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Intrahepatic Cholestasis

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https://www.readbyqxmd.com/read/29226108/severe-alcoholic-hepatitis-atypical-presentation-with-markedly-elevated-alkaline-phosphatase
#1
Page Axley, Kirk Russ, Ashwani K Singal
Alcoholic hepatitis (AH) is an acute inflammatory liver disease with poor prognosis. Infections in AH are difficult to detect and contribute to short-term mortality. Intrahepatic cholestasis and elevated alkaline phosphatase levels are also associated with worse outcomes. This report describes an uncommon presentation of severe AH.
December 28, 2017: Journal of Clinical and Translational Hepatology
https://www.readbyqxmd.com/read/29215645/cherchez-la-femme-maternal-incidental-findings-can-explain-discordant-prenatal-cell-free-dna-sequencing-results
#2
REVIEW
Diana W Bianchi
Circulating DNA fragments in a pregnant woman's plasma derive from three sources: placenta, maternal bone marrow, and fetus. Prenatal sequencing to noninvasively screen for fetal chromosome abnormalities is performed on this mixed sample; results can therefore reflect the maternal as well as the fetoplacental DNA. Although it is recommended that pretest counseling include the possibility of detecting maternal genomic imbalance, this seldom occurs. Maternal abnormalities that can affect a prenatal screening test result include disorders that affect the size and metabolism of DNA, such as B12 deficiency, autoimmune disease, and intrahepatic cholestasis of pregnancy...
December 7, 2017: Genetics in Medicine: Official Journal of the American College of Medical Genetics
https://www.readbyqxmd.com/read/29201793/intrahepatic-cholestasis-of-pregnancy-spontaneous-vs-in-vitro-fertilization
#3
Filiz F Bolukbas, Cengiz Bolukbas, Hatice Y Balaban, Cem Aygun, Seyda Ignak, Emine Ergul, Mehtap Yazicioglu, Suat S Ersahin
Aim: Intrahepatic cholestasis of pregnancy (ICP) is the most common liver disease in pregnancy. Although it was shown that multiple pregnancy and hormone therapies increase the risk of ICP, there is limited information that compared spontaneous fertilization and in vitro fertilization (IVF) from the aspect of developing ICP. In our study, we investigated the potential relationship between ICP and IVF/ spontaneous pregnancy. Materials and methods: We reviewed the records (between June 2007 and December 2014) of pregnancies with ICP who were referred to gastroenterology clinics in three different hospitals...
July 2017: Euroasian Journal of Hepato-Gastroenterology
https://www.readbyqxmd.com/read/29196308/pseudohyponatraemia-secondary-to-hyperlipidaemia-in-obstructive-jaundice
#4
Samuel O Igbinedion, Sudha Pandit, Meher S Mavuram, Moheb Boktor
A 44-year-old man with uncontrolled diabetes and chronic pancreatitis presented with abdominal pain, jaundice and unintentional weight loss. Laboratory investigations were significant for hyponatraemia, an obstructive pattern of liver enzymes. Imaging was consistent with intrahepatic and extrahepatic biliary obstruction, and endoscopic evaluation revealed a long common bile duct stricture. Intravascular volume depletion, beer potomania and syndrome of inappropriate antidiuretic hormone (with concern for biliary or pancreatic malignancy) were considered in the work-up for the aetiology of the hyponatraemia...
December 1, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/29196240/the-pathophysiology-of-human-obstructive-cholestasis-is-mimicked-in-cholestatic-gold-syrian-hamsters
#5
Rowan F van Golen, Pim B Olthof, Lianne R de Haan, Robert-Jan Coelen, Alexandros Pechlivanis, Mark J de Keijzer, Ruud Weijer, Dirk R de Waart, André B P van Kuilenburg, Jeroen Roelofsen, Pim W Gilijamse, Martinus A Maas, Matthew R Lewis, Jeremy K Nicholson, Joanne Verheij, Michal Heger
Obstructive cholestasis causes liver injury via accumulation of toxic bile acids (BAs). Therapeutic options for cholestatic liver disease are limited, partially because the available murine disease models lack translational value. Profiling of time-related changes following bile duct ligation (BDL) in Gold Syrian hamsters revealed a biochemical response similar to cholestatic patients in terms of BA pool composition, alterations in hepatocyte BA transport and signaling, and suppression of BA production. Hamsters tolerated cholestasis well for up to 28days and progressed relatively slowly to fibrotic liver injury...
