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Polycystic liver

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https://www.readbyqxmd.com/read/28416225/b-type-natriuretic-peptide-overexpression-ameliorates-hepatorenal-fibrocystic-disease-in%C3%A2-a%C3%A2-rat%C3%A2-model-of-polycystic-kidney-disease
#1
Sara J Holditch, Claire A Schreiber, Peter C Harris, Nicholas F LaRusso, Marina Ramirez-Alvarado, Alessandro Cataliotti, Vicente E Torres, Yasuhiro Ikeda
Polycystic kidney disease (PKD) involves progressive hepatorenal cyst expansion and fibrosis, frequently leading to end-stage renal disease. Increased vasopressin and cAMP signaling, dysregulated calcium homeostasis, and hypertension play major roles in PKD progression. The guanylyl cyclase A agonist, B-type natriuretic peptide (BNP), stimulates cGMP and shows anti-fibrotic, anti-hypertensive, and vasopressin-suppressive effects, potentially counteracting PKD pathogenesis. Here, we assessed the impacts of guanylyl cyclase A activation on PKD progression in a rat model of PKD...
April 14, 2017: Kidney International
https://www.readbyqxmd.com/read/28386465/transgastric-removal-of-a-polycystic-liver-disease-using-mini-laparoscopic-excision
#2
REVIEW
Fatih Sumer, Cuneyt Kayaalp, Yılmaz Polat, Ismail Ertugrul, Servet Karagul
Surgical treatment is often necessary for patients with symptomatic or complicated polycystic liver diseases (PLD). In this paper, we describe a 52-year-old female with symptomatic PLD that had resulted in the formation of liver cysts, the largest of which was 23 cm in diameter. The patient underwent mini-laparoscopic fenestration through 5-mm abdominal trocars. The walls of the cysts were unroofed using a harmonic scalpel. Four thickened rubber-like pieces of specimens (sizes ranged between 9 × 6 × 0...
June 1, 2016: Interventional Medicine & Applied Science
https://www.readbyqxmd.com/read/28375157/isolated-polycystic-liver-disease-genes-define-effectors-of-polycystin-1-function
#3
Whitney Besse, Ke Dong, Jungmin Choi, Sohan Punia, Sorin V Fedeles, Murim Choi, Anna-Rachel Gallagher, Emily B Huang, Ashima Gulati, James Knight, Shrikant Mane, Esa Tahvanainen, Pia Tahvanainen, Simone Sanna-Cherchi, Richard P Lifton, Terry Watnick, York P Pei, Vicente E Torres, Stefan Somlo
Dominantly inherited isolated polycystic liver disease (PCLD) consists of liver cysts that are radiologically and pathologically identical to those seen in autosomal dominant polycystic kidney disease, but without clinically relevant kidney cysts. The causative genes are known for fewer than 40% of PCLD index cases. Here, we have used whole exome sequencing in a discovery cohort of 102 unrelated patients who were excluded for mutations in the 2 most common PCLD genes, PRKCSH and SEC63, to identify heterozygous loss-of-function mutations in 3 additional genes, ALG8, GANAB, and SEC61B...
April 4, 2017: Journal of Clinical Investigation
https://www.readbyqxmd.com/read/28370150/polycystic-ovary-syndrome-with-hyperandrogenism-as-a-risk-factor-for-non-obese-non-alcoholic-fatty-liver-disease
#4
J J Kim, D Kim, J Y Yim, J H Kang, K H Han, S M Kim, K R Hwang, S Y Ku, C S Suh, S H Kim, Y M Choi
BACKGROUND: Non-alcoholic fatty liver disease (NAFLD) is known to be associated with polycystic ovary syndrome (PCOS). However, most studies investigated the prevalence of NAFLD in obese PCOS patients. AIM: To compare the prevalence of non-obese NAFLD in women with or without PCOS, and to assess an independent association between PCOS and NAFLD in a non-obese Asian cohort. METHODS: This was a case-control study using a prospective PCOS cohort...
March 30, 2017: Alimentary Pharmacology & Therapeutics
https://www.readbyqxmd.com/read/28357030/factors-associated-with-long-term-survival-after-liver-transplantation-a-retrospective-cohort-study
#5
Sven Pischke, Marie C Lege, Moritz von Wulffen, Antonio Galante, Benjamin Otto, Malte H Wehmeyer, Uta Herden, Lutz Fischer, Björn Nashan, Ansgar W Lohse, Martina Sterneck
AIM: To identify predictive factors associated with long-term patient and graft survival (> 15 years) in liver transplant recipients. METHODS: Medical charts of all de novo adult liver transplant recipients (n = 140) who were transplanted in Hamburg between 1997 and 1999 were retrospectively reviewed. In total, 155 transplantations were identified in this time period (15 re-transplantations). Twenty-six orthotopic liver transplant (OLT) recipients were early lost to follow-up due to moving to other places within 1 year after transplantation...
March 18, 2017: World Journal of Hepatology
https://www.readbyqxmd.com/read/28341273/branched-chain-amino-acids-enhance-cyst-development-in-autosomal-dominant-polycystic-kidney-disease
#6
Junya Yamamoto, Saori Nishio, Fumihiko Hattanda, Daigo Nakazawa, Toru Kimura, Michio Sata, Minoru Makita, Yasunobu Ishikawa, Tatsuya Atsumi
Autosomal dominant polycystic kidney disease (ADPKD) is characterized by the progressive development of kidney and liver cysts. The mammalian target of rapamycin (mTOR) cascade is one of the important pathways regulating cyst growth in ADPKD. Branched-chain amino acids (BCAAs), including leucine, play a crucial role to activate mTOR pathway. Therefore, we administered BCAA dissolved in the drinking water to Pkd1(flox/flox):Mx1-Cre (cystic) mice from four to 22 weeks of age after polyinosinic-polycytidylic acid-induced conditional Pkd1 knockout at two weeks of age...
March 21, 2017: Kidney International
https://www.readbyqxmd.com/read/28340841/liver-transplantation-for-polycystic-liver-disease-due-to-huge-liver-with-related-complications-a-case-report
#7
S Acar, G Gencdal, M Tokac, E Eren, U Alkara, G Tellioglu, A Dinckan, M Akyildiz
Polycystic liver disease is characterized by multiple cystic lesions on the liver. It is an uncommon autosomal dominant disease. The cysts' diameters range from 20 to 30 cm to small microscopic nodules. Generally, more than half of the liver parenchyma is covered. The mass effect of the liver created by the large cysts can cause life-threatening symptoms such as weight loss, reduction of oral intake, and malnutrition. Liver transplantation is the best treatment option in symptomatic patients. We present a patient who had polycystic liver and kidney disease, and we performed liver transplantation because of his life-threatening symptoms...
April 2017: Transplantation Proceedings
https://www.readbyqxmd.com/read/28339345/alanine-aminotransferase-is-a-marker-of-lipotoxicity-consequences-and-hyperandrogenemia-in-women-with-polycystic-ovary-syndrome
#8
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
#9
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
#10
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 28, 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
#11
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
#12
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
#13
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
#14
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
#15
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
#16
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
#17
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
#18
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
#19
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
#20
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
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