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Polycystic liver

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https://www.readbyqxmd.com/read/28339345/alanine-aminotransferase-is-a-marker-of-lipotoxicity-consequences-and-hyperandrogenemia-in-women-with-polycystic-ovary-syndrome
#1
Matea Belan, Chloé Pelletier, Jean-Patrice Baillargeon
BACKGROUND: Several studies have reported higher levels of Alanine aminotransferase (ALT) in women with polycystic ovary syndrome (PCOS) compared with control subjects. Plasma ALT levels are considered a marker of hepatic lipotoxicity because of their significant associations with different hepatic metabolic dysfunctions, such as hepatic steatosis and hepatic insulin resistance. METHODS: Retrospective chart review aiming to assess, in PCOS women, the relationship between ALT levels and measures of lipotoxicity consequences that are available clinically, both during fasting and using the oral glucose tolerance test...
April 2017: Metabolic Syndrome and related Disorders
https://www.readbyqxmd.com/read/28319674/assessment-of-circulating-betatrophin-concentrations-in-lean-glucose-tolerant-women-with-polycystic-ovary-syndrome
#2
Onur Erol, Mustafa Kemal Özel, Hamit Yaşar Ellidağ, Tayfun Toptaş, Aysel Uysal Derbent, Necat Yılmaz
The aims of the current study were to investigate the betatrophin levels in lean glucose-tolerant women with polycystic ovary syndrome (PCOS), and to explore the relationships between these levels and antropometric, hormonal and metabolic parameters. The study population consisted of 50 lean (body mass index [BMI] < 25 kg/m(2)) women diagnosed with PCOS using the Rotterdam criteria, and 60 age- and BMI-matched healthy controls without any features of clinical or biochemical hyperandrogenism. Before recruitment, glucose tolerance was evaluated in all of the subjects using the 2-h 75 g oral glucose-tolerance test, and only those exhibiting normal glucose tolerance were enrolled...
March 20, 2017: Journal of Obstetrics and Gynaecology: the Journal of the Institute of Obstetrics and Gynaecology
https://www.readbyqxmd.com/read/28317394/an-update-on-the-pathophysiology-and-management-of-polycystic-liver-disease
#3
May Yw Wong, Geoffrey W McCaughan, Simone I Strasser
Polycystic liver disease (PLD) is characterized by the presence of multiple cholangiocyte-derived hepatic cysts that progressively replace liver tissue. They are classified as an inherited ciliopathy /cholangiopathy as pathology exists at the level of the primary cilia of cholangiocytes. Aberrant expression of the proteins in primary cilia can impair their structures and functions, thereby promoting cystogenesis. Areas covered: This review begins by looking at the epidemiology of PLD and its natural history...
March 18, 2017: Expert Review of Gastroenterology & Hepatology
https://www.readbyqxmd.com/read/28306734/simultaneous-liver-kidney-transplantation-and-bilateral-nephrectomy-through-a-midline-is-feasible-and-safe-in-polycystic-disease
#4
Ina Jochmans, Diethard Monbaliu, Laurens J Ceulemans, Jacques Pirenne, Jiri Fronek
In Eurotransplant, 50% of simultaneous liver kidney transplantations (SLK) are performed for polycystic disease. Classically, liver and kidney are transplanted in two steps: liver through a subcostal incision, kidney through a separate oblique incision. Liver and kidney volume can make this 'two-step' procedure challenging, especially if simultaneous native nephrectomy is indicated. A 'one-step' SLK through a xiphopubic laparotomy might be a safe alternative, facilitating mobilization of the voluminous polycystic liver and native nephrectomy whilst offering access to iliac fossae for kidney transplantation...
2017: PloS One
https://www.readbyqxmd.com/read/28303733/bi-parametric-magnetic-resonance-imaging-applied-to-obstetrics
#5
Lucia Manganaro, Valeria Vinci, Antonella Giancotti, Sandro Gerli, Denis A Cozzi, Teresa Pusiol, Michele Scialpi, Luca Roncati
Foetal magnetic resonance imaging (MRI) plays an increasingly important role in the diagnosis of foetal abnormalities. Over the years, we have successfully applied bi-parametric MRI (bp-MRI) to the following obstetric conditions: (1) neurologic vascular diseases; (2) assessment of lung parenchyma maturation; (3) renal pathologies, such as polycystic kidney, suspected renal infarction, unilateral or bilateral renal agenesis; (4) placental pathologies, as twin-twin transfusion syndrome or placenta accreta; (5) benignant and malignant congenital tumours or cysts of the liver, such as haemangioendothelioma, hepatoblastoma or metastatic neuroblastoma, of the kidney (e...
