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https://www.readbyqxmd.com/read/28807340/unusual-locations-of-hydatid-disease-a-10-year-experience-from-a-tertiary-reference-center-in-western-turkey
#1
Eylul Gun, Demet Etit, Dilara O Buyuktalanci, Fulya Cakalagaoglu
INTRODUCTION: Hydatid disease is an endemic parasitic infection caused by Echinococcus granulosus mostly seen in the Mediterranean countries. The most affected organ is the liver, however hydatidosis can be found anywhere in the human body. METHODS: The records of patients who were diagnosed with hydatid disease in our hospital from December 2005 to February 2016 were analyzed retrospectively. The cases were evaluated and recorded depending on their gender, age and the localization of the cysts...
August 2017: Annals of Diagnostic Pathology
https://www.readbyqxmd.com/read/28797981/rhabdoid-meningioma-arising-concurrent-in-pulmonary-and-intracranial-with-a-rare-malignant-clinical-progression-case-report-and-literature-review
#2
Peng Zhao, Ning Li, Jinfeng Cao, Xiangtao Lin, Changhu Liang
BACKGROUND: Rhabdoid meningioma (RM) is an unusual variant of meningioma, classified as WHO grade III. Although its recurrence is common, extracranial metastasis is rare and usually misdiagnosed. The transfer mechanism and pathway are ambiguous; once it develops, there is a poor prognosis and no effective management. The present case is the first report on concurrent intracranial and pulmonary RM with rapid and widespread metastasis. We hope this report can be a helpful reference for clinicians and radiologists...
August 7, 2017: World Neurosurgery
https://www.readbyqxmd.com/read/28794366/regression-of-crizotinib-associated-complex-cystic-lesions-after-switching-to-alectinib
#3
Kageaki Taima, Hisashi Tanaka, Yoshihito Tanaka, Masamichi Itoga, Shingo Takanashi, Sadatomo Tasaka
Crizotinib, which is effective in patients with anaplastic lymphoma kinase (ALK) positive non-small cell lung cancer, is sometimes associated with the generation of complex renal cysts. A 56-year-old man with ALK positive adenocarcinoma received crizotinib. Ten months after the introduction of crizotinib, a cystic lesion developed from his right kidney to the iliopsoas muscle, accompanied by fever, anemia, and hypoproteinemia. After 17 months of treatment, crizotinib was switched to alectinib, followed by the recovery of hypoproteinemia and systemic inflammation...
August 10, 2017: Internal Medicine
https://www.readbyqxmd.com/read/28794066/insights-into-cellular-and-molecular-basis-for-urinary-tract-infection-in-autosomal-dominant-polycystic-kidney-disease
#4
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/28793871/diagnostic-accuracy-of-contrast-enhanced-ultrasound-for-characterization-of-kidney-lesions-in-patients-with-and-without-chronic-kidney-disease
#5
Emily Hueywen Chang, Wui Kheong Chong, Sandeep Kumar Kasoji, Julia Rose Fielding, Ersan Altun, Lee B Mullin, Jung In Kim, Jason Peter Fine, Paul Alexander Dayton, Wendy Kimryn Rathmell
BACKGROUND: Patients with chronic kidney disease are at increased risk of cystic kidney disease that requires imaging monitoring in many cases. However, these same patients often have contraindications to contrast-enhanced computed tomography and magnetic resonance imaging. This study evaluates the accuracy of contrast-enhanced ultrasound (CEUS), which is safe for patients with chronic kidney disease, for the characterization of kidney lesions in patients with and without chronic kidney disease...
