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https://www.readbyqxmd.com/read/29453607/semaphorin-7a-in-circulating-regulatory-t-cells-is-increased-in-autosomal-dominant-polycystic-kidney-disease-and-decreases-with-tolvaptan-treatment
#1
Yashang Lee, Katrina Lehmann Blount, Feng Dai, Siobhan Thompson, Jonathan Kaufman Scher, Sherrie Bitterman, Madeline Droher, Erica L Herzog, Gilbert Moeckel, Anil Karihaloo, Neera K Dahl
BACKGROUND: Semaphorin 7A (SEMA7A) is an immunomodulating protein implicated in lung and liver fibrosis. In autosomal-dominant polycystic kidney disease (ADPKD), the progressive expansion of renal cysts, inflammation, and subsequent renal fibrosis leads to end-stage renal disease (ESRD). SEMA7A may play a role in renal fibrosis and in ADPKD. METHODS: We evaluated Sema7a in a mouse model of renal fibrosis and determined the expression of SEMA7A in human ADPKD kidney...
February 16, 2018: Clinical and Experimental Nephrology
https://www.readbyqxmd.com/read/29444616/endovascular-treatment-of-cerebral-aneurysm-after-renal-transplantation-in-polycystic-kidney-disease
#2
Zeferino Demartini, Jennyfer Galdino, Gelson L Koppe, Alexandre T Bignelli, Alexandre N Francisco, Luana Am Gatto
Background Patients with polycystic kidney disease have a higher prevalence of intracranial aneurysms and may progress to renal failure requiring transplantation. The endovascular treatment of intracranial aneurysms may improve prognosis, since rupture often causes premature death or disability, but the nephrotoxicity risk associated with contrast medium must be always considered in cases of renal impairment. Methods A 55-year-old female patient with polycystic kidney disease and grafted kidney associated with anterior communicant artery aneurysm was successfully treated by embolization...
January 1, 2018: Interventional Neuroradiology
https://www.readbyqxmd.com/read/29440922/is-the-light-at-the-end-of-the-tunnel-nigh-a-review-of-adpkd-focusing-on-the-burden-of-disease-and-tolvaptan-as-a-new-treatment
#3
REVIEW
Rashid A Barnawi, Rahaf Z Attar, Sultan S Alfaer, Osama Y Safdar
Autosomal dominant polycystic kidney disease (ADPKD) causes pathological cystic changes to the kidney and is characterized by numerous renal and systemic manifestations. ADPKD is the fourth most common renal disease requiring renal replacement therapy. In this report, we present a detailed review of ADPKD, with a particular focus on its major economic, psychological, and social burden in affected patients. Treatment of this disease has been based on prophylactic and supportive measures. However, in recent years, new drugs have emerged as promising agents that may retard the progression of ADPKD, such as tolvaptan...
2018: International Journal of Nephrology and Renovascular Disease
https://www.readbyqxmd.com/read/29439650/a-model-to-predict-disease-progression-in-patients-with-autosomal-dominant-polycystic-kidney-disease-adpkd-the-adpkd-outcomes-model
#4
Phil McEwan, Hayley Bennett Wilton, Albert C M Ong, Bjarne Ørskov, Richard Sandford, Francesco Scolari, Maria-Cristina V Cabrera, Gerd Walz, Karl O'Reilly, Paul Robinson
BACKGROUND: Autosomal dominant polycystic kidney disease (ADPKD) is the leading inheritable cause of end-stage renal disease (ESRD); however, the natural course of disease progression is heterogeneous between patients. This study aimed to develop a natural history model of ADPKD that predicted progression rates and long-term outcomes in patients with differing baseline characteristics. METHODS: The ADPKD Outcomes Model (ADPKD-OM) was developed using available patient-level data from the placebo arm of the Tolvaptan Efficacy and Safety in Management of ADPKD and its Outcomes Study (TEMPO 3:4; ClinicalTrials...
