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https://www.readbyqxmd.com/read/28545714/anticystogenic-activity-of-a-small-molecule-pak4-inhibitor-may-be-a-novel-treatment-for-autosomal-dominant-polycystic-kidney-disease
#1
Vicki J Hwang, Xia Zhou, Xiaonan Chen, Josephine Trott, Omran Abu Aboud, Kyuhwan Shim, Lai Kuan Dionne, Kenneth J Chmiel, William Senapedis, Erkan Baloglu, Moe R Mahjoub, Xiaogang Li, Robert H Weiss
Autosomal Dominant Polycystic Kidney Disease (ADPKD) is a common hereditary renal disease with no currently available targeted therapies. Based on the established connection between β-catenin signaling and renal ciliopathies, and on data from our and other laboratories showing striking similarities of this disease and cancer, we evaluated the use of an orally bioavailable small molecule, KPT-9274 (a dual inhibitor of the protein kinase PAK4 and nicotinamide phosphoribosyl transferase), for treatment of ADPKD...
May 23, 2017: Kidney International
https://www.readbyqxmd.com/read/28545401/the-effect-of-disease-severity-markers-on-quality-of-life-in-autosomal-dominant-polycystic-kidney-disease-a-systematic-review-meta-analysis-and-meta-regression
#2
Myrte K Neijenhuis, Wietske Kievit, Ronald D Perrone, Jeff A Sloan, Patricia Erwin, Mohammad Hassan Murad, Tom J G Gevers, Marie C Hogan, Joost P H Drenth
BACKGROUND: Little is known about determinants of quality of life (QoL) in autosomal dominant polycystic kidney disease (ADPKD). Recent studies suggest that QoL in ADPKD is determined by more factors than mere renal function. We investigated the effect of ADPKD on QoL and evaluated how Qol is affected by disease severity markers renal function, kidney volume and liver volume. METHODS: We performed a systematic review, meta-analysis and meta-regression analyses of cohort studies and randomized controlled trials investigating patient-reported QoL in adult patients with ADPKD not yet on dialysis...
May 25, 2017: BMC Nephrology
https://www.readbyqxmd.com/read/28540892/aldosterone-synthase-gene-is-not-a-major-susceptibility-gene-for-progression-of-chronic-kidney-disease-in-patients-with-autosomal-dominant-polycystic-kidney-disease
#3
Gnanasambandan Ramanathan, Ramprasad Elumalai, Soundararajan Periyasamy, Bhaskar V K S Lakkakula
Autosomal dominant polycystic kidney disease (ADPKD) is the most common heritable kidney disease and is characterized by bilateral renal cysts. Hypertension is a frequent cause of chronic kidney disease (CKD) and mortality in patients with ADPKD. The aldosterone synthase gene polymorphisms of the renin-angiotensin-aldosterone system have been extensively studied as hypertension candidate genes. The present study is aimed to investigate the potential modifier effect of CYP11B2 gene on the progression of CKD in ADPKD...
May 2017: Saudi Journal of Kidney Diseases and Transplantation
https://www.readbyqxmd.com/read/28535817/safety-and-tolerability-of-autologous-bone-marrow-mesenchymal-stromal-cells-in-adpkd-patients
#4
Atieh Makhlough, Soroosh Shekarchian, Reza Moghadasali, Behzad Einollahi, Seyedeh Esmat Hosseini, Neda Jaroughi, Tina Bolurieh, Hossein Baharvand, Nasser Aghdami
BACKGROUND: Autosomal dominant polycystic kidney disease (ADPKD) is a genetic ciliopathy disease characterized by progressive formation and enlargement of cysts in multiple organs. The kidneys are particularly affected and patients may eventually develop end-stage renal disease (ESRD). We hypothesize that bone marrow mesenchymal stromal cells (BMMSCs) are renotropic and may improve kidney function via anti-apoptotic, anti-fibrotic, and anti-inflammatory effects. In this study, we aim to assess the safety and tolerability of a BMMSC infusion in ADPKD patients...
May 23, 2017: Stem Cell Research & Therapy
https://www.readbyqxmd.com/read/28535524/genetics-and-autosomal-dominant-polycystic-kidney-disease-progression
#5
Valentina Corradi, Anna Giuliani, Fiorella Gastaldon, Massimo de Cal, Barbara Mancini, Anna Montaldi, Alberta Alghisi, Irene Capelli, Gaetano La Manna, Claudio Ronco
Autosomal dominant polycystic kidney disease (ADPKD) is the most common hereditary kidney disease, accounting for 10% of European patients on renal replacement therapy. In the previous years, many approaches to slow the progression of ADPKD were studied and many clinical trials published. In addition to having diagnostic role, the description of the genotype is even important to predict the progression of the disease and contributes, combined with several other factors, to a more precise patients classification...
