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Intrahepatic Cholestasis

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https://www.readbyqxmd.com/read/28611260/sensitivity-and-specificity-of-biochemical-tests-for-diagnosis-of-intrahepatic-cholestasis-of-pregnancy
#1
Jurate Kondrackiene, Rimantas Zalinkevicius, Jolanta Sumskiene, Vladas Gintautas, Limas Kupcinskas
BACKGROUND AND AIM: Intrahepatic cholestasis of pregnancy (ICP) is linked with increased risk of fetal complications. An accurate diagnostic test is needed to diagnose this disorder on time. We aimed to assess sensitivity and specificity of laboratory tests used for diagnosis of intrahepatic cholestasis of pregnancy and determine more reliable cut-off values of transaminases. MATERIAL AND METHODS: Sixty one symptomatic patients with ICP and 29 healthy pregnant women were included in the retrospective analysis...
August 1, 2017: Annals of Hepatology
https://www.readbyqxmd.com/read/28611259/prevalence-risk-factors-and-survival-of-patients-with-intrahepatic-cholangiocarcinoma
#2
Paulina Chinchilla-López, Nancy Aguilar-Olivos, Jaime García-Gómez, Karen Hernández-Alejandro, Fredy Chablé-Montero, Daniel Motola-Kuba, Tushar Patel, Nahum Mendez-Sánchez
PURPOSE: To investigate the prevalence, related risk factors, and survival of intrahepatic cholangiocarcinoma in a Mexican population. MATERIAL AND METHODS: We conducted a cross-sectional study at Medica Sur Hospital in Mexico City with approval of the local research ethics committee. We found cases by reviewing all clinical records of in-patients between October 2005 and January 2016 who had been diagnosed with malignant liver tumors. Clinical characteristics and comorbidities were obtained to evaluate the probable risk factors and the Charlson index...
August 1, 2017: Annals of Hepatology
https://www.readbyqxmd.com/read/28588187/primary-sclerosing-cholangitis-in-a-young-female
#3
S Biswas, C S Bala, M N Ahasan, M K Chowdhury, M M Hassan, P K Sarkar, M M Sarkar, M Haque
Primary sclerosing cholangitis (PSC) is a liver disease characterized by inflammation and fibrosis of both intrahepatic and extrahepatic bile ducts resulting in cholestasis. Due to nonspecific symptoms it is difficult to diagnose until complication arises. It is common in male and usually associated with other autoimmune diseases. Here, we report a case of PSC in a young female which was initially thought to be drug induced cholestasis without presence of any other autoimmunity.
April 2017: Mymensingh Medical Journal: MMJ
https://www.readbyqxmd.com/read/28583540/living-donor-liver-transplantation-for-progressive-familial-intrahepatic-cholestasis-type-1-two-reported-cases
#4
Y Oya, Y Sugawara, M Honda, D Yoshii, K Isono, S Hayashida, H Yamamoto, Y Inomata
BACKGROUND: Progressive familial intrahepatic cholestasis type 1 (PFIC1) is an inherited disease characterized by cholestatic features. We report two patients with PFIC1 who underwent liver retransplantation. CASE REPORT: One patient was a 3-year-old female who underwent liver transplantation for PFIC1. She presented with severe diarrhea and fatty liver, and went into liver failure. She therefore underwent liver retransplantation and external biliary diversion 8 years after the initial liver transplantation...
June 2017: Transplantation Proceedings
https://www.readbyqxmd.com/read/28583322/ocular-findings-in-patients-with-cholestatic-disorders-of-infancy-a-single-centre-experience
#5
Hanaa El-Karaksy, Dalia Hamed, Hanan Fouad, Engy Mogahed, Heba Helmy, Fotouh Hasanain
BACKGROUND AND STUDY AIMS: Neonatal cholestasis can be associated with ocular findings that might aid in its diagnosis, e.g., Alagille syndrome (AGS) and Niemann Pick disease (NPD). We aimed to investigate the frequency of ocular manifestations in infants with cholestasis. PATIENTS AND METHODS: This cross-sectional study included cholestatic infants presenting to the Paediatric Hepatology Unit, Cairo University Paediatric Hospital, Cairo, Egypt. All infants underwent examination of lid, ocular motility, anterior and posterior segments and measurement of intraocular pressure, cycloplegic refraction, ocular ultrasonography and vision...