August 9, 2017: BMC Nephrology
https://www.readbyqxmd.com/read/28776935/shorter-telomere-length-increases-age-related-tumor-risks-in-von-hippel-lindau-disease-patients
#6
Jiang-Yi Wang, Shuang-He Peng, Xiang-Hui Ning, Teng Li, Sheng-Jie Liu, Jia-Yuan Liu, Bao-An Hong, Nie-Nie Qi, Xiang Peng, Bo-Wen Zhou, Jiu-Feng Zhang, Lin Cai, Kan Gong
Von Hippel-Lindau (VHL) disease is a rare autosomal dominant cancer syndrome caused by alterations of VHL gene. Patients are predisposed to develop pheochromocytomas and solid or cystic tumors of the central nervous system, kidney, pancreas, and retina. Remarkable phenotypic heterogeneity exits in organ involvement and tumor onset age between and within VHL families. However, no reliable markers have been found to predict the age-related tumor risks in VHL patients. A large Chinese cohort composed of 300 VHL patients and 92 healthy family controls was enrolled in our study...
August 4, 2017: Cancer Medicine
https://www.readbyqxmd.com/read/28770138/anterior-urethral-valve-uncommon-association-with-renal-duplicity
#7
Amina Ben Salem, Ines Mazhoud, Rachida Laamiri, Randa Salem, Hayet Laajili, Lassaad Sahnoun, Chiraz Hafsa
Anterior urethral valves (AUVs) is an unusual cause of congenital obstruction of the male urethra, being 15-30 times less common than posterior urethral valves. We present a case of AUV diagnosed at 24th gestational week. Ultrasonography and fetal MRI revealed hydronephrotic kidneys with ureteral duplicity, a distended bladder and perineal cystic mass which confirmed dilated anterior urethra in a male fetus. Diagnosis was confirmed postnatally by voiding cystourethrogram and surgery.
April 2017: Journal of Neonatal Surgery
https://www.readbyqxmd.com/read/28768225/complex-liver-cysts-in-autosomal-dominant-polycystic-kidney-disease
#8
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/28764564/neonatal-renal-cystic-diseases
#9
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
https://www.readbyqxmd.com/read/28754558/magnetic-resonance-t2-mapping-and-diffusion-weighted-imaging-for-early-detection-of-cystogenesis-and-response-to-therapy-in-a-mouse-model-of-polycystic-kidney-disease
#10
Mareike Franke, Bettina Baeßler, Jan Vechtel, Claudia Dafinger, Martin Höhne, Lori Borgal, Heike Göbel, Friederike Koerber, David Maintz, Thomas Benzing, Bernhard Schermer, Thorsten Persigehl
Polycystic kidney disease (PKD) is among the leading causes of end-stage renal disease. Increasing evidence exists that molecular therapeutic strategies targeted to cyst formation and growth might be more efficacious in early disease stages, highlighting the growing need for sensitive biomarkers. Here we apply quantitative magnetic resonance imaging techniques of T2 mapping and diffusion-weighted imaging in the jck mouse model for PKD using a clinical 3.0 T scanner. We tested whether kidney T2 values and the apparent diffusion coefficient (ADC) are superior to anatomical imaging parameters in the detection of early cystogenesis, as shown on macro- and histopathology...
July 26, 2017: Kidney International
https://www.readbyqxmd.com/read/28752022/ultrasound-imaging-of-cystic-nephroma
#11
REVIEW
Federico Greco, Eliodoro Faiella, Domiziana Santucci, Delia De Lisi, Gianguido Lo Vullo, Bruno Beomonte Zobel, Rosario Francesco Grasso
Cystic nephroma is a rare, benign multicystic lesion of the kidney. This tumor occurs both in children and in adults. In children, it is highly prevalent in males; in adults, it is more frequent in women. The term "cystic nephroma" represents two apparently different entities: pediatric cystic nephroma, a benign form thought to originate from metanephric tissue, and adult cystic nephroma, considered as a lesion of mixed epithelial stromal tumor. The clinical presentation may be a palpable mass or nonspecific symptoms such as abdominal pain, hematuria, and urinary tract infections...
