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Polycystic liver

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https://www.readbyqxmd.com/read/27919367/familial-partial-lipodystrophy-presenting-as-metabolic-syndrome
#1
Darwin Chan, Adam D McIntyre, Robert A Hegele, Andrew C Don-Wauchope
We report the first described case of a heterozygous p.R545H (c.1634 G > A) missense mutation in the LMNA gene with clinical features compatible with Dunnigan-type 2 familial partial lipodystrophy (FPLD2). The case presented as metabolic syndrome to a specialist clinical service and highlights the overlap between FPLD2 and the metabolic syndrome. The associations with type 2 diabetes mellitus, fatty liver disease, polycystic ovarian syndrome, and hypertriglyceridemia are highlighted. The importance of evaluating patients for these associated conditions is discussed, and the potential mechanisms of disease are briefly outlined...
November 2016: Journal of Clinical Lipidology
https://www.readbyqxmd.com/read/27891514/evidence-for-a-pathogenic-triumvirate-in-congenital-hepatic-fibrosis-in-autosomal-recessive-polycystic-kidney-disease
#2
REVIEW
Lu Jiang, Pingping Fang, James L Weemhoff, Udayan Apte, Michele T Pritchard
Autosomal recessive polycystic kidney disease (ARPKD) is a severe monogenic disorder that occurs due to mutations in the PKHD1 gene. Congenital hepatic fibrosis (CHF) associated with ARPKD is characterized by the presence of hepatic cysts derived from dilated bile ducts and a robust, pericystic fibrosis. Cyst growth, due to cyst wall epithelial cell hyperproliferation and fluid secretion, is thought to be the driving force behind disease progression. Liver fibrosis is a wound healing response in which collagen accumulates in the liver due to an imbalance between extracellular matrix synthesis and degradation...
2016: BioMed Research International
https://www.readbyqxmd.com/read/27862900/long-term-renal-allograft-survival-after-sequential-liver-kidney-transplantation-from-a-single-living-donor
#3
Kumiko Kitajima, Yuichi Ogawa, Katsuyuki Miki, Kotaro Kai, Akihito Sannomiya, Kazuhiro Iwadoh, Toru Murakami, Ichiro Koyama, Ichiro Nakajima, Shohei Fuchinoue
Background Combined liver-kidney transplantation (CLKT) is well-established as a definitive therapy with the potential to provide complete recovery for certain liver-kidney diseases, although the results might be contingent on the cause of transplantation. The purposes of the present study were to review the long-term outcome of renal allografts in CLKT patients from single living donors and to investigate the beneficial factors, compared with solitary renal transplantation. Materials and Methods Thirteen patients underwent sequential liver (LTx) and kidney (KTx) transplantations from single living donors...
November 10, 2016: Liver Transplantation
https://www.readbyqxmd.com/read/27829402/intracystic-magnetic-resonance-imaging-in-patients-with-autosomal-dominant-polycystic-kidney-disease-features-of-severe-cyst-infection-in-a-case-control-study
#4
Tatsuya Suwabe, Yoshifumi Ubara, Toshiharu Ueno, Noriko Hayami, Junichi Hoshino, Aya Imafuku, Masahiro Kawada, Rikako Hiramatsu, Eiko Hasegawa, Naoki Sawa, Satoshi Saitoh, Itsuko Okuda, Kenmei Takaichi
BACKGROUND: The purpose of this study was to investigate the usefulness of intracystic MRI features for detection of severe cyst infection that is usually refractory to antibiotic therapy alone in patients with autosomal dominant polycystic kidney disease. METHODS: Seventy-six patients (88 episodes) with positive cyst cultures treated from January 2006 to December 2013 were enrolled as the cases for this case-control study, while 147 patients who continued to attend our hospital from January 2011 to December 2013 and did not have cyst infection diagnosed during that period were enrolled as the controls...
