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Intrahepatic Cholestasis

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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
#1
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
#2
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
#3
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
#4
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
#5
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...
May 11, 2017: American Journal of Pathology
https://www.readbyqxmd.com/read/28497648/intrahepatic-cholangiojejunostomy-for-complex-biliary-stenosis-after-pediatric-living-donor-liver-transplantation
#6
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
https://www.readbyqxmd.com/read/28497004/progressive-familial-intrahepatic-cholestasis-type-2-in-an-indian-child
#7
Ira Shah, Sujeet Chilkar
Progressive familial intrahepatic cholestasis (PFIC) is a chronic cholestasis syndrome that begins in infancy and usually progresses to cirrhosis within the first decade of life. There are three varieties of PFIC described: PFIC-1 occurs due to mutations in the ATP8B1 gene mapped to 18q21.31, PFIC-2 due to mutations in ABCB11 mapped to 2q24, and PFIC-3 due to mutations in ABCB4 located on 7q21.12. We report an Indian child whose mutation analysis was suggestive of PFIC-2. He underwent a biliary diversion at 3½ years of age but subsequently died secondary to massive hematemesis...
June 2017: Journal of Pediatric Genetics
https://www.readbyqxmd.com/read/28494557/-clinicopathologic-features-of-drug-induced-vanishing-bile-duct-syndrome
#8
L H Ye, C K Wang, H C Zhang, Z Q Liu, H W Zheng
Vanishing bile duct syndrome (VBDS) manifests as progressive destruction and disappearance of the intrahepatic bile duct caused by various factors and cholestasis. VBDS associated with drug-induced liver injury (D-VBDS) is an important etiology of VBDS, and immune disorder or immune imbalance may be the main pathogenesis. According to its clinical symptoms, serological markers, and course of the disease, D-VBDS is classified into major form and minor form, and its clinical features are based on various pathomorphological findings...
April 20, 2017: Zhonghua Gan Zang Bing za Zhi, Zhonghua Ganzangbing Zazhi, Chinese Journal of Hepatology
https://www.readbyqxmd.com/read/28484399/biochemical-benefits-diagnosis-and-clinical-risks-evaluation-of-kratom
#9
REVIEW
Dimy Fluyau, Neelambika Revadigar
BACKGROUND: Kratom (Mitragyna speciosa) is a tropical tree with a long history of traditional use in parts of Africa and Southeast Asia. Kratom is also known as Thom, Thang, and Biak. Its leaves and the teas brewed from them have long been used by people in that region to manage pain and opioid withdrawal and to stave off fatigue. Kratom is actually consumed throughout the world for its stimulant effects and as an opioid substitute (in form of tea, chewed, smoked, or ingested in capsules)...
2017: Frontiers in Psychiatry
https://www.readbyqxmd.com/read/28477356/epigallocatechin-3-gallate-ameliorates-intrahepatic-cholestasis-of-pregnancy-by-inhibiting-matrix-metalloproteinases-2-and-9
#10
Mei Zhang, Meimei Xu
Matrix metalloproteinase (MMP)-2 and -9 are involved in many illnesses affecting pregnant women, including intrahepatic cholestasis of pregnancy (ICP), a serious liver abnormality during pregnancy. Epigallocatechin 3-gallate (EGCG) has been widely reported to inhibit activities of MMP-2 and MMP-9. We aimed to investigate the role of EGCG in ameliorating ICP symptoms in a rat model. Using 17α-ethinylestradiol to induce ICP in pregnant rats, we investigated the efficacy of EGCG administration on ICP symptoms, including bile flow rate, total bile acids (TBA) and MMP-2 and MMP-9 activities...
May 5, 2017: Fundamental & Clinical Pharmacology
https://www.readbyqxmd.com/read/28476903/endoscopic-nasobiliary-drainage-an-effective-treatment-option-for-benign-recurrent-intrahepatic-cholestasis-bric
#11
Ashok Choudhury, Anand V Kulkarni, Bishnupriya Sahoo, Chhagan Bihari
Benign recurrent intrahepatic cholestasis (BRIC) is characterised by recurrent episodes of jaundice, severe pruritus and low or normal serum γ-glutamyltransferase activity lasting from several weeks to months. BRIC is an autosomal recessive disorder caused by the mutation in either of the two hepatic transporter genes-ATP8B1 or ABCB11 gene. The disease is very well known for episodic flare of jaundice with cholestatic symptoms that are spontaneous or perpetuated by acute insults, followed by self-recovery...
May 5, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28465853/novel-heterozygous-mutations-in-jag1-and-notch2-genes-in-a-neonatal-patient-with-alagille-syndrome
#12
Alisa Brennan, Anil Kesavan
Alagille Syndrome (ALGS) is a rare autosomal dominant disorder that affects multiple organ systems. Cholestasis as a result of a paucity of intrahepatic bile ducts and congenital heart defects are the two most common features of ALGS. We describe a case of ALGS with novel mutations of JAG1 and NOTCH2 genes in a newborn girl with complex congenital heart disease, bilateral dysplastic kidneys, and malrotation with volvulus.
2017: Case Reports in Pediatrics
https://www.readbyqxmd.com/read/28462673/an-alternative-method-for-measuring-oxidative-stress-in-intrahepatic-cholestasis-of-pregnancy-thiol-disulphide-homeostasis
#13
Cem Yasar Sanhal, Korkut Daglar, Ozgur Kara, Zehra Vural Yılmaz, Gulenay Gencosmanoglu Turkmen, Ozcan Erel, Dilek Uygur, Aykan Yucel
PURPOSE: The aim of our study was to evaluate the oxidative stress (OS) in pregnant women with intrahepatic cholestasis of pregnancy (ICP) by evaluating thiol/disulphide homeostasis using an alternative technique. METHODS: A total of 57 pregnant women with ICP were compared with 50 gestational age and body mass index matched controls. A recently defined method was used for the measurement of plasma native-total thiol and disulphide levels. The independent two-sample t test, Mann-Whitney-U test, Chi-square test, binary logistic regression with backward elimination and receiver operating characteristic (ROC) curve was performed for statistical analyses...
May 2, 2017: Journal of Maternal-fetal & Neonatal Medicine
https://www.readbyqxmd.com/read/28450276/pregnancy-and-bile-acid-disorders
#14
Vanessa Pataia, Peter H Dixon, Catherine Williamson
During pregnancy, extensive adaptations in maternal metabolic and immunological physiology occur. Consequently, pre-existing disease may be exacerbated or attenuated and new disease susceptibility may be unmasked. Cholestatic diseases, characterized by a supraphysiological raise in bile acid levels, require careful monitoring during pregnancy. This review describes the latest advances in the knowledge of intrahepatic cholestasis of pregnancy (ICP), the commonest bile acid disorder specific to pregnancy, with a focus on the disease etiology and potential mechanisms of ICP-associated adverse pregnancy outcomes, including fetal demise...
April 27, 2017: American Journal of Physiology. Gastrointestinal and Liver Physiology
https://www.readbyqxmd.com/read/28437442/relationship-between-early-onset-severe-intrahepatic-cholestasis-of-pregnancy-and-higher-risk-of-meconium-stained-fluid
#15
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
#16
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
#17
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
#18
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
#19
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, for example, in progressive familial intrahepatic cholestasis or acquired, 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
#20
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
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