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https://www.readbyqxmd.com/read/29038287/genetic-complexity-of-autosomal-dominant-polycystic-kidney-and-liver-diseases
#1
Emilie Cornec-Le Gall, Vicente E Torres, Peter C Harris
Data indicate significant phenotypic and genotypic overlap, plus a common pathogenesis, between two groups of inherited disorders, autosomal dominant polycystic kidney diseases (ADPKD), a significant cause of ESRD, and autosomal dominant polycystic liver diseases (ADPLD), which result in significant PLD with minimal PKD. Eight genes have been associated with ADPKD (PKD1 and PKD2), ADPLD (PRKCSH, SEC63, LRP5, ALG8, and SEC61B), or both (GANAB). Although genetics is only infrequently used for diagnosing these diseases and prognosing the associated outcomes, its value is beginning to be appreciated, and the genomics revolution promises more reliable and less expensive molecular diagnostic tools for these diseases...
October 16, 2017: Journal of the American Society of Nephrology: JASN
https://www.readbyqxmd.com/read/29034246/tolvaptan-for-the-treatment-of-enlarged-polycystic-liver-disease
#2
Hiroki Mizuno, Junichi Hoshino, Tatsuya Suwabe, Keiichi Sumida, Akinari Sekine, Yoichi Oshima, Masahiko Oguro, Kyohei Kunizawa, Masahiro Kawada, Rikako Hiramatsu, Noriko Hayami, Eiko Hasegawa, Masayuki Yamanouchi, Naoki Sawa, Kenmei Takaichi, Yoshifumi Ubara
A 44-year-old Japanese woman with autosomal dominant polycystic kidney disease was admitted to our hospital for evaluation of abdominal distension. Her eGFR was 53.7 mL/min/1.73 m(2). Total kidney volume was 2,614 mL. Tolvaptan (60 mg/day) was started to treat renal involvement. The patient's abdominal fullness began to improve and liver volume, indicating advanced polycystic liver disease (PLD), decreased from 9,750 mL to 8,345 mL after 17 months of tolvaptan treatment, though there was no significant change in kidney volume...
May 2017: Case Reports in Nephrology and Dialysis
https://www.readbyqxmd.com/read/29033415/liver-cyst-infection-after-colon-endoscopic-mucosal-resection-in-a-patient-with-autosomal-dominant-polycystic-kidney-disease-on-maintenance-hemodialysis
#3
Shota Shimano, Miki Tsuda, Seiya Fuyuno, Yoshihide Arimura, Fumio Nanishi
A 60-year-old Japanese man with autosomal dominant polycystic kidney disease (ADPKD) on maintenance hemodialysis underwent colonoscopy and endoscopic mucosal resection (EMR). He was hospitalized after 4 days of fever that began the day following colonoscopy. We detected Klebsiella pneumoniae in a blood culture and a ring-shaped integration in the liver cyst by gallium scintigraphy. He recovered with antibiotics and percutaneous drainage. The patient was believed to have contracted the liver cyst infection via an injured colonic mucosa and portal vein...
October 16, 2017: Internal Medicine
https://www.readbyqxmd.com/read/29030588/impaired-branched-chain-amino-acid-metabolism-may-underlie-the-nonalcoholic-fatty-liver-disease-like-pathology-of-neonatal-testosterone-treated-female-rats
#4
Álvaro Anzai, Rodrigo R Marcondes, Thiago H Gonçalves, Kátia C Carvalho, Manuel J Simões, Natália Garcia, José M Soares, Vasantha Padmanabhan, Edmund C Baracat, Ismael D C G da Silva, Gustavo A R Maciel
Polycystic ovary syndrome (PCOS) is frequently associated with non-alcoholic fatty liver disease (NAFLD), but the mechanisms involved in the development of NAFLD in PCOS are not well known. We investigated histological changes and metabolomic profile in the liver of rat models of PCOS phenotype induced by testosterone or estradiol. Two-day old female rats received sc injections of 1.25 mg testosterone propionate (Testos; n = 10), 0.5 mg estradiol benzoate (E2; n = 10), or vehicle (control group, CNT; n = 10)...
October 13, 2017: Scientific Reports
https://www.readbyqxmd.com/read/29024702/polycystic-ovarian-syndrome-pcos-long-term-metabolic-consequences
#5
Panagiotis Anagnostis, Basil C Tarlatzis, Robert P Kauffman
Polycystic ovary syndrome (PCOS) is the most common endocrine disorder in women during their reproductive ages, associated with a plethora of cardiometabolic consequences, with obesity, insulin resistance and hyperandrogenemia playing a major role in the degree of such manifestations. These consequences include increased risk of glucose intolerance and diabetes mellitus (both type 2 and gestational), atherogenic dyslipidemia, systemic inflammation, non-alcoholic fatty liver disease, hypertension and coagulation disorders...
