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autosomal dominant polycystic kidney

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https://www.readbyqxmd.com/read/28208936/supra-hepatic-inferior-vena-cava-thrombosis-surgical-challenges
#1
Senthil Muthuraman, Anand Ramamurthy, Mahesh Gopashetty, Cuddalore Sadasivam Vijayshankar, Anand Khakhar
Autosomal Dominant Polycystic Kidney Disease (ADPKD) is a chronic affliction characterized by numerous liver and kidney cysts. There is a gradual but progressive renal and liver impairment which may require combined liver-kidney transplantation. Compression of the retrohepatic Inferior Vena Cava (IVC) by an enlarged polycystic liver may impede clear visualization on pre-operative imaging and miss an underlying thrombosis or obliteration. This may result in an intra-operative surprise. Management can be challenging requiring modification of conventional surgical approach...
December 2016: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28207635/kidney-transplantation-rates-across-glomerulonephritis-subtypes-in-the-united-states
#2
Michelle M O'Shaughnessy, Sai Liu, Maria E Montez-Rath, Richard A Lafayette, Wolfgang C Winkelmayer
BACKGROUND: Whether kidney transplantation rates differ by glomerulonephritis (GN) subtype remains largely unknown. METHODS: Using the US Renal Data System, we identified all adult patients with ESRD attributed to 1 of 6 GN subtypes who initiated dialysis in the US (1996-2013). Patients with diabetic nephropathy (DN) and autosomal-dominant polycystic kidney disease (ADPKD) served as "external" non-GN comparators. Using Cox proportional hazards regression, with death considered a competing risk, we estimated hazard ratios [HRs (95% confidence intervals)] for first kidney transplantation, controlling for year, demographics, comorbidities, socioeconomic factors, and Organ Procurement Organization (OPO)...
February 15, 2017: Transplantation
https://www.readbyqxmd.com/read/28205547/microrna-17-family-promotes-polycystic-kidney-disease-progression-through-modulation-of-mitochondrial-metabolism
#3
Sachin Hajarnis, Ronak Lakhia, Matanel Yheskel, Darren Williams, Mehran Sorourian, Xueqing Liu, Karam Aboudehen, Shanrong Zhang, Kara Kersjes, Ryan Galasso, Jian Li, Vivek Kaimal, Steven Lockton, Scott Davis, Andrea Flaten, Joshua A Johnson, William L Holland, Christine M Kusminski, Philipp E Scherer, Peter C Harris, Marie Trudel, Darren P Wallace, Peter Igarashi, Edmund C Lee, John R Androsavich, Vishal Patel
Autosomal dominant polycystic kidney disease (ADPKD) is the most frequent genetic cause of renal failure. Here we identify miR-17 as a target for the treatment of ADPKD. We report that miR-17 is induced in kidney cysts of mouse and human ADPKD. Genetic deletion of the miR-17∼92 cluster inhibits cyst proliferation and PKD progression in four orthologous, including two long-lived, mouse models of ADPKD. Anti-miR-17 treatment attenuates cyst growth in short-term and long-term PKD mouse models. miR-17 inhibition also suppresses proliferation and cyst growth of primary ADPKD cysts cultures derived from multiple human donors...
February 16, 2017: Nature Communications
https://www.readbyqxmd.com/read/28194574/tolvaptan-treatment-for-severe-neonatal-autosomal-dominant-polycystic-kidney-disease
#4
Rodney D Gilbert, Hazel Evans, Kazeem Olalekan, Arvind Nagra, Mushfequr R Haq, Mark Griffiths
BACKGROUND: Severe neonatal autosomal-dominant polycystic kidney disease (ADPKD) is rare and easily confused with recessive PKD. Managing such infants is difficult and often unsuccessful. CASE DIAGNOSIS/TREATMENT: A female infant with massive renal enlargement, respiratory compromise and hyponatraemia was treated with the arginine vasopressin receptor 2 antagonist tolvaptan. This resolved hyponatraemia, and there was no further increase in renal size. CONCLUSION: Tolvaptan may be a useful treatment for severe neonatal PKD...
