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https://www.readbyqxmd.com/read/29215110/quercetin-inhibits-renal-cyst-growth-in-vitro-and-via-parenteral-injection-in-a-polycystic-kidney-disease-mouse-model
#1
Yangyang Zhu, Tian Teng, Hu Wang, Hao Guo, Lei Du, Baoxue Yang, Xiaoxing Yin, Ying Sun
Autosomal dominant polycystic kidney disease (ADPKD) is a common monogenic disease characterized by massive enlargement of fluid-filled cysts in the kidney. There is an urgent need to develop effective ADPKD therapies. We used an in vitro Madin-Darby canine kidney (MDCK) cyst model and a murine embryonic kidney cyst model to evaluate whether quercetin inhibits cyst development. We then used a polycystic kidney disease (PKD) mouse model to further determine the in vivo effects of quercetin (100 mg per kg body weight twice per day) on PKD mice via subcutaneous injections...
December 7, 2017: Food & Function
https://www.readbyqxmd.com/read/29181500/perinatal-diagnosis-management-and-follow-up-of-cystic-renal-diseases-a-clinical-practice-recommendation-with-systematic-literature-reviews
#2
Charlotte Gimpel, Fred E Avni, Carsten Bergmann, Metin Cetiner, Sandra Habbig, Dieter Haffner, Jens König, Martin Konrad, Max C Liebau, Lars Pape, Georg Rellensmann, Andrea Titieni, Constantin von Kaisenberg, Stefanie Weber, Paul J D Winyard, Franz Schaefer
Importance: Prenatal and neonatal cystic kidney diseases are a group of rare disorders manifesting as single, multiple unilateral, or bilateral cysts or with increased echogenicity of the renal cortex without macroscopic cysts. They may be accompanied by grossly enlarged kidneys, renal oligohydramnios, pulmonary hypoplasia, extrarenal abnormalities, and neonatal kidney failure. The prognosis is extremely variable from trivial to very severe or even uniformly fatal, which poses significant challenges to prenatal counseling and management...
November 27, 2017: JAMA Pediatrics
https://www.readbyqxmd.com/read/29169385/standardised-outcomes-in-nephrology-polycystic-kidney-disease-song-pkd-study-protocol-for-establishing-a-core-outcome-set-in-polycystic-kidney-disease
#3
Yeoungjee Cho, Benedicte Sautenet, Gopala Rangan, Jonathan C Craig, Albert C M Ong, Arlene Chapman, Curie Ahn, Dongping Chen, Helen Coolican, Juliana Tze-Wah Kao, Ron Gansevoort, Ronald Perrone, Tess Harris, Vicente Torres, York Pei, Peter G Kerr, Jessica Ryan, Talia Gutman, Martin Howell, Angela Ju, Karine E Manera, Armando Teixeira-Pinto, Lorraine A Hamiwka, Allison Tong
BACKGROUND: Autosomal dominant polycystic kidney disease (ADPKD) is the most common potentially life threatening inherited kidney disease and is responsible for 5-10% of cases of end-stage kidney disease (ESKD). Cystic kidneys may enlarge up to 20 times the weight of a normal kidney due to the growth of renal cysts, and patients with ADPKD have an increased risk of morbidity, premature mortality, and other life-time complications including renal and hepatic cyst and urinary tract infection, intracranial aneurysm, diverticulosis, and kidney pain which impair quality of life...
November 23, 2017: Trials
https://www.readbyqxmd.com/read/29167286/a-novel-prrt2-pathogenic-variant-in-a-family-with-paroxysmal-kinesigenic-dyskinesia-and-benign-familial-infantile-seizures
#4
Jacqueline G Lu, Juliet Bishop, Sarah Cheyette, Igor B Zhulin, Su Guo, Nara Sobreira, Steven E Brenner
Paroxysmal Kinesigenic Dyskinesia (PKD) is a rare neurological disorder characterized by recurrent attacks of dyskinetic movements without alteration of consciousness that are often triggered by the initiation of voluntary movements. Whole exome sequencing has revealed a cluster of pathogenic variants in PRRT2 (proline-rich transmembrane protein), a gene with a function in synaptic regulation that remains poorly understood. Here, we report the discovery of a novel PRRT2 pathogenic variant inherited in an autosomal dominant pattern in a family with PKD and Benign Familial Infantile Seizures (BFIS)...