March 17, 2017: Journal of Obstetrics and Gynaecology: the Journal of the Institute of Obstetrics and Gynaecology
https://www.readbyqxmd.com/read/28301536/prognostic-impact-of-peritonitis-in-hemodialysis-patients-a-national-wide-longitudinal-study-in-taiwan
#6
Yueh-An Lu, Kun-Hua Tu, Cheng-Chia Lee, Patricia W Wu, Chee-Jen Chang, Ya-Chung Tian, Chih-Wei Yang, Pao-Hsien Chu
BACKGROUND: Peritonitis has been independently associated with increased morbidity and mortality in peritoneal dialysis patients. However, there are few reports on peritonitis in hemodialysis patients. We aim at investigating both the risk profiles and prognostic impact of peritonitis in hemodialysis patients. METHODS: This nation-wide longitudinal study uses claims data obtained from the Taiwan National Health Insurance Research Database. A total of 80,733 incident hemodialysis patients of age ≥ 20 years without a history of peritonitis were identified between January 1, 1998 and December 31, 2009...
2017: PloS One
https://www.readbyqxmd.com/read/28296980/recent-progress-of-the-aregpkd-registry-study-on-autosomal-recessive-polycystic-kidney-disease
#7
Kathrin Ebner, Franz Schaefer, Max Christoph Liebau
Autosomal recessive polycystic kidney disease (ARPKD) is a rare monogenic disease with a severe phenotype often presenting prenatally or in early childhood. With its obligate renal and hepatic involvement, ARPKD is one of the most important indications for liver and/or kidney transplantation in childhood. Marked phenotypic variability is observed, the genetic basis of which is largely unknown. Treatment is symptomatic and largely empiric as evidence-based guidelines are lacking. Therapeutic initiatives for ARPKD face the problem of highly variable cohorts and lack of clinical or biochemical risk markers without clear-cut clinical end points...
2017: Frontiers in Pediatrics
https://www.readbyqxmd.com/read/28296808/waitlisted-candidates-with-polycystic-liver-disease-are-more-likely-to-be-transplanted-than-those-with-chronic-liver-failure
#8
Sahil D Doshi, Therese Bittermann, Thomas D Schiano, David Seth Goldberg
BACKGROUND: Polycystic liver disease (PCLD) is characterized by cystic replacement of the hepatic parenchyma, leading to hepatic dysfunction, portal hypertension, and hepatomegaly. Patients with liver dysfunction and/or symptomatic disease are eligible for liver transplantation. However, little is known about these patients' waitlist outcomes relative to others with chronic liver disease. METHODS: We used OPTN/UNOS data from February 27, 2002 to December 31, 2015 to compare waitlist outcomes of adult patients with PCLD to those with chronic liver failure (CLF) and hepatocellular carcinoma (HCC)...
March 15, 2017: Transplantation
https://www.readbyqxmd.com/read/28285710/role-of-immune-cells-in-obesity-induced-low-grade-inflammation-and-insulin-resistance
#9
REVIEW
Ambreen Asghar, Nadeem Sheikh
The frequency of obesity is enormously growing worldwide. Obesity results when energy intake exceeds, energy expenditure. Excess adiposity is a major risk factor in the progress of various metabolic disorders accounting insulin resistance, hypertension, Type 2 diabetes, nonalcoholic fatty liver disease, polycystic ovarian disease and several types of cancers. Obesity is characterized by pro-inflammatory condition in which hypertrophied adipose tissue along with immune cells contribute to increase the level of pro-inflammatory cytokines...
March 7, 2017: Cellular Immunology
https://www.readbyqxmd.com/read/28285615/consensus-on-the-integrated-traditional-chinese-and-western-medicine-criteria-of-diagnostic-classification-in-polycystic-ovary-syndrome-draft
#10
Jin Yu, Chao-Qin Yu, Qi Cao, Li Wang, Wen-Jun Wang, Li-Rong Zhou, Jing Li, Qiao-Hong Qian
Polycystic ovary syndrome (PCOS) is the most common endocrine and metabolic disorder of women, with complex pathogenesis and heterogeneous manifestations. Professor Jin Yu recently wrote an article entitled "Proposal of Diagnosis and Diagnostic Classification of PCOS in Integrated Traditional Chinese and Western Medicine."From this, the Obstetrics and Gynecology branches of the Chinese Association of Integrative Medicine and the China Association of Chinese Medicine collaborated with the Gynecology branch of the Chinese Association for Research and Advancement of Chinese Medicine to draft a report on the consensus of criteria for the diagnosis and classification of PCOS in integrated traditional Chinese and Western medicine...