February 13, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28194573/hyponatremia-and-cyst-growth-in-neonatal-polycystic-kidney-disease-a-case-for-aquaretics
#5
EDITORIAL
Detlef Bockenhauer
Hyponatremia is a common complication in neonatal polycystic kidney disease and is thought to be due to water retention. Aquaretics are drugs that promote free water excretion by blocking the arginine vasopressin receptor type 2 (AVPR2) in the collecting duct and thus impair urinary concentration. AVPR2 is also a key stimulant for cyclic AMP production in the collecting duct and in this way promotes cyst proliferation and pathologic kidney growth in autosomal dominant polycystic kidney disease (ADPKD). Consequently, the aquaretic tolvaptan is now used to slow down progression of ADPKD in adult patients...
February 13, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28191534/the-relation-of-anthropometric-measurements-and-insulin-resistance-in-patients-with-polycystic-kidney-disease
#6
Bennur Esen, Emel Sağlam Gokmen, Mahmut Kaya, Burak Ozkan, Ahmet Engin Atay
OBJECTIVE: To examine the frequency of insulin resistance (IR) and its relation with anthropometric measurements in patients with autosomal dominant polycystic kidney disease (ADPKD). MATERIAL AND METHODS: Nonobese 82 patients with ADPKD and 58 age matched healthy controls were enrolled into the study. None of participants were diabetic or receiving renal replacement therapies (RRT). IR was determined by homeostasis model assessment of insulin resistance (HOMA-IR) formula...
September 1, 2016: Journal of Translational Internal Medicine
https://www.readbyqxmd.com/read/28186577/vascular-dysfunction-in-children-and-young-adults-with-autosomal-dominant-polycystic-kidney-disease
#7
Kristen L Nowak, Heather Farmer, Melissa A Cadnapaphornchai, Berenice Gitomer, Michel Chonchol
No abstract text is available yet for this article.
February 1, 2017: Nephrology, Dialysis, Transplantation
https://www.readbyqxmd.com/read/28179401/high-serum-soluble-cd200-levels-in-patients-with-autosomal-dominant-polycystic-kidney-disease
#8
Funda Sari, Saadet Gumuslu, Ramazan Cetinkaya, Metin Sarikaya, Arzu Didem Yalcin
CD200 is a novel immune-effective molecule, existing in a cell membrane-bound form, as well as in a soluble form in serum, which performs to modulate inflammatory and acquired immune responses. Autosomal dominant polycystic kidney disease (ADPKD) is characterized by the development of large renal cysts and progressive loss of renal function. As defects in cell cycle arrest and apoptosis of renal tubular epithelial cells occur in ADPKD, we asked whether serum soluble CD200 might underlie and effect on ADPKD...
February 8, 2017: Journal of Investigative Medicine: the Official Publication of the American Federation for Clinical Research
https://www.readbyqxmd.com/read/28166521/rationale-and-design-of-a-clinical-trial-investigating-tolvaptan-safety-and-efficacy-in-autosomal-dominant-polycystic-kidney-disease
#9
Vicente E Torres, Olivier Devuyst, Arlene B Chapman, Ron T Gansevoort, Ronald D Perrone, John Ouyang, Jaime D Blais, Frank S Czerwiec, Olga Sergeyeva
BACKGROUND: In TEMPO 3:4, the vasopressin V2-receptor antagonist tolvaptan slowed kidney growth and function decline in autosomal dominant polycystic kidney disease (ADPKD) patients with relatively preserved kidney function. METHODS: Prospective, phase 3b, multi-center, randomized-withdrawal, placebo-controlled, double-blind trial of tolvaptan in ADPKD patients with late stage 2 to early stage 4 chronic kidney disease (CKD). The primary endpoint was estimated glomerular filtration rate (eGFR) change from pre-treatment baseline to post-treatment follow-up...
February 7, 2017: American Journal of Nephrology
https://www.readbyqxmd.com/read/28159317/novel-treatment-protocol-for-ameliorating-refractory-chronic-pain-in-patients-with-autosomal-dominant-polycystic-kidney-disease
#10
Niek F Casteleijn, Maatje D A van Gastel, Peter J Blankestijn, Joost P H Drenth, Rosa L de Jager, Anna M Leliveld, Ruud Stellema, Andreas P Wolff, Gerbrand J Groen, Ron T Gansevoort
Autosomal dominant polycystic kidney disease (ADPKD) patients can suffer from chronic pain that can be refractory to conventional treatment, resulting in a wish for nephrectomy. This study aimed to evaluate the effect of a multidisciplinary treatment protocol with sequential nerve blocks on pain relief in ADPKD patients with refractory chronic pain. As a first step a diagnostic, temporary celiac plexus block with local anesthetics was performed. If substantial pain relief was obtained, the assumption was that pain was relayed via the celiac plexus and major splanchnic nerves...
