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https://www.readbyqxmd.com/read/27921040/new-insights-into-the-molecular-mechanisms-targeting-tubular-channels-transporters-in-pkd-development
#1
REVIEW
Ming Wu, Shengqiang Yu
BACKGROUND: Autosomal dominant polycystic kidney disease (PKD) or autosomal recessive PKD is caused by a mutation in the PKD1, PKD2 or PKHD1 gene, which encodes polycystin-1, polycystin-2 or fibrocystin, respectively. Embryonic and postnatal mutation studies show that transport or channel function is dysregulated before the initiation of cystogenesis, suggesting that the abnormality of transport or channel function plays a critical role in the pathology of PKD. SUMMARY: Polycystin-2 by itself is a calcium-permeable cation channel, and its channel function can be regulated by polycystin-1 or fibrocystin...
October 2016: Kidney Diseases
https://www.readbyqxmd.com/read/27921039/clinical-manifestation-and-management-of-adpkd-in-western-countries
#2
REVIEW
Claudia Sommerer, Martin Zeier
BACKGROUND: Autosomal dominant polycystic kidney disease (ADPKD) is the most common hereditary kidney disease in Western countries. The prevalence is between 2.4/10,000 and 3.9/10,000. ADPKD represents a systemic disease resulting in deterioration in renal function. Until now, mutations in two genes (PKD1 and PKD2) have been identified. Recently, the European Medicines Agency (EMA) approved the use of the vasopressin V2 receptor antagonist tolvaptan to slow the progression of cyst development and renal insufficiency connected with ADPKD in adult patients with chronic kidney disease stages 1-3 at initiation of treatment with evidence of rapidly progressing disease...
October 2016: Kidney Diseases
https://www.readbyqxmd.com/read/27920423/de-novo-renal-neoplasia-after-kidney-transplantation-according-to-new-2016-who-classification-of-renal-tumors
#3
Albino Eccher, Luigino Boschiero, Brett Delahunt, Luca Cima, Francesca Fior, Francesco Nacchia, Momo Rostand, Amedeo Carraro, Umberto Tedeschi, Gianluigi Zaza, Marilena Casartelli Liviero, Laura Zampicinini, Marco Chilosi, Giuseppe Feltrin, Claudio Rago, Antonietta D'Errico, Claudio Ghimenton, Guido Martignoni, Matteo Brunelli
BACKGROUND De novo renal neoplasia developing after kidney transplantation at Verona Kidney Transplant Center were reviewed according to new 2016 WHO Renal Tumor Classification. MATERIAL AND METHODS Primary renal tumors developed in native or transplanted kidneys de novo following renal transplantation were retrieved and histologically reviewed by three expert uropathologists. Immunoexpression of the diagnostic antigens CD13, CD10, CK7, CK34bE12, AMACR, CAIX, AE1/AE3, CK14, GATA-3, HMB-45, cathepsin-k, S100A1, and parvalbumin was assessed...
December 6, 2016: Annals of Transplantation: Quarterly of the Polish Transplantation Society
https://www.readbyqxmd.com/read/27899951/the-first-case-of-benign-multicystic-mesothelioma-presenting-as-a-splenic-mass
#4
Antonio D'Antonio, Carlo Baldi, Maria Addesso, Carmine Napolitano
Multicystic mesothelioma (MM) is a relatively rare tumour arising in the pelvic peritoneum of the tuboovarian region of young woman. Exceptionally, MM occurs on the serosal surfaces of various organs including kidney, bladder, lymph nodes, and liver. We report here the first case of MM wherein a 58-year-old woman with a previous history of endometriosis of the right ovary presented with a large multicystic mass of the spleen. The diagnosis of MM was made on a surgical specimen after splenectomy. A histopathologic examination is always necessary for the diagnosis of MM which should be differentiated from other lesions particularly from cystic lymphangioma...
2016: Ecancermedicalscience
https://www.readbyqxmd.com/read/27895019/nephrotoxicity-during-vancomycin-therapy-in-combination-with-piperacillin-tazobactam-or-cefepime
#5
W Cliff Rutter, Jessica N Cox, Craig A Martin, Donna R Burgess, David S Burgess
BACKGROUND: Recent reports have demonstrated that vancomycin (VAN) may lead to an increase in acute kidney injury (AKI) when combined with anti-pseudomonal beta-lactams. This study compared the incidence of AKI associated with VAN plus piperacillin-tazobactam (TZP) or cefepime (FEP). METHODS: This was a retrospective, matched cohort study at an academic medical center between September 2010 and September 2014 including adult patients receiving TZP-VAN or FEP-VAN for at least 48 hours and without severe chronic or structural kidney disease, dialysis, pregnancy, cystic fibrosis, or hospital transfer...
