keyword
MENU ▼
Read by QxMD icon Read
search

polycystic kidney

keyword
https://www.readbyqxmd.com/read/29042084/adenylyl-cyclase-5-deficiency-reduces-renal-cyclic-amp-and-cyst-growth-in-an-orthologous-mouse-model-of-polycystic-kidney-disease
#1
Qian Wang, Patricia Cobo-Stark, Vishal Patel, Stefan Somlo, Pyung-Lim Han, Peter Igarashi
Cyclic AMP promotes cyst growth in polycystic kidney disease (PKD) by stimulating cell proliferation and fluid secretion. Previously, we showed that the primary cilium of renal epithelial cells contains a cAMP regulatory complex comprising adenylyl cyclases 5 and 6 (AC5/6), polycystin-2, A-kinase anchoring protein 150, protein kinase A, and phosphodiesterase 4C. In Kif3a mutant cells that lack primary cilia, the formation of this regulatory complex is disrupted and cAMP levels are increased. Inhibition of AC5 reduces cAMP levels in Kif3a mutant cells, suggesting that AC5 may mediate the increase in cAMP in PKD...
October 14, 2017: Kidney International
https://www.readbyqxmd.com/read/29038287/genetic-complexity-of-autosomal-dominant-polycystic-kidney-and-liver-diseases
#2
Emilie Cornec-Le Gall, Vicente E Torres, Peter C Harris
Data indicate significant phenotypic and genotypic overlap, plus a common pathogenesis, between two groups of inherited disorders, autosomal dominant polycystic kidney diseases (ADPKD), a significant cause of ESRD, and autosomal dominant polycystic liver diseases (ADPLD), which result in significant PLD with minimal PKD. Eight genes have been associated with ADPKD (PKD1 and PKD2), ADPLD (PRKCSH, SEC63, LRP5, ALG8, and SEC61B), or both (GANAB). Although genetics is only infrequently used for diagnosing these diseases and prognosing the associated outcomes, its value is beginning to be appreciated, and the genomics revolution promises more reliable and less expensive molecular diagnostic tools for these diseases...
October 16, 2017: Journal of the American Society of Nephrology: JASN
https://www.readbyqxmd.com/read/29037159/bowel-perforation-by-a-peritoneal-dialysis-catheter-report-of-two-cases
#3
Maki Fujiwara, Takeshi Soda, Takuya Okada, Hiroshi Kanamaru, Takahiro Inoue, Osamu Ogawa
BACKGROUND: Complications of peritoneal dialysis (PD) such as pain and catheter leakage are frequently reported. Delayed bowel perforation of a PD catheter is a rare adverse event but a serious complication associated with significant mortality. Bowel perforation of a PD catheter is difficult to differentiate from PD-related peritonitis and likely to result in a delay in diagnosis. Here, we report two cases of bowel perforation after PD catheter insertion by the stepwise initiation of PD using the Moncrief and Popovich technique (SMAP) and peritoneal wall anchor technique (PWAT)...
October 16, 2017: BMC Nephrology
https://www.readbyqxmd.com/read/29035198/affected-parent-sex-and-severity-of-autosomal-dominant-polycystic-kidney-disease-a-retrospective-cohort-study%C3%A2
#4
Kristen L Nowak, Michel Chonchol, Zhiying You, Malika Gupta, Berenice Gitomer
OBJECTIVE: Parental inheritance may differentially affect autosomal dominant polycystic kidney disease (-ADPKD) severity via genetic imprinting or in utero epigenetic modifications; however, evidence is inconsistent. We conducted a longitudinal retrospective cohort study to assess the association between sex of the affected parent and time to hypertension diagnosis, end-stage renal disease (ESRD), and death in patients with the PKD1 genotype. MATERIALS AND METHODS: 814 individuals who participated in research at the University of Colorado were studied...
October 16, 2017: Clinical Nephrology
https://www.readbyqxmd.com/read/29035195/effects-of-fasting-during-the-month-of-ramadan-on-renal-function-in-patients-with-autosomal-dominant-polycystic-kidney-disease%C3%A2
#5
Iskender Ekinci, Reha Erkoc, Meltem Gursu, Elif Ece Dogan, Elif Kilic, Egemen Cebeci, Savas Ozturk, Rumeyza Kazancioglu
BACKGROUND: In this study, we aimed to examine the impact of fasting during the month of Ramadan on autosomal dominant polycystic kidney disease (-ADPKD) patients with normal to near-normal glomerular filtration rate (GFR). MATERIALS AND METHODS: This was a prospective observational study of patients with ADPKD, the majority of whom had normal or near-normal GFR. Patients were divided into two groups: the fasting group (FG) and the nonfasting group (NFG). Assessments in the NFG were performed 1 week before and 1 month after Ramadan, while FG patients were assessed on the last day of fasting in addition to the abovementioned visits...