January 31, 2017: Kidney International
https://www.readbyqxmd.com/read/28154010/extracellular-loops-are-essential-for-the-assembly-and-function-of-polycystin-receptor-ion-channel-complexes
#11
Zahra Salehi-Najafabadi, Bin Li, Victoria Valentino, Courtney Ng, Hannah Martin, Yang Yu, Zhifei Wang, Parul Kashyap, Yong Yu
Polycystin complexes, or TRPP/PKD complexes, made of transient receptor potential channel polycystin (TRPP)4 and polycystic kidney disease (PKD) proteins, play key roles in coupling extracellular stimuli with intracellular Ca2+ signals. For example, the TRPP2/PKD1 complex has a crucial function in renal physiology, with mutations in either protein causing autosomal dominant polycystic kidney disease (ADPKD). In contrast, the TRPP3/PKD1L3 complex responds to low pH and was proposed to be a sour taste receptor candidate...
February 2, 2017: Journal of Biological Chemistry
https://www.readbyqxmd.com/read/28151755/renal-cystic-disease-and-associated-ciliopathies
#12
Karl O Kagan, Andreas Dufke, Ulrich Gembruch
PURPOSE OF REVIEW: To review disorders that are associated with renal cystic disease during prenatal life and to highlight the strong association between renal cystic disease and ciliopathies. RECENT FINDINGS: There are numerous causative genes for ciliopathies that can present with cystic kidney disease. In the group of single gene ciliopathies, autosomal dominant polycystic kidney disease is by far the most prevalent one. Other examples are autosomal recessive polycystic kidney disease, nephronophthisis, Bardet-Biedl syndrome, Meckel-Gruber syndrome, Joubert syndrome and related disorders as well as X-linked orofaciodigital syndrome type 1, respectively...
February 1, 2017: Current Opinion in Obstetrics & Gynecology
https://www.readbyqxmd.com/read/28118643/the-association-of-baseline-and-longitudinal-change-in-endothelial-microparticle-count-with-mortality-in-chronic-kidney-disease
#13
Darren Green, Sarah Skeoch, M Yvonne Alexander, Philip A Kalra, Ben Parker
BACKGROUND: Chronic kidney disease (CKD) is associated with a unique milieu of vascular pathology, and effective biomarkers of active vascular damage are lacking. A candidate biomarker is the quantification of circulating endothelial microparticles (EMPs). This study observed baseline and longitudinal EMP change (δEMP) and established the association of these with all-cause mortality and cardiovascular events in CKD. METHOD: An observational study in adults with CKD (estimated glomerular filtration rate [eGFR] <60 mL/min/1...
January 25, 2017: Nephron
https://www.readbyqxmd.com/read/28115652/candidate-risk-factors-and-mechanisms-for-tolvaptan-induced-liver-injury-are-identified-using-a-collaborative-cross-approach
#14
Merrie Mosedale, Yunjung Kim, William J Brock, Sharin E Roth, Tim Wiltshire, J Scott Eaddy, Gregory R Keele, Robert W Corty, Yuying Xie, William Valdar, Paul B Watkins
Clinical trials of tolvaptan showed it to be a promising candidate for the treatment of Autosomal Dominant Polycystic Kidney Disease (ADPKD) but also revealed potential for idiosyncratic drug-induced liver injury (DILI) in this patient population. To identify risk factors and mechanisms underlying tolvaptan DILI, 8 mice in each of 45 strains of the genetically diverse Collaborative Cross (CC) mouse population were treated with a single oral dose of either tolvaptan or vehicle. Significant elevations in plasma alanine aminotransferase (ALT) were observed in tolvaptan-treated animals in 3 of the 45 strains...
January 23, 2017: Toxicological Sciences: An Official Journal of the Society of Toxicology
https://www.readbyqxmd.com/read/28104302/stat5-drives-abnormal-proliferation-in-autosomal-dominant-polycystic-kidney-disease
#15
Maria Fragiadaki, Morgane Lannoy, Madeleine Themanns, Barbara Maurer, Wouter N Leonhard, Dorien J M Peters, Richard Moriggl, Albert C M Ong
Autosomal dominant polycystic kidney disease (ADPKD) leads to renal failure. The hallmark of ADPKD is increased epithelial proliferation, which has been proposed to be due to atypical signaling including abnormal JAK-STAT activity. However, the relative contribution of JAK-STAT family members in promoting proliferation in ADPKD is unknown. Here, we present siRNA JAK-STAT-focused screens discovering a previously unknown proliferative role for multiple JAK-STAT components (including STAT1, STAT2, STAT4, STAT5a, and STAT5b)...
