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https://www.readbyqxmd.com/read/28729967/tesevatinib-ameliorates-progression-of-polycystic-kidney-disease-in-rodent-models-of-autosomal-recessive-polycystic-kidney-disease
#1
William E Sweeney, Philip Frost, Ellis D Avner
AIM: To investigate the therapeutic potential of tesevatinib (TSV), a unique multi-kinase inhibitor currently in Phase II clinical trials for autosomal dominant polycystic kidney disease (ADPKD), in well-defined rodent models of autosomal recessive polycystic kidney disease (ARPKD). METHODS: We administered TSV in daily doses of 7.5 and 15 mg/kg per day by I.P. to the well characterized bpk model of polycystic kidney disease starting at postnatal day (PN) 4 through PN21 to assess efficacy and toxicity in neonatal mice during postnatal development and still undergoing renal maturation...
July 6, 2017: World Journal of Nephrology
https://www.readbyqxmd.com/read/28724316/molecular-diagnosis-of-autosomal-dominant-polycystic-kidney-disease
#2
Xuewen Song, Amirreza Haghighi, Ioan-Andrei Iliuta, York Pei
Autosomal dominant polycystic kidney disease (ADPKD) is the most common inherited kidney disease that accounts for 5-10% of end-stage renal disease in developed countries. Mutations in PKD1 and PKD2 account for a majority of cases. Mutation screening of PKD1 is technically challenging largely due to the complexity resulting from duplication of its first 33 exons in six highly homologous pseudogenes (i.e. PKD1P1-P6). Protocol using locus-specific long-range and nested PCR has enabled comprehensive PKD1 mutation screening but is labor-intensive and costly...
July 20, 2017: Expert Review of Molecular Diagnostics
https://www.readbyqxmd.com/read/28709639/ganoderma-triterpenes-retard-renal-cyst-development-by-downregulating-ras-mapk-signaling-and-promoting-cell-differentiation
#3
Limin Su, Liying Liu, Yingli Jia, Lei Lei, Jiangfeng Liu, Shuai Zhu, Hong Zhou, Ruoyun Chen, Hua Ann Jenny Lu, Baoxue Yang
Autosomal dominant polycystic kidney disease (ADPKD) is a common monogenetic disease characterized by the progressive development of renal cysts with further need for effective therapy. Here our aim was to investigate the effect of Ganoderma triterpenes (GT) on the development of kidney cysts. Importantly, GT attenuated cyst development in two mouse models of ADPKD with phenotypes of severe cystic kidney disease. Assays for tubulogenesis showed that GT promoted epithelial tubule formation in MDCK cells, suggesting a possible effect on epithelial cell differentiation...
July 11, 2017: Kidney International
https://www.readbyqxmd.com/read/28701694/defective-cftr-leads-to-aberrant-%C3%AE-catenin-activation-and-kidney-fibrosis
#4
Jie Ting Zhang, Yan Wang, Jun Jiang Chen, Xiao Hu Zhang, Jian Da Dong, Lai Ling Tsang, Xiao Ru Huang, Zhiming Cai, Hui Yao Lan, Xiao Hua Jiang, Hsiao Chang Chan
Cystic fibrosis transmembrane conductance regulator (CFTR), known as a cAMP-activated Cl(-) channel, is widely expressed at the apical membrane of epithelial cells in a wide variety of tissues. Of note, despite the abundant expression of CFTR in mammalian kidney, the role of CFTR in kidney disease development is unclear. Here, we report that CFTR expression is downregulated in the UUO (unilateral ureteral obstruction)-induced kidney fibrosis mouse model and human fibrotic kidneys. Dysfunction or downregulation of CFTR in renal epithelial cells leads to alteration of genes involved in Epithelial-Mesenchymal Transition (EMT) and kidney fibrosis...
July 12, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28701314/cux1-promotes-cell-proliferation-and-polycystic-kidney-disease-progression-in-an-adpkd-mouse-model
#5
Binu Porath, Safia Livingston, Erica L Andres, Alexandra M Petrie, Joshua C Wright, Anna E Woo, Carol G Carlton, Richard Baybutt, Gregory B Vanden Heuvel
Autosomal Dominant Polycystic Kidney Disease (ADPKD) is one of the most common monogenic hereditary disorders in humans characterized by fluid-filled cysts, primarily in the kidneys. Cux1, a cell cycle regulatory gene highly expressed during kidney development, is elevated in the cyst-lining cells of Pkd1 mutant mice, and in human ADPKD cells. However, forced expression of Cux1 is insufficient to induce cystic disease in transgenic mice, or to induce rapid cyst formation after cilia disruption in the kidneys of adult mice...