October 9, 2017: Metabolism: Clinical and Experimental
https://www.readbyqxmd.com/read/29023824/cholangiocyte-autophagy-contributes-to-hepatic-cystogenesis-in-polycystic-liver-disease-and-represents-a-potential-therapeutic-target
#6
Anatoliy I Masyuk, Tatyana V Masyuk, Maria J Lorenzo Pisarello, Jingyi Francess Ding, Lorena Loarca, Bing Q Huang, Nicholas F LaRusso
Polycystic liver disease (PLD) is a group of genetic disorders with limited treatment and significant morbidity. Hepatic cysts arise from cholangiocytes exhibiting a hyperproliferative phenotype. Considering that hyperproliferation of many cell types is associated with alterations in autophagy, we hypothesized that autophagy is altered in PLD cholangiocytes, contributes to hepatic cystogenesis, and might represent a potential therapeutic target. We employed Functional Pathway Cluster Analysis (FPCA) and NextGen Sequencing (NGS), transmission electron microscopy, immunofluorescence confocal microscopy, and western blotting to assess autophagy in human and rodent PLD cholangiocytes...
October 10, 2017: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://www.readbyqxmd.com/read/29023812/polycystic-liver-disease-hepatic-venous-outflow-obstruction-lesions-of-the-non-cystic-parenchyma-have-major-consequences
#7
Louise Barbier, Maxime Ronot, Béatrice Aussilhou, François Cauchy, Claire Francoz, Prof Valérie Vilgrain, Olivier Soubrane, Valérie Paradis, Jacques Belghiti
BACKGROUND: In patients with polycystic liver disease, development of cysts induces hepatic venous outflow obstruction (HVOO) and parenchymal modifications, challenging the paradigm of a normal non-cystic liver parenchyma. The aims were to reappraise the pathology of the non-cystic parenchyma, by focusing on HVOO lesions; and to investigate the association with outflow obstruction at imaging and perioperative course after liver resection. METHODS: This is a retrospective study conducted in one tertiary center between 1993 and 2014...
October 10, 2017: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://www.readbyqxmd.com/read/28973524/liver-cyst-gene-knockout-in-cholangiocytes-inhibits-cilium-formation-and-wnt-signaling
#8
Edgar S Wills, René H M Te Morsche, Jeroen van Reeuwijk, Nicola Horn, Iris Geomini, Liyanne F M van de Laarschot, Dorus A Mans, Marius Ueffing, Karsten Boldt, Joost P H Drenth, Ronald Roepman
Mutations in the PRKCSH, SEC63, and LRP5 genes cause autosomal dominant polycystic liver disease (ADPLD). The proteins products of PRKCSH (alias GIIB) and SEC63 function in protein quality control and processing in the endoplasmic reticulum (ER), while LRP5 is implicated in Wnt/β-catenin signaling. To identify common denominators in the PLD pathogenesis, we mapped the PLD interactome by affinity proteomics, employing both HEK293T cells and H69 cholangiocytes. Identification of known complex members, such as glucosidase IIA (GIIA) for PRKCSH, and SEC61A1 and SEC61B for SEC63, confirmed the specificity of the analysis...
August 14, 2017: Human Molecular Genetics
https://www.readbyqxmd.com/read/28965028/angiopoietin-like-protein-8-an-attractive-biomarker-for-the-evaluation-of-subjects-with-insulin-resistance-and-related-disorders
#9
REVIEW
Su Wang, Xiafei Hong, Zhigang Tu, Guoyue Yuan
Insulin resistance is prevalent worldwide and is associated with many metabolic diseases, in particular, type 2 diabetes mellitus (T2DM), obesity, nonalcoholic fatty liver disease (NAFLD), polycystic ovary syndrome (PCOS) and metabolic syndrome (MetS). Angiopoietin-like protein 8 (ANGPTL8), a newly-identified secreted protein composing of 198 amino acids, is enriched in the liver of human. Considering its promising potential for β-cell proliferation and therapeutic prospect for diabetes, ANGPTL8 has aroused extensive interests...
September 8, 2017: Diabetes Research and Clinical Practice
https://www.readbyqxmd.com/read/28951847/successful-treatment-with-korean-herbal-medicine-and-lifestyle-management-in-an-obese-woman-with-polycystic-ovarian-syndrome
#10
Ji Hyeon Lee, Junyoung Jo
In Korea, herbal remedies have been widely used to treat polycystic ovarian syndrome (PCOS). We report the case of a woman with obese-type PCOS who was successfully treated with Korean herbal medicine (KHM) and lifestyle management. A 23-year-old female patient was first examined at our clinic in April 2015. She had amenorrhea for the previous 9 months and presented with obesity and abnormal reproductive hormone levels. She was treated using KHM and lifestyle management. We observed her menstrual cycles and re-evaluated her hormonal levels during the treatment...