June 2, 2017: Arab Journal of Gastroenterology: the Official Publication of the Pan-Arab Association of Gastroenterology
https://www.readbyqxmd.com/read/28581354/perinatal-outcomes-associated-with-intrahepatic-cholestasis-of-pregnancy
#6
Christina Annette Herrera, Tracy A Manuck, Gregory J Stoddard, Michael W Varner, Sean Esplin, Erin A S Clark, Robert M Silver, Alexandra G Eller
OBJECTIVE: The objective of this study is to examine perinatal outcomes associated with cholestasis of pregnancy according to bile acid level and antenatal testing practice. STUDY DESIGN: Retrospective cohort study of women with symptoms and bile acid testing from 2005 to 2014. Women were stratified by bile acid level: no cholestasis (<10 μmol/L), mild (10-39 μmol/L), moderate (40-99 μmol/L), and severe (≥100 μmol/L). The primary outcome was composite neonatal morbidity (hypoxic ischemic encephalopathy, severe intraventricular hemorrhage, bronchopulmonary dysplasia, necrotizing enterocolitis, or death)...
June 5, 2017: Journal of Maternal-fetal & Neonatal Medicine
https://www.readbyqxmd.com/read/28577654/sickle-cell-intrahepatic-cholestasis-unresponsive-to-exchange-blood-transfusion-a-case-report
#7
Juliana Albano de Guimarães, Luciana Cristina Dos Santos Silva
No abstract text is available yet for this article.
April 2017: Revista Brasileira de Hematologia e Hemoterapia
https://www.readbyqxmd.com/read/28575098/mir-148a-mediated-estrogen-induced-cholestasis-in-intrahepatic-cholestasis-of-pregnancy-role-of-pxr-mrp3
#8
Zhou-Zhou Rao, Xiao-Wen Zhang, Yi-Ling Ding, Meng-Yuan Yang
Intrahepatic cholestasis of pregnancy (ICP) is an idiopathic liver disease while the biochemical characteristic is the elevated level of total bile acid (TBA). The present study investigated whether miR-148a mediates the induced effect of estrogen on the development of ICP and the proper mechanism: PXR/MRP3 signal pathway. mRNA expression was detected by qPCR, protein expression was detected by western blotting, the concentration of estrogen and TBA were detected by reagent kit respectively. In the cinical research, it was found that miR-148a expression was positive related with the concentration of TBA in the serum of ICP patients...
2017: PloS One
https://www.readbyqxmd.com/read/28574590/pregnancy-outcomes-after-liver-transplantation-in-finland
#9
Mirjami Mattila, Helena Kemppainen, Helena Isoniemi, Päivi Polo-Kantola
Pregnancy after liver transplantation (LT) is possible but associated with increased risk of obstetrical complications. We report here for the first time the pregnancy outcomes after LT in Finland MATERIAL AND METHODS: All of the 25 pregnancies ending in deliveries after LT in Finland in 1998-2015 were analyzed. The data were collected from the mothers' medical records. The main outcome measures included pregnancy complications and the mode of the delivery. Neonatal outcome measures were birth weight, 5 minute Apgar score and umbilical artery pH RESULTS: There were 26 infants born...
June 2, 2017: Acta Obstetricia et Gynecologica Scandinavica
https://www.readbyqxmd.com/read/28570655/circulating-fgf19-closely-correlates-with-bile-acid-synthesis-and-cholestasis-in-patients-with-primary-biliary-cirrhosis
#10
Zhanyi Li, Bingliang Lin, Guoli Lin, Yuankai Wu, Yusheng Jie, Xiangyong Li, Brian Ko, Yutian Chong, Jian Luo
BACKGROUND AND AIM: Bile acid (BA) synthesis in the liver is regulated by Fibroblast Growth Factor 19 (FGF19) secreted from the ileum as an enterohepatic feedback mechanism. Although FGF19 mRNA is absent in normal liver, FGF19 gene expression was reported to increase in response to both extrahepatic and intrahepatic cholestasis. The impact of upregulated FGF19 expression on BA synthesis is unclear and the overall role of circulating FGF19 and BA synthesis under cholestatic conditions needs to be further investigated...
2017: PloS One
https://www.readbyqxmd.com/read/28566572/importance-of-reverse-translational-research-rtr
#11
Yuichi Sugiyama
 When events lead to clinical problems, the mechanisms involved often remain unclear. This is true for medications and therapies, in addition to problems inherent in an underlying disease. However, the recent development of modeling and metric methods makes it possible to estimate the relationship between side effects and various factors to explain inter-individual differences, such as genetic polymorphisms, co-administered drugs, age, gender, dysfunction of the liver/kidney based upon the database for side effects [such as Food and Drug Administration-Adverse Event Reporting System (FDA-AERS)] and the database in a patient's medical records...