November 22, 2017: Cold Spring Harbor Molecular Case Studies
https://www.readbyqxmd.com/read/29136299/malignancy-in-renal-transplant-recipients-exposed-to-cyclophosphamide-prior-to-transplantation-for-the-treatment-of-native-glomerular-disease
#5
M R Jorgenson, J L Descourouez, T Singh, Brad C Astor, Sarah E Panzer
STUDY OBJECTIVE: To evaluate the risk of post-transplantation malignancy in renal transplant recipients exposed to pre-transplantation cyclophosphamide for treatment of glomerular nephropathy (GN). DESIGN: Retrospective cohort study. SETTING: Tertiary academic medical center. PATIENTS: Six hundred adult renal transplant recipients transplanted between 1993 and 2014; 54 patients were exposed to pre-transplantation cyclophosphamide for treatment of GN (GN-CYC group), and 546 patients with polycystic kidney disease were not exposed to pre-transplantation cyclophosphamide (PKD group)...
November 14, 2017: Pharmacotherapy
https://www.readbyqxmd.com/read/29132464/-clinical-manifestations-and-genetic-diagnosis-of-paroxysmal-kinesigenic-dyskinesia
#6
Xiao-Ming Zhu, Yu-Hong Gong, Si Lu, Shou-Chao Cheng, Bao-Zhen Yao
The clinical manifestations of five children with paroxysmal kinesigenic dyskinesia (PKD) were retrospectively analyzed and their gene mutations were analyzed by high-throughput sequencing and chromosome microarray. The 5 patients consisted of 4 males and 1 female and the age of onset was 6-9 years. Dyskinesia was induced by sudden turn movement, scare, mental stress, or other factors. These patients were conscious and had abnormal posture of unilateral or bilateral extremities, athetosis, facial muscle twitching, and abnormal body posture...
November 2017: Zhongguo Dang Dai Er Ke za Zhi, Chinese Journal of Contemporary Pediatrics
https://www.readbyqxmd.com/read/29129530/glutamylation-regulates-transport-specializes-function-and-sculpts-the-structure-of-cilia
#7
Robert O'Hagan, Malan Silva, Ken C Q Nguyen, Winnie Zhang, Sebastian Bellotti, Yasmin H Ramadan, David H Hall, Maureen M Barr
Ciliary microtubules (MTs) are extensively decorated with post-translational modifications (PTMs), such as glutamylation of tubulin tails. PTMs and tubulin isotype diversity act as a "tubulin code" that regulates cytoskeletal stability and the activity of MT-associated proteins such as kinesins. We previously showed that, in C. elegans cilia, the deglutamylase CCPP-1 affects ciliary ultrastructure, localization of the TRP channel PKD-2 and the kinesin-3 KLP-6, and velocity of the kinesin-2 OSM-3/KIF17, whereas a cell-specific α-tubulin isotype regulates ciliary ultrastructure, intraflagellar transport, and ciliary functions of extracellular vesicle (EV)-releasing neurons...
November 20, 2017: Current Biology: CB
https://www.readbyqxmd.com/read/29113359/hepatointestinal-complications-in-polycystic-kidney-disease
#8
Shih-Ting Huang, Ya-Wen Chuang, Tung-Min Yu, Cheng-Li Lin, Long-Bin Jeng
Background: The objective of this study was to determine the incidence of major hepatointestinal complications in patients with polycystic kidney disease (PKD). Methods: We analyzed the Taiwan National Health Insurance claims data (2000-2010) of 6031 patients with PKD and 23,976 non-PKD hospitalized controls. The control cohort was propensity score matched with the PKD cohort at a 1:4 ratio. All patients were followed up from the index date to the first inpatient diagnosis of hepatointestinal complications, death, or 31 December, 2011...
