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https://www.readbyqxmd.com/read/28087011/acute-aortic-syndrome-in-autosomal-dominant-polycystic-kidney-disease
#1
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
#2
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
https://www.readbyqxmd.com/read/28078995/sphingolipids-in-genetic-and-acquired-forms-of-chronic-kidney-diseases
#3
Norishi Ueda
Sphingolipids (SLs) regulate apoptosis, proliferation, and stress response. SLs, including ceramide, glycosphingolipids (glucosylceramide, lactosylceramide, and gangliosides) and sphingosine-1-phosphate (S1P), play a role in the pathogenesis and progression of genetic (lysosomal storage disease, congenital nephrotic syndrome and polycystic kidney disease) and non-genetic forms of chronic kidney diseases (CKDs). SLs metabolism defects promote complications (cardiovascular events, etc.) via oxidant stress in CKDs...
January 12, 2017: Current Medicinal Chemistry
https://www.readbyqxmd.com/read/28077374/parallel-microarray-profiling-identifies-erbb4-as-a-determinant-of-cyst-growth-in-adpkd-and-a-prognostic-biomarker-for-disease-progression
#4
Andrew J Streets, Tajdida A Magayr, Linghong Huang, Laura Vergoz, Sandro Rossetti, Roslyn J Simms, Peter C Harris, Dorien J M Peters, Albert Cm Ong
Autosomal Dominant Polycystic Kidney Disease (ADPKD) is the fourth most common cause of end-stage renal disease. The disease course can be highly variable and treatment options are limited. To identify new therapeutic targets and prognostic biomarkers of disease, we conducted parallel discovery microarray profiling in normal and diseased human PKD1 cystic kidney cells. A total of 1515 genes and 5 miRNA were differentially expressed by more than two-fold in PKD1 cells. Functional enrichment analysis identified 30 dysregulated signalling pathways including the epidermal growth factor (EGF) receptor pathway...
January 11, 2017: American Journal of Physiology. Renal Physiology
https://www.readbyqxmd.com/read/28072739/risk-factors-and-outcomes-associated-with-a-higher-use-of-inotropes-in-kidney-transplant-recipients
#5
Jae Moon Choi, Jun-Young Jo, Jae-Won Baik, Sooyoung Kim, Chan Sik Kim, Sung-Moon Jeong
Preservation of adequate perfusion pressures to the graft is a main focus of intraoperative management during kidney transplantation. We undertook this study to investigate the incidence of the higher use of inotropes in kidney transplant recipients and identify the patient outcomes and preoperative and intraoperative variables related to this.We retrospectively analyzed 1053 patients who underwent kidney transplantation at Asan Medical Center between January 2006 and February 2012, stratified by their inotropic score ([dopamine] + [dobutamine] + [epinephrine × 100] + [norepinephrine × 100]) <7 versus ≥7, wherein all doses are expressed as μg/kg/min...
January 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28063418/focal-segmental-glomerulosclerosis-in-atypical-polycystic-kidney-disease
#6
Eun Hui Bae, Hyunsuk Kim, Sung Sun Kim, Seong Kwon Ma, Soo Wan Kim
No abstract text is available yet for this article.
January 5, 2017: Korean Journal of Internal Medicine
https://www.readbyqxmd.com/read/28058003/ruptured-emphysematous-renal-cyst
#7
Abdelrahman Yahya Mohamed, Fahad Quhal
Renal Cyst infections are rare, account for approximately 10% of causes for hospitalization in patients with adult polycystic kidney disease. We reported a rare case of ruptured emphysematous renal cyst with infection extending into the right perinephric space with gas forming organism involving the renal collecting system and the urinary bladder.
October 2016: Urology Annals
https://www.readbyqxmd.com/read/28057871/copeptin-a-surrogate-marker-for-arginine-vasopressin-is-associated-with-disease-severity-and-progression-in-iga-nephropathy-patients
#8
Debbie Zittema, Jan A J G van den Brand, Stephan J L Bakker, Jack F Wetzels, Ron T Gansevoort
BACKGROUND: Besides its essential role for water homeostasis, arginine vasopressin (AVP) may have deleterious effects on the kidney. Copeptin, a surrogate marker for AVP, has been shown to be related to renal outcome in patients with diabetic nephropathy and polycystic kidney disease. We investigated the association of copeptin with disease severity and progression in immunoglobulin A nephropathy (IgAN). METHODS: We included a prospective cohort of 59 patients with biopsy proven IgAN...
