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Cholestasis

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https://www.readbyqxmd.com/read/28805978/obeticholic-acid-a-selective-farnesoid-x-receptor-agonist-regulates-bile-acid-homeostasis-in-sandwich-cultured-human-hepatocytes
#1
Yuanyuan Zhang, Jonathan P Jackson, Robert L St Claire, Kimberly Freeman, Kenneth R Brouwer, Jeffrey E Edwards
Farnesoid X receptor (FXR) is a master regulator of bile acid homeostasis through transcriptional regulation of genes involved in bile acid synthesis and cellular membrane transport. Impairment of bile acid efflux due to cholangiopathies results in chronic cholestasis leading to abnormal elevation of intrahepatic and systemic bile acid levels. Obeticholic acid (OCA) is a potent and selective FXR agonist that is 100-fold more potent than the endogenous ligand chenodeoxycholic acid (CDCA). The effects of OCA on genes involved in bile acid homeostasis were investigated using sandwich-cultured human hepatocytes...
August 2017: Pharmacology Research & Perspectives
https://www.readbyqxmd.com/read/28802084/the-parameters-that-help-to-differentiate-biliary-atresia-from-other-diseases
#2
Makoto Hayashida, Toshiharu Matsuura, Yoshiaki Kinoshita, Genshiro Esumi, Koichiro Yoshimaru, Yusuke Yanagi, Yoshiaki Takahashi, Tomoaki Taguchi
BACKGROUND: The diagnosis of biliary atresia (BA) is still challenging. The aim of this study was to analyze the clinical features and parameters that contribute to a diagnosis of BA. METHODS: From January 2000 to December 2013, 37 patients who underwent operative cholangiography were evaluated retrospectively. The patients were divided into two groups: a BA group, and a group with other cholestatic diseases (non-BA group). The demographic data and preoperative liver function test results were analyzed...
August 12, 2017: Pediatrics International: Official Journal of the Japan Pediatric Society
https://www.readbyqxmd.com/read/28799842/midterm-safety-and-efficacy-of-irreversible-electroporation-of-malignant-liver-tumors-located-close-to-major-portal-or-hepatic-veins
#3
Martina Distelmaier, Alexandra Barabasch, Philipp Heil, Nils A Kraemer, Peter Isfort, Sebastian Keil, Christiane K Kuhl, Philipp Bruners
Purpose To investigate the efficacy and safety of irreversible electroporation (IRE) in the treatment of hepatic tumors not suitable for thermal ablation (radiofrequency ablation [RFA] or microwave ablation). Materials and Methods This was an institutional review board-approved prospective study in 29 patients (15 men, 14 women; mean age, 63 years ± 12 [standard deviation]) with 43 primary (n = 8) or secondary (n = 35) malignant liver tumors who underwent computed tomography (CT)-guided IRE. All target tumors were located immediately adjacent to major hepatic veins, portal veins, or both; thus, they were not considered suitable for RFA or microwave ablation...
August 11, 2017: Radiology
https://www.readbyqxmd.com/read/28798896/pregnancy-outcomes-in-hiv-infected-women-experience-from-a-tertiary-care-center-in-india
#4
Vatsla Dadhwal, Aparna Sharma, Kavita Khoiwal, Dipika Deka, Plaboni Sarkar, P Vanamail
BACKGROUND AND OBJECTIVES: There is conflicting data on the effect of HIV infection as well as antiretroviral therapy (ART) on pregnancy outcome. The objectives of this study were to compare pregnancy outcomes in women with and without HIV infection, and to evaluate the effect of HAART on pregnancy in HIV-infected women. METHODS: This is a prospective case record analysis of 212 HIV-infected women delivering between 2002 and 2015, in a tertiary health care center in India...
2017: International Journal of MCH and AIDS
https://www.readbyqxmd.com/read/28796059/human-cholestatic-hepatitis-owing-to-polyoxyethylene-nonylphenol-ingestion-a-case-report
#5
Jihye Min, Joohye Han, Kyungju Kim, Samel Park, Sunhyo Lee, Jungrak Hong, Hyowook Gil, Hoyeon Song, Saeyong Hong
RATIONALE: The purpose of this study was to identify the chemical responsible for cholestatic hepatitis in a 55-year-old woman who ingested 1,1'-iminodi (octamethylene) diguanidinium triacetate (iminoctadine triacetate), a fungicide. The fungicide formulation was also composed of polyoxyethylene nonylphenol (NP-40) and methanol. PATIENT CONCERNS: Severe cholestatic hepatitis developed, which led to the patient's death on day 88 of hospitalization. Post-mortem necropsy of the liver showed focal hepatocyte necrosis involving mostly the mid-zone, along with intracytoplasmic and intracanalicular cholestasis...
