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https://www.readbyqxmd.com/read/28820206/a-cross-platform-metabolomics-workflow-for-volume-restricted-tissue-samples-application-to-an-animal-model-for-polycystic-kidney-disease
#1
E Sánchez-López, H Happé, E Steenvoorden, A L Crego, M L Marina, D J M Peters, O A Mayboroda
Metabolic profiling provides an unbiased view of the physiological status of an organism as a "function" of the metabolic composition of a measured sample. Here, we propose a simple LC-MS based workflow for metabolic profiling of volume-restricted samples, namely individual 20 μm-thick histological sections of a mouse kidney. The main idea of this workflow is to re-use the material after an RPLC-MS run, namely using the volume remaining in the vial after injection, and then introducing a phase changing step to enable HILIC-MS analysis...
August 18, 2017: Molecular BioSystems
https://www.readbyqxmd.com/read/28816297/-perirenal-cystic-lymphangioma-in-an-adult-a-case-report-and-literature-review
#2
W He, Y C Hao, H Z Xia, R Z Ma, B Yang, J Lu
Lymphangioma is a rare, benign mesenchymal neoplasm, which is characterized by numerous intercommunicating cystic spaces containing lymphatic fluid. It is considered a congenital disease resulting from the obstruction of regional lymph drainage during the developmental period. Lymphangioma frequently occurs in the cervical neck and axilla, also in the retroperitoneum, mediastinum, mesentery, omentum, colon, and pelvis, rarely in the perirenal space. These tumors usually present in childhood, but infrequently, these also present in adults...
August 18, 2017: Beijing da Xue Xue Bao. Yi Xue Ban, Journal of Peking University. Health Sciences
https://www.readbyqxmd.com/read/28815206/autosomal-recessive-polycystic-kidney-disease
#3
Ashley S Hafer, Richard M Conran
The following fictional case is intended as a learning tool within the Pathology Competencies for Medical Education (PCME), a set of national standards for teaching pathology. These are divided into three basic competencies: Disease Mechanisms and Processes, Organ System Pathology, and Diagnostic Medicine and Therapeutic Pathology. For additional information, and a full list of learning objectives for all three competencies, see http://journals.sagepub.com/doi/10.1177/2374289517715040.
January 2017: Acad Pathol
https://www.readbyqxmd.com/read/28814334/abernethy-malformation-associated-with-caroli-s-syndrome-in-a-patient-with-a-pkhd1-mutation-a-case-report
#4
Xiao-Xiao Mi, Xiao-Guang Li, Zi-Rong Wang, Ling Lin, Chun-Hai Xu, Jun-Ping Shi
BACKGROUND: Abernethy malformation is a rare congenital anomaly characterised by the partial or complete absence of the portal vein and the subsequent development of an extrahepatic portosystemic shunt. Caroli's disease is a rare congenital condition characterised by non-obstructive saccular intrahepatic bile duct dilation. Caroli's disease combined with congenital hepatic fibrosis and/or renal cystic disease is referred to - Caroli's syndrome. The combination of Abernethy malformation and Caroli's syndrome has not been reported previously...
August 16, 2017: Diagnostic Pathology
https://www.readbyqxmd.com/read/28810844/effect-of-tolvaptan-on-renal-handling-of-water-and-sodium-gfr-and-central-hemodynamics-in-autosomal-dominant-polycystic-kidney-disease-during-inhibition-of-the-nitric-oxide-system-a-randomized-placebo-controlled-double-blind-crossover-study
#5
Safa Al Therwani, My Emma Sofie Malmberg, Jeppe Bakkestroem Rosenbaek, Jesper Noergaard Bech, Erling Bjerregaard Pedersen
BACKGROUND: Tolvaptan slows progression of autosomal dominant polycystic kidney disease (ADPKD) by antagonizing the vasopressin-cAMP axis. Nitric oxide (NO) stimulates natriuresis and diuresis, but its role is unknown during tolvaptan treatment in ADPKD. METHODS: Eighteen patients with ADPKD received tolvaptan 60 mg or placebo in a randomized, placebo-controlled, double blind, crossover study. L-NMMA (L-NG-monomethyl-arginine) was given as a bolus followed by continuous infusion during 60 min...