October 6, 2017: Oncotarget
https://www.readbyqxmd.com/read/29104045/5-ht2a-receptor-is-the-predominant-receptor-mediating-contraction-of-the-isolated-porcine-distal-ureter-to-5-ht-in-young-and-old-animals
#9
Iris Lim, Russ Chess-Williams, Donna Sellers
Isolated ureteral strips develop spontaneous phasic contractile activity which is enhanced by 5-hydroytryptamine (5-HT). The aim of this study was to identify the receptor subtype mediating these responses and to determine whether responses to 5-HT change with age. The frequency of contractions and the overall contractile activity (measured as the area under the curve, AUC) were recorded in strips of porcine distal ureter isolated from young (3 months) and old (2 years) pigs. Responses to 5-HT were examined in the absence and presence of selective 5-HT receptor subtype antagonists...
November 7, 2017: European Journal of Pharmacology
https://www.readbyqxmd.com/read/29078391/integrated-field-laboratory-and-theoretical-study-of-pkd-spread-in-a-swiss-prealpine-river
#10
Luca Carraro, Enrico Bertuzzo, Lorenzo Mari, Inês Fontes, Hanna Hartikainen, Nicole Strepparava, Heike Schmidt-Posthaus, Thomas Wahli, Jukka Jokela, Marino Gatto, Andrea Rinaldo
Proliferative kidney disease (PKD) is a major threat to wild and farmed salmonid populations because of its lethal effect at high water temperatures. Its causative agent, the myxozoan Tetracapsuloides bryosalmonae, has a complex lifecycle exploiting freshwater bryozoans as primary hosts and salmonids as secondary hosts. We carried out an integrated study of PKD in a prealpine Swiss river (the Wigger). During a 3-year period, data on fish abundance, disease prevalence, concentration of primary hosts' DNA in environmental samples [environmental DNA (eDNA)], hydrological variables, and water temperatures gathered at various locations within the catchment were integrated into a newly developed metacommunity model, which includes ecological and epidemiological dynamics of fish and bryozoans, connectivity effects, and hydrothermal drivers...
November 7, 2017: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/29071385/protein-kinase-d-inhibitor-crt0066101-suppresses-bladder-cancer-growth-in-vitro-and-xenografts-via-blockade-of-the-cell-cycle-at-g2-m
#11
Qingdi Quentin Li, Iawen Hsu, Thomas Sanford, Reema Railkar, Navin Balaji, Carole Sourbier, Cathy Vocke, K C Balaji, Piyush K Agarwal
The protein kinase D (PKD) family of proteins are important regulators of tumor growth, development, and progression. CRT0066101, an inhibitor of PKD, has antitumor activity in multiple types of carcinomas. However, the effect and mechanism of CRT0066101 in bladder cancer are not understood. In the present study, we show that CRT0066101 suppressed the proliferation and migration of four bladder cancer cell lines in vitro. We also demonstrate that CRT0066101 blocked tumor growth in a mouse flank xenograft model of bladder cancer...
October 25, 2017: Cellular and Molecular Life Sciences: CMLS
https://www.readbyqxmd.com/read/29062716/clear-cell-tubopapillary-renal-cell-carcinoma-mimicking-polycystic-kidney-disease-a-case-report
#12
Gricelda Gomez, Adam Althaus, Christine E Gruessner, Michelle S Hirsch, Graeme S Steele
Clear cell tubopapillary renal cell carcinoma (CCTP-RCC) is a distinct histologic subtype of RCC recognized for its unique clinicopathologic and immunohistochemical features. A 72-year-old man with presumed polycystic kidney disease (PKD) and bilateral clear cell RCC (CC-RCC) underwent left radical nephrectomy and right partial nephrectomy 20 years ago at an outside hospital. On surveillance imaging, RCC recurrence was suspected and right radical nephrectomy was performed. Histologic and gross examination of the right remaining kidney was consistent with CCTP-RCC...