August 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28792861/predictive-value-of-the-aspartate-aminotransferase-to-platelet-ratio-index-for-parenteral-nutrition-associated-cholestasis-in-premature-infants-with-intestinal-perforation
#6
Kannikar Vongbhavit, Mark A Underwood
BACKGROUND: Parenteral nutrition-associated cholestasis (PNAC) is a major cause of morbidity and mortality in premature infants. Early predictors of PNAC would have clinical value. We sought to evaluate risk factors and liver function testing as predictors of PNAC in premature infants with intestinal perforation. METHODS: Medical records of infants with a gestational age <34 weeks, birth weight <2000 g, and intestinal perforation due to either necrotizing enterocolitis or spontaneous intestinal perforation were reviewed...
August 1, 2017: JPEN. Journal of Parenteral and Enteral Nutrition
https://www.readbyqxmd.com/read/28792647/ultrashort-bowel-is-an-independent-risk-factor-for-liver-fibrosis-in-adults-with-home-parenteral-nutrition
#7
Dominique Cazals-Hatem, Lore Billiauws, Pierre-Emmanuel Rautou, Vanessa Bondjemah, Nicolas Pote, Olivier Corcos, Valérie Paradis, Francisca Joly
BACKGROUND & AIMS: Intestinal failure-associated liver disease is rare in adults and risk factors are unclear. The aim of this study was to determine risk factors of liver fibrosis in adults receiving home parenteral nutrition (HPN) for intestinal failure and its impact on survival. METHODS: We retrospectively analyzed patients with irreversible intestinal failure who underwent a liver biopsy between 2000 and 2013. Significant liver fibrosis was defined as ≥F2 according to NASH-CRN score...
August 9, 2017: Liver International: Official Journal of the International Association for the Study of the Liver
https://www.readbyqxmd.com/read/28792089/plasma-exchange-for-the-management-of-refractory-pruritus-of-cholestasis-a-report-of-three-cases-and-review-of-literature
#8
Rati Chkheidze, Ranjit Joseph, James Burner, Karen Matevosyan
BACKGROUND: Intractable pruritus of cholestasis leads to significant morbidity. Therapeutic plasma exchange (TPE) has been shown to be an effective alternative in the setting of refractory pruritus associated with cholestatic liver disease based on several individual reports. Due to rarity of this approach to intractable pruritus, the literature is sparse and therefore TPE, as a treatment for refractory pruritus is currently not in the apheresis guidelines. We present three additional patients with severe intractable pruritus of cholestasis successfully treated with plasma exchange to add to the mounting literature showing this as an effective and safe adjunctive therapy...
August 9, 2017: Journal of Clinical Apheresis
https://www.readbyqxmd.com/read/28789951/hepatoprotective-activities-of-rosmarinic-acid-against-extrahepatic-cholestasis-in-rats
#9
Shih-Yi Lin, Ya-Yu Wang, Wen-Ying Chen, Su-Lan Liao, Su-Tze Chou, Ching-Ping Yang, Chun-Jung Chen
Though rosmarinic acid possesses nutritional, pharmaceutical, and toxic properties and shows therapeutic potential on liver diseases, its therapeutic effects against cholestatic liver diseases have not been proven. Using an extrahepatic cholestasis rat model by bile-duct ligation (BDL), daily oral administration of rosmarinic acid showed improvement effects on liver histology, serum biochemicals, ductular reaction, oxidative stress, inflammation, and fibrosis. Rosmarinic acid alleviated BDL-induced transforming growth factor beta-1 (TGF-β1) production and hepatic collagen deposition, and the anti-fibrotic effects were accompanied by reductions in matrix-producing cells and Smad2/3...
August 5, 2017: Food and Chemical Toxicology
https://www.readbyqxmd.com/read/28780310/outcome-of-neonates-with-gastroschisis-at-different-gestational-ages-using-a-national-database
#10
Ruby Gupta, Erwin T Cabacungan
BACKGROUND/PURPOSE: The optimal time for delivery of neonates with a prenatal diagnosis of gastroschisis (GS) is controversial. We compared the outcomes for GS at three different gestational ages (GAs), 33-34 weeks, 35-36 weeks, and ≥ 37 weeks. METHODS: We analyze hospital discharge data of neonates with GS using the 2006, 2009 and 2012 Healthcare Cost and Utilization Project Kids' Inpatient Database (HCUPKIDS). Multivariable analysis was used to compare the association between GS outcomes and the three GAs...