November 28, 2017: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/29185945/alagille-syndrome-an-overview
#6
Dalacy Jesina
Alagille syndrome (AGS) is a highly complex, multisystem, autosomal dominant disorder that is caused by a defect in the Notch signaling pathway. This syndrome mainly affects the liver, causing significant cholestasis, which is caused by a paucity of intrahepatic bile ducts. There can be cardiac involvement, including, but not limited to, pulmonary stenosis and tetralogy of Fallot. Patients can also present with butterfly vertebra, ocular issues, and vascular events. Because this syndrome follows an autosomal dominant inheritance, it can have variable expression even in the same family line...
November 1, 2017: Neonatal Network: NN
https://www.readbyqxmd.com/read/29174177/long-term-outcomes-of-six-patients-after-partial-internal-biliary-diversion-for-progressive-familial-intrahepatic-cholestasis
#7
Basak Erginel, Feryal Gun Soysal, Ozlem Durmaz, Alaattin Celik, Tansu Salman
BACKGROUND: Partial internal biliary diversion (PIBD) is an alternative approach for the treatment of devastating pruritus in patients with progressive familial intrahepatic cholestasis (PFIC). In these patients quality of life can be improved and progression of liver disease can be delayed while waiting for liver transplantation. The aim of our study was to evaluate six patients with PFIC who have undergone PIBD in long-term follow-up. METHODS: Retrospective review of the records of six patients who underwent PIBD for PFIC between 2008 and 2010 was conducted to evaluate age, growth, clinical and laboratory studies for long-term outcome...
November 23, 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/29164395/primary-biliary-cholangitis-a-comprehensive-overview
#8
REVIEW
Ana Lleo, Simona Marzorati, Juan-Manuel Anaya, M Eric Gershwin
Primary biliary cholangitis (PBC) is an autoimmune liver disease characterized by biliary destruction, progressive cholestasis, and potentially liver cirrhosis. Patients develop a well-orchestrated immune reaction, both innate and adaptive, against mitochondrial antigens that specifically targets intrahepatic biliary cells. A puzzling feature of PBC is that the immune attack is predominantly organ specific, although the mitochondrial autoantigens are found in all nucleated cells. The disease results from a combination of genetic and environmental risk factors; however, the exact pathogenesis remains unclear...
November 21, 2017: Hepatology International
https://www.readbyqxmd.com/read/29152073/molecular-diagnosis-of-citrin-deficiency-in-an-infant-with-intrahepatic-cholestasis-identification-of-a-21-7kb-gross-deletion-that-completely-silences-the-transcriptional-and-translational-expression-of-the-affected-slc25a13-allele
#9
Zhan-Hui Zhang, Wei-Xia Lin, Qi-Qi Zheng, Li Guo, Yuan-Zong Song
Neonatal Intrahepatic Cholestasis caused by Citrin Deficiency (NICCD) arises from biallelic SLC25A13 mutations, and SLC25A13 analysis provides reliable evidences for NICCD definite diagnosis. However, novel large insertions/deletions in this gene could not be detected just by conventional DNA analysis. This study aimed to explore definite diagnostic evidences for an infant highly-suspected to have NICCD. Prevalent mutation screening and Sanger sequencing of SLC25A13 gene just revealed a paternally-inherited mutation c...
October 20, 2017: Oncotarget
https://www.readbyqxmd.com/read/29141329/-association-between-illness-and-mental-status-in-pregnant-women-and-birth-defects
#10
D D Zhao, Y X Dai, L Q Guo, R Liu, H L Wang, B B Mi, S N Dang, H Yan
Objective: To understand the prevalence of birth defects, related diseases and mental status of women during pregnancy in Shaanxi province and to analyse the major risk factors on birth defects and congenital heart disease. Possible association between maternal diseases or mental status and the risk of birth defects, was also explored. Methods: A cross-sectional design was used in this study and stratified multistage random sampling method was used. The whole survey was from Jury 2013 to November 2013. Logistic regression method was used to analyze the association between maternal diseases, mental status during pregnancy and birth defects...
November 10, 2017: Zhonghua Liu Xing Bing Xue za Zhi, Zhonghua Liuxingbingxue Zazhi
https://www.readbyqxmd.com/read/29137101/analysis-of-islet-beta-cell-functions-and-their-correlations-with-liver-dysfunction-in-patients-with-neonatal-intrahepatic-cholestasis-caused-by-citrin-deficiency-niccd
#11
Chun-Ting Lu, Jing Yang, Si-Min Huang, Lie Feng, Ze-Jian Li
Neonatal intrahepatic cholestasis caused by citrin deficiency (NICCD) primarily manifests in neonates or infants with hepatomegaly, liver dysfunction, and hypoglycemia. This study investigated the functions of islet beta cells and their correlations with liver dysfunction in NICCD patients.We retrospectively analyzed clinical data on liver function and islet beta cell functions for 36 patients diagnosed with NICCD and 50 subjects as the control group. The NICCD group had significantly higher total bilirubin (TBIL), direct bilirubin (DBIL), alanine aminotransferase (ALT), aspartate amino transferase (AST), gamma-glutamyl transpeptidase (GGT), alkaline phosphatase (ALP) and alpha-fetoprotein (AFP) levels and albumin/globulin ratio (A/G) (P < ...
