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Intrahepatic Cholestasis

Luca Mannini, Flavia Sorbi, Ivo Noci, Viola Ghizzoni, Federica Perelli, Mariarosaria Di Tommaso, Alberto Mattei, Massimiliano Fambrini
PURPOSE: The main aim of this study was to evaluate the incidence of endometriosis and intrahepatic cholestasis (ICP) and induction of labor in pregnant women with endometriosis compared with women without endometriosis. The secondary aim was to confirm increased incidence of already known endometriosis-related pregnancy complications in these patients. METHODS: This is a retrospective cohort study performed at a tertiary hospital between January 2009 and December 2014 to compare obstetrics outcome between women with endometriosis and women without endometriosis...
October 21, 2016: Archives of Gynecology and Obstetrics
Linling Zhu, Yu Zhang, Yifeng Liu, Runjv Zhang, Yiqing Wu, Yun Huang, Feng Liu, Meigen Li, Saijun Sun, Lanfeng Xing, Yimin Zhu, Yiyi Chen, Li Xu, Liangbi Zhou, Hefeng Huang, Dan Zhang
This study was carried out to explore associations between assisted reproductive technology (ART) and maternal and neonatal outcomes compared with similar outcomes following spontaneously conceived births. We conducted a retrospective cohort study of pregnancies conceived by ART (N = 2641) during 2006-2014 compared to naturally conceived pregnancies (N = 5282) after matching for maternal age and birth year. Pregnancy complications, perinatal complications and neonatal outcomes of enrolled subjects were investigated and analysed by multivariate logistic regression...
October 20, 2016: Scientific Reports
Giovanni Vitale, Giulia Simonetti, Martina Pirillo, Gianfranco Taruschio, Pietro Andreone
Bile Salt Export Pump (BSEP) Deficiency disease, including Progressive Familial Intrahepatic Cholestasis type 2 (PFIC2), is a rare disease, usually leading within the first ten years to portal hypertension, liver failure, hepatocellular carcinoma. Often liver transplantation is needed. Sodium 4-phenylbutyrate (4-PB) seems to be a potential therapeutic compound for PFIC2. Psychiatric side effects in the adolescent population are little known and little studied since the drug used to treat children and infants...
September 2016: Psychiatry Investigation
Xiang Kong, Yan Kong, Fangyuan Zhang, Tingting Wang, Jin Yan
BACKGROUND: Intrahepatic cholestasis of pregnancy (ICP) is a specific pregnancy-related disorder without standard medical therapies. Ursodeoxycholic acid (UDCA) is the most used medicine, but the efficacy and safety of UDCA remain uncertain. Several meta-analyses had been made to assess the effects of UDCA in ICP. However, the samples were not large enough to convince obstetricians to use UDCA. We conducted a meta-analysis to evaluate the effects and safety of UDCA in patients with ICP, which included only randomized controlled trials (RCTs)...
October 2016: Medicine (Baltimore)
Sajan Agarwal, Bikrant Bihari Lal, Dinesh Rawat, Archana Rastogi, Kishore G S Bharathy, Seema Alam
OBJECTIVE: To study the clinical and laboratory profile of children with progressive familial intrahepatic cholestasis (PFIC) and evaluate their outcome. METHODS: The study is a retrospective review of all cases diagnosed with PFIC between January 2011 and July 2015. All children underwent histopathological examination and immunostaining. Management was done as per institute's protocol. RESULTS: There were a total of 24 PFIC cases (PFIC 1-2, PFIC 2-19, PFIC 3-3)...
September 2016: Journal of Clinical and Experimental Hepatology
Xiaojiaoyang Li, Zihang Yuan, Runping Liu, Hozeifa M Hassan, Hang Yang, Rong Sun, Luyong Zhang, Zhenzhou Jiang
Estrogen-induced cholestasis, known as intrahepatic cholestasis of pregnancy (ICP), is an estrogen-related liver disease that is widely recognized as female or pregnancy-specific. Our previous findings showed that the synthetic estrogen, 17α-ethinylestradiol (EE), induced cholestatic injury through ERK1/2-LKB1-AMP-activated protein kinase (AMPK) signaling pathway and its mediated suppression of farnesoid X receptor (FXR). To investigate the role played by bile acids in EE-induced cholestasis, we evaluated the effects of chenodeoxycholic acid (CDCA), ursodeoxycholic acid (UDCA) and deoxycholic acid (DCA) on sandwich cultured rat primary hepatocytes (SCRHs) and an in vivo rat model...