July 12, 2017: American Journal of Physiology. Renal Physiology
https://www.readbyqxmd.com/read/28685422/chronic-diseases-as-barriers-to-oxygen-delivery-a-unifying-hypothesis-of-tissue-reoxygenation-therapy
#6
G A Perdrizet
Modern medical practice has resulted in the accumulation of a growing number of incurable chronic diseases, many of which are inflammatory in nature. Inflammation establishes a hypoxic microenvironment within tissues, a condition of inflammatory hypoxia (IH). Tissues thus affected become severely compromised, are unable to elicit adaptive responses and eventually develop fibrosis and fixed microvascular deficits. Previous work has demonstrated that tissue hypoxia exits even within the simple human model of self-resolving inflammation, the tuberculin reaction...
2017: Advances in Experimental Medicine and Biology
https://www.readbyqxmd.com/read/28684259/zinner-syndrome-a-diagnostic-challenge-the-aid-of-morphology-embryology-and-immunohistochemistry
#7
Michela Campora, Alberto Olivero, Carlo Toncini, Bruno Spina, Ezio Fulcheri, Carlo Terrone, Valerio Gaetano Vellone
We investigate a patient with right kidney agenesis. Imaging showed the presence of a cystic mass dislocating the bladder. The specimen showed three formations: a kidney remnant, a ureter with blind-ending branch and a cysts, from this departed another tubular structure, considered the deferential duct. The specimen was sampled. The supposed kidney was formed by cystic structures. Examination of the tubular structures disclosed smooth muscle fibers with no lumen, while the cyst was surrounded by fibrous and hemorrhagic walls...
July 3, 2017: Urology
https://www.readbyqxmd.com/read/28679080/renal-cystic-adenocarcinoma-in-a-flowerhorn-fish-with-metastatic-involvement-of-the-spleen
#8
Hooman Rahmati-Holasoo, Sara Shokrpoor, Majid Masoudifard, HosseinAli Ebrahimzadeh Mousavi, Ali Haddadi, Amir Tavakkoli
A 480 g flowerhorn fish hybrid cichlid with severe bilateral abdominal swelling, bulla-like structures on the skin, bilateral exophthalmia and prolapsed intestine was presented. Radiographs showed a compression of the posterior part of swim bladder and abdominal distention. Ultrasonography of visceral organs showed a heterogenous mass with hypo to anechoic polycystic parenchyma and free fluid in abdominal cavity. At necropsy free fluid in the abdominal cavity and a large polycystic mass, originating from the posterior kidney, was observed...
July 5, 2017: Journal of Aquatic Animal Health
https://www.readbyqxmd.com/read/28678013/-major-complications-of-acquired-renal-cystic-disease-w%C3%A3-nderlich-syndrome-and-papilar-carcinoma
#9
Enrique Rodríguez-Zarco, Ana Vallejo-Benítez, Antonio Delgado Cotán, Sofía Pereira-Gallardo
OBJETIVE: We report a case of acquired renal cystic disease associated with renal dialysis and endstage renal disease. The patient suffered the two major complications related with acquired renal cystic disease; hemorrhage and renal carcinoma. METHODS: Our case is a patient with acquired renal cystic disease, single kidney after surgery for renal clear cell carcinoma four years earlier, who developed a Wünderlich syndrome (WS). RESULTS: The histological study of the nephrectomy specimen showed a renal papillary carcinoma in the context of acquired renal cystic disease after surgery for a WS...
July 2017: Archivos Españoles de Urología
https://www.readbyqxmd.com/read/28676554/diabetic-rats-present-higher-urinary-loss-of-proteins-and-lower-renal-expression-of-megalin-cubilin-clc-5-and-cftr
#10
Miriam F Figueira, Raquel C Castiglione, Carolina M de Lemos Barbosa, Felipe M Ornellas, Geórgia da Silva Feltran, Marcelo M Morales, Rodrigo N da Fonseca, Jackson de Souza-Menezes
Diabetic nephropathy (DN) occurs in around 40% of those with diabetes. Proteinuria is the main characteristic of DN and develops as a result of increased permeability of the glomerulus capillary wall and/or decreased proximal tubule endocytosis. The goal of this work was to evaluate renal function and the expression of megalin, cubilin, CFTR (cystic fibrosis transmembrane conductance regulator), and ClC-5 in the proximal tubule and renal cortex of rats with type 1 diabetes. Male Wistar rats were randomly assigned to control (CTRL) and diabetic (DM) groups for 4 weeks...
