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Polycystic kidney disease

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https://www.readbyqxmd.com/read/28937383/multicentric-papillary-and-chromophobe-renal-cell-carcinomas-in-a-patient-with-autosomal-dominant-polycystic-kidney-disease-report-of-a-rare-case
#1
Nibedita Sahoo, Susama Patra, Swagatika Senapati, Tushar S Mishra
The causal relationship of autosomal dominant polycystic kidney disease (ADPKD) with the development of renal cell carcinoma (RCC) is still not known. We describe a case of bilateral PKD complicated with a large enhancing mass and multiple small nodules in the left kidney. The histopathological study of the nephrectomy specimen revealed the synchronous occurrence of eosinophilic variant of chromophobe RCC (EVCRCC) and multicentric papillary RCC (PRCC) in a background of ADPKD. To the best of our knowledge, this case is the first to describe the collision tumor of EVCRCC and multicentric PRCC in ADPKD...
July 2017: Indian Journal of Pathology & Microbiology
https://www.readbyqxmd.com/read/28937072/epidemiology-of-end-stage-renal-disease-in-dubai-single-center-data
#2
Fakhriya Alalawi, Maseer Ahmed, Hind AlNour, Malaz Noralla, Amna Alhadari
Hemodialysis (HD) was first established in Dubai in the year 1980 and was in its full capacity by the year 1983. Since then, the HD population has been growing rapidly. This report represents the demographic data and clinical characteristics of our HD patients during the period between January 2012 and October 2016. Diabetic nephropathy (57%) and hypertension (12.4%) are emerging as the most common causes of end-stage renal disease (ESRD) in our data, followed by undetermined causes in those who presented as ESRD (10...
September 2017: Saudi Journal of Kidney Diseases and Transplantation
https://www.readbyqxmd.com/read/28933340/renal-histology-and-mri-findings-in-a-%C3%A2-37-year-old-japanese-patient-with-autosomal-recessive-polycystic-kidney-disease%C3%A2
#3
Yusuke Ito, Akinari Sekine, Daisuke Takada, Junko Yabuuchi, Yuta Kogure, Toshiharu Ueno, Keiichi Sumida, Masayuki Yamanouchi, Noriko Hayami, Tatsuya Suwabe, Junichi Hoshino, Naoki Sawa, Kenmei Takaichi, Keiichi Kinowaki, Takeshi Fujii, Kenichi Ohashi, Hiroaki Kikuchi, Shintaro Mandai, Motoko Chiga, Takayasu Mori, Eisei Sohara, Shinichi Uchida, Yoshifumi Ubara
A 37-year-old Japanese man with a serum creatinine level of 2.5 mg/dL and hepatomegaly was admitted to our hospital for investigation of renal failure. Magnetic resonance imaging (MRI) showed hepatomegaly with small cystic lesions that had high signal intensity on T2-weighted images. There was no splenomegaly, and the kidneys were nearly normal in size with a few small cystic lesions. Renal biopsy revealed that interstitial fibrosis and tubular atrophy affected 60% of the cortex. There was cystic tubular dilation, mainly affecting the distal loop of Henle and distal tubules, since immunohistochemical staining of the dilated tubules was positive for cytokeratin 7 and Tamm-Horsfall protein but was negative for aquaporin 3 and CD10...
September 21, 2017: Clinical Nephrology
https://www.readbyqxmd.com/read/28927462/generation-of-special-autosomal-dominant-polycystic-kidney-disease-ipscs-with-the-capability-of-functional-kidney-like-cell-differentiation
#4
Jiahui Huang, Shumin Zhou, Xin Niu, Bin Hu, Qing Li, Feng Zhang, Xue Zhang, Xiujuan Cai, Yuanlei Lou, Fen Liu, Chenming Xu, Yang Wang
BACKGROUND: Human induced pluripotent stem cells (iPSCs) have been verified as a powerful cell model for the study of pathogenesis in hereditary disease. Autosomal dominant polycystic kidney disease (ADPKD) is caused by mutations of PKD or non-PKD genes. The pathogenesis of ADPKD remains unexplored because of the lack of a true human cell model. METHODS: Six ADPKD patients and four healthy individuals were recruited as donors of somatic cells from a Chinese ADPKD family without mutations of the PKD genes but carrying SAMSN1 gene deletion...