2017: Journal of Kidney Cancer and VHL
https://www.readbyqxmd.com/read/28750036/generation-and-phenotypic-characterization-of-pde1a-mutant-mice
#12
Xiaofang Wang, Satsuki Yamada, Wells B LaRiviere, Hong Ye, Jason L Bakeberg, María V Irazabal, Fouad T Chebib, Jan van Deursen, Peter C Harris, Caroline R Sussman, Atta Behfar, Christopher J Ward, Vicente E Torres
It has been proposed that a reduction in intracellular calcium causes an increase in intracellular cAMP and PKA activity through stimulation of calcium inhibitable adenylyl cyclase 6 and inhibition of phosphodiesterase 1 (PDE1), the main enzymes generating and degrading cAMP in the distal nephron and collecting duct, thus contributing to the development and progression of autosomal dominant polycystic kidney disease (ADPKD). In zebrafish pde1a depletion aggravates and overexpression ameliorates the cystic phenotype...
2017: PloS One
https://www.readbyqxmd.com/read/28737145/congenital-hepatic-fibrosis-with-polycystic-kidney-disease-an-unusual-cause-of-neonatal-cholestasis
#13
Vani Bharani, G Vybhav Venkatesh, Uma Nahar Saikia, B R Thapa
Congenital hepatic fibrosis is characterized by hepatic fibrosis, portal hypertension, and renal cystic disease. Typical presentation of congenital hepatic fibrosis is in the form of portal hypertension, in adolescents and young adults. We present an unusual case of neonatal cholestasis with rapid deterioration within first 4 months of life, who was diagnosed to have congenital hepatic fibrosis with polycystic kidney disease on autopsy.
July 15, 2017: Indian Pediatrics
https://www.readbyqxmd.com/read/28736435/extracellular-vesicles-in-renal-disease
#14
REVIEW
Diana Karpman, Anne-Lie Ståhl, Ida Arvidsson
Extracellular vesicles, such as exosomes and microvesicles, are host cell-derived packages of information that allow cell-cell communication and enable cells to rid themselves of unwanted substances. The release and uptake of extracellular vesicles has important physiological functions and may also contribute to the development and propagation of inflammatory, vascular, malignant, infectious and neurodegenerative diseases. This Review describes the different types of extracellular vesicles, how they are detected and the mechanisms by which they communicate with cells and transfer information...
July 24, 2017: Nature Reviews. Nephrology
https://www.readbyqxmd.com/read/28729967/tesevatinib-ameliorates-progression-of-polycystic-kidney-disease-in-rodent-models-of-autosomal-recessive-polycystic-kidney-disease
#15
William E Sweeney, Philip Frost, Ellis D Avner
AIM: To investigate the therapeutic potential of tesevatinib (TSV), a unique multi-kinase inhibitor currently in Phase II clinical trials for autosomal dominant polycystic kidney disease (ADPKD), in well-defined rodent models of autosomal recessive polycystic kidney disease (ARPKD). METHODS: We administered TSV in daily doses of 7.5 and 15 mg/kg per day by I.P. to the well characterized bpk model of polycystic kidney disease starting at postnatal day (PN) 4 through PN21 to assess efficacy and toxicity in neonatal mice during postnatal development and still undergoing renal maturation...
July 6, 2017: World Journal of Nephrology
https://www.readbyqxmd.com/read/28724316/molecular-diagnosis-of-autosomal-dominant-polycystic-kidney-disease
#16
Xuewen Song, Amirreza Haghighi, Ioan-Andrei Iliuta, York Pei
Autosomal dominant polycystic kidney disease (ADPKD) is the most common inherited kidney disease that accounts for 5-10% of end-stage renal disease in developed countries. Mutations in PKD1 and PKD2 account for a majority of cases. Mutation screening of PKD1 is technically challenging largely due to the complexity resulting from duplication of its first 33 exons in six highly homologous pseudogenes (i.e. PKD1P1-P6). Protocol using locus-specific long-range and nested PCR has enabled comprehensive PKD1 mutation screening but is labor-intensive and costly...
July 20, 2017: Expert Review of Molecular Diagnostics
https://www.readbyqxmd.com/read/28709639/ganoderma-triterpenes-retard-renal-cyst-development-by-downregulating-ras-mapk-signaling-and-promoting-cell-differentiation
#17
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
#18
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
#19
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
#20
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
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