January 5, 2017: Nephrology, Dialysis, Transplantation
https://www.readbyqxmd.com/read/28050373/histopathologic-findings-in-autopsies-with-emphasis-on-interesting-and-incidental-findings-a-pathologist-s-perspective
#9
Sapna Patel, B R Rajalakshmi, G V Manjunath
INTRODUCTION: Autopsy aids to the knowledge of pathology by unveiling the rare lesions which are a source of learning from a pathologist's perspective Some of them are only diagnosed at autopsy as they do not cause any functional derangement. This study emphasizes the various incidental lesions which otherwise would have been unnoticed during a person's life. AIM: The aim of this study was to determine the spectrum of histopathological findings including neoplastic lesions related or unrelated to the cause of death...
November 2016: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28049233/baseline-cardiovascular-characteristics-of-adult-patients-with-chronic-kidney-disease-from-the-korean-cohort-study-for-outcomes-in-patients-with-chronic-kidney-disease-know-ckd
#10
Hyoungnae Kim, Tae Hyun Yoo, Kyu Hun Choi, Kook Hwan Oh, Joongyub Lee, Soo Wan Kim, Tae Hee Kim, Suah Sung, Seung Hyeok Han
Cardiovascular disease (CVD) is the most common cause of death in patients with chronic kidney disease (CKD). We report the baseline cardiovascular characteristics of 2,238 participants by using the data of the KoreaN Cohort Study for Outcomes in Patients With Chronic Kidney Disease (KNOW-CKD) study. The cohort comprises 5 subcohorts according to the cause of CKD: glomerulonephritis (GN), diabetic nephropathy (DN), hypertensive nephropathy (HTN), polycystic kidney disease (PKD), and unclassified. The average estimated glomerular filtration rate (eGFR) was 50...
February 2017: Journal of Korean Medical Science
https://www.readbyqxmd.com/read/28049232/baseline-general-characteristics-of-the-korean-chronic-kidney-disease-report-from-the-korean-cohort-study-for-outcomes-in-patients-with-chronic-kidney-disease-know-ckd
#11
Eunjeong Kang, Miyeun Han, Hyunsuk Kim, Sue Kyung Park, Joongyub Lee, Young Youl Hyun, Yong Soo Kim, Wookyung Chung, Hyo Jin Kim, Yun Kyu Oh, Curie Ahn, Kook Hwan Oh
The KoreaN Cohort Study for Outcomes in Patients With Chronic Kidney Disease (KNOW-CKD) was developed to investigate various clinical courses and risk factors for progression of Korean chronic kidney disease (CKD). The KNOW-CKD study consists of nine clinical centers in Korea, and patients aged between 20 and 75 years with CKD from stage 1 to 5 (predialysis) were recruited. At baseline, blood and urine samples were obtained and demographic data including comorbidities, drugs, quality of life, and health behaviors were collected...
February 2017: Journal of Korean Medical Science
https://www.readbyqxmd.com/read/28039480/nutrient-induced-fnip-degradation-by-scf%C3%AE-trcp-regulates-flcn-complex-localization-and-promotes-renal-cancer-progression
#12
Katsuyuki Nagashima, Hidefumi Fukushima, Kouhei Shimizu, Aya Yamada, Masumi Hidaka, Hisashi Hasumi, Tetsuro Ikebe, Satoshi Fukumoto, Koji Okabe, Hiroyuki Inuzuka
Folliculin-interacting protein 1 and 2 (FNIP1 and FNIP2) play critical roles in preventing renal malignancy through their association with the tumor suppressor FLCN. Mutations in FLCN are associated with Birt-Hogg-Dubé (BHD) syndrome, a rare disorder with increased risk of renal cancer. Recent studies indicated that FNIP1/FNIP2 double knockout mice display enlarged polycystic kidneys and renal carcinoma, which phenocopies FLCN knockout mice, suggesting that these two proteins function together to suppress renal cancer...