October 12, 2016: Toxicology and Applied Pharmacology
Romana Furrer, Katharina Winter, Leonhard Schäffer, Roland Zimmermann, Tilo Burkhardt, Christian Haslinger
OBJECTIVE: To evaluate postpartum blood loss in women with treated intrahepatic cholestasis of pregnancy. METHODS: In a retrospective case-control study, 15,083 deliveries including 348 women with intrahepatic cholestasis of pregnancy (2.3%) were analyzed from 2004 to 2014. To adjust for differences in baseline characteristics, a propensity analysis was performed and women in the control group were matched to the women in the intrahepatic cholestasis of pregnancy group in a 5:1 ratio...
October 6, 2016: Obstetrics and Gynecology
Divakar A, Banerjee S, Pai R, Sharma Rn
No abstract text is available yet for this article.
January 2016: Journal of the Association of Physicians of India
Neng-Li Wang, Yu-Lan Lu, Ping Zhang, Mei-Hong Zhang, Jing-Yu Gong, Yi Lu, Xin-Bao Xie, Yi-Ling Qiu, Yan-Yan Yan, Bing-Bing Wu, Jian-She Wang
BACKGROUND AND AIMS: Large indels are commonly identified in patients but are not detectable by routine Sanger sequencing and panel sequencing. We specially designed a multi-gene panel that could simultaneously test known large indels in addition to ordinary variants, and reported the diagnostic yield in patients with intrahepatic cholestasis. METHODS: The panel contains 61 genes associated with cholestasis and 25 known recurrent large indels. The amplicon library was sequenced on Ion PGM system...
2016: PloS One
Dan Shan, Yayi Hu, Peiyuan Qiu, Bechu Shelley Mathew, Yun Chen, Si Li, Yuan Hu, Lijun Lin, Zhi Wang, Luping Li
The aim of the present work was to determine maternal and fetal outcomes of intrahepatic cholestasis of pregnancy (ICP) in twin pregnancies. All twin pregnancies delivered above 28 gestational weeks in West China Second University Hospital from January 2013 to May 2015 were included. Data on maternal demographics and obstetric complications together with fetal outcomes were collected. The risk of adverse maternal and fetal outcomes were determined in relation to ICP by crude odds ratios (OR) and adjusted ORs (aOR) with 95% confidence intervals (CI)...
September 28, 2016: Twin Research and Human Genetics: the Official Journal of the International Society for Twin Studies
Mohammad S Khuroo, Ajaz A Rather, Naira S Khuroo, Mehnaaz S Khuroo
Portal biliopathy refers to cholangiographic abnormalities which occur in patients with portal cavernoma. These changes occur as a result of pressure on bile ducts from bridging tortuous paracholedochal, epicholedochal and cholecystic veins. Bile duct ischemia may occur due prolonged venous pressure effect or result from insufficient blood supply. In addition, encasement of ducts may occur due fibrotic cavernoma. Majority of patients are asymptomatic. Portal biliopathy is a progressive disease and patients who have long standing disease and more severe bile duct abnormalities present with recurrent episodes of biliary pain, cholangitis and cholestasis...
September 21, 2016: World Journal of Gastroenterology: WJG
Anne Tr Noll, Thorsten Cramer, Steven Wm Olde Damink, Frank G Schaap
Cholangiocarcinoma (CCA) is a relatively rare malignancy of the intra- or extra-hepatic bile ducts that is classified according to its anatomical localization as intrahepatic, perihilar or distal. Overall, CCA has a dismal prognosis due to typical presentation at an advanced irresectable stage, lack of effective non-surgical treatments, and a high rate of disease recurrence. CCA frequently arises on a background of chronic liver inflammation and cholestasis. Chronic inflammation is accompanied by enhanced cell turnover with generation of additional inflammatory stimuli, and a microenvironment rich in pro-inflammatory mediators and proliferative factors that enable accumulation of mutations, transformation and expansion of mutated cells...
September 18, 2016: World Journal of Hepatology
Nalini Sharma, S Panda, Ahanthem Santa Singh
OBJECTIVE: To study the incidence, maternal, and perinatal outcomes in intrahepatic cholestasis of pregnancy with active management. MATERIALS AND METHODS: This observational study included 48 cases diagnosed as intrahepatic cholestasis of pregnancy. Medical treatment and active management (fetal surveillance and termination of pregnancy at 37-38 weeks) were offered to all. Maternal and perinatal outcomes are studied. RESULTS: Incidence of ICP was 2...
