological and mechanism data are limited. In our work, the animals of rats and mice, and Sandwich-cultured hepatocytes (SCHs) were caused by ANIT, and dioscin was used for the treatment...
2017: Frontiers in Pharmacology
https://www.readbyqxmd.com/read/28333693/pediatric-intestinal-failure-associated-liver-disease
#9
Cathleen M Courtney, Brad W Warner
PURPOSE OF REVIEW: The goal of this review is to provide updates on the definition, pathophysiology, treatment, and prevention of intestinal failure-associated liver disease (IFALD) that are relevant to care of pediatric patients. RECENT FINDINGS: Current literature emphasizes the multifactorial nature of IFALD. The pathogenesis is still largely unknown; however, molecular pathways have been identified. Key to these pathways are proinflammatory cytokines involved in hepatic inflammation and bile acids synthesis such as Toll-like receptor 4 and farnesoid X receptor, respectively...
March 22, 2017: Current Opinion in Pediatrics
https://www.readbyqxmd.com/read/28326516/elevated-red-blood-cell-distribution-width-is-associated-with-intrahepatic-cholestasis-of-pregnancy
#10
Zehra Vural Yilmaz, Gulenay Gencosmanoglu Turkmen, Korkut Daglar, Elif Yılmaz, Ozgur Kara, Dilek Uygur
OBJECTIVES: Intrahepatic cholestasis of pregnancy is the most common pregnancy specific liver disease and related with adverse maternal and perinatal outcome. Red blood cell distribution width, an anisocytosis marker in a complete blood count, has been used as an inflammation marker in various diseases. However the association of red blood cell distribution width with intrahepatic cholestasis of pregnancy is unknown. We aimed to evaluate the relationship between red blood cell distribution width and intrahepatic cholestasis of pregnancy...
2017: Ginekologia Polska
https://www.readbyqxmd.com/read/28319428/two-cases-of-first-onset-intrahepatic-cholestasis-of-pregnancy-associated-with-moderate-ovarian-hyperstimulation-syndrome-after-ivf-treatment-and-review-of-the-literature
#11
Mehmet Firat Mutlu, Koray Aslan, Ismail Guler, Ilknur Mutlu, Mehmet Erdem, Nuray Bozkurt, Ahmet Erdem
Intrahepatic cholestasis of pregnancy (ICP) is an uncommon disorder, which generally occurs in the second and third trimester of pregnancy with symptoms of pruritus. The cause of ICP is unknown but genetic, hormonal and environmental factors contribute to its pathogenesis. The aetiology of ICP is unclear but elevation in oestrogen levels thought to cause ICP is typically seen in the third trimester of pregnancy, and for this reason it is not usually considered in the differential diagnosis of pruritus and liver function disorders in the first trimester of the pregnancy...
March 20, 2017: Journal of Obstetrics and Gynaecology: the Journal of the Institute of Obstetrics and Gynaecology
https://www.readbyqxmd.com/read/28298162/expression-and-significance-of-dendritic-cells-and-th17-treg-in-serum-and-placental-tissues-of-patients-with-intrahepatic-cholestasis-of-pregnancy
#12
Xiang Kong, Yan Kong, Fangyuan Zhang, Tingting Wang, Xiaotong Zhu
PURPOSE: Dendritic cells (DCs) are involved in immune system, which can also regulate the differentiation of T helper 17 (Th17) and regulatory T cells (Treg). DCs and Th17/Treg participate in preeclampsia and recurrent spontaneous abortion (RSA), but there is still lack of research in intrahepatic cholestasis of pregnancy (ICP). The aim was to evaluate the expression and significance of CD83(+)DCs, CD1a(+)DCs, interleukin-17 (IL-17) and IL-35 in serum and placental tissues of patients with ICP...
March 15, 2017: Journal of Maternal-fetal & Neonatal Medicine
https://www.readbyqxmd.com/read/28292174/extrahepatic-manifestations-of-primary-biliary-cholangitis
#13
REVIEW
Sara L Chalifoux, Peter G Konyn, Gina Choi, Sammy Saab
Primary biliary cholangitis (PBC) is an autoimmune liver disease characterized by progressive destruction of the intrahepatic bile ducts, leading to cholestasis. PBC is known to have both hepatic and extrahepatic manifestations. Extrahepatic manifestations are seen in up to 73% of patients with PBC, with the most common being Sjogren's syndrome, thyroid dysfunction and systemic sclerosis. It is thought that patients with PBC are at increased risk of developing these extrahepatic manifestations, almost all of which are autoimmune, because patients with autoimmune disease are at higher risk of developing another autoimmune condition...
March 16, 2017: Gut and Liver
https://www.readbyqxmd.com/read/28271527/the-role-of-lncrna-h19-in-gender-disparity-of-cholestatic-liver-injury-in-mdr2-mice
#14
Xiaojiaoyang Li, Runping Liu, Jing Yang, Lixin Sun, Luyong Zhang, Zhenzhou Jiang, Puneet Puri, Emily C Gurley, Guanhua Lai, Yuping Tang, Zhiming Huang, William M Pandak, Phillip B Hylemon, Huiping Zhou
The multi-drug resistance 2 knockout (Mdr2(-/-) ) mouse is a well-established model of cholestatic cholangiopathies. Female Mdr2(-/-) mice develop more severe hepatobiliary damage than male Mdr2(-/-) mice, which is correlated with a higher proportion of taurocholate (TCA) in bile. Although estrogen has been identified as an important player in intrahepatic cholestasis, the underlying molecular mechanisms of gender-based disparity of cholestatic injury remain unclear. The long non-coding RNA H19 is an imprinted, maternally expressed and estrogen-targeted gene, which is significantly induced in human fibrotic/cirrhotic liver and bile duct ligated mouse liver...