September 19, 2017: Stem Cell Research & Therapy
https://www.readbyqxmd.com/read/28915934/increased-yap-activation-is-associated-with-hepatic-cyst-epithelial-cell-proliferation-in-arpkd-chf
#5
Lu Jiang, Lina Sun, Genea Edwards, Michael Manley, Darren Wallace, Seth Septer, Chirag Manohar, Michele T Pritchard, Udayan Apte
Autosomal recessive polycystic kidney disease/congenital hepatic fibrosis (ARPKD/CHF) is a rare but fatal genetic disease characterized by progressive cyst development in the kidneys and liver. Liver cysts arise from aberrantly proliferative cholangiocytes accompanied by pericystic fibrosis and inflammation. Yes associated protein (YAP), the downstream effector of the Hippo signaling pathway, is implicated in human hepatic malignancies such as hepatocellular carcinoma, cholangiocarcinoma, and hepatoblastoma, but its role in hepatic cystogenesis in CHF/ARPKD is unknown...
September 15, 2017: Gene Expression
https://www.readbyqxmd.com/read/28915698/risk-of-aortic-aneurysm-and-dissection-in-patients-with-autosomal-dominant-polycystic-kidney-disease-a-nationwide-population-based-cohort-study
#6
Pei-Hsun Sung, Yao-Hsu Yang, Hsin-Ju Chiang, John Y Chiang, Chi-Jen Chen, Chien-Ting Liu, Cheuk-Man Yu, Hon-Kan Yip
Although cardiovascular complications are the most common cause of death in patients with autosomal-dominant polycystic kidney disease (ADPKD), the incidence and risk of aortic aneurysm and dissection (AAD) in ADPKD remains unclear due to limited data and insufficient cases. We utilized the data from Taiwan National Health Insurance Research Database (NHIRD) to do a population-based cohort study (1997-2008). After excluding those patients with age <18 years old and initially concomitant diagnoses of end-stage renal disease and AAD, a total of 2076 ADPKD patients were selected from 1,000,000 of general population...
August 22, 2017: Oncotarget
https://www.readbyqxmd.com/read/28912780/inflammatory-markers-for-arterial-stiffness-in-cardiovascular-diseases
#7
REVIEW
Ioana Mozos, Clemens Malainer, Jarosław Horbańczuk, Cristina Gug, Dana Stoian, Constantin Tudor Luca, Atanas G Atanasov
Arterial stiffness predicts an increased risk of cardiovascular events. Inflammation plays a major role in large arteries stiffening, related to atherosclerosis, arteriosclerosis, endothelial dysfunction, smooth muscle cell migration, vascular calcification, increased activity of metalloproteinases, extracellular matrix degradation, oxidative stress, elastolysis, and degradation of collagen. The present paper reviews main mechanisms explaining the crosstalk between inflammation and arterial stiffness and the most common inflammatory markers associated with increased arterial stiffness, considering the most recent clinical and experimental studies...
2017: Frontiers in Immunology
https://www.readbyqxmd.com/read/28905441/the-vasopressin-system-new-insights-for-patients-with-kidney-diseases-epidemiological-evidence-and-therapeutic-perspectives
#8
REVIEW
W F Clark, O Devuyst, R Roussel
People with chronic kidney disease (CKD) are at risk of severe outcomes, such as end-stage renal disease or cardiovascular disease, and CKD is a globally increasing health burden with a high personal and economic cost. Despite major progresses in prevention and therapeutics in last decades, research is still needed to reverse this epidemic trend. The regulation of water balance and the state of activation of the vasopressin system have emerged as factors tightly associated with kidney health, in the general population but also in specific conditions; among them, various stages of CKD, diabetes and autosomal dominant polycystic kidney disease (ADPKD)...