2017: Yakugaku Zasshi: Journal of the Pharmaceutical Society of Japan
https://www.readbyqxmd.com/read/28565794/novel-two-step-derivation-method-for-the-synchronous-analysis-of-inherited-metabolic-disorders-using-urine
#12
Xiao-Qi Sheng, Yi-Chao Wang
The aim of the present study was to conduct preliminary clinical screening and monitoring using a novel two-step derivatization process of urine in five categories of inherited metabolic disease (IMD). Urine samples (100 µl, containing 2.5 mmol/l creatinine) were taken from patients with IMDs. The collected urine was then treated using a two-step derivatization method (with oximation and silylation at room temperature), where urea and protein were removed. In the first step of the derivatization, α-ketoacids and α-aldehyde acids were prepared by oximation using novel oximation reagents...
May 2017: Experimental and Therapeutic Medicine
https://www.readbyqxmd.com/read/28553227/srt1720-alleviates-anit-induced-cholestasis-in-a-mouse-model
#13
Linxi Yu, Xiaoxin Liu, Zihang Yuan, Xiaojiaoyang Li, Hang Yang, Ziqiao Yuan, Lixin Sun, Luyong Zhang, Zhengzhou Jiang
Intrahepatic cholestasis is a kind of clinical syndrome along with hepatotoxicity which caused by intrahepatic and systemic accumulations of bile acid. There are several crucial generating factors of the pathogenesis of cholestasis, such as inflammation, dysregulation of bile acid transporters and oxidative stress. SIRT1 is regarded as a class III histone deacetylase (HDAC). According to a set of researches, SIRT1 is one of the most important factors which can regulate the hepatic bile acid metabolism. SRT1720 is a kind of activator of SIRT1 which is 1000 times more potent than resveratrol, and this paper is aimed to study its protective influence on hepatotoxicity and cholestasis induced by alpha-naphthylisothiocyanate (ANIT) in mice...
2017: Frontiers in Pharmacology
https://www.readbyqxmd.com/read/28552021/maternal-copeptin-levels-in-intrahepatic-cholestasis-of-pregnancy
#14
Ahmet Tayyar, İlkbal Temel Yüksel, Nadiye Köroğlu, Ahter Tanay Tayyar, Ebru Alıcı Davutoğlu, Asuman Akkaya Fırat, Berna Aslan Çetin
PURPOSE: To investigate copeptin levels in women with intrahepatic cholestasis of pregnancy (ICP) compared to women with uncomplicated pregnancies. MATERIALS AND METHODS: This cross-sectional study was conducted in 40 pregnant women with ICP and 38 randomly selected healthy pregnant women, who formed the control group. Serum copeptin concentrations were measured using an enzyme-linked immunosorbent assay. RESULTS: Maternal age, body mass index at assessment, and gestational age at blood sampling were similar between the two groups...
May 28, 2017: Journal of Maternal-fetal & Neonatal Medicine
https://www.readbyqxmd.com/read/28524363/donor-transmitted-mutation-of-the-abcb11-gene-and-ensuing-intra-hepatic-cholestasis-of-pregnancy-in-a-liver-transplant-recipient
#15
Tiong Yeng Lim, Iona Coltart, Pierre Foskett, Richard Thompson, Sandra Strautnieks, Leonie Penna, Catherine Williamson, Rosa Miquel, Michael A Heneghan
In liver transplant (LT) recipients, the cause of graft dysfunction in pregnancy is often difficult to ascertain. Moreover, a liver biopsy in late pregnancy is often avoided as a consequence of patient and physician factors. Management of graft dysfunction can be difficult in this setting. We report a 30-year-old female LT recipient who developed acutely deranged liver biochemistry during the third trimester of her first pregnancy. At 29 weeks gestation, her liver function test (LFT) became abnormal; AST peaked at 978 IU/L (normal range 10-50), GGT 25 IU/L (normal range 1-55), bilirubin 1...
