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https://www.readbyqxmd.com/read/29234037/dynamic-regulation-of-trek1-gating-by-polycystin-2-via-a-filamin-a-mediated-cytoskeletal-mechanism
#1
Steven Li Fraine, Amanda Patel, Fabrice Duprat, Reza Sharif-Naeini
Mechanosensing is essential for several physiological functions including touch and pain sensations, osmoregulation, and controlling the myogenic tone of resistance arteries. Understanding how mechanosensitive ion channels (MSCs) are gated can provide important information regarding these processes. We have previously demonstrated that during pathological conditions such as polycystic kidney disease, polycystin 2 (TRPP2) inhibits the activity of potassium-selective MSCs through a filamin A-mediated cytoskeletal effect, and renders tubular epithelial cells susceptible to apoptosis...
December 12, 2017: Scientific Reports
https://www.readbyqxmd.com/read/29230688/therapeutic-effects-of-histone-deacetylase-inhibitors-on-kidney-disease
#2
REVIEW
Pusoon Chun
Increasing evidence has shown the involvement of histone deacetylases (HDACs) in the development and progression of various renal diseases, highlighting its inhibition as a promising therapeutic strategy to prevent kidney diseases. Accordingly, numerous studies have shown that HDAC inhibitors protect the kidneys from various diseases through their effects on multiple pathways, such as suppression of transforming growth factor-β signaling pathway and nuclear factor-κB signaling pathways, augmentation of apoptosis, and inhibition of angiogenesis...
December 11, 2017: Archives of Pharmacal Research
https://www.readbyqxmd.com/read/29230552/identification-of-clustered-phosphorylation-sites-in-pkd2l1-how-pkd2l1-channel-activation-is-regulated-by-cyclic-adenosine-monophosphate-signaling-pathway
#3
Eunice Yon June Park, Misun Kwak, Kotdaji Ha, Insuk So
Polycystic kidney disease 2-like-1 (PKD2L1), or polycystin-L or TRPP2, formerly TRPP3, is a transient receptor potential (TRP) superfamily member. It is a calcium-permeable non-selective cation channel that regulates intracellular calcium concentration and thereby calcium signaling. PKD2L1 has been reported to take part in hedgehog signaling in renal primary cilia and sour tasting coupling with PKD1L3. In addition to the previous reports, PKD2L1 is recently found to play a crucial role in localization with β2-adrenergic receptor (β2AR) on the neuronal primary cilia...
December 11, 2017: Pflügers Archiv: European Journal of Physiology
https://www.readbyqxmd.com/read/29226648/fibulin-levels-in-autosomal-dominant-polycystic-kidney-disease-and-its-relationship-to-arterial-stiffness
#4
A Inci, O Akin, H Y Ellidag, E Eren, S Dolu, F Sari
BACKGROUND: The objective of the present study was to evaluate fibulin 1 levels in different stages of patients with autosomal dominant polycystic kidney disease (ADPKD) and investigate possible connections between fibulin-1 and arterial stiffness. METHODS: For this cross-sectional study, we included 74 patients with ADPKD (mean age, 50.92 ± 15.70 years) and 32 healthy controls (mean age, 49.53 ± 7.32 years). Patients with ADPKD were classified based on CKD epidemiology collaboration (CKD-EPI) equation assessments of estimated glomerular filtration rate (eGFR)...
November 1, 2017: Clinical Laboratory
https://www.readbyqxmd.com/read/29225875/an-88-year-old-patient-with-adpkd-underscoring-the-importance-of-risk-factor-modification
#5
Abhilash Koratala, Freddy R Malpartida, Amir Kazory
The mean age of onset of end-stage renal disease (ESRD) in autosomal dominant polycystic kidney disease (ADPKD) is between 56 and 69. Our case represents one of the oldest patients with ADPKD, whose chronic kidney disease (CKD) progression rate could be successfully reduced by optimal risk factor modification.
