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https://www.readbyqxmd.com/read/28545401/the-effect-of-disease-severity-markers-on-quality-of-life-in-autosomal-dominant-polycystic-kidney-disease-a-systematic-review-meta-analysis-and-meta-regression
#1
Myrte K Neijenhuis, Wietske Kievit, Ronald D Perrone, Jeff A Sloan, Patricia Erwin, Mohammad Hassan Murad, Tom J G Gevers, Marie C Hogan, Joost P H Drenth
BACKGROUND: Little is known about determinants of quality of life (QoL) in autosomal dominant polycystic kidney disease (ADPKD). Recent studies suggest that QoL in ADPKD is determined by more factors than mere renal function. We investigated the effect of ADPKD on QoL and evaluated how Qol is affected by disease severity markers renal function, kidney volume and liver volume. METHODS: We performed a systematic review, meta-analysis and meta-regression analyses of cohort studies and randomized controlled trials investigating patient-reported QoL in adult patients with ADPKD not yet on dialysis...
May 25, 2017: BMC Nephrology
https://www.readbyqxmd.com/read/28543567/tgr5-contributes-to-hepatic-cystogenesis-in-rodents-with-polycystic-liver-diseases-via-camp-g%C3%AE-s-signaling
#2
Tatyana V Masyuk, Anatoliy I Masyuk, Maria Lorenzo Pisarello, Brynn N Howard, Bing Q Huang, Pui-Yuen Lee, Xavier Fung, Eduard Sergienko, Robert J Ardesky, Thomas Dy Chung, Anthony B Pinkerton, Nicholas F LaRusso
Hepatic cystogenesis in Polycystic Liver Disease (PLD) is associated with increased levels of cAMP in cholangiocytes lining liver cysts. TGR5, a G protein-coupled bile acid receptor, is linked to cAMP and expressed in cholangiocytes. Therefore, we hypothesized that TGR5 might contribute to disease progression. We examined expression of TGR5 and Gα proteins in cultured cholangiocytes and in livers of animal models and humans with PLD. In vitro, we assessed cholangiocyte proliferation, cAMP levels, and cyst growth in response to: (i) TGR5 agonists [taurolithocholic acid (TLCA), oleanolic acid (OA) and two synthetic compounds]; (ii) a novel TGR5 antagonist (SBI-115); and (iii) a combination of SBI-115 and pasireotide, a somatostatin receptor (SSTR) analog...
May 23, 2017: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://www.readbyqxmd.com/read/28515418/automatic-segmentation-of-kidneys-using-deep-learning-for-total-kidney-volume-quantification-in-autosomal-dominant-polycystic-kidney-disease
#3
Kanishka Sharma, Christian Rupprecht, Anna Caroli, Maria Carolina Aparicio, Andrea Remuzzi, Maximilian Baust, Nassir Navab
Autosomal Dominant Polycystic Kidney Disease (ADPKD) is the most common inherited disorder of the kidneys. It is characterized by enlargement of the kidneys caused by progressive development of renal cysts, and thus assessment of total kidney volume (TKV) is crucial for studying disease progression in ADPKD. However, automatic segmentation of polycystic kidneys is a challenging task due to severe alteration in the morphology caused by non-uniform cyst formation and presence of adjacent liver cysts. In this study, an automated segmentation method based on deep learning has been proposed for TKV computation on computed tomography (CT) dataset of ADPKD patients exhibiting mild to moderate or severe renal insufficiency...
May 17, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28509136/primary-hepatic-leiomyosarcoma-in-a-patient-with-autosomal-dominant-polycystic-kidney-disease
#4
Takashi Iida, Tamaki Maeda, Yoshifumi Amari, Takatomi Yurugi, Yoshitane Tsukamoto, Fumitaka Nakajima
Primary hepatic leiomyosarcoma is an extremely rare tumor. The diagnosis is difficult, and its etiologic factors have not been clarified. A 63-year-old woman with numerous cysts in her kidneys and liver was diagnosed with autosomal dominant polycystic kidney disease (ADPKD). Several members of her family also had ADPKD. She underwent treatment with tolvaptan to inhibit cyst growth and slow the decline in kidney function. Eight months after the start of the therapy, she was hospitalized with fatigue and fever of unknown origin...
May 2017: CEN Case Reports
https://www.readbyqxmd.com/read/28508983/effect-of-tolvaptan-in-a-patient-with-autosomal-dominant-polycystic-kidney-disease-after-living-donor-liver-transplantation
#5
Kiyotaka Uchiyama, Kazuya Honda, Ryochi Yoshida, Yuka Kamijo, Mai Yanagi, Mineo Nakatsuka, Yoshitaka Ishibashi
Recently, a large randomized placebo-controlled trial indicated a beneficial effect of tolvaptan on the progression of autosomal dominant polycystic kidney disease (ADPKD) with near-normal kidney function. Meanwhile, the evidence of tolvaptan's efficacy in ADPKD with severe renal insufficiency was limited and higher frequency of liver enzyme elevations were observed in patients taking tolvaptan. Liver transplantation (LT) is the only curative treatment for patients with severe polycystic liver disease associated with ADPKD, but considering that liver injuries should be avoided particularly in patients who underwent LT, we must be careful to start tolvaptan in post-LT ADPKD patients...
