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Polycystic liver

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
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
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
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
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
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...
September 3, 2016: Nephron
Agata Mierzwicka, Marek Bolanowski
Among new peptides responsible for the pathogenesis of metabolic disorders and carbohydrate metabolism, adipokines are of great importance. Adipokines are substances of hormonal character, secreted by adipose tissue. Apart from the well-known adipokines, adropin and preptin are relatively newly discovered, hence their function is not fully understood. They are peptides not secreted by adipose tissue but their role in the metabolic regulations seems to be significant. Preptin is a 34-amino acid peptide, a derivative of proinsulin growth factor II (pro-IGF-II), secreted by pancreatic β cells, considered to be a physiological enhancer of insulin secretion...
2016: Postȩpy Higieny i Medycyny Doświadczalnej
Romina Mancinelli, Antonio Franchitto, Shannon Glaser, Antonella Vetuschi, Julie Venter, Roberta Sferra, Luigi Pannarale, Francesca Olivero, Guido Carpino, Gianfranco Alpini, Paolo Onori, Eugenio Gaudio
The neurohypophysial hormone arginine vasopressin (AVP) acts by three distinct receptor subtypes: V1a, V1b, and V2. In the liver, AVP is involved in ureogenesis, glycogenolysis, neoglucogenesis and regeneration. No data exist about the presence of AVP in the biliary epithelium. Cholangiocytes are the target cells in a number of animal models of cholestasis, including bile duct ligation (BDL), and in several human pathologies, such as polycystic liver disease characterized by the presence of cysts that bud from the biliary epithelium...
August 29, 2016: Laboratory Investigation; a Journal of Technical Methods and Pathology
Djalila Mekahli, Karlijn J van Stralen, Marjolein Bonthuis, Kitty J Jager, Ayşe Balat, Elisa Benetti, Nathalie Godefroid, Vidar O Edvardsson, James G Heaf, Augustina Jankauskiene, Larissa Kerecuk, Svetlana Marinova, Flora Puteo, Tomas Seeman, Aleksandra Zurowska, Jacques Pirenne, Franz Schaefer, Jaap W Groothoff
BACKGROUND: The choice for either kidney or combined liver-kidney transplantation in young people with kidney failure and liver fibrosis due to autosomal recessive polycystic kidney disease (ARPKD) can be challenging. We aimed to analyze the characteristics and outcomes of transplantation type in these children, adolescents, and young adults. STUDY DESIGN: Cohort study. SETTING & PARTICIPANTS: We derived data for children, adolescents, and young adults with ARPKD with either kidney or combined liver-kidney transplants for 1995 to 2012 from the ESPN/ERA-EDTA Registry, a European pediatric renal registry collecting data from 36 European countries...
August 20, 2016: American Journal of Kidney Diseases: the Official Journal of the National Kidney Foundation
Edgar S Wills, Wybrich R Cnossen, Joris A Veltman, Rob Woestenenk, Marloes Steehouwer, Jody Salomon, René H M Te Morsche, Meritxell Huch, Jayne Y Hehir-Kwa, Martijn J Banning, Rolph Pfundt, Ronald Roepman, Alexander Hoischen, Joost P H Drenth
Autosomal dominant polycystic liver disease (ADPLD) is caused by variants in PRKCSH, SEC63, and LRP5, whereas autosomal dominant polycystic kidney disease is caused by variants in PKD1 and PKD2. Liver cyst development in these disorders is explained by somatic loss-of-heterozygosity (LOH) of the wild-type allele in the developing cyst. We hypothesize that we can use this mechanism to identify novel disease genes that reside in LOH regions. In this study, we aim to map abnormal genomic regions using high-density SNP microarrays to find novel PLD genes...
August 24, 2016: European Journal of Human Genetics: EJHG
Tung-Min Yu, Ya-Wen Chuang, Mei-Ching Yu, Cheng-Hsu Chen, Cheng-Kuang Yang, Shih-Ting Huang, Cheng-Li Lin, Kuo-Hsiung Shu, Chia-Hung Kao
BACKGROUND: Data for the risk of any solid cancer in patients with polycystic kidney disease are scarce. Therefore, we did a nationwide cohort study in Taiwan to establish the risk of cancer in patients with polycystic kidney disease without either chronic kidney disease or end-stage renal disease. METHODS: From inpatient claims of the Taiwan National Health Insurance Research Database, we included patients aged 20 years and older and diagnosed with polycystic kidney disease between January, 1998 and December, 2010, in the polycystic kidney disease cohort...
October 2016: Lancet Oncology
Audrey Binette, Kerry Howatt, Ashley Waddington, Robert L Reid
Despite the introduction of promising products into the contraceptive market, the rate of unintended pregnancies remains high. Women with underlying medical conditions should have access to safe and effective contraceptive methods for various reasons, including the potential deleterious effect of the disease on the pregnancy or the effect of the pregnancy on the disease process. Healthcare providers are often confronted with cases in which contraception counseling is problematic due to controversial evidence and persistent myths...
August 22, 2016: Journal of Women's Health
Marie Neuville, Roland Hustinx, Jessica Jacques, Jean-Marie Krzesinski, François Jouret
BACKGROUND: Acute febrile abdomen represents a diagnostic challenge in patients with autosomal dominant polycystic kidney disease (ADPKD). Although criteria have been proposed for cyst infection (CyI) and hemorrhage (CyH), there is a lack of comparative assessments. Furthermore, distinguishing cystic from non-cystic complications remains problematic. DESIGN: ADPKD patients presenting with abdominal pain and/or fever between 01/2005 and 06/2015 were retrospectively identified in a systematic computerized billing database...
