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Intrahepatic Cholestasis

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https://www.readbyqxmd.com/read/28196718/the-neuropeptide-galanin-is-up-regulated-during-cholestasis-and-contributes-to-cholangiocyte-proliferation
#1
Matthew McMillin, Gabriel Frampton, Stephanie Grant, Sharon DeMorrow
During the course of cholestatic liver diseases, mitotically dormant cholangiocytes proliferate and subsequently acquire a neuroendocrine phenotype. Galanin is a neuroendocrine factor responsible for regulation of physiological responses, such as feeding behavior and mood, and has been implicated in the development of fatty liver disease, although its role in biliary hyperplasia is unknown. Biliary hyperplasia was induced in rats via bile duct ligation (BDL) surgery, and galanin was increased in serum and liver homogenates from BDL rats...
February 11, 2017: American Journal of Pathology
https://www.readbyqxmd.com/read/28195083/progressive-familial-intrahepatic-cholestasis-a-comprehensive-review-of-a-challenging-liver-disease
#2
REVIEW
Kavita Gaur, Puja Sakhuja
Cholestatic liver disease in children represents a diagnostic and therapeutic challenge. The requirement of a multidisciplinary approach, high levels of professional expertise, and the costs of genetic testing are a few of the reasons why such patients may suffer for want of an accurate diagnosis. Progressive familial intrahepatic cholestasis (PFIC) is a hereditary cholestatic liver disease, afflicted children often progressing to liver failure. Despite its potential to cause significant morbidity, it has seldom been studied in India...
January 2017: Indian Journal of Pathology & Microbiology
https://www.readbyqxmd.com/read/28163717/four-major-factors-contributing-to-intrahepatic-stones
#3
REVIEW
Xi Ran, Baobing Yin, Baojin Ma
Intrahepatic stone is prevalent in Asian countries; though the incidence declines in recent years, the number of patients is still in a large quantity. Because of multiple complications, high recurrence rates, serious systemic damage, and a lack of extremely effective procedure for the management, it is more important to find out the etiology and pathogenesis of intrahepatic stones to prevent the disease from happening and developing rather than curing. A number of factors contribute to the development of the disease, such as cholestasis, infection, and anatomic abnormity of bile duct and bile metabolic defect...
2017: Gastroenterology Research and Practice
https://www.readbyqxmd.com/read/28150711/generation-of-a-bile-salt-export-pump-deficiency-model-using-patient-specific-induced-pluripotent-stem-cell-derived-hepatocyte-like-cells
#4
Kazuo Imagawa, Kazuo Takayama, Shigemi Isoyama, Ken Tanikawa, Masato Shinkai, Kazuo Harada, Masashi Tachibana, Fuminori Sakurai, Emiko Noguchi, Kazumasa Hirata, Masayoshi Kage, Kenji Kawabata, Ryo Sumazaki, Hiroyuki Mizuguchi
Bile salt export pump (BSEP) plays an important role in hepatic secretion of bile acids and its deficiency results in severe cholestasis and liver failure. Mutation of the ABCB11 gene encoding BSEP induces BSEP deficiency and progressive familial intrahepatic cholestasis type 2 (PFIC2). Because liver transplantation remains standard treatment for PFIC2, the development of a novel therapeutic option is desired. However, a well reproducible model, which is essential for the new drug development for PFIC2, has not been established...
February 2, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28149148/pediatric-liver-transplantation-our-experiences
#5
Ahmet Basturk, Aygen Yılmaz, Ersin Sayar, Ayhan Dinçhan, İbrahim Aliosmanoğlu, Halil Erbiş, Bülent Aydınlı, Reha Artan
OBJECTIVE: The aim of our study was to evaluate our liver transplant pediatric patients and to report our experience in the complications and the long-term follow-up results. MATERIALS AND METHODS: Patients between the ages of 0 and 18 years, who had liver transplantation in the organ transplantation center of our university hospital between 1997 and 2016, were included in the study. The age, sex, indications for the liver transplantation, complications after the transplantation, and long-term follow-up findings were retrospectively evaluated...
October 2016: Eurasian Journal of Medicine
https://www.readbyqxmd.com/read/28133487/upregulation-of-pdzk1-by-calculus-bovis-sativus-may-play-an-important-role-in-restoring-biliary-transport-function-in-intrahepatic-cholestasis
#6
Dong Xiang, Tao Wu, Cheng-Yang Feng, Xi-Ping Li, Yan-Jiao Xu, Wen-Xi He, Kai Lei, Hong-Jiao Cai, Cheng-Liang Zhang, Dong Liu
Intrahepatic cholestasis is a main cause of hepatic accumulation of bile acids leading to liver injury, fibrosis, and liver failure. Our previous studies proved that Calculus Bovis Sativus (CBS) can restore biliary transport function through upregulating the multidrug resistance-associated protein 2 (MRP2) and breast cancer resistance protein (BCRP) in 17α-ethynylestradiol- (EE-) induced intrahepatic cholestasis rats. The regulation mechanism of CBS on these transporters, however, remains unclear. This study was designed to evaluate the possible relationship between the effect of CBS on transport activities and the regulation of CBS on the expression of PDZK1, a mainly scaffold protein which can regulate MRP2 and BCRP...
