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https://www.readbyqxmd.com/read/29880041/urinary-exosomal-expression-of-activator-of-g-protein-signaling-3-in-polycystic-kidney-disease
#1
Krishna C Keri, Kevin R Regner, Aaron T Dall, Frank Park
OBJECTIVE: PKD is a genetic disease that is characterized by abnormally proliferative epithelial cells in the kidney and liver. Urinary exosomes have been previously examined as a source of unique proteins that may be used to diagnose and monitor the progression of PKD. Previous studies by our group have shown that AGS3, which is a receptor-independent regulator G-proteins, was markedly upregulated in RTECs during kidney injury including PKD. In this study, our goal was to determine whether AGS3 could be measured in exosomes using animals and humans with PKD...
June 7, 2018: BMC Research Notes
https://www.readbyqxmd.com/read/29873585/nash-in-nondiabetic-endocrine-disorders
#2
Timothy Wang, Wei Yang, Sidika Karakas, Souvik Sarkar
Nonalcoholic fatty liver disease (NAFLD) encompasses a spectrum of disease, including hepatic steatosis, inflammation, and fibrosis. NAFLD carries the risk of progression to cirrhosis with its associated complications and hepatocellular carcinoma. It is now the most common liver disease in the Western world and its prevalence is increasing. While the association between NAFLD and type 2 diabetes has been well documented, there is significantly less understanding of the pathophysiology and progression of NAFLD in patients with other endocrine disorders affecting metabolism in various ways...
June 6, 2018: Metabolic Syndrome and related Disorders
https://www.readbyqxmd.com/read/29862868/inhibition-of-mast-cell-degranulation-with-cromolyn-sodium-exhibits-organ-specific-effects-in-polycystic-kidney-pck-rats
#3
Lu Jiang, Pingping Fang, Seth Septer, Udayan Apte, Michele T Pritchard
Autosomal recessive polycystic kidney disease (ARPKD) is a monogenic disease characterized by development of hepatorenal cysts, pericystic fibrosis, and inflammation. Previous studies show that mast cell (MC) mediators such as histamine induce proliferation of cholangiocytes. We observed robust MC accumulation around liver cysts, but not kidney cysts, in polycystic kidney (PCK) rats (an animal model of ARPKD). Therefore, we hypothesized that MCs contribute to hepatic cyst growth in ARPKD. To test this hypothesis, we treated PCK rats with 1 of 2 different MC stabilizers, cromolyn sodium (CS) or ketotifen, or saline...
January 1, 2018: International Journal of Toxicology
https://www.readbyqxmd.com/read/29859544/do-inositol-supplements-enhance-phosphatidylinositol-supply-and-thus-support-endoplasmic-reticulum-function
#4
Robert H Michell
This review attempts to explain why consuming extra myoinositol (Ins), an essential component of membrane phospholipids, is often beneficial for patients with conditions characterised by insulin resistance, non-alcoholic fatty liver disease and endoplasmic reticulum (ER) stress. For decades we assumed that most human diets provide an adequate Ins supply, but newer evidence suggests that increasing Ins intake ameliorates several disorders, including polycystic ovary syndrome, gestational diabetes, metabolic syndrome, poor sperm development and retinopathy of prematurity...
June 3, 2018: British Journal of Nutrition
https://www.readbyqxmd.com/read/29850365/combination-of-modified-bentall-procedure-and-orthotopic-liver-transplantation
#5
Philipp C Seppelt, Rebecca von Haken, Jens Werner, Klaus Kallenbach
Indication for combined cardiac surgery and orthotopic liver transplantation is rare and patients are at high risks. Individual surgical strategy must be developed since a general standard of such procedure does not exist. We report the case of a 45-year-old woman who underwent simultaneously modified Bentall procedure and orthotopic liver transplantation. Underlying diseases were end-stage polycystic liver, aneurysm of the ascending aorta, and severe aortic regurgitation. To avoid prolonged bypass times, both teams worked simultaneously...