October 16, 2017: Clinical Nephrology
https://www.readbyqxmd.com/read/29034881/generation-of-induced-pluripotent-stem-cells-derived-from-an-autosomal-dominant-polycystic-kidney-disease-patient-with-a-p-ser1457fs-mutation-in-pkd1
#6
Ching-Ying Huang, Ming-Ching Ho, Jia-Jung Lee, Daw-Yang Hwang, Hui-Wen Ko, Yu-Che Cheng, Yu-Hung Hsu, Huai-En Lu, Hung-Chun Chen, Patrick C H Hsieh
Autosomal dominant polycystic kidney disease is one of the most prevalent forms of inherited cystic kidney disease, and can be characterized by kidney cyst formation and enlargement. Here we report the generation of a Type 1 ADPKD disease iPS cell line, IBMS-iPSC-012-12, which retains the conserved deletion of PKD1, normal karyotype and exhibits the properties of pluripotent stem cells such as ES-like morphology, expression of pluripotent markers and capacity to differentiate into all three germ layers. Our results show that we have successfully generated a patient-specific iPS cell line with a mutation in PKD1 for study of renal disease pathophysiology...
October 2017: Stem Cell Research
https://www.readbyqxmd.com/read/29034246/tolvaptan-for-the-treatment-of-enlarged-polycystic-liver-disease
#7
Hiroki Mizuno, Junichi Hoshino, Tatsuya Suwabe, Keiichi Sumida, Akinari Sekine, Yoichi Oshima, Masahiko Oguro, Kyohei Kunizawa, Masahiro Kawada, Rikako Hiramatsu, Noriko Hayami, Eiko Hasegawa, Masayuki Yamanouchi, Naoki Sawa, Kenmei Takaichi, Yoshifumi Ubara
A 44-year-old Japanese woman with autosomal dominant polycystic kidney disease was admitted to our hospital for evaluation of abdominal distension. Her eGFR was 53.7 mL/min/1.73 m(2). Total kidney volume was 2,614 mL. Tolvaptan (60 mg/day) was started to treat renal involvement. The patient's abdominal fullness began to improve and liver volume, indicating advanced polycystic liver disease (PLD), decreased from 9,750 mL to 8,345 mL after 17 months of tolvaptan treatment, though there was no significant change in kidney volume...
May 2017: Case Reports in Nephrology and Dialysis
https://www.readbyqxmd.com/read/29033453/polycystic-kidney-disease-modulation-of-cystogenesis-by-the-microenviroment
#8
Ellen F Carney
No abstract text is available yet for this article.
October 16, 2017: Nature Reviews. Nephrology
https://www.readbyqxmd.com/read/29033415/liver-cyst-infection-after-colon-endoscopic-mucosal-resection-in-a-patient-with-autosomal-dominant-polycystic-kidney-disease-on-maintenance-hemodialysis
#9
Shota Shimano, Miki Tsuda, Seiya Fuyuno, Yoshihide Arimura, Fumio Nanishi
A 60-year-old Japanese man with autosomal dominant polycystic kidney disease (ADPKD) on maintenance hemodialysis underwent colonoscopy and endoscopic mucosal resection (EMR). He was hospitalized after 4 days of fever that began the day following colonoscopy. We detected Klebsiella pneumoniae in a blood culture and a ring-shaped integration in the liver cyst by gallium scintigraphy. He recovered with antibiotics and percutaneous drainage. The patient was believed to have contracted the liver cyst infection via an injured colonic mucosa and portal vein...
October 16, 2017: Internal Medicine
https://www.readbyqxmd.com/read/29033332/disruption-of-core-planar-cell-polarity-signaling-regulates-renal-tubule-morphogenesis-but-is-not-cystogenic
#10
Koshi Kunimoto, Roy D Bayly, Eszter K Vladar, Tyson Vonderfecht, Anna-Rachel Gallagher, Jeffrey D Axelrod
Oriented cell division (OCD) and convergent extension (CE) shape developing renal tubules, and their disruption has been associated with polycystic kidney disease (PKD) genes, the majority of which encode proteins that localize to primary cilia. Core planar cell polarity (PCP) signaling controls OCD and CE in other contexts, leading to the hypothesis that disruption of PCP signaling interferes with CE and/or OCD to produce PKD. Nonetheless, the contribution of PCP to tubulogenesis and cystogenesis is uncertain, and two major questions remain unanswered...
October 11, 2017: Current Biology: CB
https://www.readbyqxmd.com/read/29032669/xp11-2-translocation-renal-cell-carcinoma-in-the-autosomal-dominant-polycystic-kidney-disease-patient-with-preserved-renal-function
#11
Hyuk Huh, Hyung Ah Jo, YongJin Yi, Seung Hyup Kim, Kyung Chul Moon, Curie Ahn, Hayne Cho Park