July 2017: Physiological Reports
https://www.readbyqxmd.com/read/28676441/chronic-toxicity-and-oncogenicity-of-octamethylcyclotetrasiloxane-d4-in-the-fischer-344-rat
#11
Paul A Jean, Kathleen P Plotzke
Octamethylcyclotetrasiloxane (D4) is a cyclic volatile methylsiloxane primarily used in the synthesis of silicon-based materials used in in a variety of consumer products. This paper details the chronic toxicity and oncogenicity evaluation of D4 in the Fischer 344 rat. Animals were exposed to 0, 10, 30, 150, or 700ppm D4 vapor for 6h/day, 5 days/week for up to 104 weeks in whole-body inhalation chambers. Effects of two year chronic exposure included increased liver, kidney, testes, and uterine weight with correlating microscopic findings of hepatocellular hypertrophy (males only), chronic nephropathy (both sexes), interstitial cell hyperplasia, and cystic endometrial hyperplasia and endometrial adenoma, respectively...
July 1, 2017: Toxicology Letters
https://www.readbyqxmd.com/read/28672087/interventions-for-chronic-kidney-disease-in-people-with-sickle-cell-disease
#12
REVIEW
Noemi Ba Roy, Patricia M Fortin, Katherine R Bull, Carolyn Doree, Marialena Trivella, Sally Hopewell, Lise J Estcourt
BACKGROUND: Sickle cell disease (SCD) is one of the commonest severe monogenic disorders in the world, due to the inheritance of two abnormal haemoglobin (beta-globin) genes. SCD can cause severe pain, significant end-organ damage, pulmonary complications, and premature death. Kidney disease is a frequent and potentially severe complication in people with SCD.Chronic kidney disease is defined as abnormalities of kidney structure or function, present for more than three months. Sickle cell nephropathy refers to the spectrum of kidney complications in SCD...
July 3, 2017: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/28666097/novel-transgenic-mouse-model-of-polycystic-kidney-disease
#13
Yusuke Kito, Chiemi Saigo, Tamotsu Takeuchi
Transmembrane protein 207 (TMEM207) is characterized as an important molecule for invasiveness of gastric signet-ring cell carcinoma cells. To clarify the pathobiological effects of TMEM207, we generated 13 transgenic mouse strains, designated C57BL/6-transgenic (Tg) (ITF-TMEM207), where the mouse Tmem207 is ectopically expressed under the proximal promoter of the murine intestinal trefoil factor gene. A C57BL/6-Tg (ITF-TMEM207) mouse strain unexpectedly exhibited a high incidence of spontaneous kidney cysts with histopathological features resembling human polycystic kidney disease, which were found in approximately all mice within 1 year...
June 27, 2017: American Journal of Pathology
https://www.readbyqxmd.com/read/28664407/combined-use-of-electron-microscopy-and-intravital-imaging-captures-morphological-and-functional-features-of-podocyte-detachment
#14
REVIEW
James L Burford, Georgina Gyarmati, Isao Shirato, Wilhelm Kriz, Kevin V Lemley, János Peti-Peterdi
The development of podocyte injury and albuminuria in various glomerular pathologies is still incompletely understood due to technical limitations in studying the glomerular filtration barrier (GFB) in real-time. We aimed to directly visualize the early morphological and functional changes of the GFB during the development of focal segmental glomerulosclerosis (FSGS) using a combination of transmission electron microscopy (TEM) and in vivo multiphoton microscopy (MPM) in the rat puromycin aminonucleoside (PAN) model...
June 29, 2017: Pflügers Archiv: European Journal of Physiology
https://www.readbyqxmd.com/read/28663537/algorithm-for-bosniak-2f-cyst-in-kidney-donation
#15
Robert C Minnee, Hendrikus J A N Kimenai, Paul C Verhagen, Jan H von der Thüsen, Roy S Dwarkasing, Jacqueline van de Wetering, Jan N IJzermans
BACKGROUND The Bosniak system for radiological classification of renal cysts offers a tool for surgical decision-making in clinical practice. Although 95% of Bosniak 2F cysts remain benign, a consensus on the management of Bosniak 2F cysts in kidney donation has not been developed. CASE REPORT We present a donor with a Bosniak 2F cyst, who successfully donated her kidney after partial resection of the Bosniak 2F cyst. Postoperative pathology examination of the partially resected cystic wall revealed a multilocular cystic renal cell carcinoma...