2017: Contributions to Nephrology
https://www.readbyqxmd.com/read/28532709/image-texture-features-predict-renal-function-decline-in-patients-with-autosomal-dominant-polycystic-kidney-disease
#6
Timothy L Kline, Panagiotis Korfiatis, Marie E Edwards, Kyongtae T Bae, Alan Yu, Arlene B Chapman, Michal Mrug, Jared J Grantham, Douglas Landsittel, William M Bennett, Bernard F King, Peter C Harris, Vicente E Torres, Bradley J Erickson
Magnetic resonance imaging (MRI) examinations provide high-resolution information about the anatomic structure of the kidneys and are used to measure total kidney volume (TKV) in patients with Autosomal Dominant Polycystic Kidney Disease (ADPKD). Height-adjusted TKV (HtTKV) has become the gold-standard imaging biomarker for ADPKD progression at early stages of the disease when estimated glomerular filtration rate (eGFR) is still normal. However, HtTKV does not take advantage of the wealth of information provided by MRI...
May 20, 2017: Kidney International
https://www.readbyqxmd.com/read/28522688/polycystic-kidney-disease-without-an-apparent-family-history
#7
Ioan-Andrei Iliuta, Vinusha Kalatharan, Kairong Wang, Emilie Cornec-Le Gall, John Conklin, Marina Pourafkari, Ryan Ting, Chen Chen, Alessia C Borgo, Ning He, Xuewen Song, Christina M Heyer, Sarah R Senum, Young-Hwan Hwang, Andrew D Paterson, Peter C Harris, Korosh Khalili, York Pei
The absence of a positive family history (PFH) in 10%-25% of patients poses a diagnostic challenge for autosomal dominant polycystic kidney disease (ADPKD). In the Toronto Genetic Epidemiology Study of Polycystic Kidney Disease, 210 affected probands underwent renal function testing, abdominal imaging, and comprehensive PKD1 and PKD2 mutation screening. From this cohort, we reviewed all patients with and without an apparent family history, examined their parental medical records, and performed renal imaging in all available parents of unknown disease status...
May 18, 2017: Journal of the American Society of Nephrology: JASN
https://www.readbyqxmd.com/read/28522687/integrin-linked-kinase-signaling-promotes-cyst-growth-and-fibrosis-in-polycystic-kidney-disease
#8
Archana Raman, Gail A Reif, Yuqiao Dai, Aditi Khanna, Xiaogang Li, Lindsay Astleford, Stephen C Parnell, James P Calvet, Darren P Wallace
Autosomal dominant polycystic kidney disease (ADPKD) is characterized by innumerous fluid-filled cysts and progressive deterioration of renal function. Previously, we showed that periostin, a matricellular protein involved in tissue repair, is markedly overexpressed by cyst epithelial cells. Periostin promotes cell proliferation, cyst growth, interstitial fibrosis, and the decline in renal function in PKD mice. Here, we investigated the regulation of these processes by the integrin-linked kinase (ILK), a scaffold protein that links the extracellular matrix to the actin cytoskeleton and is stimulated by periostin...
May 18, 2017: Journal of the American Society of Nephrology: JASN
https://www.readbyqxmd.com/read/28515418/automatic-segmentation-of-kidneys-using-deep-learning-for-total-kidney-volume-quantification-in-autosomal-dominant-polycystic-kidney-disease
#9
Kanishka Sharma, Christian Rupprecht, Anna Caroli, Maria Carolina Aparicio, Andrea Remuzzi, Maximilian Baust, Nassir Navab
Autosomal Dominant Polycystic Kidney Disease (ADPKD) is the most common inherited disorder of the kidneys. It is characterized by enlargement of the kidneys caused by progressive development of renal cysts, and thus assessment of total kidney volume (TKV) is crucial for studying disease progression in ADPKD. However, automatic segmentation of polycystic kidneys is a challenging task due to severe alteration in the morphology caused by non-uniform cyst formation and presence of adjacent liver cysts. In this study, an automated segmentation method based on deep learning has been proposed for TKV computation on computed tomography (CT) dataset of ADPKD patients exhibiting mild to moderate or severe renal insufficiency...
May 17, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28509136/primary-hepatic-leiomyosarcoma-in-a-patient-with-autosomal-dominant-polycystic-kidney-disease
#10
Takashi Iida, Tamaki Maeda, Yoshifumi Amari, Takatomi Yurugi, Yoshitane Tsukamoto, Fumitaka Nakajima
Primary hepatic leiomyosarcoma is an extremely rare tumor. The diagnosis is difficult, and its etiologic factors have not been clarified. A 63-year-old woman with numerous cysts in her kidneys and liver was diagnosed with autosomal dominant polycystic kidney disease (ADPKD). Several members of her family also had ADPKD. She underwent treatment with tolvaptan to inhibit cyst growth and slow the decline in kidney function. Eight months after the start of the therapy, she was hospitalized with fatigue and fever of unknown origin...