July 22, 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/28763485/cxcr2-signaling-and-the-microbiome-suppress-inflammation-bile-duct-injury-and-the-phenotype-of-experimental-biliary-atresia
#11
Junbae Jee, Reena Mourya, Pranavkumar Shivakumar, Lin Fei, Michael Wagner, Jorge A Bezerra
Biliary atresia is progressive fibro-inflammatory cholangiopathy of young children. Central to pathogenic mechanisms of injury is the tissue targeting by the innate and adaptive immune cells. Among these cells, neutrophils and the IL-8/Cxcl-8 signaling via its Cxcr2 receptor have been linked to bile duct injury. Here, we aimed to investigate whether the intestinal microbiome modulates Cxcr2-dependent bile duct injury and obstruction. Adult wild-type (WT) and Cxcr2-/- mice were fed a diet supplemented with sulfamethoxazole/trimethoprim (SMZ/TMP) during pregnancy and lactation, and their pups were injected intraperitoneally with rhesus rotavirus (RRV) within 24 hours of life to induce experimental biliary atresia...
2017: PloS One
https://www.readbyqxmd.com/read/28759700/interventions-for-treating-intrahepatic-cholestasis-in-people-with-sickle-cell-disease
#12
REVIEW
Arturo J Martí-Carvajal, Cristina Elena Martí-Amarista
BACKGROUND: Sickle cell disease is the most common hemoglobinopathy occurring worldwide and sickle cell intrahepatic cholestasis is a complication long recognized in this population. Cholestatic liver diseases are characterized by impaired formation or excretion (or both) of bile from the liver. There is a need to assess the clinical benefits and harms of the interventions used to treat intrahepatic cholestasis in people with sickle cell disease. This is an update of a previously published Cochrane Review...
July 31, 2017: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/28759390/cholestasis-and-hepatic-iron-deposition-in-an-infant-with-complex-glycerol-kinase-deficiency
#13
Diana Montoya-Williams, Meredith Mowitz
We present a 6-week-old male infant with persistent hyperbilirubinemia, hypertriglyceridemia, elevated creatine kinase levels, and transaminitis since the second week of life. When he developed hyperkalemia, clinical suspicion was raised for adrenal insufficiency despite hemodynamic stability. A full endocrine workup revealed nearly absent adrenocorticotropic hormone. Coupled with his persistent hypertriglyceridemia (peak of 811 mg/dL) and elevated creatine kinase levels (>20 000 U/L), his corticotropin level lead to a clinical diagnosis of complex glycerol kinase deficiency (GKD), also known as Xp21 deletion syndrome...
July 2017: Pediatrics
https://www.readbyqxmd.com/read/28757911/baicalin-ameliorates-experimental-liver-cholestasis-in-mice-by-modulation-of-oxidative-stress-inflammation-and-nrf2-transcription-factor
#14
Kezhen Shen, Xiaowen Feng, Hao Pan, Feng Zhang, Haiyang Xie, Shusen Zheng
Experimental cholestatic liver fibrosis was performed by bile duct ligation (BDL) in mice, and significant liver injury was observed in 15 days. Administration of baicalin in mice significantly ameliorates liver fibrosis. Experimental cholestatic liver fibrosis was associated with induced gene expression of fibrotic markers such as collagen I, fibronectin, alpha smooth muscle actin (SMA), and connective tissue growth factor (CTGF); increased inflammatory cytokines (TNFα, MIP1α, IL1β, and MIP2); increased oxidative stress and reactive oxygen species- (ROS-) inducing enzymes (NOX2 and iNOS); dysfunctional mitochondrial electron chain complexes; and apoptotic/necrotic cell death markers (DNA fragmentation, caspase 3 activity, and PARP activity)...
2017: Oxidative Medicine and Cellular Longevity
https://www.readbyqxmd.com/read/28757171/genetic-determinants-of-cholangiopathies-molecular-and-systems-genetics
#15
REVIEW
Matthias C Reichert, Rabea A Hall, Marcin Krawczyk, Frank Lammert
Familial cholangiopathies are rare but potentially severe diseases. Their spectrum ranges from fairly benign conditions as, for example, benign recurrent intrahepatic cholestasis to low-phospholipid associated cholelithiasis and progressive familial intrahepatic cholestasis (PFIC). Many cholangiopathies such as primary biliary cholangitis (PBC) or primary sclerosing cholangitis (PSC) affect first the bile ducts ("ascending pathophysiology") but others, such as PFIC, start upstream in hepatocytes and cause progressive damage "descending" down the biliary tree and leading to end-stage liver disease...
