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https://www.readbyqxmd.com/read/28341273/branched-chain-amino-acids-enhance-cyst-development-in-autosomal-dominant-polycystic-kidney-disease
#1
Junya Yamamoto, Saori Nishio, Fumihiko Hattanda, Daigo Nakazawa, Toru Kimura, Michio Sata, Minoru Makita, Yasunobu Ishikawa, Tatsuya Atsumi
Autosomal dominant polycystic kidney disease (ADPKD) is characterized by the progressive development of kidney and liver cysts. The mammalian target of rapamycin (mTOR) cascade is one of the important pathways regulating cyst growth in ADPKD. Branched-chain amino acids (BCAAs), including leucine, play a crucial role to activate mTOR pathway. Therefore, we administered BCAA dissolved in the drinking water to Pkd1(flox/flox):Mx1-Cre (cystic) mice from four to 22 weeks of age after polyinosinic-polycytidylic acid-induced conditional Pkd1 knockout at two weeks of age...
March 21, 2017: Kidney International
https://www.readbyqxmd.com/read/28341240/overexpression-of-exogenous-kidney-specific-ngal-attenuates-progressive-cyst-development-and-prolongs-lifespan-in-a-murine-model-of-polycystic-kidney-disease
#2
Ellian Wang, Yuan-Yow Chiou, Wen-Yih Jeng, Hsiu-Kuan Lin, Hsi-Hui Lin, Hsian-Jean Chin, Chi-Kuang Leo Wang, Shang-Shiuan Yu, Shih-Chieh Tsai, Chih-Ying Chiang, Po-Hao Cheng, Hong-Jie Lin, Si-Tse Jiang, Sou-Tyau Chiu, Hsiu Mei Hsieh-Li
Neutrophil gelatinase-associated lipocalin (Ngal) is a biomarker for acute and chronic renal injuries, including polycystic kidney disease (PKD). However, the effect of Ngal on PKD progression remains unexplored. To study this, we generated 3 strains of mice with different expression levels of Ngal within an established PKD model (Pkd1(L3/L3)): Pkd1(L3/L3) (with endogenous Ngal), Pkd1(L3/L3); Ngal(Tg/Tg) (with endogenous and overexpression of exogenous kidney-specific Ngal) and Pkd1(L3/L3); Ngal(-/-) mice (with Ngal deficiency)...
February 2017: Kidney International
https://www.readbyqxmd.com/read/28340841/liver-transplantation-for-polycystic-liver-disease-due-to-huge-liver-with-related-complications-a-case-report
#3
S Acar, G Gencdal, M Tokac, E Eren, U Alkara, G Tellioglu, A Dinckan, M Akyildiz
Polycystic liver disease is characterized by multiple cystic lesions on the liver. It is an uncommon autosomal dominant disease. The cysts' diameters range from 20 to 30 cm to small microscopic nodules. Generally, more than half of the liver parenchyma is covered. The mass effect of the liver created by the large cysts can cause life-threatening symptoms such as weight loss, reduction of oral intake, and malnutrition. Liver transplantation is the best treatment option in symptomatic patients. We present a patient who had polycystic liver and kidney disease, and we performed liver transplantation because of his life-threatening symptoms...
April 2017: Transplantation Proceedings
https://www.readbyqxmd.com/read/28340010/non-proteinuric-rather-than-proteinuric-renal-diseases-are-the-leading-cause-of-end-stage-kidney-disease
#4
Davide Bolignano, Carmine Zoccali
Proteinuria is a distinguishing feature in primary and secondary forms of chronic glomerulonephritis, which contribute to no more than the 20% of the end-stage kidney disease (ESKD) population. The contribution of non-proteinuric nephropathies to the global ESKD burden is still poorly focused and scarce research efforts are dedicated to the elucidation of risk factors and mechanistic pathways triggering ESKD in these diseases. We abstracted information on proteinuria in the main renal diseases other than glomerulonephritides that may evolve into ESKD...
March 1, 2017: Nephrology, Dialysis, Transplantation
https://www.readbyqxmd.com/read/28326182/birt-hogg-dub%C3%A3-syndrome-a-case-report-and-a-review-of-the-literature
#5
REVIEW
Dea Kejlberg Jensen, Anders Villumsen, Anne-Bine Skytte, Mia Gebauer Madsen, Mette Sommerlund, Elisabeth Bendstrup
Background: Birt-Hogg-Dubé syndrome (BHDS) is a rare autosomal dominant inherited syndrome caused by mutations in the folliculin coding gene (FLCN). The clinical manifestations of the syndrome involve the skin, lungs, and kidneys. Because of the rarity of the syndrome, guidelines for diagnosis and management of the patients with BHDS are lacking. Objective: To present a case story and a review of the literature on BHDS in order to give an update on genetics, clinical manifestations, diagnosis, treatment, prognosis and follow-up strategies...