November 9, 2016: BMC Nephrology
https://www.readbyqxmd.com/read/27826057/adenylyl-cyclase-5-links-changes-in-calcium-homeostasis-to-camp-dependent-cyst-growth-in-polycystic-liver-disease
#5
Carlo Spirli, Valeria Mariotti, Ambra Villani, Luca Fabris, Romina Fiorotto, Mario Strazzabosco
BACKGROUND/AIMS: Genetic defects in Polycystins -1 or -2 (PC1 or PC2) cause polycystic liver disease associated with ADPKD (PLD-ADPKD). Progressive cyst growth is sustained by a cAMP-dependent Ras/ERK/HIFα pathway leading to increased autocrine/paracrine VEGF-A signalling. In PC2-defective cholangiocytes, store-operated Ca(2+) entry (SOCE), intracellular and endoplasmic reticulum [Ca(2+)]ER levels are reduced, while cAMP production in response to [Ca(2+)]ER depletion is increased. We hypothesized that in PC2-defective cells, in response to [Ca(2+)]ER depletion, the Ca(2+)-inhibitable adenylyl-cyclases AC5 or AC6 are activated by the ER chaperon STIM1 resulting in cAMP/PKA-dependent Ras/ERK/HIFα pathway activation...
November 5, 2016: Journal of Hepatology
https://www.readbyqxmd.com/read/27823607/metformin-from-research-to-clinical-practice
#6
REVIEW
Meng H Tan, Hussain Alquraini, Kara Mizokami-Stout, Mark MacEachern
Metformin is the recommended first-line oral glucose-lowering drug initiated to control hyperglycemia in type 2 diabetes mellitus. It acts in the liver, small intestines, and skeletal muscles with its major effect on decreasing hepatic gluconeogenesis. It is safe, inexpensive, and weight neutral and can be associated with weight loss. It can reduce microvascular complication risk and its use is associated with a lower cardiovascular mortality compared with sulfonylurea therapy. It is also used to delay the onset of type 2 diabetes mellitus, in treating gestational diabetes, and in women with polycystic ovary syndrome...
December 2016: Endocrinology and Metabolism Clinics of North America
https://www.readbyqxmd.com/read/27804265/hepatic-steatosis-is-common-in-adolescents-with-obesity-and-pcos-and-relates-to-de-novo-lipogenesis-but-not-insulin-resistance
#7
Melanie Cree-Green, Bryan C Bergman, Gregory V Coe, Lindsey Newnes, Amy D Baumgartner, Samantha Bacon, Ann Sherzinger, Laura Pyle, Kristen J Nadeau
OBJECTIVE: Increased liver fat and type 2 diabetes are prevalent in women with polycystic ovarian syndrome (PCOS) and cause excess mortality, yet little is known about their development during adolescence. The objective of this study was to measure hepatic steatosis and related metabolic contributors in girls with obesity, with and without PCOS. METHODS: Nondiabetic adolescents with obesity, 41 with PCOS (PCOS; age 15.0 [13.0-16.0] years, BMI 35.2 ± 0.61 kg/m(2) ) and 30 without PCOS (OB; age 14...
November 2016: Obesity
https://www.readbyqxmd.com/read/27798284/assessment-of-growth-and-metabolism-characteristics-in-offspring-of-dehydroepiandrosterone-induced-polycystic-ovary-syndrome-adults
#8
Ying Huang, Jiang-Man Gao, Chun-Mei Zhang, Hong-Cui Zhao, Yue Zhao, Rong Li, Yang Yu, Jie Qiao
Polycystic ovary syndrome (PCOS) is a common reproductive disorder that has many characteristic features including hyperandrogenemia, insulin resistance and obesity, which may have significant implications for pregnancy outcomes and long-term health of women. Daughters born to PCOS mothers constitute a high-risk group for metabolic and reproductive derangements, but no report has described potential growth and metabolic risk factors for such female offspring. Hence, we used a mouse model of dehydroepiandrosterone (DHEA)-induced PCOS to study the mechanisms underlying the pathology of PCOS by investigating the growth, developmental characteristics, metabolic indexes and expression profiles of key genes of offspring born to the models...