July 27, 2017: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/28754450/pathogenesis-of-biliary-fibrosis
#16
REVIEW
Massimo Pinzani, Tu Vinh Luong
Chronic cholestatic liver diseases such as primary biliary cholangitis (PBC) and primary sclerosing cholangitis (PSC) are associated with active hepatic fibrogenesis, and, ultimately, to the development of cirrhosis. However, the precise relationship between cholestasis, in its broad meaning, and liver tissue fibrosis is still poorly defined. Fibrogenesis is currently viewed as a dynamic process that appears strictly related to the extent and duration of parenchymal injury. This relationship is clearly evident in the presence of reiterative hepatocellular necrosis due to viral infection or alcohol abuse...
July 25, 2017: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/28751522/liver-allograft-biopsies-with-histological-cholestasis-a-clinicopathological-study-of-254-cases-from-a-single-centre
#17
Regina Cheuk-Lam Lo, Kristy Kwan-Shuen Chan, Kenneth Siu-Ho Chok, Irene Oi-Lin Ng
AIMS: Liver allograft biopsy is important in the management of liver transplant (LT) recipients. Cholestasis is an indicator of liver dysfunction, and histological evidence of cholestasis can be observed in a wide range of pathological entities in the post-LT setting. In this study, we describe the clinicopathological features and significance of liver allograft biopsies with histological cholestasis over 11 years in our centre. METHODS: Liver allograft biopsies performed in Queen Mary Hospital, Hong Kong from 2004 to 2014 showing histological cholestasis were retrieved from the pathology archive...
July 27, 2017: Journal of Clinical Pathology
https://www.readbyqxmd.com/read/28751425/melatonin-inhibits-hypothalamic-gonandotropin-releasing-hormone-release-and-reduces-biliary-hyperplasia-and-fibrosis-in-cholestatic-rats
#18
Matthew McMillin, Sharon DeMorrow, Shannon Glaser, Julie Venter, Konstantina Kyritsi, Tianhao Zhou, Stephanie Grant, Thao Giang, John F Greene, Nan Wu, Brandi Jefferson, Fanyin Meng, Gianfranco Alpini
Melatonin is a hormone produced by the pineal gland with increased circulating levels shown to inhibit biliary hyperplasia and fibrosis during cholestasis. Melatonin also has the capability to suppress the release of hypothalamic gonadotropin releasing hormone (GnRH), a hormone that promotes cholangiocyte proliferation when serum levels are elevated. However, the interplay and contribution of neural melatonin and GnRH to cholangiocyte proliferation and fibrosis in bile duct-ligated (BDL) rats has not been investigated...
July 27, 2017: American Journal of Physiology. Gastrointestinal and Liver Physiology
https://www.readbyqxmd.com/read/28748913/d-chiro-inositol-effectively-attenuates-cholestasis-in-bile-duct-ligated-rats-by-improving-bile-acid-secretion-and-attenuating-oxidative-stress
#19
Shuang-Shuang Zhao, Na-Ren Li, Wu-Li Zhao, Hong Liu, Mao-Xu Ge, Yi-Xuan Zhang, Long-Yin Zhao, Xue-Fu You, Hong-Wei He, Rong-Guang Shao
Cholestatic liver diseases are important causes of liver cirrhosis and liver transplantation, but few drugs are available for treatment. D-chiro-inositol (DCI), an isomer of inositol found in many Leguminosae plants and in animal viscera, is used clinically for the treatment of polycystic ovary syndrome (PCOS) and diabetes mellitus. In this study, we investigated whether DCI exerted an anti-cholestatic effect and its underlying mechanisms. A cholestatic rat model was established via bile duct ligation (BDL)...
July 27, 2017: Acta Pharmacologica Sinica
https://www.readbyqxmd.com/read/28745070/management-options-for-cholestatic-liver-disease-in-children
#20
Andrea Catzola, Pietro Vajro
Due to a peculiar age-dependent increased susceptibility, neonatal cholestasis affects the liver of approximately 1 in every 2500 term infants. A high index of suspicion is the key to an early diagnosis, and to implement timely, often life-saving treatments. Even when specific treatment is not available or curative, prompt medical management and optimization of nutrition are of paramount importance to survival and avoidance of complications. Areas covered: The present article will prominently focus on a series of newer diagnostic and therapeutic options of cholestasis in neonates and infants blended with consolidated established paradigms...
July 28, 2017: Expert Review of Gastroenterology & Hepatology
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