No abstract text is available yet for this article.
October 17, 2017: Korean Journal of Internal Medicine
https://www.readbyqxmd.com/read/29026418/heterozygosity-analysis-of-polycystic-kidney-disease-1-gene-microsatellite-markers-for-linkage-analysis-of-autosomal-dominant-polycystic-kidney-disease-type-1-in-the-iranian-population
#12
Razieh Fatehi, Sharifeh Khosravi, Maryam Abedi, Rasoul Salehi, Yousof Gheisari
BACKGROUND: Autosomal dominant polycystic kidney disease (ADPKD) is the most common genetic cause of end-stage renal disease. Although imaging techniques are a means of accurate diagnosis when the cysts appear in the third or fourth decades of the patient's life, they are of little value for early diagnosis. Genetic tests are required for preimplantation genetic diagnosis, decision-making for kidney donation to an affected relative. Although mutation of the polycystic kidney disease (PKD1) gene is solely responsible for the most cases of ADPKD, direct genetic testing is limited by the large size of this gene and the presence of many mutations without hot spots...
2017: Journal of Research in Medical Sciences: the Official Journal of Isfahan University of Medical Sciences
https://www.readbyqxmd.com/read/29024476/prevalence-and-outcomes-of-cystic-lesions-of-the-transplant-pancreas-the-university-of-wisconsin-experience
#13
Talal M Al-Qaoud, Eric J Martinez, Hans W Sollinger, Dixon B Kaufman, Robert R Redfield, Bridget Welch, Glen Leverson, Jon S Odorico
Literature on the behavior of cystic lesions in pancreas transplants is scarce, and hence a better understanding is warranted. Data on recipients and their respective donors that underwent simultaneous kidney and pancreas, pancreas transplant alone, and pancreas after kidney between 1994-2015 were reviewed (n=1185). Cystic lesions of the transplant pancreas developed in 22 patients (1.8%): 12 pseudocysts, 2 cysts/remnants, 4 intraductal papillary mucinous neoplasms (IPMN), 2 adenocarcinomas, 1 low grade intraepithelial pancreatic neoplasia, and 1 case of polycystic kidney disease...
October 11, 2017: American Journal of Transplantation
https://www.readbyqxmd.com/read/29021226/attenuation-of-accelerated-renal-cystogenesis-in-pkd1-mice-by-renin-angiotensin-system-blockade
#14
Wayne R Fitzgibbon, Yujing Dang, Marlene A Bunni, Catalin F Baicu, Michael R Zile, Adam E Mullick, Takamitsu Saigusa
BACKGROUND: The intrarenal renin angiotensin system (RAS) is activated in polycystic kidney disease. We have recently shown in the Pkd1 mouse that Gen 2 antisense oligonucleotide (ASO), which suppresses angiotensinogen (Agt) synthesis, is efficacious in slowing kidney cyst formation compared to lisinopril. The aim of this current study was to determine 1) if unilateral nephrectomy accelerates cystogenesis in Pkd1 mice (as previously shown in cilia knockout mice), and 2) whether Agt ASO can slow the progression in this accelerated cystic mouse model...
October 11, 2017: American Journal of Physiology. Renal Physiology
https://www.readbyqxmd.com/read/28993480/mitochondrial-abnormality-facilitates-cyst-formation-in-autosomal-dominant-polycystic-kidney-disease
#15
Yu Ishimoto, Reiko Inagi, Daisuke Yoshihara, Masanori Kugita, Shizuko Nagao, Akira Shimizu, Norihiko Takeda, Masaki Wake, Kenjiro Honda, Jing Zhou, Masaomi Nangaku
Autosomal dominant polycystic kidney disease (ADPKD) constitutes the most common inherited kidney disease. Mutations in the PKD1 and PKD2 genes, encoding respective polycystin-1 and polycystin-2 Ca(2+) ion channels, results in tubular epithelial cell-derived renal cysts. Recent clinical studies demonstrate oxidative stress as present early in ADPKD. Mitochondria comprise the primary reactive oxygen species source and also their main effector target; however, the pathophysiological role of mitochondria in ADPKD remains uncharacterized...
October 9, 2017: Molecular and Cellular Biology
https://www.readbyqxmd.com/read/28993351/haematuria-in-adpkd-not-always-benign-be-aware
#16
Manav Agarwal, Ashok Kumar Sokhal, Manoj Kumar, Sunny Goel
Haematuria in patients with autosomal dominant polycystic kidney disease (ADPKD) is a very common manifestation. The cause of haematuria is often benign with the most common cause being haemorrhage within the renal cyst. But haematuria may also be caused by a coincident malignancy, the diagnosis of which may be missed if not investigated thoroughly. Herein, we present a case of ADPKD who presented to us with haematuria and was later found to have bladder cancer.