November 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29125588/glucocorticoids-cause-gender-dependent-reversal-of-hepatic-fibrosis-in-the-mdr2-knockout-mouse-model
#12
Anca D Petrescu, Stephanie Grant, Gabriel Frampton, Jessica Kain, Karam Hadidi, Elaina Williams, Matthew McMillin, Sharon DeMorrow
Hepatic cholestasis is associated with a significant suppression of the hypothalamus-pituitary-adrenal axis (HPA). In the present study, we tested the hypothesis that activation of the HPA axis by corticosterone treatment can reverse liver inflammation and fibrosis in a multidrug resistance protein 2 knockout (MDR2KO) transgenic mouse model of hepatic cholestasis. Friend Virus B NIH-Jackson (FVBN) control and MDR2KO male and female mice were treated with vehicle or corticosterone for two weeks, then serum and liver analyses of hepatic cholestasis markers were performed...
November 10, 2017: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/29121800/protective-effects-of-ginsenosides-on-17-formula-see-text-ethynyelstradiol-induced-intrahepatic-cholestasis-via-anti-oxidative-and-anti-inflammatory-mechanisms-in-rats
#13
Yan-Jiao Xu, Zao-Qin Yu, Cheng-Liang Zhang, Xi-Ping Li, Cheng-Yang Feng, Kai Lei, Wen-Xi He, Dong Liu
The present study was designed to assess the effects and potential mechanisms of ginsenosides on 17[Formula: see text]-ethynyelstradiol (EE)-induced intrahepatic cholestasis (IC). Ginsenoside at doses of 30, 100, 300[Formula: see text]mg/kg body weight was intragastrically (i.g.) given to rats for 5 days to examine the effect on EE-induced IC. Serum levels of alanine aminotransferase (ALT), aspartate aminotransferase (AST), alkaline phosphatase (ALP), and total bile acid (TBA) were measured. Hepatic malondialdehyde (MDA) content and superoxide dismutase (SOD) activity were determined...
November 9, 2017: American Journal of Chinese Medicine
https://www.readbyqxmd.com/read/29118315/balancing-medical-and-non-accidental-causes-of-multiple-fractures-in-a-child-with-progressive-familial-intrahepatic-cholestasis
#14
Hisham Abdelrhim, Sami Khan, Paul Heaton, Rajeev Peeka
BACKGROUND All medical practitioners must be vigilant for child abuse and neglect (CAN) so that opportunities to intervene, prevent, and improve outcomes are not missed. However, child abuse is often overlooked in practice, and no sign or pattern of presentation of fractures is absolutely specific for child abuse. CASE REPORT Here, we present the case of a 22-month-old girl with progressive familial intrahepatic cholestasis (PFIC) type 2 who presented with "red flag" fractures indicative of child abuse. Biochemistry showed vitamin D deficiency and a skeletal survey revealed rickets and multiple pathological fractures...
November 9, 2017: American Journal of Case Reports
https://www.readbyqxmd.com/read/29104077/assessment-of-atp8b1-deficiency-in-pediatric-patients-with-cholestasis-using-peripheral-blood-monocyte-derived-macrophages
#15
Hisamitsu Hayashi, Sotaro Naoi, Takao Togawa, Yu Hirose, Hiroki Kondou, Yasuhiro Hasegawa, Daiki Abukawa, Mika Sasaki, Koji Muroya, Satoshi Watanabe, Satoshi Nakano, Kei Minowa, Ayano Inui, Akinari Fukuda, Mureo Kasahara, Hironori Nagasaka, Kazuhiko Bessho, Mitsuyoshi Suzuki, Hiroyuki Kusuhara
Progressive familial intrahepatic cholestasis type 1 (PFIC1), a rare inherited recessive disease resulting from a genetic deficiency in ATP8B1, progresses to liver failure. Because of the difficulty of discriminating PFIC1 from other subtypes of PFIC based on its clinical and histological features and genome sequencing, an alternative method for diagnosing PFIC1 is desirable. Herein, we analyzed human peripheral blood monocyte-derived macrophages (HMDM) and found predominant expression of ATP8B1 in interleukin-10 (IL-10)-induced M2c, a subset of alternatively activated macrophages...
