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https://www.readbyqxmd.com/read/28709639/ganoderma-triterpenes-retard-renal-cyst-development-by-downregulating-ras-mapk-signaling-and-promoting-cell-differentiation
#1
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
#2
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
#3
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
#4
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/28678013/-major-complications-of-acquired-renal-cystic-disease-w%C3%A3-nderlich-syndrome-and-papilar-carcinoma
#5
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/28672087/interventions-for-chronic-kidney-disease-in-people-with-sickle-cell-disease
#6
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
#7
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/28660330/quantitative-mri-of-kidneys-in-renal-disease
#8
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
#9
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
#10
(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
#11
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
#12
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
https://www.readbyqxmd.com/read/28640449/polycystic-kidney-disease
#13
Joseph Ghata, Benjamin D Cowley
Renal cysts, which arise from renal tubules, can be seen in a variety of hereditary and nonhereditary entities. Common mechanisms associated with renal cyst formation include increased cell proliferation, epithelial fluid secretion, and extracellular matrix remodeling. Hereditary polycystic kidney disease (PKD) is seen as a component of numerous diseases. Autosomal dominant (AD) PKD is the most common potentially fatal hereditary disease in humans, causes renal failure in approximately 50% of affected individuals, and accounts for approximately 5% of end stage renal disease cases in the United States...
June 18, 2017: Comprehensive Physiology
https://www.readbyqxmd.com/read/28630289/green-mamba-peptide-targets-type-2-vasopressin-receptor-against-polycystic-kidney-disease
#14
Justyna Ciolek, Helen Reinfrank, Loïc Quinton, Say Viengchareun, Enrico A Stura, Laura Vera, Sabrina Sigismeau, Bernard Mouillac, Hélène Orcel, Steve Peigneur, Jan Tytgat, Laura Droctové, Fabrice Beau, Jerome Nevoux, Marc Lombès, Gilles Mourier, Edwin De Pauw, Denis Servent, Christiane Mendre, Ralph Witzgall, Nicolas Gilles
Polycystic kidney diseases (PKDs) are genetic disorders that can cause renal failure and death in children and adults. Lowering cAMP in cystic tissues through the inhibition of the type-2 vasopressin receptor (V2R) constitutes a validated strategy to reduce disease progression. We identified a peptide from green mamba venom that exhibits nanomolar affinity for the V2R without any activity on 155 other G-protein-coupled receptors or on 15 ionic channels. Mambaquaretin-1 is a full antagonist of the V2R activation pathways studied: cAMP production, beta-arrestin interaction, and MAP kinase activity...
July 3, 2017: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/28623191/renal-capillary-haemangioma-associated-with-renal-cell-carcinoma-and-polycythaemia-in-acquired-cystic-disease
#15
Matthew Beamer, Matthew Love, Seyed Ghasemian
Capillary haemangiomas are relatively common tumours, typically occurring in the subcutaneous tissue during childhood. However, visceral occurrence is very rare. These tumours make up a subset of vascular lesions that have previously, although rarely, been described in case reports in association with the kidney. Here we review the literature and describe a capillary haemangioma occurring in the renal hilum found to be coexistent with end-stage renal disease, renal cell carcinoma and polycythaemia. To our knowledge, this is the first case report to describe the occurrence of this tumour in the renal hilum in association with this constitution of renal pathologies...
June 16, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28622163/novel-roles-for-mucin-1-in-the-kidney
#16
Mohammad M Al-Bataineh, Rebecca P Hughey
PURPOSE OF REVIEW: Recent studies in the kidney have revealed that the well characterized tumor antigen mucin 1 (MUC1/Muc1) also has numerous functions in the normal and injured kidney. RECENT FINDINGS: Mucin 1 is a transmembrane mucin with a robust glycan-dependent apical targeting signal and efficient recycling from endosomes. It was recently reported that the TRPV5 calcium channel is stabilized on the cell surface by galectin-dependent cross-linking to mucin 1, providing a novel mechanism for regulation of ion channels and normal electrolyte balance...