December 2017: Clinical Case Reports
https://www.readbyqxmd.com/read/29224797/kidney-transplant-with-and-without-native-nephrectomy-for-polycystic-kidney-disease-results-of-the-national-inpatient-sample-and-the-rationale-for-a-2-staged-procedure
#6
Raymond A Jean, Mehida Alexandre, Peter S Yoo
BACKGROUND: Polycystic kidney disease (PKD) is one of the most common causes of end-stage renal disease requiring hemodialysis or transplantation. In patients requiring transplant, there are several indications for native nephrectomy, including recurrent cyst infection, bleeding, or to provide room for the graft. There is disagreement about whether it is advisable to perform kidney transplant alone (KT), or to perform kidney transplant with simultaneous native nephrectomy (KTN). We compared postoperative outcomes of KTN and KT in a large national cohort...
December 7, 2017: Journal of the American College of Surgeons
https://www.readbyqxmd.com/read/29217884/how-harmful-can-herbal-remedies-be-a-case-of-severe-acute-tubulointerstitial-nephritis
#7
P Beniwal, N Gaur, S K Singh, N Raveendran, V Malhotra
Acute interstitial nephritis (AIN) is a condition in which acute kidney injury (AKI) is characterized by the histological finding of interstitial inflammation. Hyponidd is an ayurvedic drug containing Momordica charantia, Gymnema sylvestre, Swertia chirata, etc., used for the treatment of Type 2 diabetes mellitus (DM) and polycystic ovarian disease as an insulin sensitizer. There are no case reports of AIN caused by this drug yet. We report a biopsy-proven case of AKI due to severe AIN associated with the use of hyponidd tablet in a 60-year-old male with DM and hypertension...
November 2017: Indian Journal of Nephrology
https://www.readbyqxmd.com/read/29217372/acute-peritoneal-dialysis-in-neonatal-intensive-care-unit-an-8-year-experience-of-a-referral-hospital
#8
Aslihan Kara, Metin Kaya Gurgoze, Mustafa Aydin, Erdal Taskin, Unal Bakal, Aysen Orman
BACKGROUND: The aim of present study was to evaluate the indications, complications and outcomes of acute peritoneal dialysis (APD) in neonates at a referral university hospital during the previous 8 years. METHODS: This retrospective analysis included a total of 52 newborn infants who underwent APD in a neonatal intensive care unit between January 2008 and March 2016. Demographic, clinical, laboratory and microbiological data were extracted from patients' medical files...
November 16, 2017: Pediatrics and Neonatology
https://www.readbyqxmd.com/read/29215110/quercetin-inhibits-renal-cyst-growth-in-vitro-and-via-parenteral-injection-in-a-polycystic-kidney-disease-mouse-model
#9
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/29208861/etiology-of-early-renal-allograft-dysfunction-and-comparison-between-dysfunction-and-function-group-a-single-center-study
#10
K M Hadiuzzaman, S F Islam, M O Faroque, R M Hossain, M Munirunnessa, S I Selim, M Morshed
Over a period of two years thirty five renal allograft recipients & donors were evaluated to find out the aetiology of early renal allograft dysfunction, in the Department of Nephrology, Bangabandhu Sheikh Mujib Medical University, Dhaka, Bangladesh from March 2010 to February 2012. A comparison was made between dysfunction & functioning graft group. Mean age of recipients were (36.4±9.4) years, mean age of donors were (41.7±8.3) years, with a male and female ratio of 3:1. Fifty percent recipients showed one heliotype match, ninety percent recipients were anti CMV antibody IgG positive, few were anti CMV antibody IgM positive...
October 2017: Mymensingh Medical Journal: MMJ
https://www.readbyqxmd.com/read/29208045/joubert-s-syndrome-and-related-disorders-and-home-based-peritoneal-dialysis-in-east-africa-a-case-report
#11
Grace M Musiime, Doris M W Kinuthia, Donald P Oyatsi, Wangui Manguyu
BACKGROUND: Joubert's syndrome is a rare condition affecting an estimated 1:80,000-1:100,000 individuals. There is underdevelopment of the cerebellar vermis resulting in a characteristic molar tooth sign on cross sectional axial magnetic resonance imaging. It can occur in association with multi-organ involvement; in such cases it is classified as Joubert's syndrome and related disorders. To date, there are no cases of Joubert's syndrome and related disorders from sub-Saharan Africa described in the literature...
