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Intrahepatic Cholestasis

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https://www.readbyqxmd.com/read/29914448/intrahepatic-cholestasis-of-pregnancy-and-associated-causes-of-death-a-cohort-study-with-follow-up-of-27-46-years
#1
Suvi-Tuulia Hämäläinen, Kaisa Turunen, Kari J Mattila, Markku Sumanen
BACKGROUND: The aim of this study was to determine whether intrahepatic cholestasis of pregnancy (ICP) is associated with causes of death during on average 35 years follow-up after the delivery. METHODS: The study population comprised 571 women with ICP in at least one pregnancy seen at Tampere University Hospital, Finland, between 1969 and 1988. ICP was verified from patient records. The previous and following subjects in the maternity ward diary were taken as controls for each ICP case...
June 19, 2018: BMC Women's Health
https://www.readbyqxmd.com/read/29904153/mouse-models-of-hepatocellular-carcinoma-an-overview-and-highlights-for-immunotherapy-research
#2
REVIEW
Zachary J Brown, Bernd Heinrich, Tim F Greten
Mouse models are the basis of preclinical and translational research in hepatocellular carcinoma (HCC). Multiple methods exist to induce tumour formation in mice, including genetically engineered mouse models, chemotoxic agents, intrahepatic or intrasplenic injection of tumour cells and xenograft approaches. Additionally, as HCC generally develops in the context of diseased liver, methods exist to induce liver disease in mice to mimic viral hepatitis, fatty liver disease, fibrosis, alcohol-induced liver disease and cholestasis...
June 14, 2018: Nature Reviews. Gastroenterology & Hepatology
https://www.readbyqxmd.com/read/29894336/diffuse-18f-fdg-avidity-in-liver-associated-with-x-linked-protoporphyria-on-pet-ct
#3
Zhanli Fu, Meng Liu, Xueqi Chen, Xing Yang, Qian Li
An F-FDG PET/CT was performed on a 43-year-old woman with photosensitive skin rash, abnormal liver function, and pancytopenia, which demonstrated prominent hepatomegaly, splenomegaly, and diffuse liver F-FDG avidity. The liver biopsy revealed intrahepatic cholestasis with biliary fibrosis resulting from the deposition of protoporphyrin. X-linked erythroid-specific 5-aminolevulinate synthase gene analysis proved the diagnosis of X-linked protoporphyria.
June 12, 2018: Clinical Nuclear Medicine
https://www.readbyqxmd.com/read/29893994/predictive-factors-for-successful-cervical-ripening-using-a-double-balloon-catheter-after-previous-cesarean-delivery
#4
Mathilde Vital, Joséphine Grange, Aurélie Le Thuaut, Jérôme Dimet, Guillaume Ducarme
OBJECTIVE: To identify predictors of successful cervical ripening using double-balloon catheter (DBC) for labor induction among women with previous cesarean delivery (PCD) and unfavorable cervix at term. METHODS: The present prospective observational study was conducted among women who underwent cervical ripening with DBC at a French tertiary care hospital between January 1, 2014, and December 31, 2017. Inclusion criteria were PCD; singleton term fetus; cephalic presentation; and unfavorable cervix (Bishop score <6)...
June 12, 2018: International Journal of Gynaecology and Obstetrics
https://www.readbyqxmd.com/read/29891454/-role-of-endoplasmic-reticulum-stress-induced-apoptosis-of-trophoblasts-in-intrahepatic-cholestasis-during-pregnancy
#5
Hai-Zhen Wang, Dan-Chun Cai, Dan-Dan Liao, Mei Zhong, Yun-Fei Gao, Chao Sheng
OBJECTIVE: To investigate the role of endoplasmic reticulum stress (ERS)-induced trophoblast apoptosis in the development of intrahepatic cholestasis during pregnancy (ICP). METHODS: Twenty pregnant women with ICP and 20 normal pregnant women undergoing cesarean section were enrolled in this study. The number of placenta syncytial knots in these women was determined using HE staining. The mRNA expressions of GRP78, CHOP, caspase-3, and caspase-7 were detected using RT-PCR in the placental tissues of the women and also in HTR-8/SVneo cells treated with different doses of deoxycholic acid (DCA)...
