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Polycystic liver

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https://www.readbyqxmd.com/read/29224111/prenatal-testosterone-programming-of-insulin-resistance-in-the%C3%A2-female-sheep
#1
Muraly Puttabyatappa, Vasantha Padmanabhan
Insulin resistance, a common feature of metabolic disorders such as obesity, nonalcoholic fatty liver disease, metabolic syndrome, and polycystic ovary syndrome, is a risk factor for development of diabetes. Because sex hormones orchestrate the establishment of sex-specific behavioral, reproductive, and metabolic differences, a role for them in the developmental origin of insulin resistance is also to be expected. Female sheep exposed to male levels of testosterone during fetal life serve as an excellent translational model for delineating programming of insulin resistance...
2017: Advances in Experimental Medicine and Biology
https://www.readbyqxmd.com/read/29211938/polycystic-liver-disease-the-interplay-of-genes-causative-for-hepatic-and-renal-cystogenesis
#2
Tatyana V Masyuk, Anatoliy I Masyuk, Nicholas F LaRusso
No abstract text is available yet for this article.
December 6, 2017: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://www.readbyqxmd.com/read/29209995/early-clinical-expressions-of-insulin-resistance-the-real-enemy-to-look-for
#3
René Rodríguez-Gutiérrez, Alejandro Salcido-Montenegro, José Gerardo González-González
The type 2 diabetes mellitus epidemic threatens public healthcare systems worldwide. Efforts to prevent chronic complications of diabetes and reduce their associated mortality have been ineffective. Hence, early prevention of type 2 diabetes mellitus and cardiovascular disease needs to be prioritized. This strategy, however, must be centered not on an approach based on hyperglycemia but on early pathophysiologic mechanisms, such as insulin resistance. Non-alcoholic fatty liver disease, androgenic alopecia, acanthosis nigricans, and polycystic ovarian syndrome are all well-accepted early clinical manifestations of insulin resistance that represent, in themselves, a risk for further development of type 2 diabetes and that appear years before hyperglycemia...
December 5, 2017: Diabetes Therapy: Research, Treatment and Education of Diabetes and related Disorders
https://www.readbyqxmd.com/read/29184820/metabolic-syndrome-in-children-and-adolescents
#4
REVIEW
Dania Al-Hamad, Vandana Raman
Prevalence of metabolic syndrome in children and adolescents is increasing, in parallel with the increasing trends in obesity rates. Varying definitions of this syndrome have hindered the development of a consensus for the diagnostic criteria in the pediatric population. While pathogenesis of metabolic syndrome is not completely understood, insulin resistance and subsequent inflammation are thought to be among its main mechanistic underpinnings. Overweight and obesity are cardinal features, along with abnormal glucose metabolism, dyslipidemia, and hypertension...
October 2017: Translational Pediatrics
https://www.readbyqxmd.com/read/29175241/clinical-management-of-polycystic-liver-disease
#5
REVIEW
René M M van Aerts, Liyanne F M van de Laarschot, Jesus M Banales, Joost P H Drenth
In this Grand Round we present a typical case of a woman with polycystic liver disease (PLD). This case prompts questions which both patients and clinicians may face in clinical practice. This article aims to provide guidance to clinicians in the care of PLD patients in relation to recent key development in the field. We discuss the latest novelties in pathophysiology, natural course of disease, complications and treatment options. Finally, we discuss other potential new and effective therapies.
November 23, 2017: Journal of Hepatology
https://www.readbyqxmd.com/read/29170905/irisin-in-metabolic-diseases
#6
REVIEW
Stergios A Polyzos, Athanasios D Anastasilakis, Zoe A Efstathiadou, Polyzois Makras, Nikolaos Perakakis, Jannis Kountouras, Christos S Mantzoros
INTRODUCTION: Irisin is a myokine/adipokine induced by the exercise in mice and humans, which is proposed to induce "browning" of white adipose tissue, its primary target, thus increasing thermogenesis and energy expenditure. Since its identification, irisin has been linked to favorable effects on metabolic diseases, including obesity, type 2 diabetes mellitus (T2DM), lipid metabolism and cardiovascular disease (CVD), nonalcoholic fatty liver disease (NAFLD), polycystic ovary syndrome (PCOS), and metabolic bone diseases...
November 23, 2017: Endocrine
https://www.readbyqxmd.com/read/29167138/recent-advances-in-management-of-autosomal-dominant-polycystic-kidney-disease
#7
REVIEW
Jacob W Potts, Shaker A Mousa
PURPOSE: Promising developments in the search for effective pharmacotherapies for autosomal-dominant polycystic kidney disease (ADPKD) are reviewed. SUMMARY: The formation and development of cysts characteristic of ADPKD result in inexorable renal and extrarenal manifestations that give rise to more rapid disease progression and more widespread complications than are seen with other forms of chronic kidney disease. To date, no agent has gained Food and Drug Administration marketing approval for use in patients with ADPKD, complicating efforts to meet the medical needs of this population...
December 1, 2017: American Journal of Health-system Pharmacy: AJHP
https://www.readbyqxmd.com/read/29161258/insulin-resistance-and-hyperandrogenism-drive-steatosis-and-fibrosis-risk-in-young-females-with-pcos
#8
Salvatore Petta, Alessandro Ciresi, Jessica Bianco, Vincenzo Geraci, Roberta Boemi, Luigi Galvano, Franco Magliozzo, Giovanni Merlino, Antonio Craxì, Carla Giordano
BACKGROUND AND AIMS: Nonalcoholic fatty liver disease (NAFLD) and polycystic ovary syndrome (PCOS) recognize obesity and insulin resistance (IR) as common pathogenic background. We assessed 1) whether PCOS is a risk factor for steatosis, and 2) the impact, in PCOS patients, of IR and hyperandrogenism on steatosis and fibrosis. METHODS: We considered 202 consecutive Italian PCOS nondiabetic patients and 101 age-matched controls. PCOS was diagnosed applying the Rotterdam diagnostic criteria...