2018: Case Reports in Transplantation
https://www.readbyqxmd.com/read/29804940/hyperhomocysteinemia-in-polycystic-ovary-syndrome-decreased-betaine-homocysteine-methyltransferase-and-cystathionine-%C3%AE-synthase-mediated-homocysteine-metabolism
#6
Da Li, Hong-Xiang Liu, Yuan-Yuan Fang, Jia-Ning Huo, Qi-Jun Wu, Tian-Ren Wang, Yi-Ming Zhou, Xiu-Xia Wang, Xiao-Xin Ma
RESEARCH QUESTION: What are the metabolic characteristics of homocysteine in polycystic ovary syndrome (PCOS)? DESIGN: Homocysteine concentrations were determined in serum samples from non-obese and obese control subjects and PCOS patients. Homocysteine metabolism was studied in a rat model of PCOS established using dehydroepiandrosterone (DHEA) or DHEA in combination with a high-fat diet (HFD). RESULTS: It was shown that (i) serum homocysteine concentrations were greater in PCOS patients than in control subjects in the obese group (P < 0...
May 22, 2018: Reproductive Biomedicine Online
https://www.readbyqxmd.com/read/29797773/baseline-characteristics-of-the-autosomal-dominant-polycystic-kidney-disease-subcohort-of-the-korean-cohort-study-for-outcomes-in-patients-with-chronic-kidney-disease-know-ckd
#7
Hyunsuk Kim, Junga Koh, Sue K Park, Kook Hwan Oh, Yeong Hoon Kim, Yaeni Kim, Curie Ahn, Yun Kyu Oh
AIM: The aim of this study was to describe the baseline characteristics of autosomal dominant polycystic kidney disease (ADPKD) in a cohort of Korean patients with chronic kidney disease (CKD). METHODS: From April 2011 to February 2016, patients with CKD stage 1 to 5 (pre-dialysis) were enrolled as an ADPKD subcohort of the KoreaN Cohort Study for Outcomes in Patients With Chronic Kidney Disease. Baseline characteristics, the correlation of kidney and liver volume and kidney function, and the factors associated with kidney function were analyzed...
May 24, 2018: Nephrology
https://www.readbyqxmd.com/read/29779024/a-randomized-clinical-trial-of-metformin-to-treat-autosomal-dominant-polycystic-kidney-disease
#8
Stephen L Seliger, Kaleab Z Abebe, Kenneth R Hallows, Dana C Miskulin, Ronald D Perrone, Terry Watnick, Kyongtae Tae Bae
BACKGROUND: Metformin inhibits cyclic AMP generation and activates AMP-activated protein kinase (AMPK), which inhibits the cystic fibrosis transmembrane conductance regulator and Mammalian Target of Rapamycin pathways. Together these effects may reduce cyst growth in autosomal dominant polycystic kidney disease (ADPKD). METHODS: A phase II, double-blinded randomized placebo-controlled trial of 26 months duration. Participants will include nondiabetic adults (n = 96) aged 18-60 years, with an estimated glomerular filtration rate (eGFR) ≥50 mL/min/1...
May 18, 2018: American Journal of Nephrology
https://www.readbyqxmd.com/read/29766146/the-effect-of-resveratrol-on-oxidative-stress-in-the-liver-and-serum-of-a-rat-model-of-polycystic-ovary-syndrome-an-experimental-study
#9
Mahnaz Ghowsi, Homayoun Khazali, Sajjad Sisakhtnezhad
Background: Studies of oxidative status in polycystic ovarian syndrome (PCOS) patients are limited with inconsistent results. The effects of resveratrol as a natural antioxidant on oxidative status in PCOS aren't clear. Objective: This study evaluated effects of resveratrol on oxidative stress in the liver and serum of the PCOS rats. Materials and Methods: Fifteen female Wistar rats (3 wk old) were divided into 3 groups (n=5/each e): Control group, PCO-Control group, and PCO-Resveratrol group...