September 2017: Integrative medicine research
https://www.readbyqxmd.com/read/28944807/conjugated-fatty-acid-rich-oil-from-gynostrmma-pentaphyllum-seed-can-ameliorate-lipid-and-glucose-metabolism-in-type-2-diabetes-mellitus-mice
#11
Mingxing Zhang, Nan Du, Lu Wang, Xiaobing Wang, Yaping Xiao, Kun Zhang, Quanhong Liu, Pan Wang
Gynostrmma pentaphyllum seed oil (GPSO), extracted from G. pentaphyllum seeds, is rich in conjugated linolenic acid, which is a special fatty acid consisting of cis-9, trans-11, trans-13 isomers. Type 2 diabetes mellitus (T2DM) is characterized by hyperglycemia resulting from insulin resistance, and is usually accompanied by hypertension, hyperlipidemia, atherosclerosis (i.e., the metabolic syndrome, or syndrome X), and polycystic ovarian disease. This study aimed to investigate the effect of GPSO on T2DM hepatic lipid metabolism and the underlying mechanism involving level of protein expression...
September 25, 2017: Food & Function
https://www.readbyqxmd.com/read/28943540/amyloid-polyneuropathy-and-myocardial-amyloidosis-10-years-after-domino-liver-transplantation-from-a-patient-with-a-transthyretin-ser50arg-mutation
#12
Masaaki Matsushima, Ichiro Yabe, Masaya Tsuda, Mamoru Sakakibara, Tsuyoshi Shimamura, Hidenao Sasaki
A 54-year-old man with polycystic liver disease received a domino liver transplantation (DLT) from a patient of hereditary ATTR amyloidosis with the transthyretin Ser50Arg mutation. Ten years after transplantation, he felt a slight numbness in his toes, and cardiac amyloidosis was simultaneously suspected upon a heart function evaluation. Biopsy specimens from the myocardium revealed transthyretin amyloidosis with the Ser50Arg mutation. Oral tafamidis therapy has inhibited the progression of neurological and cardiovascular symptoms this far...
September 25, 2017: Internal Medicine
https://www.readbyqxmd.com/read/28942551/androgen-excess-and-metabolic-disorders-in-women-with-pcos-beyond-the-body-mass-index
#13
REVIEW
R A Condorelli, A E Calogero, M Di Mauro, L M Mongioi', R Cannarella, G Rosta, S La Vignera
BACKGROUND: Insulin resistance is a common feature among women with polycystic ovary syndrome (PCOS), especially in those patients with hyperandrogenism and chronic anovulation. PCOS women are at risk for developing metabolic syndrome, impaired glucose tolerance and type II diabetes mellitus (DM II). OBJECTIVE: The aim of this review is to explore the existing knowledge of the interplay between androgen excess, pancreatic β-cell function, non-alcoholic fatty liver disease (NAFLD), intra-abdominal and subcutaneous (SC) abdominal adipocytes in PCOS, providing a better comprehension of the molecular mechanisms of diabetologic interest...
September 23, 2017: Journal of Endocrinological Investigation
https://www.readbyqxmd.com/read/28915934/increased-yap-activation-is-associated-with-hepatic-cyst-epithelial-cell-proliferation-in-arpkd-chf
#14
Lu Jiang, Lina Sun, Genea Edwards, Michael Manley, Darren P Wallace, Seth Septer, Chirag Manohar, Michele T Pritchard, Udayan Apte
Autosomal recessive polycystic kidney disease/congenital hepatic fibrosis (ARPKD/CHF) is a rare but fatal genetic disease characterized by progressive cyst development in the kidneys and liver. Liver cysts arise from aberrantly proliferative cholangiocytes accompanied by pericystic fibrosis and inflammation. Yes associated protein (YAP), the downstream effector of the Hippo signaling pathway, is implicated in human hepatic malignancies such as hepatocellular carcinoma, cholangiocarcinoma, and hepatoblastoma, but its role in hepatic cystogenesis in CHF/ARPKD is unknown...