January 2018: Urology Case Reports
https://www.readbyqxmd.com/read/29058957/ctgf-is-expressed-during-cystic-remodeling-in-the-pkd-mhm-cy-rat-model-for-autosomal-dominant-polycystic-kidney-disease-adpkd
#13
Stefan Gauer, Yvonne Holzmann, Bettina Kränzlin, Sigrid C Hoffmann, Norbert Gretz, Ingeborg A Hauser, Margarete Goppelt-Struebe, Helmut Geiger, Nicholas Obermüller
Connective tissue growth factor (CTGF, also named CCN2) plays an important role in the development of tubulointerstitial fibrosis, which most critically determines the progression to end-stage renal failure in autosomal-dominant polycystic kidney disease (ADPKD), the most common genetically caused renal disease. We determined CTGF expression in a well-characterized animal model of human ADPKD, the PKD/Mhm (cy/+) rat. Kidneys of 12 weeks old (cy/+) as well as (+/+) non-affected rats were analyzed for CTGF RNA and protein expression by RT-PCR, Northern and Western blot analyses, in situ hybridization, and IHC...
October 1, 2017: Journal of Histochemistry and Cytochemistry: Official Journal of the Histochemistry Society
https://www.readbyqxmd.com/read/29056747/prrt2-deficiency-induces-paroxysmal-kinesigenic-dyskinesia-by-regulating-synaptic-transmission-in-cerebellum
#14
Guo-He Tan, Yuan-Yuan Liu, Lu Wang, Kui Li, Ze-Qiang Zhang, Hong-Fu Li, Zhong-Fei Yang, Yang Li, Dan Li, Ming-Yue Wu, Chun-Lei Yu, Juan-Juan Long, Ren-Chao Chen, Li-Xi Li, Lu-Ping Yin, Ji-Wei Liu, Xue-Wen Cheng, Qi Shen, You-Sheng Shu, Kenji Sakimura, Lu-Jian Liao, Zhi-Ying Wu, Zhi-Qi Xiong
Mutations in the proline-rich transmembrane protein 2 (PRRT2) are associated with paroxysmal kinesigenic dyskinesia (PKD) and several other paroxysmal neurological diseases, but the PRRT2 function and pathogenic mechanisms remain largely obscure. Here we show that PRRT2 is a presynaptic protein that interacts with components of the SNARE complex and downregulates its formation. Loss-of-function mutant mice showed PKD-like phenotypes triggered by generalized seizures, hyperthermia, or optogenetic stimulation of the cerebellum...
October 20, 2017: Cell Research
https://www.readbyqxmd.com/read/29042084/adenylyl-cyclase-5-deficiency-reduces-renal-cyclic-amp-and-cyst-growth-in-an-orthologous-mouse-model-of-polycystic-kidney-disease
#15
Qian Wang, Patricia Cobo-Stark, Vishal Patel, Stefan Somlo, Pyung-Lim Han, Peter Igarashi
Cyclic AMP promotes cyst growth in polycystic kidney disease (PKD) by stimulating cell proliferation and fluid secretion. Previously, we showed that the primary cilium of renal epithelial cells contains a cAMP regulatory complex comprising adenylyl cyclases 5 and 6 (AC5/6), polycystin-2, A-kinase anchoring protein 150, protein kinase A, and phosphodiesterase 4C. In Kif3a mutant cells that lack primary cilia, the formation of this regulatory complex is disrupted and cAMP levels are increased. Inhibition of AC5 reduces cAMP levels in Kif3a mutant cells, suggesting that AC5 may mediate the increase in cAMP in PKD...