March 2018: International Journal of Reproductive Biomedicine (Yazd, Iran)
https://www.readbyqxmd.com/read/29747678/a-potential-link-between-polycystic-ovary-syndrome-and-non-alcoholic-fatty-liver-disease-an-update-meta-analysis
#10
Jia Wu, Xin-Yu Yao, Ru-Xia Shi, Su-Fen Liu, Xiao-Yong Wang
BACKGROUND: Epidemiological literature regarding the effect of polycystic ovary syndrome (PCOS) as a risk factor for non-alcoholic fatty liver disease (NAFLD) remains inconsistent. Furthermore, it remains debatable whether NAFLD is associated with PCOS as a consequence of shared risk factors or whether PCOS contributes to NAFLD in an independent fashion. Therefore, this meta-analysis was conducted. METHODS: This meta-analysis was conducted in accordance with the guidelines of the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA)...
May 10, 2018: Reproductive Health
https://www.readbyqxmd.com/read/29746821/lansoprazole-reduces-renal-cyst-in-polycystic-kidney-disease-via-inhibition-of-cell-proliferation-and-fluid-secretion
#11
Jiriporn Nantavishit, Varanuj Chatsudthipong, Sunhapas Soodvilai
Renal cyst development and expansion in autosomal dominant polycystic kidney disease (ADPKD) is mediated by abnormal cyst-ling cell proliferation and fluid accumulation. Liver X receptor (LXR)-activating ligands suppresses renal cyst enlargement by modulation of cysticfibrosis transmembrane conductance regulator (CFTR)-mediated fluid accumulation. Lansoprazole has been reported as agonist of LXR, and shows an anti-proliferative effect in cancer cells. Here, lansoprazole's pharmacological effect and underlying mechanism on renal cyst development and expansion in in vitro; human ADPKD cyst-lining epithelial cell line and Type I Mardin Darby Canine Kidney (MDCK) cells, and in vivo models was investigated...
May 7, 2018: Biochemical Pharmacology
https://www.readbyqxmd.com/read/29725371/analyses-of-risk-factors-for-polycystic-ovary-syndrome-complicated-with-non-alcoholic-fatty-liver-disease
#12
Jianhai Zhang, Jian Hu, Chunxia Zhang, Yanni Jiao, Xiang Kong, Wei Wang
The risk factors related to polycystic ovary syndrome (PCOS) patients complicated with non-alcoholic fatty liver disease (NAFLD) were investigated. A total of 188 PCOS patients treated in Shengli Oilfield Central Hospital (Dongying, China) from February 2014 to February 2015 were retrospectively analyzed as PCOS group, and PCOS group was further divided into NAFLD group and non-NAFLD (N-NAFLD) group according to the liver B ultrasound. In the same time-period, 65 healthy people were selected as normal control group...
May 2018: Experimental and Therapeutic Medicine
https://www.readbyqxmd.com/read/29719598/hyperandrogenism-and-insulin-resistance-contribute-to-hepatic-steatosis-and-inflammation-in-female-rat-liver
#13
Yuehui Zhang, Fanci Meng, Xiaoyan Sun, Xue Sun, Min Hu, Peng Cui, Edvin Vestin, Xin Li, Wei Li, Xiao-Ke Wu, John-Olov Jansson, Linus R Shao, Håkan Billig
Women with polycystic ovary syndrome (PCOS) are at high risk for nonalcoholic fatty liver disease (NAFLD). While insulin resistance is a common trait for both PCOS and NAFLD, hyperandrogenism is also considered to be a key factor contributing to PCOS, and the molecular mechanisms behind the interactions between insulin resistance and hyperandrogenism in the female liver remain largely unexplored. Using chronic treatment with insulin and/or human chorionic gonadotropin (hCG), we showed that all female rats with different treatments induced imbalance between de novo lipogenesis and mitochondrial β-oxidation via the Pparα/β-Srebp1/2-Acc1 axis, resulting in varying degrees of hepatic steatosis...