September 15, 2017: Gene Expression
https://www.readbyqxmd.com/read/28911175/hyperandrogenemia-induced-by-letrozole-treatment-of-pubertal-female-mice-results-in-hyperinsulinemia-prior-to-weight-gain-and-insulin-resistance
#15
Danalea V Skarra, Angelina Hernández-Carretero, Alissa J Rivera, Arya R Anvar, Varykina G Thackray
Women with polycystic ovary syndrome (PCOS) diagnosed with hyperandrogenism and ovulatory dysfunction have an increased risk of developing metabolic disorders, including type 2 diabetes and cardiovascular disease. We previously developed a model that uses letrozole to elevate endogenous testosterone levels in female mice. This model has hallmarks of PCOS, including hyperandrogenism, anovulation, and polycystic ovaries, as well as increased abdominal adiposity and glucose intolerance. In the current study, we further characterized the metabolic dysfunction that occurs after letrozole treatment to determine whether this model represents a PCOS-like metabolic phenotype...
September 1, 2017: Endocrinology
https://www.readbyqxmd.com/read/28903946/ppara-agonist-fenofibrate-enhances-fatty-acid-%C3%AE-oxidation-and-attenuates-polycystic-kidney-and-liver-disease-in-mice
#16
Ronak Lakhia, Matanel Yheskel, Andrea Flaten, Ezekiel B Quittner-Strom, William L Holland, Vishal Patel
PPARA is nuclear hormone receptor that promotes fatty acid β-oxidation (FAO) and oxidative phosphorylation (OXPHOS). We and others have recently shown that PPARA and its target genes are downregulated, and FAO and OXPHOS are impaired in autosomal dominant polycystic kidney disease (ADPKD). However, whether PPARA and FAO/OXPHOS are causally linked to ADPKD progression is not entirely clear. We report that expression of PPARA and FAO/OXPHOS genes is downregulated and in-vivo β-oxidation rate of 3H-labelled triolein is reduced in Pkd1RC/RC mice, a slowly-progressing orthologous model of ADPKD that closely mimics the human ADPKD phenotype...
September 13, 2017: American Journal of Physiology. Renal Physiology
https://www.readbyqxmd.com/read/28901968/medical-comorbidity-in-polycystic-ovary-syndrome-with-special-focus-on-cardiometabolic-autoimmune-hepatic-and-cancer-diseases-an-updated-review
#17
Dorte Glintborg, Marianne Andersen
PURPOSE OF REVIEW: Polycystic ovary syndrome (PCOS) is defined by hyperandrogenism, irregular menses and polycystic ovaries when other causes are excluded. The possible implication of increased morbidity in PCOS for screening and follow-up is uncertain and is reviewed in this article. RECENT FINDINGS: The increased risk of type 2 diabetes and cardiovascular disease in PCOS is closely associated with BMI. Women with PCOS should be screened for the elements of the metabolic syndrome upon diagnosis...
September 8, 2017: Current Opinion in Obstetrics & Gynecology
https://www.readbyqxmd.com/read/28881341/the-association-of-combined-total-kidney-and-liver-volume-with-pain-and-gastrointestinal-symptoms-in-patients-with-later-stage-autosomal-dominant-polycystic-kidney-disease
#18
Hedwig M A D'Agnolo, Niek F Casteleijn, Tom J G Gevers, Hans de Fijter, Maartje D A van Gastel, Annemarie L Messchendorp, Dorien J M Peters, Mahdi Salih, Darius Soonawala, Edwin M Spithoven, Folkert W Visser, Jack F M Wetzels, Robert Zietse, Ron T Gansevoort, Joost P H Drenth
BACKGROUND: There is an ongoing debate if and how kidney and liver volume are associated with pain and gastrointestinal (GI) symptoms in autosomal dominant polycystic kidney disease (ADPKD) patients. Since both kidney and liver volume could interact, we investigated whether combined total kidney and liver volume had stronger associations with ADPKD-related pain and GI symptoms than the volumes of the organs separately. METHODS: We used baseline data from the DIPAK-1 study, which included ADPKD patients with an estimated glomerular filtration rate (eGFR) between 30 and 60 mL/min/1...
2017: American Journal of Nephrology
https://www.readbyqxmd.com/read/28877812/spontaneous-hepatic-hemorrhage-a-single-institution-s-16-year-experience
#19
Armando Rosales, Florencia G Que
Spontaneous hemorrhage from hepatic tumors is an uncommon but serious complication. Recently, interventional radiologic (IR) techniques are being used increasingly in the management of these patients. We report our 16-year experience in managing spontaneous hemorrhage from liver tumors. Twenty-six consecutive patients were diagnosed with spontaneous liver hemorrhage between 1995 and 2011. Initial management was operative in eight, IR in six, and supportive in 12 patients. Of those managed operatively, five were segmentectomies; one hemihepatectomy; one wedge resection; and one packing who later died from coagulopathy...
November 1, 2016: American Surgeon
https://www.readbyqxmd.com/read/28862642/isolated-polycystic-liver-disease-genes-define-effectors-of-polycystin-1-function
#20
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
No abstract text is available yet for this article.
September 1, 2017: Journal of Clinical Investigation
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