June 15, 2017: Current Opinion in Nephrology and Hypertension
https://www.readbyqxmd.com/read/28618971/inversin-correlates-with-the-malignant-phenotype-of-non-small-cell-lung-cancer-and-promotes-the-invasiveness-of-lung-cancer-cells
#17
Gui-Yang Jiang, Yong Zhang, Xiu-Peng Zhang, Xu-Yong Lin, Juan-Han Yu, En-Hua Wang
Inversin, encoded by NPHP2, is one of the 10 NPHP proteins known to be involved in nephronophthisis (an autosomal recessive cystic kidney). Although the previous reports showed that inversin played an important role in embryonic development and renal diseases, its function in cancer was not revealed clearly so far. As measured by immunohistochemical staining, inversin was highly expressed in the cytoplasm of lung cancer samples (63.4%, 161/254) compared with adjacent normal lung tissues (22.0%, 11/50, p < 0...
June 2017: Tumour Biology: the Journal of the International Society for Oncodevelopmental Biology and Medicine
https://www.readbyqxmd.com/read/28615245/the-regulatory-1%C3%AE-subunit-of-protein-kinase-a-modulates-renal-cystogenesis
#18
Hong Ye, Xiaofang Wang, Megan M Constans, Caroline R Sussman, Fouad Chebib, Maria V Irazabal, William F Young, Peter C Harris, Lawrence S Kirschner, Vicente E Torres
The failure of the polycystins (PCs) to function in primary cilia is thought to be responsible for Autosomal Dominant Polycystic Kidney Disease (ADPKD). Primary cilia integrate multiple cellular signaling pathways, including calcium, cAMP, Wnt, and Hedgehog, which control cell proliferation and differentiation. It has been proposed that mutated PCs result in reduced intracellular calcium, which in turn upregulates cAMP, protein kinase A (PKA) signaling, and subsequently other proliferative signaling pathways...
June 14, 2017: American Journal of Physiology. Renal Physiology
https://www.readbyqxmd.com/read/28604386/lysine-methyltransferase-smyd2-promotes-cyst-growth-in-autosomal-dominant-polycystic-kidney-disease
#19
Linda Xiaoyan Li, Lucy X Fan, Julie Xia Zhou, Jared J Grantham, James P Calvet, Julien Sage, Xiaogang Li
Autosomal dominant polycystic kidney disease (ADPKD) is driven by mutations in PKD1 and PKD2 genes. Recent work suggests that epigenetic modulation of gene expression and protein function may play a role in ADPKD pathogenesis. In this study, we identified SMYD2, a SET and MYND domain protein with lysine methyltransferase activity, as a regulator of renal cyst growth. SMYD2 was upregulated in renal epithelial cells and tissues from Pkd1-knockout mice as well as in ADPKD patients. SMYD2 deficiency delayed renal cyst growth in postnatal kidneys from Pkd1 mutant mice...
June 30, 2017: Journal of Clinical Investigation
https://www.readbyqxmd.com/read/28591983/-clinical-and-pathologic-analysis-of-414-cases-of-renal-angiomyolipomain-in-a-single-institution
#20
H L Bao, X Chen, Y X An, H B Sun, H Y Wang, A T Guo
Objective: To study the different clinicopathological characteristics between classic and epithelioid renal angiomyolipoma, and the relationships between clinicopathological characteristics and biological behaviors as basis for clinical treatment. Methods: The clinicopathological and follow-up data for the patients diagnosed with renal angiomyolipoma between 2004 and 2011 were retrospectively reviewed and analyzed. Results: There were 414 cases of renal angiomyolipoma diagnosed over 8 years ago, accounting for 8...
June 8, 2017: Zhonghua Bing Li Xue za Zhi Chinese Journal of Pathology
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