2017: European Clinical Respiratory Journal
https://www.readbyqxmd.com/read/28324003/biliary-anomalies-in-patients-with-hnf1b-diabetes
#6
Jarno Lt Kettunen, Helka Parviainen, Päivi J Miettinen, Martti Färkkilä, Marjo Tamminen, Pia Salonen, Eila Lantto, Tiinamaija Tuomi
Context: The clinical spectrum of organogenetic anomalies associated with HNF1B mutations is heterogeneous. Besides cystic kidney disease, diabetes, and various other manifestations, odd cases of mainly neonatal and post-transplant cholestasis have been described. The biliary phenotype is incompletely defined. Objective: To systematically characterize HNF1B-related anomalies in the bile ducts by imaging with MRI or MRCP. Setting and Patients: Fourteen patients with HNF1B mutations in the catchment area of the Helsinki University hospital were evaluated with upper abdominal MRI and MRCP...
March 17, 2017: Journal of Clinical Endocrinology and Metabolism
https://www.readbyqxmd.com/read/28296808/waitlisted-candidates-with-polycystic-liver-disease-are-more-likely-to-be-transplanted-than-those-with-chronic-liver-failure
#7
Sahil D Doshi, Therese Bittermann, Thomas D Schiano, David Seth Goldberg
BACKGROUND: Polycystic liver disease (PCLD) is characterized by cystic replacement of the hepatic parenchyma, leading to hepatic dysfunction, portal hypertension, and hepatomegaly. Patients with liver dysfunction and/or symptomatic disease are eligible for liver transplantation. However, little is known about these patients' waitlist outcomes relative to others with chronic liver disease. METHODS: We used OPTN/UNOS data from February 27, 2002 to December 31, 2015 to compare waitlist outcomes of adult patients with PCLD to those with chronic liver failure (CLF) and hepatocellular carcinoma (HCC)...
March 15, 2017: Transplantation
https://www.readbyqxmd.com/read/28283827/prenatal-ultrasound-genotype-and-outcome-in-a-large-cohort-of-prenatally-affected-patients-with-autosomal-recessive-polycystic-kidney-disease-and-other-hereditary-cystic-kidney-diseases
#8
Florian Erger, Nadina Ortiz Brüchle, Ulrich Gembruch, Klaus Zerres
PURPOSE: To investigate the sonographic and clinical genotype-phenotype correlations in autosomal recessive polycystic kidney disease (ARPKD) and other cystic kidney diseases (CKD) in a large cohort of prenatally detected fetuses with hereditary CKD. METHODS: We retrospectively studied the clinical and diagnostic data of 398 patients referred with prenatal ultrasound findings suggestive of CKD between 1994 and 2010. Cases with confirmed hereditary CKD (n = 130) were analyzed as to their prenatal ultrasound findings, genotype, and possible predictors of clinical outcome...
April 2017: Archives of Gynecology and Obstetrics
https://www.readbyqxmd.com/read/28283013/-molecular-characterization-of-echinococcus-granulosus-isolates-obtained-from-different-hosts
#9
Emrah Erdoğan, Bora Özkan, Fatih Mutlu, Serkan Karaca, İzzet Şahin
Echinococcus granulosus is a parasite that can be seen throughout the world. So far, five species of genus Echinococcus have been identified as parasite in people: E.granulosus, E.multilocularis, E.vogeli, E.oligarthrus, E.shiquicus. Larval (metacestod) form of parasite settles in internal organs of hoofed animals (cattle, goats, pigs, horses, sheep, etc.) and human; the adult form is found in small intestine of final host, canine. Disease caused by parasite called as "Cystic echinococcosis" (CE) is an important health problem and causes economic losses in many countries including our country that livestock is common...
January 2017: Mikrobiyoloji Bülteni
https://www.readbyqxmd.com/read/28250993/imaging-the-abdominal-manifestations-of-cystic-fibrosis
#10
REVIEW
C D Gillespie, M K O'Reilly, G N Allen, S McDermott, V O Chan, C A Ridge
Cystic fibrosis (CF) is a multisystem disease with a range of abdominal manifestations including those involving the liver, pancreas, and kidneys. Recent advances in management of the respiratory complications of the disease has led to a greater life expectancy in patients with CF. Subsequently, there is increasing focus on the impact of abdominal disease on quality of life and survival. Liver cirrhosis is the most important extrapulmonary cause of death in CF, yet significant challenges remain in the diagnosis of CF related liver disease...