2017: PloS One
https://www.readbyqxmd.com/read/29154852/the-mitochondrial-uncoupling-protein-2-gene-is-causal-for-the-spontaneous-polycystic-liver-diseases-in-mice
#9
Misa Hirose, Paul Schilf, Sarah Rohde, Yask Gupta, Tiphaine Sancerni, Marie-Clotilde Alves-Guerra, Christian Sina, Robert Jaster, Bruno Miroux, Saleh M Ibrahim
Polycystic liver diseases (PCLDs) are autosomal dominant disorders. To date, 3 genes are known to be associated with the disease, SEC63 and PRKCSH and LRP5. Here, we report that mice deficient in the mitochondrial uncoupling protein 2 gene (Ucp2(-/-)) spontaneously developed PCLDs when they were over 12months old. Macroscopical observation, blood chemistry as well as histopathological analysis demonstrated the PCLDs found in Ucp2(-/-) mice were very similar to the findings in human PCLDs. This is the first report describing the gene encoding mitochondrial protein is causative for PCLDs...
November 14, 2017: Mitochondrion
https://www.readbyqxmd.com/read/29142941/the-longitudinal-study-of-liver-cysts-in%C3%A2-patients-with-autosomal-dominant-polycystic-kidney-disease-and-polycystic-liver-disease
#10
Ryo Matsuura, Kenjiro Honda, Yoshifumi Hamasaki, Kent Doi, Eisei Noiri, Masaomi Nangaku
Introduction: Although polycystic liver disease (PCLD) is one of the extrarenal complications in patients with autosomal dominant polycystic kidney disease (ADPKD), longitudinal changes and the association with total liver volume (TLV) have not been clearly elucidated yet. Methods: Patients with ADPKD were chosen who underwent computed tomography or magnetic resonance imaging twice or more during August 2003 through December 2015. TLV, each cyst volume, and the proportion of parenchyma were measured...
January 2017: KI Reports
https://www.readbyqxmd.com/read/29128060/risk-factors-for-the-development-of-nonalcoholic-fatty-liver-disease-nonalcoholic-steatohepatitis-including-genetics
#11
REVIEW
Huei-Wen Lim, David E Bernstein
Nonalcoholic fatty liver disease is emerging as the most common cause of chronic liver disease worldwide. This trend is, in part, secondary, to the growing incidence of obesity, type 2 diabetes, and metabolic syndrome. Other risk factors include age, gender, race/ethnicity, genetic predisposition, and polycystic ovarian disease. With the introduction of genome-wide association studies, genetic mutations contributing to inherited susceptibility to steatosis have been identified, which hold keys to future improvement in diagnosis and management...
February 2018: Clinics in Liver Disease
https://www.readbyqxmd.com/read/29114377/nonalcoholic-fatty-liver-disease-in-a-sample-of-iranian-women-with-polycystic-ovary-syndrome
#12
Ferdous Mehrabian, Roya Jahanmardi
Introduction: Polycystic ovary syndrome (PCOS) is the most common endocrinopathy in women in reproductive age that is associated with insulin resistance (IR) and metabolic abnormalities which are also a part of metabolic syndrome (Met S). This study was aimed to determine the prevalence of nonalcoholic fatty liver disease (NAFLD) women diagnosed with PCOS based on the Rotterdam criteria from January 2013 to June 2014. Methods: In this cross-sectional study, 75 women with PCOS and 75 healthy controls were enrolled...
2017: International Journal of Preventive Medicine
https://www.readbyqxmd.com/read/29098121/using-zebrafish-to-model-liver-diseases-where-do-we-stand
#13
Duc-Hung Pham, Changwen Zhang, Chunyue Yin
Purpose of Review: The liver is the largest internal organ and performs both exocrine and endocrine function that is necessary for survival. Liver failure is among the leading causes of death and represents a major global health burden. Liver transplantation is the only effective treatment for end-stage liver diseases. Animal models advance our understanding of liver disease etiology and hold promise for the development of alternative therapies. Zebrafish has become an increasingly popular system for modeling liver diseases and complements the rodent models...
June 2017: Current Pathobiology Reports
https://www.readbyqxmd.com/read/29055424/a-new-epitope-tagged-pkhd1-allele-sheds-light-on-fibrocystin%C3%A2-signaling
#14
Wendy A Lea, Christopher J Ward
In this issue of Kidney International, Outeda et al. present a new epitope-tagged allele of murine Pkhd1 that allows the monitoring of functional fibrocystin in vivo from the extreme C-terminus of the molecule. This work also shows that the removal of two-thirds of the intracellular tail of fibrocystin does not result in cystogenesis in either the liver or kidney, with major implications for our understanding of Pkhd1 function and polycystic kidney disease in general.
November 2017: Kidney International
https://www.readbyqxmd.com/read/29038287/genetic-complexity-of-autosomal-dominant-polycystic-kidney-and-liver-diseases
#15
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
#16
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 m2. 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
#17
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
#18
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
#19
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 10, 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
#20
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
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