April 6, 2018: Oncotarget
https://www.readbyqxmd.com/read/29707880/risk-factors-for-adverse-maternal-and-perinatal-outcomes-in-women-with-preeclampsia-analysis-of-1396-cases
#14
Xun Li, Weishe Zhang, Jianhua Lin, Huai Liu, Zujing Yang, Yincheng Teng, Si Duan, Xinxiu Lin, Yingming Xie, Yuanqiu Li, Liangqun Xie, Qiaozhen Peng, Lu Xia
Preeclampsia is a major cause of adverse maternal and perinatal outcomes, but how to identify women and fetuses at increased risk for later adverse events is a challenge. This study aimed to investigate the risk factors for adverse maternal and perinatal outcomes in women with preeclampsia. Data from 1396 women with preeclampsia were retrospectively collected and analyzed. Eighteen candidate risk factors and 12 adverse outcomes were investigated. The following factors were found to be significantly associated with at least one adverse outcome: maternal age 35 years or older, multiple birth, the usage of assisted reproductive technology, living in a rural area, history of pregnancy-induced hypertension, male fetus, multigravida, or having polycystic ovary syndrome, hemolysis, elevated liver enzymes, and low platelet count syndrome, intrahepatic cholestasis of pregnancy, cardiovascular disease, gestational diabetes mellitus, systemic lupus erythematosus, thyroid disease, or liver disease...
April 30, 2018: Journal of Clinical Hypertension
https://www.readbyqxmd.com/read/29706351/monoallelic-mutations-to-dnajb11-cause-atypical-autosomal-dominant-polycystic-kidney-disease
#15
Emilie Cornec-Le Gall, Rory J Olson, Whitney Besse, Christina M Heyer, Vladimir G Gainullin, Jessica M Smith, Marie-Pierre Audrézet, Katharina Hopp, Binu Porath, Beili Shi, Saurabh Baheti, Sarah R Senum, Jennifer Arroyo, Charles D Madsen, Claude Férec, Dominique Joly, François Jouret, Oussamah Fikri-Benbrahim, Christophe Charasse, Jean-Marie Coulibaly, Alan S Yu, Korosh Khalili, York Pei, Stefan Somlo, Yannick Le Meur, Vicente E Torres, Peter C Harris
Autosomal-dominant polycystic kidney disease (ADPKD) is characterized by the progressive development of kidney cysts, often resulting in end-stage renal disease (ESRD). This disorder is genetically heterogeneous with ∼7% of families genetically unresolved. We performed whole-exome sequencing (WES) in two multiplex ADPKD-like pedigrees, and we analyzed a further 591 genetically unresolved, phenotypically similar families by targeted next-generation sequencing of 65 candidate genes. WES identified a DNAJB11 missense variant (p...
May 3, 2018: American Journal of Human Genetics
https://www.readbyqxmd.com/read/29678444/biliary-epithelium-a-neuroendocrine-compartment-in-cholestatic-liver-disease
#16
REVIEW
Laurent Ehrlich, Marinda Scrushy, Fanyin Meng, Terry C Lairmore, Gianfranco Alpini, Shannon Glaser
Hepatic fibrosis is characterized by abnormal accumulation of extracellular matrix (ECM) that can lead to ductopenia, cirrhosis, and even malignant transformation. In this review, we examine cholestatic liver diseases characterized by extensive biliary fibrosis such as primary sclerosing cholangitis (PSC), primary biliary cholangitis (PBC), polycystic liver disease (PLD), and MDR2-/- and BDL mouse models. Following biliary injury, cholangiocytes, the epithelial cells that line the bile ducts, become reactive and adopt a neuroendocrine phenotype in which they secrete and respond to neurohormones and neuropeptides in an autocrine and paracrine fashion...