2017: International Journal of Hepatology
https://www.readbyqxmd.com/read/28249589/diagnostic-and-therapeutic-challenges-of-an-ambiguous-cystic-kidney-disease-in-a-resource-limited-setting-a-case-report
#11
Christian Akem Dimala, Ndemazie Nkafu Bechem, Benjamin Momo Kadia, Vitalis Fambombi Feteh, Simeon Pierre Choukem
BACKGROUND: Unilateral renal cystic disease is a rare condition that shares morphological similarities with multicystic dysplastic kidney, the former often distinguished from the latter on some clinical and histopathological grounds. However serious diagnostic and therapeutic dilemmas set in when there is a considerable overlap in the distinguishing features between these entities. CASE PRESENTATION: A 19-year-old African female presented with a chronic severe debilitating right lower quadrant abdominal pain refractory to analgesics...
March 1, 2017: BMC Research Notes
https://www.readbyqxmd.com/read/28249268/bile-acids-in-polycystic-liver-diseases-triggers-of-disease-progression-and-potential-solution-for-treatment
#12
Maria J Perugorria, Ibone Labiano, Aitor Esparza-Baquer, Marco Marzioni, Jose J G Marin, Luis Bujanda, Jesús M Banales
Polycystic liver diseases (PLDs) are a group of genetic hereditary cholangiopathies characterized by the development and progressive growth of cysts in the liver, which are the main cause of morbidity. Current therapies are based on surgical procedures and pharmacological strategies, which show short-term and modest beneficial effects. Therefore, the determination of the molecular mechanisms of pathogenesis appears to be crucial in order to find new potential targets for pharmacological therapy. Ductal plate malformation during embryogenesis and abnormal cystic cholangiocyte growth and secretion are some of the key mechanisms involved in the pathogenesis of PLDs...
2017: Digestive Diseases
https://www.readbyqxmd.com/read/28247443/acute-kidney-injury-in-patients-treated-with-iv-beta-lactam-beta-lactamase-inhibitor-combinations
#13
W Cliff Rutter, David S Burgess
STUDY OBJECTIVE: Increased acute kidney injury (AKI) incidence has been reported in patients receiving piperacillin-tazobactam (PTZ) therapy compared to other beta-lactams. This study sought to determine if the addition of beta-lactamase inhibitors impact AKI incidence by comparing patients treated with PTZ or ampicillin-sulbactam (SAM). DESIGN: Retrospective cohort study. SETTING: Large academic tertiary care hospital. PATIENTS: Overall, 2,448 patients received PTZ (n=1,836) or SAM (n=612) for at least 48 hours between September 1, 2007 and September 30, 2015...
March 1, 2017: Pharmacotherapy
https://www.readbyqxmd.com/read/28244683/rapamycin-treatment-dose-dependently-improves-the-cystic-kidney-in-a-new-adpkd-mouse-model-via-the-mtorc1-and-cell-cycle-associated-cdk1-cyclin-axis
#14
Ao Li, Song Fan, Yuchen Xu, Jialin Meng, Xufeng Shen, Jun Mao, Li Zhang, Xiansheng Zhang, Gilbert Moeckel, Dianqing Wu, Guanqing Wu, Chaozhao Liang
Although translational research into autosomal dominant polycystic kidney disease (ADPKD) and its pathogenesis has made considerable progress, there is presently lack of standardized animal model for preclinical trials. In this study, we developed an orthologous mouse model of human ADPKD by cross-mating Pkd2 conditional-knockout mice (Pkd2(f3) ) to Cre transgenic mice in which Cre is driven by a spectrum of kidney-related promoters. By systematically characterizing the mouse model, we found that Pkd2(f3/f3) mice with a Cre transgene driven by the mouse villin-1 promoter (Vil-Cre;Pkd2(f3/f3) ) develop overt cysts in the kidney, liver and pancreas and die of end-stage renal disease (ESRD) at 4-6 months of age...