October 2017: Journal of Internal Medicine
https://www.readbyqxmd.com/read/28904867/the-asparagine-533-residue-in-the-outer-pore-loop-region-of-the-mouse-pkd2l1-channel-is-essential-for-its-voltage-dependent-inactivation
#9
Takahiro Shimizu, Taiga Higuchi, Toshihiro Toba, Chie Ohno, Takuto Fujii, Bernd Nilius, Hideki Sakai
Voltage-dependent inactivation of ion channels contributes to the regulation of the membrane potential of excitable cells. Mouse polycystic kidney disease 2-like 1 (PKD2L1) forms voltage-dependent nonselective cation channels, which are activated but subsequently inactivated in response to membrane depolarization. Here, we found that the mutation of an asparagine 533 residue (N533Q) in the outer pore loop region of PKD2L1 caused a marked increase in outward currents induced by depolarization. In addition, the tail current analysis demonstrated that the N533Q mutants are activated during depolarization but the subsequent inactivation does not occur...
September 2017: FEBS Open Bio
https://www.readbyqxmd.com/read/28904368/polycystin-1-inhibits-eif2%C3%AE-phosphorylation-and-cell-apoptosis-through-a-pkr-eif2%C3%AE-pathway
#10
Yan Tang, Zuocheng Wang, JungWoo Yang, Wang Zheng, Di Chen, Guanqing Wu, Richard Sandford, Jingfeng Tang, Xing-Zhen Chen
Autosomal dominant polycystic kidney disease (ADPKD) is caused by mutations in PKD1 or PKD2 which encodes polycystin-1 (PC1) and polycystin-2, respectively. PC1 was previously shown to slow cell proliferation and inhibit apoptosis but the underlying mechanisms remain elusive or controversial. Here we showed in cultured mammalian cells and Pkd1 knockout mouse kidney epithelial cells that PC1 and its truncation mutant comprising the last five transmembrane segments and the intracellular C-terminus (PC1-5TMC) down-regulate the phosphorylation of protein kinase R (PKR) and its substrate eukaryotic translation initiation factor 2 alpha (eIF2α)...
September 13, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28903946/ppara-agonist-fenofibrate-enhances-fatty-acid-%C3%AE-oxidation-and-attenuates-polycystic-kidney-and-liver-disease-in-mice
#11
Ronak Lakhia, Matanel Yheskel, Andrea Flaten, Ezekiel B Quittner-Strom, William L Holland, Vishal Patel
PPARA is nuclear hormone receptor that promotes fatty acid β-oxidation (FAO) and oxidative phosphorylation (OXPHOS). We and others have recently shown that PPARA and its target genes are downregulated, and FAO and OXPHOS are impaired in autosomal dominant polycystic kidney disease (ADPKD). However, whether PPARA and FAO/OXPHOS are causally linked to ADPKD progression is not entirely clear. We report that expression of PPARA and FAO/OXPHOS genes is downregulated and in-vivo β-oxidation rate of 3H-labelled triolein is reduced in Pkd1RC/RC mice, a slowly-progressing orthologous model of ADPKD that closely mimics the human ADPKD phenotype...
September 13, 2017: American Journal of Physiology. Renal Physiology
https://www.readbyqxmd.com/read/28892094/deletion-of-pkd1-in-renal-stromal-cells-causes-defects-in-the-renal-stromal-compartment-and-progressive-cystogenesis-in-the-kidney
#12
Xuguang Nie, Lois J Arend
Autosomal dominant polycystic kidney disease (ADPKD), caused by PKD1 and PKD2 gene mutations, is one of the most common genetic diseases, affecting up to 1 in 500 people. Mutations of PKD1 account for over 85% of ADPKD cases. However, mechanisms of disease progression and explanations for the wide range in disease phenotype remain to be elucidated. Moreover, functional roles of PKD1 in the renal stromal compartment are poorly understood. In this work, we tested if Pkd1 is essential for development and maintenance of the renal stromal compartment and if this role contributes to pathogenesis of polycystic kidney disease using a novel tissue-specific knockout mouse model...