December 6, 2017: BMC Research Notes
https://www.readbyqxmd.com/read/29204517/proteomic-analysis-of-aqp11-null-kidney-proximal-tubular-type-polycystic-kidney-disease
#12
Tatsuya Saito, Yasuko Tanaka, Yoshiyuki Morishita, Kenichi Ishibashi
Autosomal Dominant Polycystic Kidney Disease (ADPKD) is caused by the mutation of polycystins (PC-1 or PC-2), in which cysts start from the collecting duct to extend to all nephron segments with eventual end stage renal failure. The cyst development is attenuated by a vasopressin V2 receptor antagonist tolvaptan which, however, will not affect proximal tubule cysts devoid of V2 receptor. Aquaporin-11 (AQP11) is expressed selectively in the proximal tubule of the kidney and AQP11-null kidneys have a disruptive PC-1 trafficking to the plasma membrane to develop polycystic kidneys...
March 2018: Biochemistry and Biophysics Reports
https://www.readbyqxmd.com/read/29203870/ift-proteins-spatially-control-the-geometry-of-cleavage-furrow-ingression-and-lumen-positioning
#13
Nicolas Taulet, Benjamin Vitre, Christelle Anguille, Audrey Douanier, Murielle Rocancourt, Michael Taschner, Esben Lorentzen, Arnaud Echard, Benedicte Delaval
Cytokinesis mediates the physical separation of dividing cells and, in 3D epithelia, provides a spatial landmark for lumen formation. Here, we unravel an unexpected role in cytokinesis for proteins of the intraflagellar transport (IFT) machinery, initially characterized for their ciliary role and their link to polycystic kidney disease. Using 2D and 3D cultures of renal cells, we show that IFT proteins are required to correctly shape the central spindle, to control symmetric cleavage furrow ingression and to ensure central lumen positioning...
December 4, 2017: Nature Communications
https://www.readbyqxmd.com/read/29200897/insulinoma-and-chronic-kidney-disease-an-uncommon-conundrum-not-to-be-overlooked
#14
Luca Foppiani, Serena Panarello, Marco Filauro, Maria Concetta Scirocco, Stefano Cappato, Andrea Parodi, Simona Sola, Giancarlo Antonucci
A hypertensive man with chronic kidney disease (CKD) secondary to polycystic disease was hospitalized for symptoms related to hypoglycemia. Fasting test elicited symptomatic hypoglycemia after 12 hours, which was associated with inappropriately unsuppressed normal insulin and C-peptide levels. Neither ultrasonography (US) nor magnetic resonance imaging detected any pancreatic tumor. Endoscopic ultrasonography (EUS) showed a small isoechogenic nodule suspect for neuroendocrine tumor in the pancreatic head...
2017: Clinical Medicine Insights. Endocrinology and Diabetes
https://www.readbyqxmd.com/read/29195058/essential-role-of-ift140-in-promoting-dentinogenesis
#15
G Li, M Liu, S Zhang, H Wan, Q Zhang, R Yue, X Yan, X Wang, Z Wang, Y Sun
Primary cilia, with highly regulated cellular sensory functions, play key roles in tissue development and function maintenance. Intraflagellar transport 140 (IFT140) is a subunit of IFT complex A, which is specialized for retrograde transportation in cilia. Mutations of Ift140 are usually associated with syndromic ciliopathy and may cause isolated diseases such as retinal dystrophy, short ribs, and polycystic kidney. However, the role of IFT140 in tooth development has not been well investigated. In this study, a close relationship between IFT140 and dentin formation is disclosed...