May 20, 2018: Nan Fang Yi Ke da Xue Xue Bao, Journal of Southern Medical University
https://www.readbyqxmd.com/read/29890866/assessment-of-circulating-betatrophin-levels-in-intrahepatic-cholestasis-of-pregnancy
#6
Özgür Kara, Ayşe Kirbas, Kadriye Yakut, Korkut Daglar, Hakan Timur, Zeynep Ozturk İnal, Yaprak Engin-Ustun
OBJECTIVE: To investigate maternal serum levels of betatrophin and their relationship with total bile acid (TBA) levels in patients with intrahepatic cholestasis of pregnancy (ICP). MATERIALS AND METHODS: Fifty-nine pregnant women with ICP (31 patients with severe and 28 patients with mild disease classifications) and 23 healthy women with uncomplicated pregnancies as the control group included the study. The maternal betatrophin, fasting blood glucose, fasting insulin (FI), and homeostatic model assessment of insulin resistance (HOMA-IR) levels of the groups were compared...
June 11, 2018: Journal of Maternal-fetal & Neonatal Medicine
https://www.readbyqxmd.com/read/29886774/labor-induction-in-indicated-moderate-to-late-preterm-birth
#7
Charline Bertholdt, Olivier Morel, Matthieu Dap, Marion Choserot, Hélène Minebois
INTRODUCTION: The primary objective of the study was to evaluate the success of labor induction for indicated moderate and late preterm birth. As secondary objectives, the mode of delivery was assessed. MATERIAL AND METHODS: This is an observational study conducted in a tertiary care unit between 2013 and 2015. All patients who underwent labor induction for indicated preterm birth between 32 + 0 and 36 + 6 weeks of gestation (as premature rupture of membranes, preeclampsia, intrauterine growth restriction, fetomaternal alloimmunization, or intrahepatic cholestasis) were included...
June 10, 2018: Journal of Maternal-fetal & Neonatal Medicine
https://www.readbyqxmd.com/read/29886607/-relationship-between-phenotype-and-genotype-of-abcb11-deficiency-in-siblings-and-literature-review
#8
X R Peng, Y Lu, M H Zhang, L T Li, X B Xie, J Y Gong, J S Wang
Objective: To explore the relationship between genotype and phenotype of ABCB11 deficiency. Methods: Clinical data of two siblings with ABCB11 deficiency were retrospectively analyzed. Related literature from PubMed, CNKI and Wangfang databases was reviewed to date (up to August 2017) with 'ABCB11 gene' or 'bile salt export pump', 'cholestasis' and 'child' as key words. Results: The patients were siblings. Both of them presented as jaundice, pruritus and hepatosplenomegaly since 3 days after birth. Significant laboratory findings on admission of the older sister included high total bilirubin, 170 µmol/L;conjugated bilirubin, 115...
June 2, 2018: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
https://www.readbyqxmd.com/read/29867509/yinchenhao-decoction-ameliorates-alpha-naphthylisothiocyanate-induced-intrahepatic-cholestasis-in-rats-by-regulating-phase-ii-metabolic-enzymes-and-transporters
#9
Ya-Xiong Yi, Yue Ding, Yong Zhang, Ning-Hui Ma, Feng Shi, Ping Kang, Zhen-Zhen Cai, Tong Zhang
Yinchenhao Decoction (YCHD), a famous traditional Chinese formula, has been used for treating cholestasis for 1000s of years. The cholagogic effect of YCHD has been widely reported, but its pharmacodynamic material and underlying therapeutic mechanism remain unclear. By using ultra-high-performance liquid chromatography (UHPLC)-quadrupole time-of-flight mass spectrometry, 11 original active components and eight phase II metabolites were detected in rats after oral administration of YCHD, including three new phase II metabolites...
2018: Frontiers in Pharmacology
https://www.readbyqxmd.com/read/29851770/total-bile-acid-concentration-in-duodenal-fluid-is-a-useful-preoperative-screening-marker-to-rule-out-biliary-atresia
#10
Tomoya Fukuoka, Kazuhiko Bessho, Makiko Tachibana, Yoshinori Satomura, Akiko Konishi, Kie Yasuda, Takeshi Kimura, Yasuhiro Hasegawa, Takehisa Ueno, Yoko Miyoshi, Keiichi Ozono
OBJECTIVES: Duodenal tube test (DTT) is used as a preoperative screening to rule out biliary atresia (BA). In previous reports, DTT was assessed by the color of the duodenal fluid, but there were no quantitative criteria. The aim of this study was to examine the efficacy of DTT based on the total bile acid (TBA) concentration in duodenal fluid. METHODS: This is a single-center retrospective study of infants with cholestasis who underwent DTT from 2008 to 2016 at the Osaka University Hospital...