August 15, 2017: BMC Nephrology
https://www.readbyqxmd.com/read/28810709/parallel-evolution-of-sperm-hyper-activation-ca2-channels
#6
Jacob C Cooper, Nitin Phadnis
Sperm hyper-activation is a dramatic change in sperm behavior where mature sperm burst into a final sprint in the race to the egg. The mechanism of sperm hyper-activation in many metazoans, including humans, consists of a jolt of Ca2+ into the sperm flagellum via CatSper ion channels. Surprisingly, all nine CatSper genes have been independently lost in several animal lineages. In Drosophila, sperm hyper-activation is performed through the cooption of the polycystic kidney disease 2 (pkd2) Ca2+ channel. The parallels between CatSpers in primates and pkd2 in Drosophila provide a unique opportunity to examine the molecular evolution of the sperm hyper-activation machinery in two independent, nonhomologous calcium channels separated by > 500 million years of divergence...
July 1, 2017: Genome Biology and Evolution
https://www.readbyqxmd.com/read/28810583/kidney-dysfunction-following-adrenalectomy-in-autosomal-dominant-polycystic-kidney-disease-complicated-with-primary-aldosteronism-a-case-report
#7
Hiroyuki Hirai, Makoto Kanno, Tsuyoshi Watanabe, Hiroaki Satoh
The present study reported a case of autosomal dominant polycystic kidney disease complicated with primary aldosteronism in a 49-year-old woman. The patient was referred for refractory hypertension. Laboratory examinations revealed low potassium and renin levels. Computed tomography indicated a right adrenal tumor and multiple renal cysts. Adrenal vein sampling revealed a high aldosterone level on the side of the tumor. The patient was diagnosed with autosomal dominant polycystic kidney disease complicated by primary aldosteronism and adrenalectomy was performed...
August 2017: Experimental and Therapeutic Medicine
https://www.readbyqxmd.com/read/28808579/a-novel-bbs10-mutation-identified-in-a-patient-with-bardet-biedl-syndrome-with-a-violent-emotional-outbreak
#8
Tatsuyuki Ohto, Takashi Enokizono, Ryuta Tanaka, Mai Tanaka, Hisato Suzuki, Aiko Sakai, Kazuo Imagawa, Hiroko Fukushima, Takashi Fukushima, Ryo Sumazaki, Tomoko Uehara, Toshiki Takenouchi, Kenjiro Kosaki
We report a 10-year-old girl with Bardet-Biedl syndrome caused by a novel mutation in the Bardet-Biedl syndrome 10 (BBS10) gene. She had multiple malformations, including a dysmorphic face, postaxial polydactyly, polycystic kidney and amblyopia. She presented with typical BBS features, including intellectual disability with emotional outbursts and mild obesity. Whole-exome sequencing identified compound heterozygous mutations with NM_024685.3:c.1677C>A [p.(Tyr559*)] and c.1974T>G [p.(Tyr658*)]. To our knowledge, the latter mutation has never been reported previously...
2017: Human Genome Variation
https://www.readbyqxmd.com/read/28806944/real-world-costs-of-autosomal-dominant-polycystic-kidney-disease-in-the-nordics
#9
Daniel Eriksson, Linda Karlsson, Oskar Eklund, Hans Dieperink, Eero Honkanen, Jan Melin, Kristian Selvig, Johan Lundberg
BACKGROUND: There is limited real-world data on the economic burden of patients with autosomal dominant polycystic kidney disease (ADPKD). The objective of this study was to estimate the annual direct and indirect costs of patients with ADPKD by severity of the disease: chronic kidney disease (CKD) stages 1-3; CKD stages 4-5; transplant recipients; and maintenance dialysis patients. METHODS: A retrospective study of ADPKD patients was undertaken April-December 2014 in Denmark, Finland, Norway and Sweden...
August 15, 2017: BMC Health Services Research
https://www.readbyqxmd.com/read/28802235/steviol-stabilizes-polycystin-1-expression-and-promotes-lysosomal-degradation-of-cftr-and-%C3%AE-catenin-proteins-in-renal-epithelial-cells
#10
Chaowalit Yuajit, Chatchai Muanprasat, Sureeporn Homvisasevongsa, Varanuj Chatsudthipong
Malfunction of polycystin 1 (PC1) is linked to abnormally high epithelial cell proliferation and fluid secretion, eventually leading to renal cyst development and declined renal function as found in autosomal dominant polycystic kidney disease (ADPKD). Currently, there is no effective therapy for ADPKD. Recent studies report PC1 regulates CFTR chloride channels and β-catenin levels in normal renal epithelial cells. Concurrently, our previous study found steviol retarded renal cyst enlargement in an in vitro and in an in vivo models by reducing CFTR expression and activity...