June 30, 2017: American Journal of Case Reports
https://www.readbyqxmd.com/read/28660330/quantitative-mri-of-kidneys-in-renal-disease
#16
Timothy L Kline, Marie E Edwards, Ishan Garg, Maria V Irazabal, Panagiotis Korfiatis, Peter C Harris, Bernard F King, Vicente E Torres, Sudhakar K Venkatesh, Bradley J Erickson
PURPOSE: To evaluate the reproducibility and utility of quantitative magnetic resonance imaging (MRI) sequences for the assessment of kidneys in young adults with normal renal function (eGFR ranged from 90 to 130 mL/min/1.73 m(2)) and patients with early renal disease (autosomal dominant polycystic kidney disease). MATERIALS AND METHODS: This prospective case-control study was performed on ten normal young adults (18-30 years old) and ten age- and sex-matched patients with early renal parenchymal disease (autosomal dominant polycystic kidney disease)...
June 28, 2017: Abdominal Radiology
https://www.readbyqxmd.com/read/28658845/large-multicystic-dysplastic-kidney-mimicking-a-large-cystic-renal-neoplasm
#17
Priyank Yadav, Sanjoy Kumar Sureka, M S Ansari, Rahul Soni, Hira Lal
Multicystic Dysplastic Kidney (MCDK) is one of the most common renal conditions seen in paediatric population. The natural history typically involves involution and many of the patients with unilateral disease may actually never become symptomatic. The initial evaluation is usually done on Ultrasonography (USG) while cross-sectional imaging and nuclear scan are reserved for diagnostic dilemmas. Management is conservative and surgery is done for selected patients with symptomatic cysts or suspicion of neoplasm...
May 2017: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28650600/clear-cell-neuroendocrine-tumor-of-the-pancreas-in-von-hippel-lindau-disease-a-case-report-and-literature-review
#18
(no author information available yet)
Clear cell neuroendocrine tumor (NET) of the pancreas is found in von Hippel-Lindau (VHL) disease, multiple endocrine neoplasia type I (MEN I), and sporadic form. Clear cell NETs are often misdiagnosed as metastatic renal cell carcinoma. A 47-year-old woman with VHL was found to have a mass in the pancreatic tail and two masses in the right kidney with two cysts. A distal pancreatectomy and right radical nephrectomy were performed. The pancreatic lesion was a well-circumscribed, golden-yellow solid mass, which was lobulated by septal fibrosis...
May 26, 2017: Neuro Endocrinology Letters
https://www.readbyqxmd.com/read/28648202/a-review-on-autosomal-dominant-tubulointerstitial-kidney-disease
#19
Nadia Ayasreh Fierro, Rosa Miquel Rodríguez, Ana Matamala Gastón, Elisabet Ars Criach, Roser Torra Balcells
In recent years there has been a reclassification of hereditary tubulointerstitial renal diseases. The old concepts of nephronoptisis or medullary cystic disease have been reordered based on the discovery of new genes. The 2015 KDIGO guidelines proposed a unification of terminology, diagnostic criteria and monitoring. So far 4genes causing autosomal dominant tubulointerstitial kidney disease have been described: MUC1, UMOD, HNF1B and REN. Although the mutation in each of them causes distinctive features in how they present, all have in common the progressive tubulointerstitial damage and renal fibrosis...
May 2017: Nefrología: Publicación Oficial de la Sociedad Española Nefrologia
https://www.readbyqxmd.com/read/28642870/imaging-and-management-of-incidental-renal-lesions
#20
REVIEW
Silvio Mazziotti, Giuseppe Cicero, Tommaso D'Angelo, Maria Adele Marino, Carmela Visalli, Ignazio Salamone, Giorgio Ascenti, Alfredo Blandino
The increased use of imaging modalities in the last years has led to a greater incidence in depicting abdominal incidental lesions. In particular, "incidentalomas" of the kidney are discovered in asymptomatic patients or patients who suffer from diseases not directly related to the kidneys. The aim of this paper is to provide the radiologist with a useful guide to recognize and classify the main incidental renal findings with the purpose of establishing the correct management. First we describe the so-called "pseudotumors" which are important to recognize in order to avoid a misdiagnosis...
2017: BioMed Research International
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