October 2016: Journal of Obstetrics and Gynaecology of India
Gurleen Wander, Francesa Neuberger, Mandish K Dhanjal, Catherine Nelson-Piercy, May Ching Soh
Most published cases of cytomegalovirus infection in pregnancy relate to congenital abnormalities in neonates infected in early pregnancy, while the mother remains asymptomatic. We describe a diagnostically challenging case of an immunosuppressed woman with scleroderma who developed deranged liver function tests attributed to intrahepatic cholestasis of pregnancy and haemolysis, elevated liver enzymes and low platelets syndrome but was ultimately found to have disseminated cytomegalovirus. Cytomegalovirus can present in a myriad of ways...
September 2016: Obstetric Medicine
Song Zhaoyi, Shi Qingyun
Intrahepatic cholestasis of pregnancy (ICP) is a disorder of bile acid (BA) synthesis, excretion, and metabolism, with systemic accumulation of BAs, which can lead to prematurity, fetal distress, and intrauterine death. Here, we investigate the expression of peroxisome proliferator-activated receptor alpha and cytochrome P450 oxysterol 7alpha-hydroxylase by exposing to 17α-ethynylestradiol with or without the estrogen receptor signaling pathway in pregnant rats with intrahepatic cholestasis. In vivo and in vitro evidences showed that estrogen receptor alpha (ERα) may be the key point of occurrence and development of intrahepatic cholestasis in pregnant rats...
September 14, 2016: Reproductive Sciences
Mu-Jun Chang, Yan-Jiao Xu, Wen-Xi He, Cheng-Liang Zhang, Xi-Ping Li, Dong Liu
AIMS: Although the intimate relationship between liver and gut has been previously reported in physiological and pathological conditions, intestinal involvement in the process of intrahepatic cholestasis of pregnancy has been unclear. The aim of this study was to investigate the intestinal changes in 17α-ethynylestradiol-induced rat model. METHODS: Liver injury was assessed by hematoxylin and eosin staining and measurement of serum biochemical parameters. Intestinal transit was determined by a method using ink...
September 13, 2016: Journal of Digestive Diseases
Yasir Abdulqader, Keng-Yu Chuang, Jyotsna Ravi, Abdul Nadir
We report a case of secondary sclerosing cholangitis that manifested itself during pregnancy. A tentative diagnosis of intrahepatic cholestasis of pregnancy was considered, but after her third delivery, a liver biopsy and imaging, as well as review of past records, confirmed the diagnosis of secondary sclerosing cholangitis. Maternal and fetal outcomes of primary sclerosis cholangitis have been reported, and this case highlights the importance of considering other diseases besides the benign intrahepatic cholestasis of pregnancy as a cause of cholestasis in pregnancy...
July 2016: ACG Case Reports Journal
Viara Shoumnalieva-Ivanova, Ivan Tanev, Yani Zdravkov, Simeon Monov, Russka Shumnalieva
Aagenaes syndrome, also called lymphoedema cholestasis syndrome 1 (LSC1), is characterized by neonatal intrahepatic cholestasis, often lessening and becoming intermittent with age and severe chronic lymphoedema, mainly affecting the lower extremities. The condition is autosomal recessively inherited, and the gene is located on chromosome 15q. The locus, LCS1, was mapped to a 6.6 cM region on chromosome 15. Angioid streaks are visible irregular crack-like dehiscences in bruch's membrane that are associated with atrophic degeneration of the overlying retinal pigment epithelium...
September 10, 2016: International Ophthalmology
C Guth, U Beuers, K Beckh
Intermittent cholestatic liver disease may indicate an inherited deficiency of bile salt transport proteins. Episodes of cholestasis may start during pregnancy or during use of oral contraceptives or other medication. We describe the case of a 22-year-old mother with increasing jaundice and severe pruritus two weeks after starting hormonal contraception. A few months before she was suffering from intrahepatic cholestasis of pregnancy (ICP). Liver biopsy showed bland cholestasis with canalicular bile plugs...
September 2016: Zeitschrift Für Gastroenterologie
Claudia D Fuchs, Gustav Paumgartner, Annika Wahlström, Philipp Schwabl, Thomas Reiberger, Nadja Leditznig, Tatjana Stojakovic, Nataliya Rohr-Udilova, Peter Chiba, Hanns-Ulrich Marschall, Michael Trauner
BACKGROUND & AIMS: Cholestasis is characterized by intrahepatic accumulation of potentially cytotoxic bile acids (BAs) subsequently leading to liver injury with disruption of hepatocellular integrity, inflammation, fibrosis and ultimately liver cirrhosis. Bile salt export pump (BSEP/ABCB11) is the main canalicular BA transporter and therefore the rate limiting step for hepatobiliary BA excretion. In this study we aimed to investigate the role of BSEP/ABCB11 in the development of acquired cholestatic liver and bile duct injury...
September 1, 2016: Journal of Hepatology
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