December 25, 2016: Oncotarget
https://www.readbyqxmd.com/read/28038444/an-association-between-autosomal-dominant-polycystic-kidney-disease-and-the-risk-of-acute-myocardial-infarction-in-asian-population-results-of-a-nationwide-study
#13
Pei-Hsun Sung, Hsin-Ju Chiang, Yao-Hsu Yang, Chi-Jen Chen, John Y Chiang, Hon-Kan Yip
Cardiovascular complications are the leading causes of death in patients with autosomal-dominant polycystic kidney disease (ADPKD) in the Western countries. However, theprevalence and risk of acute myocardial infarction (AMI) in patients with ADPKD remain unknown, especially in Asian population. We utilized the data from Taiwan National Health Insurance Research Database (NHIRD) to perform a population-based cohort study (1997-2008). A total of 2062 patients with ADPKD were selected from one million of general population after excluding those patients with age less than 18 years old, receiving renal replacement therapy, and concomitant diagnoses of AMI...
December 27, 2016: Oncotarget
https://www.readbyqxmd.com/read/28029746/respiratory-manifestations-in-38-patients-with-alstr%C3%A3-m-syndrome
#14
Caroline Boerwinkle, Jan D Marshall, Joy Bryant, William A Gahl, Kenneth N Olivier, Meral Gunay-Aygun
OBJECTIVES: Alström syndrome (AS) is a rare, multi-system condition characterized by retinal degeneration, sensorineural hearing loss, obesity, insulin-resistant diabetes, hypertriglyceridemia, cardiomyopathy, hepatorenal disease, and recurrent respiratory infections. It belongs to a group of genetic disorders known as primary ciliopathies, which includes autosomal dominant and recessive polycystic kidney diseases, as well as Joubert and Bardet-Biedl syndromes. Prior studies have suggested phenotypic overlap between primary ciliopathies affecting the non-motile, sensory cilia, and primary ciliary dyskinesia (PCD), a motile ciliopathy characterized by respiratory tract disease...
December 28, 2016: Pediatric Pulmonology
https://www.readbyqxmd.com/read/28024168/carbohydrate-antigen-19-9-is-significantly-elevated-in-autosomal-dominant-polycystic-kidney-disease
#15
Hirotaka Fukasawa, Mai Kaneko, Hiroki Niwa, Hideo Yasuda, Hiromichi Kumagai, Ryuichi Furuya
AIM: Liver cysts are the most common extrarenal manifestation in patients with autosomal dominant polycystic kidney disease (ADPKD). Carbohydrate antigen 19-9 (CA19-9) is generally used as a marker for biliopancreatic malignancies, although CA19-9 levels in patients with ADPKD are largely unknown. METHODS: A prospective observational study of 53 ADPKD patients and 83 non-ADPKD control subjects was performed. The serum levels of CA19-9 were studied to evaluate the association with clinical parameters and liver cysts...
December 26, 2016: Nephrology
https://www.readbyqxmd.com/read/28018103/portal-hypertension-in-polycystic-liver-disease-patients-does-not-affect-wait-list-or-immediate-post-liver-transplantation-outcomes
#16
Neil Rajoriya, Dhiraj Tripathi, Joanna A Leithead, Bridget K Gunson, Sophie Lord, James W Ferguson, Gideon M Hirschfield
AIM: To establish the impact of portal hypertension (PH) on wait-list/post-transplant outcomes in patients with polycystic liver disease (PCLD) listed for liver transplantation. METHODS: A retrospective single-centre case controlled study of consecutive patients listed for liver transplantation over 12 years was performed from our centre. PH in the PCLD cohort was defined by the one or more of following parameters: (1) presence of radiological or endoscopic documented varices from our own centre or the referral centre; (2) splenomegaly (> 11 cm) on radiology in absence of splenic cysts accounting for increased imaging size; (3) thrombocytopenia (platelets < 150 × 10(9)/L); or (4) ascites without radiological evidence of hepatic venous outflow obstruction from a single cyst...