2017: Evidence-based Complementary and Alternative Medicine: ECAM
https://www.readbyqxmd.com/read/28128437/schisandrol-b-protects-against-cholestatic-liver-injury-through-pregnane-x-receptor
#7
Hang Zeng, Yiming Jiang, Pan Chen, Xiaomei Fan, Dongshun Li, Aiming Liu, Xiaochao Ma, Wen Xie, Peiqing Liu, Frank J Gonzalez, Min Huang, Huichang Bi
BACKGROUND AND PURPOSE: Currently, ursodeoxycholic acid is the only FDA-approved drug for limited cholestatic liver diseases, and thus the development of new therapeutic approaches is of great importance. This study aimed to evaluate the anti-cholestasis effect of Schisandrol B (SolB), a bioactive compound isolated from Schisandra sphenanthera. EXPERIMENTAL APPROACH: Hepatoprotective effect of SolB against lithocholic acid (LCA)-induced intrahepatic extrahepatic cholestasis was evaluated in mice...
January 27, 2017: British Journal of Pharmacology
https://www.readbyqxmd.com/read/28119944/liver-transplantation-as-a-treatment-for-severe-refractory-vitamin-e-deficiency-related-to-progressive-familial-intrahepatic-cholestasis-type-2-in-a-pediatric-patient
#8
Elizabeth Collyer, Vera Hupertz, Bijan Eghtesad, Kadakkal Radhakrishnan
Refractory vitamin E deficiency is thought to have irreversible effects on neurologic function. We report an adolescent boy with severe refractory vitamin E deficiency due to progressive familial intrahepatic cholestasis (PFIC) type 2. His consequent neurologic dysfunction included severe ataxia, dysmetria, dysarthria, and cranial nerve VI palsy. He underwent liver transplantation at age 13 due to his neurologic dysfunction; and afterward, he had marked improvement in neurologic function. We demonstrate that in a patient with PFIC 2 and severe refractory vitamin E deficiency, liver transplant can improve vitamin E absorption, prevent further neurological sequelae, and reverse prior neurologic dysfunction...
August 2016: ACG Case Reports Journal
https://www.readbyqxmd.com/read/28110453/update-on-the-diagnosis-and-treatment-of-cholangiocarcinoma
#9
REVIEW
Bryan Doherty, Vinod E Nambudiri, William C Palmer
PURPOSE OF REVIEW: Cholangiocarcinoma is a rare biliary adenocarcinoma associated with poor outcomes. Cholangiocarcinoma is subdivided into extrahepatic and intrahepatic variants. Intrahepatic cholangiocarcinoma is then further differentiated into (1) peripheral mass-forming tumors and (2) central periductal infiltrating tumors. We aimed to review the currently known risk factors, diagnostic tools, and treatment options, as well as highlight the need for further clinical trials and research to improve overall survival rates...
January 2017: Current Gastroenterology Reports
https://www.readbyqxmd.com/read/28074213/-intrahepatic-cholestasis-of-pregnancy-rare-but-important
#10
REVIEW
A E Kremer, K Wolf, S Ständer
Intrahepatic cholestasis of pregnancy (ICP) is a liver-specific disorder occurring in approximately 0.5-2.0% of all pregnancies with a considerable variation in certain ethnic groups. ICP usually runs a benign course for the mother and is characterized by maternal pruritus mainly in the third trimester, elevated transaminases and fasting total serum bile salts and increased fetal adverse events. The etiology of ICP is only partially understood but seems to be multifactorial. Cholestasis-inducing effects of certain female sex hormones and their metabolites play an important role in genetically susceptible women...
January 10, 2017: Der Hautarzt; Zeitschrift Für Dermatologie, Venerologie, und Verwandte Gebiete
https://www.readbyqxmd.com/read/28074212/-specific-dermatoses-of-pregnancy
#11
REVIEW
C M Ambros-Rudolph, M Sticherling
The specific dermatoses of pregnancy represent a heterogeneous group of inflammatory skin diseases related to pregnancy and/or the postpartum period. A clinically relevant classification has been well established over the past 10 years and includes pemphigoid gestationis, polymorphic eruption of pregnancy, intrahepatic cholestasis of pregnancy, and atopic eruption of pregnancy. The hallmark of all four entities is severe pruritus that is accompanied by characteristic skin changes. While some of these dermatoses are distressing only to the mother because of pruritus, others may be associated with significant fetal risks...