April 17, 2018: Clinics and Research in Hepatology and Gastroenterology
https://www.readbyqxmd.com/read/29643536/-genetic-diagnosis-of-caroli-syndrome-with-autosomal-recessive-polycystic-kidney-disease-a-case-report-and-literature-review
#17
X Y Yang, L P Zhu, X Q Liu, C Y Zhang, Y Yao, Y Wu
This case report is about one genetically specified diagnosed infant case of Caroli syndrome with autosomal recessive polycystic kidney disease (ARPKD) in China. The patient in this case report was an eight-month infant boy with an atypical onset and the main clinical manifestation was non-symptomatic enlargement of the liver and kidneys. The imaging study demonstrated a diffused cystic dilatation of intrahepatic bile ducts as well as polycystic changes in bilateral kidneys. The basic blood biochemical tests indicated a normal hepatorenal function...
April 18, 2018: Beijing da Xue Xue Bao. Yi Xue Ban, Journal of Peking University. Health Sciences
https://www.readbyqxmd.com/read/29622386/long-term-outcome-of-transjugular-intrahepatic-portosystemic-shunt-for-portal-hypertension-in-autosomal-recessive-polycystic-kidney-disease
#18
Sarah Verbeeck, Djalila Mekhali, David Cassiman, Geert Maleux, Peter Witters
BACKGROUND: Autosomal recessive polycystic kidney disease (ARPKD) with congenital hepatic fibrosis (CHF) causes portal hypertension and its complications. A transjugular intrahepatic portosystemic shunt (TIPSS) could serve as a symptomatic treatment for portal hypertension-related symptoms in these children. AIMS: To study the effect of TIPSS on portal hypertension, liver and kidney function and the long term complications. MATERIALS AND METHODS: We report on 5 children with CHF treated with a TIPSS to manage severe portal hypertension related symptoms...
March 15, 2018: Digestive and Liver Disease
https://www.readbyqxmd.com/read/29618028/hepatic-production-of-fibroblast-growth-factor-23-in-autosomal-dominant-polycystic-kidney-disease
#19
Frank Bienaimé, Ariane Ambolet, Béatrice Aussilhou, François Brazier, Marie Fouchard, Amandine Viau, Pauline Barre, Anne-Marie Tissier, Jean-Michel Correas, Valérie Paradis, Fabiola Terzi, Gérard Friedlander, Bertrand Knebelmann, Dominique Joly, Dominique Prié
Context: the bone-derived hormone fibroblast growth factor 23 (FGF23) controls phosphate homeostasis and urinary phosphate excretion. FGF23 plasma levels increase at the early step of renal insufficiency to prevent hyperphosphatemia. Recent evidence suggests that this increase has off-target effects on cardiac and immune cells that compromises patients' health. Subjects with autosomal dominant polycystic kidney disease (ADPKD) have been reported to display higher FGF23 concentrations than non-ADPKD patients with similar renal function...
March 29, 2018: Journal of Clinical Endocrinology and Metabolism
https://www.readbyqxmd.com/read/29606941/a-surgically-resected-pancreatic-schwannoma-with-obstructive-jaundice-with-special-reference-to-differential-diagnosis-from-other-cystic-lesions-in-the-pancreas
#20
Takayoshi Watanabe, Kenichiro Araki, Norihiro Ishii, Takamichi Igarashi, Akira Watanabe, Norio Kubo, Hiroyuki Kuwano, Ken Shirabe
Pancreatic schwannomas are uncommon. About 60% of pancreatic schwannomas develop cystic lesions, and the differential diagnosis from other cystic pancreatic tumors is difficult. A 43-year-old man presented for evaluation of liver dysfunction detected during a medical checkup. Blood testing detected obstructive jaundice. A computed tomography scan revealed a well-defined polycystic tumor of about 5 cm at the pancreatic head. We performed surgical resection to treat the patient's symptoms and facilitate long-term management...
January 2018: Case Reports in Gastroenterology
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