March 2017: Journal of Integrative Medicine
https://www.readbyqxmd.com/read/28280650/polycystic-liver-disease-and-sarcoidosis-unusual-coexisting-etiologies-of-portal-hypertension
#11
Waseem Amjad, Sophia Jagroop, Rukma Parthvi
Both polycystic liver disease (PLD) and sarcoidosis can involve liver. Most of the time, liver disease in both conditions is asymptomatic, but they can rarely cause portal hypertension. Our aim is to report a case of a 51-year-old female with a history of adult dominant polycystic kidney disease (ADPKD) and sarcoidosis who presented with multiple episodes of hematemesis. An endoscopy showed grade 3 esophageal varices. A computed tomography (CT) scan of the abdomen showed ascites with polycystic liver, nodular contour, and calcified granuloma...
January 25, 2017: Curēus
https://www.readbyqxmd.com/read/28262591/robotics-in-hepatobiliary-surgery-initial-experience-first-reported-case-series-from-india
#12
S Goja, M K Singh, A S Soin
INTRODUCTION: Robotic surgical system's ability to perform complex hepatobiliary surgeries is gaining momentum with outcomes similar to open surgery and advantages of minimal access surgery. The authors present their initial experience of a heterogenous spectrum of robotic hepatobiliary cases and the first reported case series from India. METHODS: Retrospective review of hepatobiliary cases done robotically from February 2015 to January 2016 was done. RESULTS: The series has ten patients; with median age of 45 years (range 15-72)...
February 20, 2017: International Journal of Surgery Case Reports
https://www.readbyqxmd.com/read/28260467/outcome-of-kidney-transplant-in-patients-with-polycystic-kidney-disease-a-single-center-study-in-tunisia
#13
Samia Barbouch, Meriam Hajji, Hafedh Hedri, Fethi El Younsi, Fathi Ben Hamida, Mohammed Mongi Bacha, Mondher Ounissi, Ezzedine Abderrahim, Taieb Ben Abdallah
OBJECTIVES: Autosomal dominant polycystic kidney disease is a common cause of end-stage renal disease and a common indication for renal transplant. This study was undertaken to evaluate the demographics, outcomes, and complications of renal transplant in patients with autosomal dominant polycystic kidney disease compared with other nephropathies. MATERIALS AND METHODS: In a retrospective case-control design, we reviewed the records of 7 patients with autosomal dominant polycystic kidney disease from a total of 701 renal transplant patients over a 30-year period (1986-2016)...
February 2017: Experimental and Clinical Transplantation
https://www.readbyqxmd.com/read/28253873/extended-clinical-features-associated-with-novel-glis3-mutation-a-case-report
#14
K A Alghamdi, A B Alsaedi, A Aljasser, A Altawil, Naglaa M Kamal
BACKGROUND: Mutations in the GLI-similar 3 (GLIS3) gene encoding the transcription factor GLIS3 are a rare cause of neonatal diabetes and congenital hypothyroidism with 12 reported patients to date. Additional features, previously described, include congenital glaucoma, hepatic fibrosis, polycystic kidneys, developmental delay, facial dysmorphism, osteopenia, sensorineural deafness, choanal atresia, craniosynostosis and pancreatic exocrine insufficiency. CASE PRESENTATION: We report a new case for consanguineous parents with homozygous novel mutation in GLIS3 gene who presented with neonatal diabetes mellitus, severe resistant congenital hypothyroidism, cholestatic liver disease, bilateral congenital glaucoma and facial dysmorphism...
March 2, 2017: BMC Endocrine Disorders
https://www.readbyqxmd.com/read/28249268/bile-acids-in-polycystic-liver-diseases-triggers-of-disease-progression-and-potential-solution-for-treatment
#15
Maria J Perugorria, Ibone Labiano, Aitor Esparza-Baquer, Marco Marzioni, Jose J G Marin, Luis Bujanda, Jesús M Banales
Polycystic liver diseases (PLDs) are a group of genetic hereditary cholangiopathies characterized by the development and progressive growth of cysts in the liver, which are the main cause of morbidity. Current therapies are based on surgical procedures and pharmacological strategies, which show short-term and modest beneficial effects. Therefore, the determination of the molecular mechanisms of pathogenesis appears to be crucial in order to find new potential targets for pharmacological therapy. Ductal plate malformation during embryogenesis and abnormal cystic cholangiocyte growth and secretion are some of the key mechanisms involved in the pathogenesis of PLDs...