October 7, 2017: EBioMedicine
https://www.readbyqxmd.com/read/29093628/evidence-from-a-familial-case-suggests-maternal-inheritance-of-primary-biliary-cholangitis
#16
Saeam Shin, In Ho Moh, Young Sik Woo, Sung Won Jung, Jin Bae Kim, Ji Won Park, Ki Tae Suk, Hyoung Su Kim, Mineui Hong, Sang Hoon Park, Myung Seok Lee
Primary biliary cholangitis (PBC) is an idiopathic autoimmune liver disease characterized by chronic cholestasis and destruction of the intrahepatic bile ducts. Similar to other autoimmune diseases, the pathogenesis of PBC is considered to be a complex etiologic phenomenon involving the interaction of genetic and environmental factors. Although a number of common variants associated with PBC have been reported from genome-wide association studies, a precise genetic mechanism underlying PBC has yet to be identified...
October 21, 2017: World Journal of Gastroenterology: WJG
https://www.readbyqxmd.com/read/29091294/zebrafish-abcb11b-mutant-reveals-novel-strategies-to-restore-bile-excretion-impaired-by-bile-salt-export-pump-deficiency
#17
Jillian L Ellis, Kevin E Bove, Erin G Schuetz, Daniel Leino, C Alexander Valencia, John D Schuetz, Alexander Miethke, Chunyue Yin
Bile salt export pump BSEP (ABCB11) is a liver-specific adenosine triphosphate-cassette binding transporter that mediates canalicular bile salt excretion from hepatocytes. Human mutations in ABCB11 cause progressive familial intrahepatic cholestasis type II (PFIC2). Although over 150 ABCB11 variants have been reported, our understanding of their biological consequences is limited by the lack of experimental model that recapitulates the patient phenotypes. We applied CRISPR/Cas9-based genome editing technology to knockout abcb11b, the ortholog of human ABCB11, in zebrafish and found that these mutants died prematurely...
November 1, 2017: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://www.readbyqxmd.com/read/29065754/diagnosis-and-prognosis-of-early-onset-intrahepatic-cholestasis-of-pregnancy-a-prospective-study
#18
Lin Jing, Gu Wei, Hou Yanyan
OBJECTIVE: To explore the gestational age of early-onset intrahepatic cholestasis (ICP) of pregnancy, and to analyze the relationship between the clinical biochemical indices and pregnancy outcomes in order to arrive at a reasonable diagnosis and administer appropriate treatment. DESIGN: This is a retrospective clinical study.
October 24, 2017: Journal of Maternal-fetal & Neonatal Medicine
https://www.readbyqxmd.com/read/29060991/-influence-of-intrahepatic-cholestasis-during-pregnancy-on-the-incidence-of-preterm-birth
#19
Maimaiti Reyihanguli, H Y Liu, W H Han, Y F Luo, A Q Hu, Y Wang, H L Wang, H Y Xiong, Y J Zheng
Objective: Intrahepatic cholestasis during pregnancy (ICP) and its relation to incidence of preterm birth (PTB) were under study. Methods: A prospective cohort study was carried out that including all the hospitalized pregnant women with live singleton births, from January 2014 to March 2015 in Anqing Municipal Hospitals. Informed consent was followed in every pregnant woman with related demographic information collected through questionnaire and hospital electronic medical record system. Both univariate and multi-variate statistical methods were used to analyze the relations between ICP and incidence of PTB...
October 10, 2017: Zhonghua Liu Xing Bing Xue za Zhi, Zhonghua Liuxingbingxue Zazhi
https://www.readbyqxmd.com/read/29056017/-research-advances-of-relationship-between-non-alcoholic-fatty-liver-disease-and-biliary-tract-diseases
#20
R X Huang, B C Wang, J G Fan
Recent studies have found that non-alcoholic fatty liver disease(NAFLD) has great impact on the development of biliary tract diseases. Here in this review, we summarized the relationship between NAFLD and the occurrence and development, risk factors and severity of cholestasis, gallstones, intrahepatic cholangiocarcinoma, primary biliary cirrhosis and bile microbiota, so as to further illuminate the pathogenesis of NAFLD and biliary tract diseases, obtain better diagnostic and therapeutic outcomes on NAFLD and biliary tract diseases...
August 20, 2017: Zhonghua Gan Zang Bing za Zhi, Zhonghua Ganzangbing Zazhi, Chinese Journal of Hepatology
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