May 2017: CEN Case Reports
https://www.readbyqxmd.com/read/28509130/acute-pulmonary-thromboembolism-occurring-during-treatment-with-tolvaptan-in-a-patient-with-autosomal-dominant-polycystic-kidney-disease
#11
Katsuhiko Morimoto, Yasuhiro Akai, Masaru Matsui, Hiroki Yano, Miho Tagawa, Ken-Ichi Samejima, Yoshihiko Saito
Autosomal-dominant polycystic kidney disease (ADPKD) is the most prevalent cystic kidney disease, with approximately half of the patients reaching end-stage renal disease by the age of 60. Tolvaptan prevents renal cyst growth by inhibiting intracellular cyclic AMP and is recommended for patients with ADPKD. Reports of thrombotic complications with tolvaptan have been limited. We report a case of a 60-year-old man who developed thromboembolisms during tolvaptan treatment for ADPKD. The patient started tolvaptan in July 2014...
May 2017: CEN Case Reports
https://www.readbyqxmd.com/read/28509123/serum-calcitriol-levels-in-a-patient-with-x-linked-hypophosphatemia-complicated-by-autosomal-dominant-polycystic-kidney-disease
#12
Satomi Kajita, Takehisa Yamamoto, Naoko Tsugawa, Hirohumi Nakayama, Takuo Kubota, Toshimi Michigami, Keiichi Ozono
Serum calcitriol [1,25(OH)2D] levels are low normal in the presence of hypophosphatemia in X-linked dominant hypophosphatemic rickets (XLH) due to elevated serum fibroblast growth factor 23 (FGF23) levels. We report a peculiar finding of markedly elevated serum 1,25(OH)2D levels in a patient with XLH complicated by autosomal dominant polycystic kidney disease (ADPKD) and retinitis pigmentosa (RP). She was diagnosed with XLH, ADPKD and RP at the age of 5, 13 and 15 years, respectively. After the diagnosis of ADPKD, the 1,25(OH)2D levels increased up to 282 pg/mL treated with a pharmacological dose of 1αOHD3 (1αOHD3)...
May 2017: CEN Case Reports
https://www.readbyqxmd.com/read/28508983/effect-of-tolvaptan-in-a-patient-with-autosomal-dominant-polycystic-kidney-disease-after-living-donor-liver-transplantation
#13
Kiyotaka Uchiyama, Kazuya Honda, Ryochi Yoshida, Yuka Kamijo, Mai Yanagi, Mineo Nakatsuka, Yoshitaka Ishibashi
Recently, a large randomized placebo-controlled trial indicated a beneficial effect of tolvaptan on the progression of autosomal dominant polycystic kidney disease (ADPKD) with near-normal kidney function. Meanwhile, the evidence of tolvaptan's efficacy in ADPKD with severe renal insufficiency was limited and higher frequency of liver enzyme elevations were observed in patients taking tolvaptan. Liver transplantation (LT) is the only curative treatment for patients with severe polycystic liver disease associated with ADPKD, but considering that liver injuries should be avoided particularly in patients who underwent LT, we must be careful to start tolvaptan in post-LT ADPKD patients...
November 2016: CEN Case Reports
https://www.readbyqxmd.com/read/28508961/bacteremic-kidney-cyst-infection-caused-by-helicobacter-cinaedi
#14
Kenta Ito, Takumi Yamamoto, Haruomi Nishio, Asako Sawaya, Masaaki Murakami, Akiko Kitagawa, Yoko Matsuo, Ken Matsuo, Satoshi Tanaka, Noriko Mori
Cyst infection is one of the major complications in patients with autosomal dominant polycystic kidney disease (ADPKD). The causative pathogen in kidney cyst infection frequently goes undetected. Although only one case report of kidney cyst infection caused by Helicobacter cinaedi (H. cinaedi) is published in English literature, it may be an important pathogen in kidney cyst infection. Kidney cyst infection and H. cinaedi infection share the common characteristic of tendency to relapse and chronic kidney disease is a major risk factor for H...
November 2016: CEN Case Reports
https://www.readbyqxmd.com/read/28502970/is-regular-screening-for-intracranial-aneurysm-necessary-in-patients-with-autosomal-dominant-polycystic-kidney-disease-a-systematic-review-and-meta-analysis
#15
Zien Zhou, Ying Xu, Candice Delcourt, Jiehui Shan, Qiang Li, Jianrong Xu, Maree L Hackett
BACKGROUND: The prevalence of intracranial aneurysm in patients with autosomal dominant polycystic kidney disease (ADPKD) is higher than that among the general population. We performed a systematic review and meta-analysis on the prevalence and natural history of intracranial aneurysm among patients with ADPKD. METHODS: Medline, Embase, Web of Science and Scopus, from inception to July 2016, were searched for studies reporting the occurrence of intracranial aneurysms among participants with ADPKD...