January 10, 2017: Der Hautarzt; Zeitschrift Für Dermatologie, Venerologie, und Verwandte Gebiete
https://www.readbyqxmd.com/read/28073941/prognostic-roles-of-tetrahydroxy-bile-acids-in-infantile-intrahepatic-cholestasis
#12
Chee-Seng Lee, Akihiko Kimura, Jia-Feng Wu, Yen-Hsuan Ni, Hong-Yuan Hsu, Mei-Hwei Chang, Hiroshi Nittono, Huey-Ling Chen
Tetrahydroxy bile acids (THBAs) are hydrophilic and are present at minimal or undetectable levels in healthy human adults but are present at high levels in bile salt export pump (abcb11)-knockout mice. The roles of THBAs in human cholestatic diseases are unclear. We aimed to investigate the presence of THBAs in patients with infantile intrahepatic cholestasis and its correlation with outcome. Urinary bile acids were analysed by GC-MS. Data were compared between good (N=21) (disease-free before 1 year old) and poor prognosis groups (N=19)...
January 10, 2017: Journal of Lipid Research
https://www.readbyqxmd.com/read/28064265/a-novel-truncation-mutation-in-atp8b1-gene-in-progressive-familial-intrahepatic-cholestasis
#13
Anjali Sharma, Ujjal Poddar, Shikha Agnihotry, Rakesh Aggarwal
BACKGROUND: Progressive familial intrahepatic cholestasis has been only infrequently reported from India. CASE CHARACTERISTICS: An Indian girl with progressive cholestatic liver disease beginning during infancy, normal gamma-glutamyl transpeptidase levels, parental consanguinity, positive family history and a fatal outcome. OBSERVATION: A novel, homozygous mutation (c.[589_592inv;592_593insA]) in ATP8B1 gene, with a markedly truncated protein (p...
December 15, 2016: Indian Pediatrics
https://www.readbyqxmd.com/read/28055006/biliary-tract-instillation-of-a-smac-mimetic-induces-trail-dependent-acute-sclerosing-cholangitis-like-injury-in-mice
#14
Maria Eugenia Guicciardi, Anuradha Krishnan, Steven F Bronk, Petra Hirsova, Thomas S Griffith, Gregory J Gores
Primary sclerosing cholangitis (PSC) is a cholestatic liver disease of unknown etiopathogenesis characterized by fibrous cholangiopathy of large and small bile ducts. Systemic administration of a murine TNF-related apoptosis-inducing ligand (TRAIL) receptor agonist induces a sclerosing cholangitis injury in C57BL/6 mice, suggesting endogenous TRAIL may contribute to sclerosing cholangitis syndromes. Cellular inhibitor of apoptosis proteins (cIAP-1 and cIAP-2) are negative regulators of inflammation and TRAIL receptor signaling...
January 5, 2017: Cell Death & Disease
https://www.readbyqxmd.com/read/28052628/management-of-cholestatic-disease-in-2017
#15
REVIEW
Elsemieke de Vries, Ulrich Beuers
Primary biliary cholangitis (PBC) and primary sclerosing cholangitis (PSC) are the most frequent chronic cholestatic liver diseases and serve as model diseases to discuss the management of cholestasis in 2017 in the lecture that is summarized in this report. PBC and PSC are characterized by inflammation and fibrosis of small intrahepatic (PBC) or larger intra- and/or extrahepatic (PSC) bile ducts. Bile duct damage leads to cholestasis and can progress to liver fibrosis and even cirrhosis. Various genetic, environmental and endogenous factors may contribute to the development of chronic cholestatic liver diseases, but the exact pathogenesis of PBC and PSC has not been clarified...
January 2017: Liver International: Official Journal of the International Association for the Study of the Liver
https://www.readbyqxmd.com/read/28049946/ursodeoxycholic-acid-ameliorates-intrahepatic-cholestasis-independent-of-biliary-bicarbonate-secretion-in-vil2-kd-kd-mice
#16
Ryo Hatano, Kotoku Kawaguchi, Fumitaka Togashi, Masato Sugata, Shizuka Masuda, Shinji Asano
Ursodeoxycholic acid (UDCA) is a hydrophilic bile acid that possesses many pharmacological effects, including increasing bile flow, changing the hydrophobicity of the bile acid pool, and modulation of the immune response. UDCA has been approved for treating cholestatic liver disease, such as primary biliary cholangitis. However, several unanticipated severe side effects of UDCA are observed in cholestatic patients, and its pharmacological benefits remain controversial. We reported that ezrin-knockdown (Vil2(kd/kd)) mice exhibited severe hepatic injury because of a functional disorder in bile duct fluidity and alkalinity regulation, resembling human intrahepatic cholestatic disease...