September 11, 2017: Laboratory Investigation; a Journal of Technical Methods and Pathology
https://www.readbyqxmd.com/read/28889140/multiple-bilateral-renal-abscesses-in-a-previously-healthy-young-patient
#13
R Y Tang, B M Cheong
The incidence of renal abscesses is not common. Patients usually have risk factors like diabetes mellitus or an underlying condition which predisposes to urinary tract infections. We report a case of a previously healthy young girl with multiple bilateral renal abscesses. Ultrasonography revealed multiple renal abscesses with a possible differential diagnosis of polycystic kidney disease with infected cysts. No renal calculi were seen. CT-scan of kidneys confirmed the diagnosis. Blood and urine cultures were repeatedly negative...
August 2017: Medical Journal of Malaysia
https://www.readbyqxmd.com/read/28887310/histone-deacetylase-6-inhibition-reduces-cysts-by-decreasing-via-camp-and-ca2-in-knockout-mouse-models-of-polycystic-kidney-disease
#14
Murali K Yanda, Qiangni Liu, Valeriu Cebotaru, William B Guggino, Liudmila Cebotaru
Autosomal dominant polycystic kidney disease (ADPKD) is associated with progressive en-largement of multiple renal cysts, often leading to renal failure which cannot be prevented by an current treatment. Two proteins encoded by two genes are associated with ADPKD, PC1 (pkd1), primarily a signaling molecule, and PC2 (pkd2), a Ca2+ channel. Dysregulation of cAMP signaling is central to ADPKD but the molecular mechanism is unresolved. Here we studied the role of histone deacetylase 6 (HDAC6) in regulating cyst growth to test the possibility that in-hibiting HDAC6 might help manage ADPKD...
September 8, 2017: Journal of Biological Chemistry
https://www.readbyqxmd.com/read/28884237/relationship-between-intracranial-aneurysms-and-the-severity-of-autosomal-dominant-polycystic-kidney-disease
#15
Hiroki Yoshida, Eiji Higashihara, Keisuke Maruyama, Kikuo Nutahara, Toshiaki Nitatori, Isao Miyazaki, Yoshiaki Shiokawa
BACKGROUND: Autosomal dominant polycystic kidney disease (ADPKD) is a hereditary kidney disease characterized by the progressive enlargement of innumerable renal cysts. Although the association of intracranial aneurysms (ICANs) with ADPKD is well known, the relationship between the ICAN and the disease severity including total kidney volume (TKV) and estimated glomerular filtration rate (eGFR) is poorly understood. METHODS: We screened 265 patients with ADPKD (mean age, 48...
September 7, 2017: Acta Neurochirurgica
https://www.readbyqxmd.com/read/28881341/the-association-of-combined-total-kidney-and-liver-volume-with-pain-and-gastrointestinal-symptoms-in-patients-with-later-stage-autosomal-dominant-polycystic-kidney-disease
#16
Hedwig M A D'Agnolo, Niek F Casteleijn, Tom J G Gevers, Hans de Fijter, Maartje D A van Gastel, Annemarie L Messchendorp, Dorien J M Peters, Mahdi Salih, Darius Soonawala, Edwin M Spithoven, Folkert W Visser, Jack F M Wetzels, Robert Zietse, Ron T Gansevoort, Joost P H Drenth
BACKGROUND: There is an ongoing debate if and how kidney and liver volume are associated with pain and gastrointestinal (GI) symptoms in autosomal dominant polycystic kidney disease (ADPKD) patients. Since both kidney and liver volume could interact, we investigated whether combined total kidney and liver volume had stronger associations with ADPKD-related pain and GI symptoms than the volumes of the organs separately. METHODS: We used baseline data from the DIPAK-1 study, which included ADPKD patients with an estimated glomerular filtration rate (eGFR) between 30 and 60 mL/min/1...