December 2016: Reproduction: the Official Journal of the Society for the Study of Fertility
https://www.readbyqxmd.com/read/27796023/-clinical-manifestations-in-autosomal-dominant-polycystic-kidney-disease-adpkd
#9
Giovanni Piscopo, Giovanna Capolongo
ADPKD is a systemic disorder, associated with numerous extrarenal manifestations, including polycystic liver disease (PCLD) and other gastrointestinal manifestations, as well as pancreatic cysts, diverticular disease, inguinal and ventral hernias which play a significant role in disease burden, particularly in the advanced stage of ADPKD. In most cases the natural history of ADPKD goes through a long period of stability followed by a progressive decline in renal function. The coexistence of hypertension, cyst infections and nephrolithiasis can influence and accelerate the progression of kidney failure...
September 2016: Giornale Italiano di Nefrologia: Organo Ufficiale Della Società Italiana di Nefrologia
https://www.readbyqxmd.com/read/27796021/-renal-transplantation-in-autosomal-dominant-polycystic-kidney-disease-adpkd
#10
Norberto Perico, Monica Cortinovis, Giuseppe Remuzzi
Transplantation is the optimal choice for renal replacement therapy in patients with autosomal dominant polycystic kidney disease (ADPKD). However, some specific issues should be addressed before transplantation, including nephrectomy of native kidneys, cystic liver involvement, screening for intracranial aneurysms and living related-donor transplantation. After kidney transplantation, patient and graft survival rates are excellent and the size of native kidneys typically declines. Nevertheless, a number of renal and extrarenal complications have been documented in kidney transplant recipients with ADPKD...
September 2016: Giornale Italiano di Nefrologia: Organo Ufficiale Della Società Italiana di Nefrologia
https://www.readbyqxmd.com/read/27796020/-treatment-of-autosomal-dominant-polycystic-kidney-disease-adpkd-somatostatin-analogues-and-mtor-inhibitors
#11
Norberto Perico, Monica Cortinovis, Giuseppe Remuzzi
Recent advances in the understanding of the molecular mechanisms underlying autosomal dominant polycystic kidney disease (ADPKD) set the stage for the development of various treatments aimed to arrest or delay disease progression. In particular, clinical trials showed that the use of somatostatin analogues in patients with ADPKD is able to slow down the increase in total kidney volume and the progressive decline in renal function over the long-term. Treatment with these agents is generally well tolerated and it also enables to control cyst growth in the liver in patients with this extra-renal manifestation of the disease...
September 2016: Giornale Italiano di Nefrologia: Organo Ufficiale Della Società Italiana di Nefrologia
https://www.readbyqxmd.com/read/27792710/-jaundice-secondary-to-bile-duct-obstruction-by-polycystic-liver-disease
#12
Fortunato Ramírez-Guillén, Oscar Rosas-Carrasco, Fritz Cajuste-Sequeira, Bernardo Barriga-Pérez Gil, Araceli Cabanillas-Morel, Anamaría Eloísa Rosales-Salinas, Jorge Anselmo Peña-Pérez
Polycystic liver disease is an autosomal dominant disorder commonly associated with autosomal dominant polycystic kidney disease. It is a rare disease that usually occurs asymptomatically in 85% of cases and diagnosis is incidentally. We present the case of 57 years old woman with progressive pruritus and jaundice secondary to stenosis of the common hepatic duct and common bile by liver cyst, rare presentation which only has been documented in case reports: Deepak, et al., Wittig, et al. and Howard, et al.1-3...