March 8, 2017: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://www.readbyqxmd.com/read/28270873/predictors-of-fetal-and-maternal-outcome-in-the-crucible-of-hepatic-dysfunction-during-pregnancy
#15
Indrajit Suresh, Vijaykumar Tr, Nandeesh Hp
BACKGROUND: Hepatic dysfunction during pregnancy places both the mother and the fetus at risk. Investigations which are efficient, cost effective and easily available for prognostication are required to tackle this global problem. We studied the etiologies and evaluated investigations for predictive efficiency. METHODS: One hundred ninety-seven pregnant women with hepatic dysfunction during pregnancy were identified. All patients were followed up till 8 weeks after termination of pregnancy or death...
February 2017: Gastroenterology Research
https://www.readbyqxmd.com/read/28262241/hemostatic-issues-in-pregnancy-induced-liver-disease
#16
Ton Lisman, William Bernal
Liver diseases may be accompanied by profound changes in the hemostatic system including thrombocytopenia, decreased plasma levels of pro- and anticoagulants, and alterations in plasma levels of fibrinolysis. The net effect of the hemostatic changes in chronic and acute liver diseases is a hemostatic system that is in relative balance due to the simultaneous decline in pro- and antihemostatic drivers. A unique category of liver diseases are those induced by pregnancy. In acute fatty liver of pregnancy, profound hemostatic changes occur, which may be caused by a combination of liver failure and disseminated intravascular coagulation...
March 2017: Thrombosis Research
https://www.readbyqxmd.com/read/28254950/assessing-the-risk-of-drug-induced-cholestasis-using-unbound-intrahepatic-concentrations
#17
Julia Riede, Birk Poller, Jörg Huwyler, Gian Camenisch
Inhibition of the bile salt export pump (BSEP) has been recognized as a key factor in the development of drug-induced cholestasis (DIC). The risk of DIC in humans has been previously assessed using in vitro BSEP inhibition data (IC50) and unbound systemic drug exposure under assumption of the "free drug hypothesis." This concept, however, is unlikely valid, as unbound intrahepatic drug concentrations are affected by active transport and metabolism. To investigate this hypothesis, we experimentally determined the in vitro liver-to-blood partition coefficients (Kpuu) for 18 drug compounds using the hepatic extended clearance model (ECM)...
May 2017: Drug Metabolism and Disposition: the Biological Fate of Chemicals
https://www.readbyqxmd.com/read/28249726/hepatobiliary-transport-kinetics-of-the-conjugated-bile-acid-tracer-11-c-csar-quantified-in-healthy-humans-and-patients-by-positron-emission-tomography-pet
#18
Nikolaj Worm Ørntoft, Ole Lajord Munk, Kim Frisch, Peter Ott, Susanne Keiding, Michael Sørensen
BACKGROUND & AIMS: Hepatobiliary secretion of bile acids is an important liver function. Here, we quantified the hepatic transport kinetics of conjugated bile acids using the bile acid tracer [N-methyl-(11)C]cholylsarcosine ((11)C-CSar) and PET. METHODS: Nine healthy subjects and eight patients with varying degrees of cholestasis were examined with (11)C-CSar PET and measurement of arterial and hepatic venous blood concentrations of (11)C-CSar. RESULTS: Results are presented as median (range)...
February 27, 2017: Journal of Hepatology
https://www.readbyqxmd.com/read/28249274/the-emerging-role-of-soluble-adenylyl-cyclase-in-primary-biliary-cholangitis
#19
Jung-Chin Chang, Ulrich Beuers, Ronald P J Oude Elferink
BACKGROUND: Primary biliary cholangitis (PBC; previously referred to as primary biliary cirrhosis) is a chronic fibrosing cholangiopathy with the signature of an autoimmune disease and features of intrahepatic cholestasis. Immunosuppressing treatments are largely unsuccessful. Responsiveness to ursodeoxycholic acid and reduced expression of anion exchanger 2 (AE2) on canalicular membranes and small bile ducts underline the importance of bicarbonate transportation in its disease mechanism...
2017: Digestive Diseases
https://www.readbyqxmd.com/read/28229073/epidemiology-and-risk-factors-of-cholangiocarcinoma
#20
Martha M Kirstein, Arndt Vogel
BACKGROUND: Cholangiocarcinoma (CCA) is the second most common primary liver cancer, being characterized by its late diagnosis and fatal outcome. Recent epidemiological reports indicate an increasing worldwide incidence of intrahepatic CCA but a decreasing incidence of extrahepatic CCA. METHODS: In this review, we present an overview of the incidence and epidemiology of CCA and possible strategies for screening and surveillance. RESULTS: Efficient strategies for the screening and surveillance of CCA have not been established so far...
December 2016: Visceral Medicine
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