September 8, 2017: American Journal of Nephrology
https://www.readbyqxmd.com/read/28877884/aberrant-smad3-phosphoisoforms-in-cyst-lining-epithelial-cells-in-the-cpk-mouse-a-model-of-autosomal-recessive-polycystic-kidney-disease
#17
Taketsugu Hama, Koichi Nakanishi, Masashi Sato, Hironobu Mukaiyama, Hiroko Togawa, Yuko Shima, Masayasu Miyajima, Kandai Nozu, Shizuko Nagao, Hisahide Takahashi, Mayumi Sako, Kazumoto Iijima, Norishige Yoshikawa, Hiroyuki Suzuki
Cystic epithelia acquire mesenchymal-like features in polycystic kidney disease (PKD). In this phenotypic alteration, it is well known that transforming growth factor (TGF)-β/Smad3 signaling is involved, however, there is emerging new data on Smad3 phosphoisoforms: Smad3 phosphorylated at linker regions (pSmad3L) and COOH terminal regions (pSmad3C) and both (pSmad3L/C). pSmad3L/C has a pathological role in colorectal cancer. Mesenchymal phenotype-specific cell responses in TGF-β/Smad3 pathway are implicated in carcinomas...
September 6, 2017: American Journal of Physiology. Renal Physiology
https://www.readbyqxmd.com/read/28877825/effectiveness-of-peritoneal-dialysis-in-treating-adult-end-stage-renal-disease-patients-with-polycystic-kidney-disease
#18
Ying Ma, Hai-Yun Wang, Zi-Juan Zhou, Yang Li, Wei Yang, Bing-Yan Liu, Jing-Hua Xia, Xue-Mei Li, Li-Meng Chen
Objective To observe the clinical characteristics,dialysis modalities,and outcomes of end stage renal disease(ESRD)patients with polycystic kidney disease(PKD)and to evaluate the feasibility of peritoneal dialysis in these population. Methods The clinical data of ESRD patient whose primary diagnosis was PKD in Peking Union Medical College Hospital were retrospectively collected from January 1993 to December 2015.PKD patients were divided into two groups according to dialysis modality,namely peritoneal dialysis group(PKD-PD)group and hemodialysis(PKD-HD)group...
August 20, 2017: Zhongguo Yi Xue Ke Xue Yuan Xue Bao. Acta Academiae Medicinae Sinicae
https://www.readbyqxmd.com/read/28875890/new-onset-diabetes-after-transplant-incidence-risk-factors-and-outcome
#19
S C Alagbe, A Voster, R Ramesar, C R Swanepoel
BACKGROUND: The outcome of renal transplantation depends on achieving effective immunosuppression while minimising the consequences of such treatment. The occurrence of new-onset diabetes in the post-transplant period has been associated with several risk factors including some immunosuppressive medication. Better understanding of the clinical and genetic risk factors associated with new-onset diabetes after transplant (NODAT) could enable risk stratification of patients in the pre-transplant period, with the goal of applying measures that will reduce the incidence...
August 25, 2017: South African Medical Journal, Suid-Afrikaanse Tydskrif Vir Geneeskunde
https://www.readbyqxmd.com/read/28873973/effect-of-renal-transcatheter-arterial-embolization-on-quality-of-life-in-patients-with-autosomal-dominant-polycystic-kidney-disease
#20
Tatsuya Suwabe, Yoshifumi Ubara, Akinari Sekine, Toshiharu Ueno, Masayuki Yamanouchi, Noriko Hayami, Junichi Hoshino, Masahiro Kawada, Rikako Hiramatsu, Eiko Hasegawa, Naoki Sawa, Kenmei Takaichi
Background: Currently, there are few strategies for improving the quality of life (QOL) in patients with autosomal dominant polycystic kidney disease (ADPKD) and massive kidneys. Renal transcatheter arterial embolization (TAE) reduces kidney volume, but its impact on QOL in ADPKD patients on hemodialysis is unknown. This study investigated the influence of renal TAE on QOL in ADPKD patients with massive kidneys receiving hemodialysis. Methods: This prospective observational study enrolled 188 ADPKD patients on hemodialysis (92 men and 96 women; mean age 56...
July 1, 2017: Nephrology, Dialysis, Transplantation
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