August 9, 2017: Biomedicine & Pharmacotherapy, Biomédecine & Pharmacothérapie
https://www.readbyqxmd.com/read/28798345/nedd4-family-e3-ligase-dysfunction-due-to-pkhd1-pkhd1-defects-suggests-a-mechanistic-model-for-arpkd-pathobiology
#11
Jun-Ya Kaimori, Cheng-Chao Lin, Patricia Outeda, Miguel A Garcia-Gonzalez, Luis F Menezes, Erum A Hartung, Ao Li, Guanqing Wu, Hideaki Fujita, Yasunori Sato, Yasuni Nakanuma, Satoko Yamamoto, Naotsugu Ichimaru, Shiro Takahara, Yoshitaka Isaka, Terry Watnick, Luiz F Onuchic, Lisa M Guay-Woodford, Gregory G Germino
Autosomal recessive polycystic kidney disease (ARPKD) is an important childhood nephropathy, occurring 1 in 20,000 live births. The major clinical phenotypes are expressed in the kidney with dilatation of the collecting ducts, systemic hypertension, and progressive renal insufficiency, and in the liver with biliary dysgenesis, portal tract fibrosis, and portal hypertension. The systemic hypertension has been attributed to enhanced distal sodium reabsorption in the kidney, the structural defects have been ascribed to altered cellular morphology, and fibrosis to increased TGF-β signaling in the kidney and biliary tract, respectively...
August 10, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28796300/apelin-apj-system-a-novel-potential-therapy-target-for-kidney-disease
#12
REVIEW
Zhen Huang, Lele Wu, Linxi Chen
Apelin is an endogenous ligand of seven-transmembrane G protein-coupled receptor APJ. Apelin and APJ are distributed in various tissues, including the heart, lung, kidney, and even in tumor tissues. Studies show that apelin mRNA is highly expressed in the inner stripe of kidney outer medulla, which plays an important role in process of water and sodium balance. Additionally, more studies also indicate that apelin/APJ system exerts a broad range of activities in kidney. Therefore, we review the role of apelin/APJ system in kidney diseases such as renal fibrosis, renal ischemia/reperfusion injury, diabetic nephropathy, polycystic kidney disease and hemodialysis...
August 10, 2017: Journal of Cellular Physiology
https://www.readbyqxmd.com/read/28794066/insights-into-cellular-and-molecular-basis-for-urinary-tract-infection-in-autosomal-dominant-polycystic-kidney-disease
#13
Chao Gao, Long Zhang, Ye Zhang, Darren Paul Wallace, Reynold I Lopez-Soler, Paul J Higgins, Wenzheng Zhang
Urinary tract infection (UTI) is a broad term referring to an infection of the kidneys, ureters, bladder and/or urethra. Due to its prevalence, frequent recurrence and rising resistance to antibiotics, UTI has become a challenge in clinical practice. Autosomal dominant polycystic kidney disease (ADPKD) is the most common monogenic disorder of the kidney and is characterized by the growth of fluid-filled cysts in both kidneys. Progressive cystic enlargement, inflammation and interstitial fibrosis result in nephron loss with subsequent decline in kidney function...
August 9, 2017: American Journal of Physiology. Renal Physiology
https://www.readbyqxmd.com/read/28792715/mutational-screening-of-pkd1-and-pkd2-genes-in-iranian-population-diagnosed-with-autosomal-dominant-polycystic-kidney-disease
#14
Fariba Ranjzad, Ahmad Tara, Abbas Basiri, Nasser Aghdami, Reza Moghadasali
BACKGROUND: Autosomal Dominant Polycystic Kidney Disease (ADPKD) is the fourth most frequent cause of endstage renal disease (ESDR), occurring at a varying frequency of 1/400 to 1/800 persons. The disease affects all ethnic groups worldwide, and there is a need for population based studies to be carried out for better diagnostic, genetic counselling, and treatment purpose. METHODS: Eighteen unrelated probands (10 males and 8 females) with a familial history of ADPKD were selected for the study...