May 13, 2017: Cerebrovascular Diseases
https://www.readbyqxmd.com/read/28502323/a-case-report-on-the-exceptional-coincidence-of-two-inherited-renal-disorders-adpkd-and-alport-syndrome%C3%A2
#16
Kathrin Ebner, Nadine Reintjes, Markus Feldkötter, Friederike Körber, Mato Nagel, Jörg Dötsch, Bernd Hoppe, Lutz Thorsten Weber, Bodo B Beck, Max C Liebau
Autosomal dominant polycystic kidney disease (ADPKD) is the most common genetic cause of dialysis-requiring end-stage renal disease in adults and is characterized by the slowly progressing replacement of renal tissue by focal macrocysts. Alport syndrome (AS; hereditary nephritis) is a rare, inherited disorder of the basement membrane associated with hematuria, proteinuria, and loss of kidney function as well as sensorineural hearing loss and ocular abnormalities. Here, we report on a family in which both ADPKD and AS are present...
May 15, 2017: Clinical Nephrology
https://www.readbyqxmd.com/read/28494735/perspectives-of-gene-therapies-in-autosomal-dominant-polycystic-kidney-disease
#17
Yuchen Xu, Ao Li, Guanqing Wu, Chaozhao Liang
Autosomal dominant polycystic kidney disease (ADPKD) is the most common inherited kidney disease in the clinic. The predominant clinical manifestation is bilateral and progressive cysts formation in the kidneys, impairs normal renal parenchyma, and ultimately leads to end-stage renal disease (ESRD). ADPKD is a heterogenic disease which is resulted from the mutations of PKD1 or PKD2 genes which encode polycystin-1 (PC1) and -2 (PC2), thereby multiple cell signaling pathways are involved. Although causative genes and aberrant signaling pathways have been investigated for decades, lack of effective and less side-effect treatment for the disease still perplex vast clinicians...
May 10, 2017: Current Gene Therapy
https://www.readbyqxmd.com/read/28490895/healthcare-resource-consumption-and-cost-of-care-among-patients-with-polycystic-kidney-disease-in-italy
#18
Luca Degli Esposti, Chiara Veronesi, Valentina Perrone, Stefano Buda, Antonio Santoro
OBJECTIVE: The aim of this study was to assess healthcare resource consumption and its associated costs among patients with polycystic kidney disease (PKD) in a real-world setting. METHODS: An observational retrospective cohort analysis was conducted using data from the administrative databases of four Italian local health units. Data for patients who were diagnosed with PKD during the inclusion period (January 1, 2010 to December 31, 2012) were extracted. The date on which a patient's first PKD hospitalization occurred during the inclusion period was defined as the index date (ID), and the ID was defined as the date of the first dialysis treatment recorded during the inclusion period for patients undergoing dialysis...
2017: ClinicoEconomics and Outcomes Research: CEOR
https://www.readbyqxmd.com/read/28473970/emerging-therapies-for-childhood-polycystic-kidney-disease
#19
REVIEW
William E Sweeney, Ellis D Avner
Cystic kidney diseases comprise a varied collection of hereditary disorders, where renal cysts comprise a major element of their pleiotropic phenotype. In pediatric patients, the term polycystic kidney disease (PKD) commonly refers to two specific hereditary diseases, autosomal recessive polycystic kidney disease (ARPKD) and autosomal dominant polycystic kidney disease (ADPKD). Remarkable progress has been made in understanding the complex molecular and cellular mechanisms of renal cyst formation in ARPKD and ADPKD...
2017: Frontiers in Pediatrics
https://www.readbyqxmd.com/read/28467389/on-the-many-actions-of-ouabain-pro-cystogenic-effects-in-autosomal-dominant-polycystic-kidney-disease
#20
REVIEW
Jessica Venugopal, Gustavo Blanco
Ouabain and other cardenolides are steroidal compounds originally discovered in plants. Cardenolides were first used as poisons, but after finding their beneficial cardiotonic effects, they were rapidly included in the medical pharmacopeia. The use of cardenolides to treat congestive heart failure remained empirical for centuries and only relatively recently, their mechanisms of action became better understood. A breakthrough came with the discovery that ouabain and other cardenolides exist as endogenous compounds that circulate in the bloodstream of mammals...
May 3, 2017: Molecules: a Journal of Synthetic Chemistry and Natural Product Chemistry
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