May 30, 2018: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/29787821/slc25a13-c-1610_1612delinsat-mutation-in-an-indian-patient-and-literature-review-of-79-cases-of-citrin-deficiency-for-genotype-phenotype-associations
#11
A Radha Rama Devi, Shaik Mohammad Naushad
Here, we report SLC25A13 c.1610_1612delinsAT mutation from India in a 13-year old boy who presented with recurrent episodes of delirium and hyperammonemia. This is the second case with this mutation; the first case was of Pakistani origin. The boy responded to diet modification, sodium benzoate and arginine supplementation. Furthermore, we have aimed to establish genotype-phenotype correlation of 79 cases of citrin deficiency (46 males and 33 females) reported in 24 studies from all over the world. Inverse association was observed between age of onset and jaundice (r = -0...
May 19, 2018: Gene
https://www.readbyqxmd.com/read/29783833/-non-transplant-surgical-intervention-in-progressive-familial-intrahepatic-cholestasis
#12
X F Li, J Y Gong, J S Wang
No abstract text is available yet for this article.
May 2, 2018: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
https://www.readbyqxmd.com/read/29761168/outcomes-of-surgical-management-of-familial-intrahepatic-cholestasis-1-and-bile-salt-export-protein-deficiencies
#13
Laura N Bull, Ludmila Pawlikowska, Sandra Strautnieks, Irena Jankowska, Piotr Czubkowski, Jennifer L Dodge, Karan Emerick, Catherine Wanty, Sami Wali, Samra Blanchard, Florence Lacaille, Jane A Byrne, Albertien M van Eerde, Kaija-Leena Kolho, Roderick Houwen, Steven Lobritto, Vera Hupertz, Patricia McClean, Giorgina Mieli-Vergani, Etienne Sokal, Philip Rosenthal, Peter F Whitington, Joanna Pawlowska, Richard J Thompson
Progressive familial intrahepatic cholestasis (PFIC) with normal circulating gamma-glutamyl transpeptidase levels can result from mutations in the ATP8B1 gene (encoding familial intrahepatic cholestasis 1 [FIC1] deficiency) or the ABCB11 gene (bile salt export protein [BSEP] deficiency). We investigated the outcomes of partial external biliary diversion, ileal exclusion, and liver transplantation in these two conditions. We conducted a retrospective multicenter study of 42 patients with FIC1 deficiency (FIC1 patients) and 60 patients with BSEP deficiency (BSEP patients) who had undergone one or more surgical procedures (57 diversions, 6 exclusions, and 57 transplants)...
May 2018: Hepatology Communications
https://www.readbyqxmd.com/read/29761167/phenotypic-spectrum-and-diagnostic-pitfalls-of-abcb4-deficiency-depending-on-age-of-onset
#14
Stephanie Barbara Schatz, Christoph Jüngst, Verena Keitel-Anselmo, Ralf Kubitz, Christina Becker, Patrick Gerner, Eva-Doreen Pfister, Imeke Goldschmidt, Norman Junge, Daniel Wenning, Stephan Gehring, Stefan Arens, Dirk Bretschneider, Dirk Grothues, Guido Engelmann, Frank Lammert, Ulrich Baumann
Genetic variants in the adenosine triphosphate-binding cassette subfamily B member 4 ( ABCB4 ) gene, which encodes hepatocanalicular phosphatidylcholine floppase, can lead to different phenotypes, such as progressive familial intrahepatic cholestasis (PFIC) type 3, low phospholipid-associated cholelithiasis, and intrahepatic cholestasis of pregnancy. The aim of this multicenter project was to collect information on onset and progression of this entity in different age groups and to assess the relevance of this disease for the differential diagnosis of chronic liver disease...
May 2018: Hepatology Communications
https://www.readbyqxmd.com/read/29739259/role-of-ischemia-modified-albumin-in-the-evaluation-of-oxidative-stress-in-intrahepatic-cholestasis-of-pregnancy
#15
A T Tayyar, S Kozalı, G Y Yıldırım, R Karakus, I T Yuksel, O Erel, S Neselioglu, M Eroglu
OBJECTIVE: To investigate the ischemia-modified albumin (IMA) level, and the IMA/albumin ratio (IMAR) in healthy pregnant women, and pregnant women with intrahepatic cholestasis of pregnancy (ICP). MATERIAL AND METHOD: This cross-sectional study included 53 women with ICP and 52 healthy pregnant women. Their serum IMA and albumin levels were analyzed, and the women were followed up to delivery. RESULTS: No significant intergroup differences were identified in maternal age, body mass index, and gestational age at the time that the blood samples were taken...