February 13, 2018: BMC Nephrology
https://www.readbyqxmd.com/read/29436846/effect-of-dimethyl-fumarate-on-renal-disease-progression-in-a-genetic-ortholog-of-nephronophthisis
#5
Oliver Oey, Padmashree Rao, Magdalena Luciuk, Carly Mannix, Natasha M Rogers, Priyanka Sagar, Annette Wong, Gopala Rangan
Dimethyl fumarate is an FDA-approved oral immunomodulatory drug with anti-inflammatory properties that induces the upregulation of the anti-oxidant transcription factor, nuclear factor erythroid-derived factor 2. The aim of this study was to determine the efficacy of dimethyl fumarate on interstitial inflammation and renal cyst growth in a preclinical model of nephronophthisis. Four-week-old female Lewis polycystic kidney disease (a genetic ortholog of human nephronophthisis-9) rats received vehicle (V), 10 mg/kg (D10) or 30 mg/kg (D30) ( n = 8-9 each) dimethyl fumarate in drinking water for eight weeks...
January 1, 2018: Experimental Biology and Medicine
https://www.readbyqxmd.com/read/29430193/tolvaptan-in-the-treatment-of-autosomal-dominant-polycystic-kidney-disease-patient-selection-and-special-considerations
#6
REVIEW
Laia Sans-Atxer, Dominique Joly
Standard of care therapies for autosomal dominant polycystic kidney disease (ADPKD) may limit morbidity and mortality due to disease-related complications, but they do not delay disease progression. Tolvaptan, a selective vasopressin V2 receptor antagonist, delays the increase in kidney volume (a surrogate marker for disease progression), slows the decline in renal function, and reduces pain in ADPKD patients with relatively preserved renal function. The most common adverse events of tolvaptan are linked to its aquaretic effect, and rare cases of idiosyncratic hepatitis were observed...
2018: International Journal of Nephrology and Renovascular Disease
https://www.readbyqxmd.com/read/29429209/-current-advances-in-pathogenesis-of-autosomal-recessive-polycystic-kidney-disease
#7
X F Shen, Y C Xu, J L Meng
No abstract text is available yet for this article.
February 2, 2018: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
https://www.readbyqxmd.com/read/29426897/a-cleavage-product-of-polycystin-1-is-a-mitochondrial-matrix-protein-that-affects-mitochondria-morphology-and-function-when-heterologously-expressed
#8
Cheng-Chao Lin, Mahiro Kurashige, Yi Liu, Takeshi Terabayashi, Yu Ishimoto, Tanchun Wang, Vineet Choudhary, Ryan Hobbs, Li-Ka Liu, Ping-Hsien Lee, Patricia Outeda, Fang Zhou, Nicholas P Restifo, Terry Watnick, Haruna Kawano, Shigeo Horie, William Prinz, Hong Xu, Luis F Menezes, Gregory G Germino
Recent studies have reported intrinsic metabolic reprogramming in Pkd1 knock-out cells, implicating dysregulated cellular metabolism in the pathogenesis of polycystic kidney disease. However, the exact nature of the metabolic changes and their underlying cause remains controversial. We show herein that Pkd1 k o /ko renal epithelial cells have impaired fatty acid utilization, abnormal mitochondrial morphology and function, and that mitochondria in kidneys of ADPKD patients have morphological alterations. We further show that a C-terminal cleavage product of polycystin-1 (CTT) translocates to the mitochondria matrix and that expression of CTT in Pkd1 ko/ko cells rescues some of the mitochondrial phenotypes...
February 9, 2018: Scientific Reports
https://www.readbyqxmd.com/read/29425828/outcomes-of-renal-transplant-from-donors-with-polycystic-kidney-disease
#9
REVIEW
Awad Shamali, Sarah Milsom-Mcquillan, Paul Gibbs
INTRODUCTION: Kidneys from donors affected by autosomal-dominant polycystic kidney disease (ADPKD) are, in general, considered unsuitable for transplantation. However, some authors report cases of patients who received kidneys from a deceased ADPKD donor showing encouraging outcomes. Our aim is to provide our experience of a patient with end stage renal failure who received a deceased donor kidney from a 29-year-old who themselves had been diagnosed with ADPKD but well maintained renal function, and to provide a comprehensive review of all the published literature...