October 9, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28992279/targeting-new-cellular-disease-pathways-in-autosomal-dominant-polycystic-kidney-disease
#17
Ming-Yang Chang, Albert C M Ong
Autosomal dominant polycystic kidney disease (ADPKD) is the most common inherited cause of end-stage renal failure. Understanding the molecular and cellular pathogenesis of ADPKD could help to identify new targets for treatment. The classic cellular cystic phenotype includes changes in proliferation, apoptosis, fluid secretion, extracellular matrix and cilia function. Hoever, recent research, suggests that the cellular cystic phenotype could be broader and that changes, such as altered metabolism, autophagy, inflammation, oxidative stress and epigenetic modification, could play important roles in the processes of cyst initiation, cyst growth or disease progression...
August 29, 2017: Nephrology, Dialysis, Transplantation
https://www.readbyqxmd.com/read/28992127/can-we-further-enrich-autosomal-dominant-polycystic-kidney-disease-clinical-trials-for-rapidly-progressive-patients-application-of-the-propkd-score-in-the-tempo-trial
#18
Emilie Cornec-Le Gall, Jaime D Blais, Maria V Irazabal, Olivier Devuyst, Ron T Gansevoort, Ron D Perrone, Arlene B Chapman, Frank S Czerwiec, John Ouyang, Christina M Heyer, Sarah R Senum, Yannick Le Meur, Vicente E Torres, Peter C Harris
Background: The PROPKD score has been proposed to stratify the risk of progression to end-stage renal disease in autosomal dominant polycystic kidney disease (ADPKD) subjects. We aimed to assess its prognostic value in a genotyped subgroup of subjects from the Tolvaptan Phase 3 Efficacy and Safety Study in Autosomal Dominant Polycystic Kidney Disease (TEMPO3/4) trial. Methods: In the post hoc analysis, PKD1 and PKD2 were screened in 770 subjects and the PROPKD score was calculated in mutation-positive subjects (male: 1 point; hypertension <35 years: 2 points; first urologic event <35 years: 2 points; nontruncating PKD1 mutation: 2 points; truncating PKD1 mutation: 4 points)...
July 19, 2017: Nephrology, Dialysis, Transplantation
https://www.readbyqxmd.com/read/28989174/new-treatment-paradigms-for-adpkd-moving-towards-precision-medicine
#19
REVIEW
Matthew B Lanktree, Arlene B Chapman
The natural history of autosomal dominant polycystic kidney disease (ADPKD) is characterized by a variable rate of cyst development and increase in total kidney volume (TKV), variable kidney function decline and age of onset of end-stage renal disease (ESRD), and variable presentation of renal and extrarenal manifestations. Precision medicine is proposed to improve patient outcomes by tailoring therapy to the specific genetic background, pathophysiology, disease burden, prognosis and status of each individual...
October 9, 2017: Nature Reviews. Nephrology
https://www.readbyqxmd.com/read/28988229/studying-kidney-disease-using-tissue-and-genome-engineering-in-human-pluripotent-stem-cells
#20
Elena Garreta, Federico González, Núria Montserrat
Kidney morphogenesis and patterning have been extensively studied in animal models such as the mouse and zebrafish. These seminal studies have been key to define the molecular mechanisms underlying this complex multistep process. Based on this knowledge, the last 3 years have witnessed the development of a cohort of protocols allowing efficient differentiation of human pluripotent stem cells (hPSCs) towards defined kidney progenitor populations using two-dimensional (2D) culture systems or through generating organoids...
October 7, 2017: Nephron
keyword
keyword
13325
1
2
Fetch more papers »
Fetching more papers... Fetching...
Read by QxMD. Sign in or create an account to discover new knowledge that matter to you.
Remove bar
Read by QxMD icon Read
×

Search Tips

Use Boolean operators: AND/OR

diabetic AND foot
diabetes OR diabetic

Exclude a word using the 'minus' sign

Virchow -triad

Use Parentheses

water AND (cup OR glass)

Add an asterisk (*) at end of a word to include word stems

Neuro* will search for Neurology, Neuroscientist, Neurological, and so on

Use quotes to search for an exact phrase

"primary prevention of cancer"
(heart or cardiac or cardio*) AND arrest -"American Heart Association"