November 2016: CEN Case Reports
https://www.readbyqxmd.com/read/28487551/high-free-androgen-index-is-associated-with-increased-risk-of-non-alcoholic-fatty-liver-disease-in-women-with-polycystic-ovary-syndrome-independently-of-obesity-and-insulin-resistance
#6
C Jie, W Chunhua, Z Yi, W Yuying, X Wendi, L Tzuchun, L Shengxian, W Lihua, Z Jun, S Yun, L Wei, T Tao
BACKGROUND/OBJECTIVECentral obesity and insulin resistance are common conditions in women with PCOS and in subjects with non-alcoholic fatty liver disease (NAFLD). However, few studies have addressed the association between hyperandrogenism (HA) and NAFLD. We aimed to determine whether variations in the free androgen index (FAI) might be associated with NAFLD prevalence.SUBJECTS/METHODSA cross-sectional study was performed including 400 Chinese women with PCOS and 100 age and body mass index (BMI)-matched women...
May 10, 2017: International Journal of Obesity: Journal of the International Association for the Study of Obesity
https://www.readbyqxmd.com/read/28465378/biliary-tract-and-liver-complications-in-polycystic-kidney-disease
#7
Parminder K Judge, Charlie H S Harper, Benjamin C Storey, Richard Haynes, Martin J Wilcock, Natalie Staplin, Raph Goldacre, Colin Baigent, Jane Collier, Michael Goldacre, Martin J Landray, Christopher G Winearls, William G Herrington
Polycystic liver disease is a well described manifestation of autosomal dominant polycystic kidney disease (ADPKD). Biliary tract complications are less well recognized. We report a 50-year single-center experience of 1007 patients, which raised a hypothesis that ADPKD is associated with biliary tract disease. We tested this hypothesis using all England Hospital Episode Statistics data (1998-2012), within which we identified 23,454 people with ADPKD and 6,412,754 hospital controls. Hospitalization rates for biliary tract disease, serious liver complications, and a range of other known ADPKD manifestations were adjusted for potential confounders...
May 2, 2017: Journal of the American Society of Nephrology: JASN
https://www.readbyqxmd.com/read/28460625/effect-of-statin-therapy-on-the-progression-of-autosomal-dominant-polycystic-kidney-disease-a-secondary-analysis-of-the-halt-pkd-trials
#8
Godela Brosnahan, Kaleab Z Abebe, Frederic F Rahbari-Oskoui, Charity G Patterson, Kyongtae T Bae, Robert W Schrier, William E Braun, Arlene B Chapman, Michael F Flessner, Peter C Harris, Ronald D Perrone, Theodore I Steinman, Vicente E Torres, The Halt Pkd Investigators
BACKGROUND: Autosomal dominant polycystic kidney disease (ADPKD) commonly results in end-stage renal disease (ESRD), yet a long-term treatment that is well tolerated is still lacking. In a small randomized trial in children and adolescents pravastatin administration for 3 years was associated with reduced renal cyst growth, but no large trial has tested the effect of statins in adults. METHODS: We performed a post-hoc analysis of the HALT PKD trials to compare outcomes of participants who never used statins with those who used statin for at least 3 years...
April 27, 2017: Current Hypertension Reviews
https://www.readbyqxmd.com/read/28457031/prepubertal-childhood-onset-type-2-diabetes-mellitus-four-case-reports
#9
Anandakumar Amutha, Ranjit Unnikrishnan, Ranjit Mohan Anjana, Viswanathan Mohan
BACKGROUND: The prevalence of childhood onset type 2 diabetes (T2D) is increasing, but prepubertal T2D is still unusual. METHODS: We report four cases of T2D with onset at or below 10 years of age registered at a tertiary diabetes centre in southern India.T2D was diagnosed based on the absence of ketosis, good beta cell reserve as shown by the C peptide assay, absence of GAD antibodies and pancreatic calculi, and response to oral hypoglycemic agents. RESULTS: All four patients were female, obese and had acanthosis nigricans...
February 2017: Journal of the Association of Physicians of India
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
#10
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
#11
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
#12
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...
May 1, 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
#13
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...
June 2017: Alimentary Pharmacology & Therapeutics
https://www.readbyqxmd.com/read/28357030/factors-associated-with-long-term-survival-after-liver-transplantation-a-retrospective-cohort-study
#14
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
#15
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
#16
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
#17
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
#18
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
#19
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
#20
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
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