February 6, 2018: International Journal of Surgery
https://www.readbyqxmd.com/read/29425281/at1-receptor-antagonism-improves-structural-functional-and-biomechanical-properties-in-resistance-arteries-in-a-rodent-chronic-kidney-disease-model
#10
K J Quek, O Z Ameer, J K Phillips
Background: The renin-angiotensin system, in particular Angiotensin II (AngII), plays a significant role in the pathogenesis of hypertension in chronic kidney disease (CKD). Effects of chronic AT1 receptor antagonism were investigated in a genetic hypertensive rat model of CKD, the Lewis polycystic kidney (LPK) rat. Methods: Mixed-sex LPK and Lewis control rats (total n=31) were split between treated (valsartan 60mg/kg/day p.o. from 4-18 weeks) and vehicle groups...
February 7, 2018: American Journal of Hypertension
https://www.readbyqxmd.com/read/29423204/practical-approaches-to-the-management-of-autosomal-dominant-polycystic-kidney-disease-patients-in-the-era-of-tolvaptan
#11
Roman-Ulrich Müller, Christian S Haas, John A Sayer
Background: Autosomal dominant polycystic kidney disease (ADPKD) is the most common inherited kidney disease worldwide. The renal phenotype is characterized by progressive cystic enlargement of the kidneys leading to a decline in renal function, hypertension and often end-stage renal disease (ESRD). Supportive care with blood pressure control and management of pain, urinary infections and renal stone disease has, until recently, been the mainstay of treatment. With the recent approval of tolvaptan for use in ADPKD, the disease progression may now be targeted specifically...
February 2018: Clinical Kidney Journal
https://www.readbyqxmd.com/read/29420817/glutamine-metabolism-via-glutaminase-1-in-autosomal-dominant-polycystic-kidney-disease
#12
Irfana Soomro, Ying Sun, Zhai Li, Lonnette Diggs, Georgia Hatzivassiliou, Ajit G Thomas, Rana Rais, Barbara S Slusher, Stefan Somlo, Edward Y Skolnik
Background: Metabolism of glutamine by glutaminase 1 (GLS1) plays a key role in tumor cell proliferation via the generation of ATP and intermediates required for macromolecular synthesis. We hypothesized that glutamine metabolism also plays a role in proliferation of autosomal-dominant polycystic kidney disease (ADPKD) cells and that inhibiting GLS1 could slow cyst growth in animal models of ADPKD. Methods: Primary normal human kidney and ADPKD human cyst-lining epithelial cells were cultured in the presence or absence of two pharmacologic inhibitors of GLS1, bis-2-(5-phenylacetamido-1,2,4-thiadiazol-2-yl)ethyl sulfide 3 (BPTES) and CB-839, and the effect on proliferation, cyst growth in collagen and activation of downstream signaling pathways were assessed...
February 5, 2018: Nephrology, Dialysis, Transplantation
https://www.readbyqxmd.com/read/29401581/ganetespib-limits-ciliation-and-cystogenesis-in-autosomal-dominant-polycystic-kidney-disease-adpkd
#13
Anna S Nikonova, Alexander Y Deneka, Anna A Kiseleva, Vladislav Korobeynikov, Anna Gaponova, Ilya G Serebriiskii, Meghan C Kopp, Harvey H Hensley, Tamina N Seeger-Nukpezah, Stefan Somlo, David A Proia, Erica A Golemis
Autosomal-dominant polycystic kidney disease (ADPKD) is associated with progressive formation of renal cysts, kidney enlargement, hypertension, and typically end-stage renal disease. In ADPKD, inherited mutations disrupt function of the polycystins (encoded by PKD1 and PKD2), thus causing loss of a cyst-repressive signal emanating from the renal cilium. Genetic studies have suggested ciliary maintenance is essential for ADPKD pathogenesis. Heat shock protein 90 (HSP90) clients include multiple proteins linked to ciliary maintenance...
January 10, 2018: FASEB Journal: Official Publication of the Federation of American Societies for Experimental Biology
https://www.readbyqxmd.com/read/29395568/-pulmonary-granuloma-in-an-immunodepressed-patient
#14
F Gadby, L Quaesaet, C Eveilleau, A Payet-Charneau, F Couturaud, C Tromeur
INTRODUCTION: Pulmonary alveolar echinococcosis is a rare but potentially severe condition. CASE REPORT: We report the case of a 50-year-old woman suffering from pulmonary alveolar echinococcosis who had had a renal transplant for polycystic liver and kidney disease. A lung opacity was identified radiologically in May 2013. Both broncho-alveolar lavage and bronchial biopsy were uninformative. In January 2014, a follow up CT-scan showed the opacity to be enlarging...