May 19, 2017: Liver Transplantation
https://www.readbyqxmd.com/read/28516797/blood-glucose-and-insulin-and-correlation-of-slc25a13-mutations-with-biochemical-changes-in-niccd-patients
#16
Chun-Ting Lu, Qi-Ping Shi, Ze-Jian Li, Jiong Li, Lie Feng
Neonatal intrahepatic cholestasis caused by citrin deficiency (NICCD) is a hereditary metabolic disease arising from biallelic mutations of SLC25A13. This study aimed to explore the characteristics of fasting blood glucose (FBG), fasting insulin (FINS) and C-peptide (C-P) levels in NICCD infants, analyze their SLC25A13 genetic mutations and further discuss the correlation between SLC25A13 genetic mutations and biochemical changes. Seventy-two cases of infants with cholestasis disease were gathered. Among them, 36 cases with NICCD diagnosis were case group...
January 1, 2017: Experimental Biology and Medicine
https://www.readbyqxmd.com/read/28506676/bile-acids-and-gestation
#17
REVIEW
Saraid McIlvride, Peter H Dixon, Catherine Williamson
There are numerous profound maternal physiological changes that occur from conception onwards and adapt throughout gestation in order to support a healthy pregnancy. By the time of late gestation, when circulating pregnancy hormones are at their highest concentrations, maternal adaptations include relative hyperlipidemia, hypercholanemia and insulin resistance. Bile acids have now been established as key regulators of metabolism, and their role in gestational changes in metabolism is becoming apparent. Bile acid homeostasis is tightly regulated by the nuclear receptor FXR, which has been shown to have reduced activity during pregnancy...
May 12, 2017: Molecular Aspects of Medicine
https://www.readbyqxmd.com/read/28506343/-current-status-of-research-on-infantile-cholestatic-liver-disease-in-china-a-visualization-analysis
#18
Qiong Liao, Chao-Min Wan, Yu Zhu, Xiao-Yan Yang, Min Shu
OBJECTIVE: To investigate the current status of research on infantile cholestatic liver disease in China and future research trends. METHODS: A co-word analysis was performed in October 2016. Document retrieval and screening were performed in the Chinese databases CNKI and Wanfang Data using "cholestasis" and "infant" as key words. Excel 2010 was used to establish a co-occurrence matrix of high-frequency key words, and Ucinet 6.0 and Netdraw were used to develop a visualized network of these high-frequency key words...
May 2017: Zhongguo Dang Dai Er Ke za Zhi, Chinese Journal of Contemporary Pediatrics
https://www.readbyqxmd.com/read/28502477/knockdown-of-hepatic-gonadotropin-releasing-hormone-by-vivo-morpholino-decreases-liver-fibrosis-in-multidrug-resistance-gene-2-knockout-mice-by-down-regulation-of-mir-200b
#19
Konstantina Kyritsi, Fanyin Meng, Tianhao Zhou, Nan Wu, Julie Venter, Heather Francis, Lindsey Kennedy, Paolo Onori, Antonio Franchitto, Francesca Bernuzzi, Pietro Invernizzi, Kelly McDaniel, Romina Mancinelli, Domenico Alvaro, Eugenio Gaudio, Gianfranco Alpini, Shannon Glaser
Hepatic fibrosis occurs during the progression of primary sclerosing cholangitis (PSC) and is characterized by accumulation of extracellular matrix proteins. Proliferating cholangiocytes and activated hepatic stellate cells (HSCs) participate in the promotion of liver fibrosis during cholestasis. Gonadotropin-releasing hormone (GnRH) is a trophic peptide hormone synthesized by hypothalamic neurons and the biliary epithelium and exerts its biological effects on cholangiocytes by interaction with the receptor subtype (GnRHR1) expressed by cholangiocytes and HSCs...
July 2017: American Journal of Pathology
https://www.readbyqxmd.com/read/28497648/intrahepatic-cholangiojejunostomy-for-complex-biliary-stenosis-after-pediatric-living-donor-liver-transplantation
#20
Fernando A Alvarez, Rodrigo Sanchez Claria, Juan Glinka, Martin de Santibañes, Juan Pekolj, Eduardo de Santibañes, Miguel A Ciardullo
The treatment of biliary stenosis after pediatric LDLT is challenging. We describe an innovative technique of peripheral IHCJ for the treatment of patients with complex biliary stenosis after pediatric LDLT in whom percutaneous treatment failed. During surgery, the percutaneous biliary drainage is removed and a flexible metal stylet is introduced trough the tract. Subsequently, the most superficial aspect of the biliary tree is recognized by palpation of the stylet's round tip in the liver surface. The liver parenchyma is then transected until the bile duct is reached...
May 12, 2017: Pediatric Transplantation
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