October 14, 2017: Kidney International
https://www.readbyqxmd.com/read/29038287/genetic-complexity-of-autosomal-dominant-polycystic-kidney-and-liver-diseases
#16
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/29033332/disruption-of-core-planar-cell-polarity-signaling-regulates-renal-tubule-morphogenesis-but-is-not-cystogenic
#17
Koshi Kunimoto, Roy D Bayly, Eszter K Vladar, Tyson Vonderfecht, Anna-Rachel Gallagher, Jeffrey D Axelrod
Oriented cell division (OCD) and convergent extension (CE) shape developing renal tubules, and their disruption has been associated with polycystic kidney disease (PKD) genes, the majority of which encode proteins that localize to primary cilia. Core planar cell polarity (PCP) signaling controls OCD and CE in other contexts, leading to the hypothesis that disruption of PCP signaling interferes with CE and/or OCD to produce PKD. Nonetheless, the contribution of PCP to tubulogenesis and cystogenesis is uncertain, and two major questions remain unanswered...
October 23, 2017: Current Biology: CB
https://www.readbyqxmd.com/read/28973148/a-constitutive-bcl2-down-regulation-aggravates-the-phenotype-of-pkd1-mutant-induced-polycystic-kidney-disease
#18
Laurence Duplomb, Nathalie Droin, Olivier Bouchot, Christel Robinet Thauvin, Ange-Line Bruel, Julien Thevenon, Patrick Callier, Guillaume Meurice, Noémie Pata-Merci, Romaric Loffroy, David Vandroux, Romain Da Costa, Virginie Carmignac, Eric Solary, Laurence Faivre
The main identified function of BCL2 protein is to prevent cell death by apoptosis. Mice knock-out for Bcl2 demonstrate growth retardation, severe polycystic kidney disease (PKD), gray hair and lymphopenia, and die prematurely after birth. Here, we report a 40-year-old male referred to for abdominal and thoracic aortic dissection with associated aortic root aneurysm, PKD, lymphocytopenia with a history of T cell lymphoblastic lymphoma, white hair since the age of 20, and learning difficulties. PKD, which was also detected in the father and sister, was related to an inherited PKD1 mutation...
September 11, 2017: Human Molecular Genetics
https://www.readbyqxmd.com/read/28967916/organoid-cystogenesis-reveals-a-critical-role-of-microenvironment-in-human-polycystic-kidney%C3%A2-disease
#19
Nelly M Cruz, Xuewen Song, Stefan M Czerniecki, Ramila E Gulieva, Angela J Churchill, Yong Kyun Kim, Kosuke Winston, Linh M Tran, Marco A Diaz, Hongxia Fu, Laura S Finn, York Pei, Jonathan Himmelfarb, Benjamin S Freedman
Polycystic kidney disease (PKD) is a life-threatening disorder, commonly caused by defects in polycystin-1 (PC1) or polycystin-2 (PC2), in which tubular epithelia form fluid-filled cysts. A major barrier to understanding PKD is the absence of human cellular models that accurately and efficiently recapitulate cystogenesis. Previously, we have generated a genetic model of PKD using human pluripotent stem cells and derived kidney organoids. Here we show that systematic substitution of physical components can dramatically increase or decrease cyst formation, unveiling a critical role for microenvironment in PKD...
October 2, 2017: Nature Materials
https://www.readbyqxmd.com/read/28965236/severity-of-diverticulitis-in-patients-with-polycystic-kidney-disease-without-transplantation
#20
Maile E Parker, Kellie L Mathis, Scott R Kelley
BACKGROUND: Patients with polycystic kidney disease (PKD) who have had a kidney transplant have an increased risk of diverticular disease and complicated diverticulitis. Literature is limited regarding the severity of diverticulitis in patients with PKD who have not had a transplant. We aim to assess whether patients with PKD, with and without renal transplant, have a similar course of diverticulitis. METHODS: A retrospective review of all adult PKD patients at our institution diagnosed with diverticulitis between 2000 and 2016 was conducted...
December 2017: International Journal of Colorectal Disease
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