January 27, 2018: Revue des Maladies Respiratoires
https://www.readbyqxmd.com/read/29395486/the-value-of-genetic-testing-in-polycystic-kidney-diseases-illustrated-by-a-family-with-pkd2-and-col4a1-mutations
#15
Emilie Cornec-Le Gall, Fouad T Chebib, Charles D Madsen, Sarah R Senum, Christina M Heyer, Brendan C Lanpher, Marc C Patterson, Robert C Albright, Alan S Yu, Vicente E Torres, Peter C Harris
The diagnosis of autosomal dominant polycystic kidney disease (ADPKD) relies on imaging criteria in the setting of a positive familial history. Molecular analysis, seldom used in clinical practice, identifies a causative mutation in >90% of cases in the genes PKD1, PKD2, or rarely GANAB. We report the clinical and genetic dissection of a 7-generation pedigree, resulting in the diagnosis of 2 different cystic disorders. Using targeted next-generation sequencing of 65 candidate genes in a patient with an ADPKD-like phenotype who lacked the familial PKD2 mutation, we identified a COL4A1 mutation (p...
January 27, 2018: American Journal of Kidney Diseases: the Official Journal of the National Kidney Foundation
https://www.readbyqxmd.com/read/29389391/reprise-tolvaptan-in-advanced-polycystic-kidney-disease
#16
Christina M Wyatt, Yannick Le Meur
No abstract text is available yet for this article.
February 2018: Kidney International
https://www.readbyqxmd.com/read/29388169/a-case-of-unilateral-nephrectomy-performed-for-autosomal-dominant-polycystic-kidney-disease-with-marked-unilateral-enlargement
#17
Shiho Makabe, Hiroshi Kataoka, Tsunenori Kondo, Kazunari Tanabe, Ken Tsuchiya, Kosaku Nitta, Toshio Mochizuki
Autosomal dominant polycystic kidney disease (ADPKD) is characterized by the occurrence of multiple cysts that increase the size of both kidneys, progressively reducing kidney function. Usually the cysts occur bilaterally, and there is no difference in the degree of cyst enlargement between the left and right. Here, we report a case of ADPKD in which kidney size increased markedly on the left side and was accompanied by severe abdominal distension and discomfort. Renal dynamic scintigraphy revealed a severe reduction in function of the left kidney compared with the right...
January 31, 2018: CEN Case Reports
https://www.readbyqxmd.com/read/29388019/volume-reduction-in-enlarged-kidneys-in-autosomal-dominant-polycystic-kidney-disease-adpkd-prior-to-renal-transplant-with-transcatheter-arterial-embolization-tae-a-systematic-review-and-meta-analysis
#18
Wenqing Ye, Maurice D Voss, Sriharsha Athreya
PURPOSE: Symptomatically enlarged kidneys observed in autosomal dominant polycystic kidney disease (ADPKD) patients can lead to compression symptoms and contraindications to renal transplantation. Surgical nephrectomy can be utilized to increase space in the abdomen prior to renal transplantation; however, not all individuals are appropriate candidates for this procedure. Transcatheter arterial embolization (TAE) of the renal arteries can provide a noninvasive way to reduce renal volume in ADPKD...
January 31, 2018: Cardiovascular and Interventional Radiology
https://www.readbyqxmd.com/read/29385372/tolvaptan-in-later-stage-polycystic-kidney-disease
#19
LETTER
(no author information available yet)
No abstract text is available yet for this article.
February 1, 2018: New England Journal of Medicine
https://www.readbyqxmd.com/read/29383633/a-fatal-case-of-amlodipine-toxicity-following-iatrogenic-hypercalcemia
#20
Narges-Sadat Zahed, Hossein Hassanian-Moghaddam, Nasim Zamani
Using calcium salts in management of amlodipine overdose is challenging. A 25-year-old male with known history of adult polycystic kidney disease presented with hypotension, tachycardia, and intact neurological status after ingestion of 450 mg of amlodipine. Immediately, normal saline infusion and norepinephrine were initiated. Two grams of calcium gluconate was injected, followed by intravenous infusion of 1.16 mg/kg/h. The patient was put on insulin-glucose protocol to maintain euglycemia and hyperinsulinemia...
January 30, 2018: Cardiovascular Toxicology
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