September 2016: Gaceta Médica de México
https://www.readbyqxmd.com/read/27792214/prevalence-etiology-and-management-of-nonalcoholic-fatty-liver-disease-in-patients-with-polycystic-ovary-syndrome
#13
Evangelia Makri, Konstantinos Tziomalos
Obesity and insulin resistance (IR) are frequently present in patients with polycystic ovary syndrome (PCOS). Both disorders also play an important role in the pathogenesis of nonalcoholic fatty liver disease (NAFLD). Accordingly, NAFLD is frequently present in patients with PCOS. In the present review, we summarize the findings of the major studies that assessed the prevalence of NAFLD in patients with PCOS. The factors driving the increased frequency of NAFLD in patients with PCOS are also analyzed. Finally, we discuss the management options of NAFLD in patients with PCOS...
October 28, 2016: Minerva Endocrinologica
https://www.readbyqxmd.com/read/27779124/automated-segmentation-of-liver-and-liver-cysts-from-bounded-abdominal-mr-images-in-patients-with-autosomal-dominant-polycystic-kidney-disease
#14
Youngwoo Kim, Sonu K Bae, Tianming Cheng, Cheng Tao, Yinghui Ge, Arlene B Chapman, Vincente E Torres, Alan S L Yu, Michal Mrug, William M Bennett, Michael F Flessner, Doug P Landsittel, Kyongtae T Bae
Liver and liver cyst volume measurements are important quantitative imaging biomarkers for assessment of disease progression in autosomal dominant polycystic kidney disease (ADPKD) and polycystic liver disease (PLD). To date, no study has presented automated segmentation and volumetric computation of liver and liver cysts in these populations. In this paper, we proposed an automated segmentation framework for liver and liver cysts from bounded abdominal MR images in patients with ADPKD. To model the shape and variations in ADPKD livers, the spatial prior probability map (SPPM) of liver location and the tissue prior probability maps (TPPMs) of liver parenchymal tissue intensity and cyst morphology were generated...
November 21, 2016: Physics in Medicine and Biology
https://www.readbyqxmd.com/read/27750233/monoethanolamine-oleate-sclerotherapy-for-polycystic-liver-disease
#15
Masahiro Takita, Mina Iwanishi, Tomohiro Minami, Masashi Kono, Hirokazo Chishina, Tadaaki Arizumi, Norihisa Yada, Satoru Hagiwara, Yasunori Minami, Hiroshi Ida, Kazuomi Ueshima, Nishida Naoshi, Masatoshi Kudo
OBJECTIVE: The objective of treatment for polycystic liver disease is to reduce the liver volume and reduce or resolve compression symptoms such as abdominal fullness and abdominal pain due to hepatomegaly. Liver cysts are treated internally by puncture and aspiration of the cyst contents or hepatic artery embolization and surgically by cyst fenestration or hepatectomy, but no clear consensus has been reached concerning their selection. We introduced monoethanolamine oleate (EO) sclerotherapy therapy for liver cysts in 1999 and reported its effectiveness...
2016: Digestive Diseases
https://www.readbyqxmd.com/read/27746590/from-placenta-to-polycystic-ovarian-syndrome-the-role-of-adipokines
#16
REVIEW
Chiara Sartori, Pietro Lazzeroni, Silvia Merli, Viviana Dora Patianna, Francesca Viaroli, Francesca Cirillo, Sergio Amarri, Maria Elisabeth Street
Adipokines are cytokines produced mainly by adipose tissue, besides many other tissues such as placenta, ovaries, peripheral-blood mononuclear cells, liver, muscle, kidney, heart, and bone marrow. Adipokines play a significant role in the metabolic syndrome and in cardiovascular diseases, have implications in regulating insulin sensitivity and inflammation, and have significant effects on growth and reproductive function. The objective of this review was to analyze the functions known today of adiponectin, leptin, resistin, and visfatin from placenta throughout childhood and adolescence...