2017: Digestive Diseases
https://www.readbyqxmd.com/read/28249265/role-of-the-g-protein-coupled-bile-acid-receptor-tgr5-in-liver-damage
#16
Maria Reich, Caroline Klindt, Kathleen Deutschmann, Lina Spomer, Dieter Häussinger, Verena Keitel
BACKGROUND: TGR5 (G protein-coupled bile acid receptor 1, M-Bar) is a G protein-coupled cell surface receptor responsive to bile acids (BA) and different steroid hormones. TGR5 mRNA is detected almost ubiquitious in human and rodent tissues with a very high expression in gallbladder, liver and intestine. In liver, TGR5 is found in sinusoidal endothelial cells, Kupffer cells and cholangiocytes. Activation of TGR5 triggers an elevation of intracellular cyclic AMP and further downstream signalling...
2017: Digestive Diseases
https://www.readbyqxmd.com/read/28244683/rapamycin-treatment-dose-dependently-improves-the-cystic-kidney-in-a-new-adpkd-mouse-model-via-the-mtorc1-and-cell-cycle-associated-cdk1-cyclin-axis
#17
Ao Li, Song Fan, Yuchen Xu, Jialin Meng, Xufeng Shen, Jun Mao, Li Zhang, Xiansheng Zhang, Gilbert Moeckel, Dianqing Wu, Guanqing Wu, Chaozhao Liang
Although translational research into autosomal dominant polycystic kidney disease (ADPKD) and its pathogenesis has made considerable progress, there is presently lack of standardized animal model for preclinical trials. In this study, we developed an orthologous mouse model of human ADPKD by cross-mating Pkd2 conditional-knockout mice (Pkd2(f3) ) to Cre transgenic mice in which Cre is driven by a spectrum of kidney-related promoters. By systematically characterizing the mouse model, we found that Pkd2(f3/f3) mice with a Cre transgene driven by the mouse villin-1 promoter (Vil-Cre;Pkd2(f3/f3) ) develop overt cysts in the kidney, liver and pancreas and die of end-stage renal disease (ESRD) at 4-6 months of age...
February 28, 2017: Journal of Cellular and Molecular Medicine
https://www.readbyqxmd.com/read/28230738/clinical-implications-of-20-hydroxyeicosatetraenoic-acid-in-the-kidney-liver-lung-and-brain-an-emerging-therapeutic-target
#18
REVIEW
Osama H Elshenawy, Sherif M Shoieb, Anwar Mohamed, Ayman O S El-Kadi
Cytochrome P450-mediated metabolism of arachidonic acid (AA) is an important pathway for the formation of eicosanoids. The ω-hydroxylation of AA generates significant levels of 20-hydroxyeicosatetraenoic acid (20-HETE) in various tissues. In the current review, we discussed the role of 20-HETE in the kidney, liver, lung, and brain during physiological and pathophysiological states. Moreover, we discussed the role of 20-HETE in tumor formation, metabolic syndrome and diabetes. In the kidney, 20-HETE is involved in modulation of preglomerular vascular tone and tubular ion transport...
February 20, 2017: Pharmaceutics
https://www.readbyqxmd.com/read/28212790/the-echinococcoses-diagnosis-clinical-management-and-burden-of-disease
#19
P Kern, A Menezes da Silva, O Akhan, B Müllhaupt, K A Vizcaychipi, C Budke, D A Vuitton
The echinococcoses are chronic, parasitic diseases that are acquired after ingestion of infective taeniid tapeworm eggs from certain species of the genus Echinococcus. Cystic echinococcosis (CE) occurs worldwide, whereas, alveolar echinococcosis (AE) is restricted to the northern hemisphere, and neotropical echinococcosis (NE) has only been identified in Central and South America. Clinical manifestations and disease courses vary profoundly for the different species of Echinococcus. CE presents as small to large cysts, and has commonly been referred to as 'hydatid disease', or 'hydatidosis'...
2017: Advances in Parasitology
https://www.readbyqxmd.com/read/28208936/supra-hepatic-inferior-vena-cava-thrombosis-surgical-challenges
#20
Senthil Muthuraman, Anand Ramamurthy, Mahesh Gopashetty, Cuddalore Sadasivam Vijayshankar, Anand Khakhar
Autosomal Dominant Polycystic Kidney Disease (ADPKD) is a chronic affliction characterized by numerous liver and kidney cysts. There is a gradual but progressive renal and liver impairment which may require combined liver-kidney transplantation. Compression of the retrohepatic Inferior Vena Cava (IVC) by an enlarged polycystic liver may impede clear visualization on pre-operative imaging and miss an underlying thrombosis or obliteration. This may result in an intra-operative surprise. Management can be challenging requiring modification of conventional surgical approach...
December 2016: Journal of Clinical and Diagnostic Research: JCDR
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