November 1, 2017: Journal of Dental Research
https://www.readbyqxmd.com/read/29181208/large-retroperitoneal-haemorrhage-following-cyst-rupture-in-a-patient-with-autosomal-dominant-polycystic-kidney-disease
#16
Holly Mabillard, Shalabh Srivastava, Philip Haslam, Maciej Karasek, John A Sayer
The complications of autosomal dominant polycystic kidney disease (ADPKD) include cyst rupture and haemorrhage leading to loin pain and frank haematuria. Risk factors include large kidney volume, hypertension, and renal impairment. We present a case of a young male who, following trauma to the kidney, had a life threatening bleed from his polycystic kidney. The case was initially treated with fluid resuscitation and blood transfusion but necessitated radiological embolization of bleeding source to control the blood loss...
2017: Case Reports in Nephrology
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
#17
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/29167138/recent-advances-in-management-of-autosomal-dominant-polycystic-kidney-disease
#18
REVIEW
Jacob W Potts, Shaker A Mousa
PURPOSE: Promising developments in the search for effective pharmacotherapies for autosomal-dominant polycystic kidney disease (ADPKD) are reviewed. SUMMARY: The formation and development of cysts characteristic of ADPKD result in inexorable renal and extrarenal manifestations that give rise to more rapid disease progression and more widespread complications than are seen with other forms of chronic kidney disease. To date, no agent has gained Food and Drug Administration marketing approval for use in patients with ADPKD, complicating efforts to meet the medical needs of this population...
December 1, 2017: American Journal of Health-system Pharmacy: AJHP
https://www.readbyqxmd.com/read/29162218/renal-histology-and-mri-in-a-25-year-old-japanese-man-with-nephronophthisis-4%C3%A2
#19
Daisuke Takada, Akinari Sekine, Junko Yabuuchi, Yuta Kogure, Toshiharu Ueno, Masayuki Yamanouchi, Keiichi Sumida, Tatsuya Suwabe, Noriko Hayami, Junichi Hoshino, Kenmei Takaichi, Keiichi Kinowaki, Takeshi Fujii, Kenichi Ohashi, Takayasu Mori, Eisei Sohara, Shinichi Uchida, Yoshifumi Ubara
We investigated a 25-year-old Japanese man who had polycystic kidneys and end-stage renal failure without a positive family history. Ultrasonography revealed enlarged kidneys with increased echogenicity and multiple cystic lesions. MRI showed replacement of both kidneys by cystic lesions without definite walls. Renal biopsy demonstrated interstitial fibrosis, especially at the corticomedullary junction. The residual tubular system showed starfish-like disruption. Tubules with cystic dilation were mainly the distal loop of Henle and the distal tubules since immunohistochemical staining was positive for cytokeratin 7 (the distal loop of Henle and the distal tubule) and Tamm-Horsfall protein (the distal loop of Henle), while being negative for aquaporin 3 (the collecting duct) and CD10 (proximal tubule)...
November 22, 2017: Clinical Nephrology
https://www.readbyqxmd.com/read/29158418/a-novel-pkhd1-mutation-interacts-with-the-nonobese-1diabetic-genetic-background-to-cause-autoimmune-cholangitis
#20
Wenting Huang, Daniel B Rainbow, Yuehong Wu, David Adams, Pranavkumar Shivakumar, Leah Kottyan, Rebekah Karns, Bruce Aronow, Jorge Bezerra, M Eric Gershwin, Laurence B Peterson, Linda S Wicker, William M Ridgway
We previously reported that NOD.c3c4 mice develop spontaneous autoimmune biliary disease (ABD) with anti-mitochondrial Abs, histopathological lesions, and autoimmune T lymphocytes similar to human primary biliary cholangitis. In this article, we demonstrate that ABD in NOD.c3c4 and related NOD ABD strains is caused by a chromosome 1 region that includes a novel mutation in polycystic kidney and hepatic disease 1 (Pkhd1). We show that a long terminal repeat element inserted into intron 35 exposes an alternative polyadenylation site, resulting in a truncated Pkhd1 transcript...
November 20, 2017: Journal of Immunology: Official Journal of the American Association of Immunologists
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