January 16, 2017: Kidney International
https://www.readbyqxmd.com/read/28098112/autosomal-dominant-polycystic-kidney-disease-study-of-clinical-characteristics-in-an-indian-population
#16
Sanjay Vikrant, Anupam Parashar
Autosomal dominant polycystic kidney disease (ADPKD) is the most common hereditary form of kidney disease. Clinical data on this multisystem disorder are scarce from developing countries. We conducted a prospective observational study of the clinical profile of ADPKD patients at a single center over a period of six years. A total of 208 patients were studied. Majority were male (60.6%) and the mean age was 45.8 ± 14.5 years. About 61.5% had early stage (Stages 1-3) of chronic kidney disease (CKD) and 38.5% had advanced CKD (Stages 4 and 5)...
January 2017: Saudi Journal of Kidney Diseases and Transplantation
https://www.readbyqxmd.com/read/28092368/molecular-insights-into-lipid-assisted-ca-2-regulation-of-the-trp-channel-polycystin-2
#17
Martin Wilkes, M Gregor Madej, Lydia Kreuter, Daniel Rhinow, Veronika Heinz, Silvia De Sanctis, Sabine Ruppel, Rebecca M Richter, Friederike Joos, Marina Grieben, Ashley C W Pike, Juha T Huiskonen, Elisabeth P Carpenter, Werner Kühlbrandt, Ralph Witzgall, Christine Ziegler
Polycystin-2 (PC2), a calcium-activated cation TRP channel, is involved in diverse Ca(2+) signaling pathways. Malfunctioning Ca(2+) regulation in PC2 causes autosomal-dominant polycystic kidney disease. Here we report two cryo-EM structures of distinct channel states of full-length human PC2 in complex with lipids and cations. The structures reveal conformational differences in the selectivity filter and in the large exoplasmic domain (TOP domain), which displays differing N-glycosylation. The more open structure has one cation bound below the selectivity filter (single-ion mode, PC2SI), whereas multiple cations are bound along the translocation pathway in the second structure (multi-ion mode, PC2MI)...
January 16, 2017: Nature Structural & Molecular Biology
https://www.readbyqxmd.com/read/28088190/total-kidney-and-liver-volume-is-a-major-risk-factor-for-malnutrition-in-ambulatory-patients-with-autosomal-dominant-polycystic-kidney-disease
#18
Hyunjin Ryu, Hyosang Kim, Hayne Cho Park, Hyunsuk Kim, Eun Jin Cho, Kyu-Beck Lee, Wookyung Chung, Kook-Hwan Oh, Jeong Yeon Cho, Young-Hwan Hwang, Curie Ahn
BACKGROUND: In patients with autosomal dominant polycystic kidney disease (ADPKD), malnutrition may develop as renal function declines and the abdominal organs become enlarged. We investigated the relationship of intra-abdominal mass with nutritional status. METHODS: This cross-sectional study was performed at a tertiary hospital outpatient clinic. Anthropometric and laboratory data including serum creatinine, albumin, and cholesterol were collected, and kidney and liver volumes were measured...
January 14, 2017: BMC Nephrology
https://www.readbyqxmd.com/read/28087011/acute-aortic-syndrome-in-autosomal-dominant-polycystic-kidney-disease
#19
Ioannis Dimarakis, Isaac Kadir
No abstract text is available yet for this article.
February 2017: Kidney International
https://www.readbyqxmd.com/read/28081165/urine-concentrating-capacity-vasopressin-and-copeptin-in-adpkd-and-iga-nephropathy-patients-with-renal-impairment
#20
Debbie Zittema, Niek F Casteleijn, Stephan J L Bakker, Lianne S M Boesten, A A Margreeth Duit, Casper F M Franssen, Carlo A J M Gaillard, Ron T Gansevoort
BACKGROUND: Autosomal Dominant Polycystic Kidney Disease (ADPKD) patients have an impaired urine concentrating capacity. Increased circulating vasopressin (AVP) concentrations are supposed to play a role in the progression of ADPKD. We hypothesized that ADPKD patients have a more severely impaired urine concentrating capacity in comparison to other patients with chronic kidney disease at a similar level of kidney function, with consequently an enhanced AVP response to water deprivation with higher circulating AVP concentrations...
2017: PloS One
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