2017: Biological & Pharmaceutical Bulletin
https://www.readbyqxmd.com/read/28045770/clinical-variability-following-partial-external-biliary-diversion-in-familial-intrahepatic-cholestasis-1-deficiency
#17
James E Squires, Neslihan Celik, Amy Morris, Kyle Soltys, George Mazariegos, Benjamin Shneider, Robert H Squires
OBJECTIVES: Familial intrahepatic cholestasis 1 (FIC1) deficiency is caused by a mutation in the ATP8B1 gene. Partial external biliary diversion (PEBD) is pursued to improve pruritus and arrest disease progression. Our aim is to describe clinical variability after PEBD in FIC1 disease. METHODS: We performed a single-center, retrospective review of genetically confirmed FIC1 deficient patients who received PEBD. Clinical outcomes after PEBD were cholestasis, pruritus, fat-soluble vitamin supplementation, growth, and markers of disease progression that included splenomegaly and aspartate aminotransferase-to-platelet ratio index...
December 30, 2016: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/28039538/maternal-liver-elasticity-determined-by-acoustic-radiation-force-impulse-elastosonography-in-intrahepatic-cholestasis-of-pregnancy
#18
Orkun Cetin, Erbil Karaman, Harun Arslan, Ibrahim Akbudak, Recep Yildizhan, Ali Kolusari
PURPOSE: Intrahepatic cholestasis of pregnancy (ICP) is a pregnancy-specific liver disorder characterized by maternal pruritus and impaired liver function. The objective of the study was to evaluate maternal liver elasticity by acoustic radiation force impulse (ARFI) elastosonography in ICP and to compare it with that in healthy pregnant women. METHODS: This descriptive, case-control study consisted of 33 women with healthy pregnancies and 22 women with ICP in the third trimester of gestation...
December 30, 2016: Journal of Medical Ultrasonics
https://www.readbyqxmd.com/read/28028430/a-child-with-debilitating-pruritus
#19
Nikhil Sonthalia, Samit S Jain, Vinay B Pawar, Vinay G Zanwar, Ravindra G Surude, Pravin M Rathi, Kshitij K Munde, Sandeep Bavdekar
We describe a case of two-year-old boy presenting with debilitating pruritus, patchy alopecia and jaundice since the age of 6 months. On evaluation he had intrahepatic cholestasis with persistently raised serum alkaline phosphatase, normal Gamma glutamyl transferase and raised serum bile acid levels. His liver biopsy showed bland cholestasis and electron microscopy showed granular bile suggestive of progressive familial intrahepatic cholestasis type I. Medical therapy with ursodeoxycholic acid, cholestyramine, rifampicin with nutritional modification was successful in alleviating the symptoms and correcting the nutritional status...
October 24, 2016: Clinics and Practice
https://www.readbyqxmd.com/read/28027587/analysis-of-surgical-interruption-of-the-enterohepatic-circulation-as-a-treatment-for-pediatric-cholestasis
#20
Kasper S Wang, Greg Tiao, Lee M Bass, Paula M Hertel, Douglas Mogul, Nanda Kerkar, Matthew Clifton, Colleen Azen, Laura Bull, Philip Rosenthal, Dylan Stewart, Riccardo Superina, Ronen Arnon, Molly Bozic, Mary L Brandt, Patrick A Dillon, Annie Fecteau, Kishore Iyer, Binita Kamath, Saul Karpen, Frederick Karrer, Kathleen M Loomes, Cara Mack, Peter Mattei, Alexander Miethke, Kyle Soltys, Yumirle P Turmelle, Karen West, Jessica Zagory, Cat Goodhue, Benjamin L Shneider
: To evaluate the efficacy of non-transplant surgery for pediatric cholestasis, 58 clinically diagnosed children, including 20 Alagille syndrome (ALGS), 16 Familial Intrahepatic Cholestasis-1 (FIC1), 18 Bile Salt Export Pump (BSEP) disease, & 4 others with low γ-glutamyl transpeptidase disease (levels <100 U/L), were identified across 14 Childhood Liver Disease Research Network (ChiLDReN) centers. Data were collected retrospectively from individuals who collectively had 39 partial external biliary diversions (PEBD), 11 ileal exclusions (IE) and 7 gallbladder-to-colon diversion (GBC)...
December 27, 2016: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
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