July 1, 2017: Clinical Laboratory
https://www.readbyqxmd.com/read/28782656/genetics-and-mechanisms-of-hepatic-cystogenesis
#15
REVIEW
L F M van de Laarschot, J P H Drenth
Polycystic liver disease (PLD) is a heterogeneous genetic condition. PKD1 and PKD2 germline mutations are found in patients with autosomal dominant polycystic kidney disease (ADPKD). Autosomal dominant polycystic liver disease (ADPLD) is associated with germline mutations in PRKCSH, SEC63, LRP5, and recently ALG8, and SEC61. GANAB mutations are found in both patient groups. Loss of heterozygosity of PLD-genes in cyst epithelium contributes to the development of hepatic cysts. A genetic interaction network is implied in hepatic cystogenesis that connects the endoplasmic glycoprotein control mechanisms and polycystin expression and localization...
August 4, 2017: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/28781369/polycystic-kidney-disease-fpc-in-arpkd
#16
Susan J Allison
No abstract text is available yet for this article.
August 7, 2017: Nature Reviews. Nephrology
https://www.readbyqxmd.com/read/28769124/metformin-inhibits-cyst-formation-in-a-zebrafish-model-of-polycystin-2-deficiency
#17
Ming-Yang Chang, Tsu-Lin Ma, Cheng-Chieh Hung, Ya-Chung Tian, Yung-Chang Chen, Chih-Wei Yang, Yi-Chuan Cheng
Autosomal dominant polycystic kidney disease (ADPKD) is a common kidney disease caused by mutations in PKD1 or PKD2. Metformin reduces cyst growth in mouse models of PKD1. However, metformin has not been studied in animal models of PKD2, and the cellular mechanism underlying its effectiveness is not entirely clear. This study investigated the effects of metformin on cyst formation in a zebrafish model of polycystin-2 deficiency resulting from morpholino knockdown of pkd2. We added metformin (2.5 to 20 mM) to the embryo media between 4 and 48 hours post fertilisation and observed pronephric cyst formation by using the wt1b promoter-driven GFP signal in Tg(wt1b:GFP) pkd2 morphants...
August 2, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28768225/complex-liver-cysts-in-autosomal-dominant-polycystic-kidney-disease
#18
Zerwa Farooq, Ashkan Heshmatzadeh Behzadi, Jon D Blumenfeld, Yize Zhao, Martin R Prince
PURPOSE: To determine prevalence of complex liver cysts in Autosomal Dominant Polycystic Kidney Disease (ADPKD). METHODS: Abdominal MRI in 186 ADPKD subjects were evaluated by two independent observers to determine prevalence of complex liver cysts. RESULTS: 23 (12%) of subjects, had at least 1 complex cyst. Only 8 (4%) were reported to have a complex cyst prospectively, representing an under-reporting rate of 65%. Median total cyst volume was 66-times greater for subjects with complex cysts compared to subjects without (p<0...
July 25, 2017: Clinical Imaging
https://www.readbyqxmd.com/read/28766875/species-differences-in-renal-development-and-associated-developmental-nephrotoxicity
#19
REVIEW
Kendall S Frazier
The developing kidney is sensitive to both morphological and functional disturbances during the gestational and postnatal phases of growth and differentiation. Exposure to drugs or chemicals during these critical windows of renal development can result in aplasia, dysplasia, polycystic kidney disease, hydronephrosis, or other features characteristic of nephrotoxicity, including tubule dilation, necrosis, or mineralization. Functional effects can occur without associated morphological abnormalities. Differences in the timing of nephrogenesis and morphologic renal development among species help to explain specific phenotypes of various gestational and postnatal teratogens and nephrotoxins...
August 2, 2017: Birth defects research
https://www.readbyqxmd.com/read/28764564/neonatal-renal-cystic-diseases
#20
Anshika Khare, Vinod Krishnappa, Deepak Kumar, Rupesh Raina
PURPOSE: Neonatal renal cystic diseases have a great impact on the morbidity and mortality of the affected neonates and infants. A good insight into the pathophysiology, diagnosis and treatment options of various neonatal renal cystic diseases aid in early diagnosis and intervention, thereby preventing complications. METHODS: PubMed search was done for articles on "neonatal renal cystic diseases" and relevant publications including reviews were considered for our article...
August 2, 2017: Journal of Maternal-fetal & Neonatal Medicine
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