2016: PloS One
Akila Rajakumar, Shiwalika Gupta, Selvakumar Malleeswaran, Joy Varghese, Ilankumaran Kaliamoorthy, Mohamed Rela
BACKGROUND AND AIMS: The perioperative management of patients presenting for simultaneous liver and kidney transplantation (SLKT) is a complex process. We analysed SLKTs performed in our institution to identify preoperative, intraoperative and post-operative challenges encountered in the management. METHODS: We retrospectively studied the case records of 12 patients who underwent SLKT between 2009 and 2014 and analysed details of pre-operative evaluation and optimisation, intraoperative anaesthetic management and the implications of use of perioperative continuous renal replacement therapy (CRRT) and the post-operative course of these patients...
July 2016: Indian Journal of Anaesthesia
K P Srikanth, Inusha Panigrahi, Babu Ram Thapa, Kim Vaiphei
We present a rare presentation of cystic fibrosis with neonatal cholestasis. Histological features of mucoviscidosis were present in liver involving the biliary tract, intestinal mucosa, pancreas, and lung. Besides, there was a rare association with autosomal dominant type of polycystic renal disease.
July 2016: Indian Journal of Pathology & Microbiology
Christian S Göbl, Johannes Ott, Latife Bozkurt, Michael Feichtinger, Victoria Rehmann, Anna Cserjan, Maike Heinisch, Helmut Steinbrecher, Ivica JustKukurova, Radka Tuskova, Michael Leutner, Elisabeth Vytiska-Binstorfer, Christine Kurz, Andrea Weghofer, Andrea Tura, Christian Egarter, Alexandra Kautzky-Willer
AIMS: There are emerging data indicating an association between PCOS (polycystic ovary syndrome) and metabolic derangements with potential impact on its clinical presentation. This study aims to evaluate the pathophysiological processes beyond PCOS with particular focus on carbohydrate metabolism, ectopic lipids and their possible interaction. Differences between the two established classifications of the disease should be additionally evaluated. METHODS: A metabolic characterization was performed in 53 untreated PCOS patients as well as 20 controls including an extended oral glucose tolerance test (OGTT, to assess insulin sensitivity, secretion and ß-cell function) in addition to a detailed examination of ectopic lipid content in muscle and liver by nuclear magnetic resonance spectroscopy...
2016: PloS One
Georgios K Dimitriadis, Ioannis Kyrou, Harpal S Randeva
BACKGROUND: Polycystic Ovary Syndrome (PCOS) is a complex heterogeneous disorder and the most common endocrinopathy amongst women of reproductive age. It is characterized by androgen excess, chronic anovulation and an altered cardiometabolic profile. PCOS is linked to impaired adipose tissue (AT) physiology and women with this disorder present with greater risk for insulin resistance (IR), hyperinsulinemia, central adiposity, nonalcoholic fatty liver disease (NAFLD) and type 2 diabetes mellitus (T2DM) than matched for age and body mass index (BMI) women without PCOS...
July 26, 2016: Current Pharmaceutical Design
Sarmad Said, Debabrata Mukherjee, Thomas F Whayne
Cardiovascular (CV) disease is the most common cause of morbidity and mortality worldwide, particularly in the presence of the metabolic syndrome (MetS). Classifications and treatment of the MetS have recently been redefined. While the majority of the cardiac components such as hypertension, diabetes mellitus (DM) and dyslipidemia are objectively measurable elements, a few disparities among the definitions have to be considered that can variably modify diagnosis, treatment and prevention. Noncardiac factors such as liver disease (including, but not limited to, alcoholic and non-alcoholic steatosis/hepatitis), renal disease, severe obesity, polycystic ovarian syndrome and obstructive sleep apnea, may have independent or synergistic relationship with complementary cardiac MetS elements, and these additional risk factors may have an incremental adverse impact on CV outcome...
July 22, 2016: Current Vascular Pharmacology
Wasawat Vutthikraivit, Montira Assanatham, Chutintorn Sriphrapradang
Autosomal dominant polycystic kidney disease (ADPKD) is the most common inherited renal disease. Hypertension is common and occurs before decline in renal function. However, the coexistence of hypertension and hypokalemia is rare in ADPKD patients. We report on a 32-year-old woman with secondary aldosteronism. Magnetic resonance imaging of the renal arteries revealed multiple cysts of varying sizes in both the kidneys and the liver, compatible with ADPKD. Increased reninangiotensin-aldosterone system activity was secondary to cyst expansion...
June 2016: Electrolyte & Blood Pressure: E & BP
Kunihisa Nezu, Takanari Sakai, Akito Kuromoto, Hidenori Kanno, Masahiko Sato, Kenji Numahata, Senji Hoshi
The patient was a 47 year-old female who had autosomal dominant polycystic kidney disease (ADPKD) with bilateral small renal cell carcinoma (RCC). We performed bilateral partial nephrectomy and radiofrequency ablation to the residual tumor. Pathological diagnosis was clear cell carcinoma,Fuhrman grade 3. Sunitinib therapy was started nine months after the operation because multiple liver metastases occurred. Twenty-six months after the operation,she died from rapid progression of liver metastasis.
June 2016: Hinyokika Kiyo. Acta Urologica Japonica
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