February 28, 2017: Journal of Cellular and Molecular Medicine
https://www.readbyqxmd.com/read/28239354/matrix-metalloproteinases-7-and-kidney-fibrosis
#15
REVIEW
Ben Ke, Chuqiao Fan, Liping Yang, Xiangdong Fang
Matrix metalloproteinase-7 (MMP-7) is a secreted zinc- and calcium-dependent endopeptidase that degrades a broad range of extracellular matrix substrates and additional substrates. MMP-7 playsa crucial role in a diverse array of cellular processes and appears to be a key regulator of fibrosis in several diseases, including pulmonary fibrosis, liver fibrosis, and cystic fibrosis. In particular, the relationship between MMP-7 and kidney fibrosis has attracted significant attention in recent years. Growing evidence indicates that MMP-7 plays an important role in the pathogenesis of kidney fibrosis...
2017: Frontiers in Physiology
https://www.readbyqxmd.com/read/28237283/ablative-therapies-for-renal-tumors-patient-selection-treatment-planning-and-follow-up
#16
Jean-Michel Correas, Christophe Delavaud, Jules Gregory, Thomas Le Guilchet, Lionel Lamhaut, Marc-Olivier Timsit, Arnaud Méjean, Olivier Hélénon
The increased use of abdominal imaging has led to a major increase in small renal tumors incidence particularly in the elderly population. Their management is evolving with the development of percutaneous ablation, particularly radiofrequency ablation, cryoablation, and microwave ablation. The typical indications that must be validated by a multidisciplinary committee include solid tumors less than 3cm in patients with multiple comorbidity factors (including age), contraindications to surgery, hereditary renal cancer, bilateral renal tumors, solitary kidney, pre-existing chronic kidney disease, or at high risk of predialysis renal function after partial nephrectomy...
February 2017: Seminars in Ultrasound, CT, and MR
https://www.readbyqxmd.com/read/28218262/cystic-kidney-disease-pals1-links-polarity-tgf-%C3%AE-and-the-hippo-pathway
#17
Andrea Aguilar
No abstract text is available yet for this article.
February 20, 2017: Nature Reviews. Nephrology
https://www.readbyqxmd.com/read/28205547/microrna-17-family-promotes-polycystic-kidney-disease-progression-through-modulation-of-mitochondrial-metabolism
#18
Sachin Hajarnis, Ronak Lakhia, Matanel Yheskel, Darren Williams, Mehran Sorourian, Xueqing Liu, Karam Aboudehen, Shanrong Zhang, Kara Kersjes, Ryan Galasso, Jian Li, Vivek Kaimal, Steven Lockton, Scott Davis, Andrea Flaten, Joshua A Johnson, William L Holland, Christine M Kusminski, Philipp E Scherer, Peter C Harris, Marie Trudel, Darren P Wallace, Peter Igarashi, Edmund C Lee, John R Androsavich, Vishal Patel
Autosomal dominant polycystic kidney disease (ADPKD) is the most frequent genetic cause of renal failure. Here we identify miR-17 as a target for the treatment of ADPKD. We report that miR-17 is induced in kidney cysts of mouse and human ADPKD. Genetic deletion of the miR-17∼92 cluster inhibits cyst proliferation and PKD progression in four orthologous, including two long-lived, mouse models of ADPKD. Anti-miR-17 treatment attenuates cyst growth in short-term and long-term PKD mouse models. miR-17 inhibition also suppresses proliferation and cyst growth of primary ADPKD cysts cultures derived from multiple human donors...
February 16, 2017: Nature Communications
https://www.readbyqxmd.com/read/28202165/ghetting-to-know-adpkd-proliferative-signaling-stat
#19
Ching-Hsien Chen, Robert H Weiss
Due to their common pathogenesis and parallel proliferative signaling pathways, the cystic diseases have been recently studied in the context of cancer biology. The present study continues this paradigm by identifying signal transducer and activator of transcription (STAT5) and growth hormone (GH) as potentially modifiable pathways in polycystic kidney disease. GH, which is a potent activator of STAT5, has the additional possibility of being a biomarker, as well as providing a potential mechanism of action of somatostatin analogs in clinical trials...
March 2017: Kidney International
https://www.readbyqxmd.com/read/28194573/hyponatremia-and-cyst-growth-in-neonatal-polycystic-kidney-disease-a-case-for-aquaretics
#20
EDITORIAL
Detlef Bockenhauer
Hyponatremia is a common complication in neonatal polycystic kidney disease and is thought to be due to water retention. Aquaretics are drugs that promote free water excretion by blocking the arginine vasopressin receptor type 2 (AVPR2) in the collecting duct and thus impair urinary concentration. AVPR2 is also a key stimulant for cyclic AMP production in the collecting duct and in this way promotes cyst proliferation and pathologic kidney growth in autosomal dominant polycystic kidney disease (ADPKD). Consequently, the aquaretic tolvaptan is now used to slow down progression of ADPKD in adult patients...
February 13, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
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