2016: Mediators of Inflammation
https://www.readbyqxmd.com/read/27733888/diagnostic-imaging-of-autosomal-dominant-polycystic-kidney-disease
#17
Monika Gradzik, Mariusz Niemczyk, Marek Gołębiowski, Leszek Pączek
Autosomal dominant polycystic kidney disease (ADPKD) is one of the most common genetic disorders caused by a single gene mutation. The disease usually manifests itself at the age of 30-40 years and is characterized by formation of renal cysts along with the enlargement of kidneys and deterioration of their function, eventually leading to renal insufficiency. Imaging studies (sonography, computed tomography, magnetic resonance imaging) play an important role in the diagnostics of the disease, the monitoring of its progression, and the detection of complications...
2016: Polish Journal of Radiology
https://www.readbyqxmd.com/read/27732751/center-is-an-important-indicator-for-choice-of-invasive-therapy-in-polycystic-liver-disease
#18
Hedwig M A D'Agnolo, Wietske Kievit, Kim N van Munster, Jouke J H Vd Laan, Frederik Nevens, Joost P H Drenth
BACKGROUND: Polycystic liver disease (PLD) is a rare genetic disorder with progressive cyst growth as the primary phenotype. Therapy consists of volume reduction through invasive surgical or radiological procedures. In order to understand the process of treatment decision, our aim was to identify factors that increased the likelihood of treatment. STUDY DESIGN: We performed a cross-sectional study using an international population of PLD patients. We collected data on the following therapies: liver transplantation, resection, fenestration and aspiration sclerotherapy...
October 12, 2016: Transplant International: Official Journal of the European Society for Organ Transplantation
https://www.readbyqxmd.com/read/27640193/differential-sensitivity-of-hepatocellular-carcinoma-cells-to-suppression-of-hepatocystin-transcription-under-hypoxic-conditions
#19
Jeong-Ju Yoo, Dong Hyeon Lee, Yuri Cho, Eun Ju Cho, Jeong-Hoon Lee, Su Jong Yu, Yoon Jun Kim, Chung Yong Kim, Jung-Hwan Yoon
Mutations in the gene encoding hepatocystin/80 K-H (PRKCSH) cause autosomal dominant polycystic liver disease. Hepatocystin deficiency impairs glucosidase II activity, which is critical for processing and folding glycoproteins in the endoplasmic reticulum (ER). Hypoxia is known as a strong stimulus for generating survival signals in hepatocellular carcinoma (HCC) cells. However, hypoxia may induce cell apoptosis under conditions of severe ER stress. Thus, we hypothesized that suppression of hepatocystin transcription induces HCC cell death under hypoxic conditions due to excessive ER stress...
September 17, 2016: Journal of Bioenergetics and Biomembranes
https://www.readbyqxmd.com/read/27599120/international-multi-specialty-delphi-survey-identification-of-diagnostic-criteria-for-hepatic-and-renal-cyst-infection
#20
Marten A Lantinga, Alexander J M Darding, Ruud G L de Sévaux, Ahsan Alam, Chantal P Bleeker-Rovers, Mickaël Bobot, Emilie Cornec-Le Gall, Tom J G Gevers, Ziad Hassoun, Esther Meijer, Michal Mrug, Frederik Nevens, Luiz F Onuchic, York Pei, Giorgina B Piccoli, Yves Pirson, Gopala K Rangan, Roser Torra, Folkert W Visser, François Jouret, Nada Kanaan, Wim J G Oyen, Tatsuya Suwabe, Vicente E Torres, Joost P H Drenth
BACKGROUND: Cyst infection is one of the complications of autosomal dominant polycystic kidney disease and polycystic liver disease. The diagnosis is typically made on a mix of clinical, laboratory and imaging abnormalities but the importance of individual items is uncertain. We aimed to perform a Delphi survey amongst physicians to achieve consensus on diagnostic criteria. METHODS: We retrieved diagnostic items from the literature and conducted physician and patient interviews...
2016: Nephron
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