December 7, 2016: World Journal of Gastroenterology: WJG
https://www.readbyqxmd.com/read/28011630/a-polycycstin-type-transient-receptor-potential-trp-channel-that-is-activated-by-atp
#17
David Traynor, Robert R Kay
ATP and ADP are ancient extra-cellular signalling molecules that in Dictyostelium amoebae cause rapid, transient increases in cytosolic calcium due to an influx through the plasma membrane. This response is independent of hetero-trimeric G-proteins, the putative IP3 receptor IplA and all P2X channels. We show, unexpectedly, that it is abolished in mutants of the polycystin-type transient receptor potential channel, TrpP. Responses to the chemoattractants cyclic-AMP and folic acid are unaffected in TrpP mutants...
December 23, 2016: Biology Open
https://www.readbyqxmd.com/read/28007903/autophagy-activators-suppress-cystogenesis-in-an-autosomal-dominant-polycystic-kidney-disease-model
#18
Ping Zhu, Cynthia J Sieben, Xiaolei Xu, Peter C Harris, Xueying Lin
Autosomal dominant polycystic kidney disease (ADPKD) is caused by mutations in either PKD1 or PKD2 It is one of the most common heritable human diseases with eventual development of renal failure; however, effective treatment is lacking. While inhibition of mechanistic target of rapamycin (mTOR) effectively slows cyst expansions in animal models, results from clinical studies are controversial, prompting further mechanistic studies of mTOR-based therapy. Here, we aim to establish autophagy, a downstream pathway of mTOR, as a new therapeutic target for PKD...
December 22, 2016: Human Molecular Genetics
https://www.readbyqxmd.com/read/27998045/how-can-a-vascular-surgeon-help-in-kidney-transplantation
#19
Anne Lejay, Fabien Thaveau, Sophie Caillard, Yannick Georg, Bruno Moulin, Philippe Wolf, Bernard Geny, Nabil Chakfe
Kidney transplantation is a surgical procedure involving both vascular and ureteric anastomoses. As a matter of fact, it can be performed either by urologists or vascular surgeons. However, vascular surgeon's expertise can be helpful at different times. In the present paper we describe how can vascular surgeons help at the different stages of kidney transplantation process in modern care: 1) before kidney transplantation for recipient preparation in order to allow subsequent graft implantation, either by performing percutaneous embolization of renal arteries in the setting of polycystic kidney disease or treatment of aneurysmal or occlusive lesions that would contra-indicate graft implantation; 2) at the time of surgery graft back table preparation and repair; and 3) after surgery for long- term follow-up, including transplant renal artery stenosis treatment or transplant nephrectomy...
December 19, 2016: Journal of Cardiovascular Surgery
https://www.readbyqxmd.com/read/27995519/hepatic-cyst-infection-during-use-of-the-somatostatin-analog-lanreotide-in-autosomal-dominant-polycystic-kidney-disease-an-interim-analysis-of-the-randomized-open-label-multicenter-dipak-1-study
#20
Marten A Lantinga, Hedwig M A D'Agnolo, Niek F Casteleijn, Johan W de Fijter, Esther Meijer, Annemarie L Messchendorp, Dorien J M Peters, Mahdi Salih, Edwin M Spithoven, Darius Soonawala, Folkert W Visser, Jack F M Wetzels, Robert Zietse, Joost P H Drenth, Ron T Gansevoort
INTRODUCTION AND AIMS: The DIPAK-1 Study investigates the reno- and hepatoprotective efficacy of the somatostatin analog lanreotide compared with standard care in patients with later stage autosomal dominant polycystic kidney disease (ADPKD). During this trial, we witnessed several episodes of hepatic cyst infection, all during lanreotide treatment. We describe these events and provide a review of the literature. METHODS: The DIPAK-1 Study is an ongoing investigator-driven, randomized, controlled, open-label multicenter trial...
December 19, 2016: Drug Safety: An International Journal of Medical Toxicology and Drug Experience
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