May 8, 2018: Journal of Maternal-fetal & Neonatal Medicine
https://www.readbyqxmd.com/read/29737914/lipidomics-reveal-aryl-hydrocarbon-receptor-ahr-regulated-lipid-metabolic-pathway-in-alpha-naphthyl-isothiocyanate-anit-induced-intrahepatic-cholestasis
#16
Bao-Long Wang, Chang-Wen Zhang, Liang Wang, Kun-Long Tang, Naoki Tanaka, Frank J Gonzalez, Yong Xu, Zhong-Ze Fang
1. Ultra-performance liquid chromatography coupled with electrospray ionization quadrupole mass spectrometry (UPLC-ESI-QTOF MS)-based lipidomics was employ to elucidate new mechanism of alpha-naphthyl isothiocyanate (ANIT)-induced intrahepatic cholestasis in mice. 2. Multiple lipid components significantly increased in ANIT-induced intrahepatic cholestasis, including PC 16:0, 20:4, PC 16:0, 22:6, PC 16:0, 18:2, LPC 18:2, PC 18:2, LPC 18:1, PC 18:1, 14:0, SM 18:1, 16:0, oleoylcarnitine, and palmitoylcarnitine...
May 8, 2018: Xenobiotica; the Fate of Foreign Compounds in Biological Systems
https://www.readbyqxmd.com/read/29729773/surgical-outcomes-in-alagille-syndrome-and-pfic-a-single-institution-s-20-year-experience
#17
Celia D Flores, Yangyang R Yu, Tamir A Miloh, John Goss, Mary L Brandt
BACKGROUND: Alagille Syndrome (AGS) and Progressive Familial Intrahepatic Cholestasis (PFIC) are rare pediatric biliary disorders that lead to progressive liver disease. This study reviews our experience with the surgical management of these disorders over the last 20years. METHODS: We retrospectively reviewed the records of children diagnosed with AGS or PFIC from January 1996 to December 2016. Data collected included demographics, surgical intervention (liver transplant or biliary diversion), and complications...
May 2018: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/29718219/xenobiotic-nuclear-receptor-signaling-determines-molecular-pathogenesis-of-progressive-familial-intrahepatic-cholestasis
#18
Kang Ho Kim, Jong Min Choi, Feng Li, Armando Arizpe, Clavia Ruth Wooton-Kee, Sayeepriyadarshini Anakk, Sung Yun Jung, Milton J Finegold, David D Moore
Progressive familial intrahepatic cholestasis (PFIC) is a genetically heterogeneous disorder of bile flow disruption due to abnormal canalicular transport or impaired bile acid (BA) metabolism, causing excess BA accumulation and liver failure. We reported an intrahepatic cholestasis mouse model based on loss of function of both farnesoid X receptor (FXR; NR1H4) and small heterodimer partner (SHP, NR0B2) (DKO), which has strong similarities to human PFIC type 5. Here, we compare the pathogenesis of the DKO liver to that of another intrahepatic cholestasis model, Bsep-/-, which represents human PFIC2...
April 26, 2018: Endocrinology
https://www.readbyqxmd.com/read/29709452/diagnostic-and-therapeutic-profiles-of-serum-bile-acids-in-women-with-intrahepatic-cholestasis-of-pregnancy-a-pseudo-targeted-metabolomics-study
#19
Yue Cui, Biao Xu, Xiaoqing Zhang, Yifan He, Yong Shao, Min Ding
BACKGROUND: Intrahepatic cholestasis of pregnancy (ICP), as a pregnancy-specific liver disorder, obtains increasing recognition due to a series of adverse outcomes. ICP is generally characterized by pruritus and jaundice, and closely related to abnormalities in the metabolism and disposition of bile acids composition. Because of its elusive pathogenesis, ICP has become an intractable issue to be diagnosed and managed for obstetricians. Analysis of metabolic profile could reveal the state of small-molecule metabolites systematically and provide comprehensively metabolic information for diseases...
August 2018: Clinica Chimica Acta; International Journal of Clinical Chemistry
https://www.readbyqxmd.com/read/29708070/drug-induced-cholestasis-mechanisms-models-and-markers
#20
Sagnik Chatterjee, Pieter Annaert
Drug-induced cholestasis is a risk factor in progression of drug candidates, and poses serious health hazard if not detected before going into human. Intrahepatic accumulation of bile acids (BAs) represents a characteristic phenomenon associated with drug-induced cholestasis. The major challenges in obtaining a complete understanding of drug-induced cholestasis lies in the complexity of BA-mediated toxicity mechanisms and the impact of bile acids at different 'targets' such as transporters, enzymes and nuclear receptors...
April 27, 2018: Current Drug Metabolism
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