November 28, 2016: Antimicrobial Agents and Chemotherapy
https://www.readbyqxmd.com/read/27894621/glomerulocystic-kidney-presenting-as-a-unilateral-kidney-mass-in-a-newborn-with-tuberous-sclerosis-report-of-a-case-and-review-of-the-literature
#6
Miguel Rito, Rafael Adame Cabrera
Glomerular cysts are defined as a 2-3 times dilation of Bowman spaces and their presence in at least 5% of the glomeruli defines the kidneys as glomerulocystic (GCK). The association between cystic kidney disease and the tuberous sclerosis complex (TSC) is well known, but its presentation as a unilateral mass with glomerulocystic pattern is rare. We describe a case of an infant with a prenatal diagnosis of TSC, with a renal mass that was believed to be a renal tumor. A four-month-old infant with maternal history of TSC and prenatally diagnosed subependymal nodules and a right renal mass underwent nephrectomy...
November 18, 2016: Pathology, Research and Practice
https://www.readbyqxmd.com/read/27894620/clinical-implications-of-a-rare-renal-entity-pleomorphic-hyalinizing-angiectatic-tumor-phat
#7
Cristina Scalici Gesolfo, Vincenzo Serretta, Fabrizio Di Maida, Giulio Giannone, Elisabetta Barresi, Vito Franco, Rodolfo Montironi
Pleomorphic Hyalinizing Angiectatic Tumor (PHAT) is a rare benign lesion characterized by slow growth, infiltrative behavior and high rate of local recurrences. Only one case has been described in retroperitoneum, at renal hilum, but not involving pelvis or parenchyma. Here we present the first case of PHAT arising in the renal parenchyma. A nodular lesion in right kidney lower pole was diagnosed to a 61 year old woman. The patient underwent right nephrectomy. Microscopically, the lesion showed solid and pseudo-cystic components with hemorrhagic areas characterized by aggregates of ectatic blood vessels...
November 17, 2016: Pathology, Research and Practice
https://www.readbyqxmd.com/read/27880955/long-term-angiotensin-ii-receptor-blockade-limits-hypertension-aortic-dysfunction-and-structural-remodeling-in-a-rat-model-of-chronic-kidney-disease
#8
Omar Z Ameer, Mark Butlin, Elena Kaschina, Manuela Sommerfeld, Alberto P Avolio, Jacqueline K Phillips
BACKGROUND/AIMS: Chronic kidney disease (CKD) is associated with large artery remodeling, endothelial dysfunction and calcification, with angiotensin II (Ang II) a known driver of these pathologies. We investigated long-term Ang II type 1 receptor inhibition with valsartan on aortic function and structure in the Lewis polycystic kidney (LPK) rat model of CKD. METHODS: Mixed sex LPK and Lewis control (total n = 28) treated (valsartan 60 mg/kg/day p.o. from 4 to 18 weeks) and vehicle groups were studied...
November 24, 2016: Journal of Vascular Research
https://www.readbyqxmd.com/read/27871310/transcriptome-analysis-reveals-manifold-mechanisms-of-cyst-development-in-adpkd
#9
Rita M C de Almeida, Sherry G Clendenon, William G Richards, Michael Boedigheimer, Michael Damore, Sandro Rossetti, Peter C Harris, Britney-Shea Herbert, Wei Min Xu, Angela Wandinger-Ness, Heather H Ward, James A Glazier, Robert L Bacallao
BACKGROUND: Autosomal dominant polycystic kidney disease (ADPKD) causes progressive loss of renal function in adults as a consequence of the accumulation of cysts. ADPKD is the most common genetic cause of end-stage renal disease. Mutations in polycystin-1 occur in 87% of cases of ADPKD and mutations in polycystin-2 are found in 12% of ADPKD patients. The complexity of ADPKD has hampered efforts to identify the mechanisms underlying its pathogenesis. No current FDA (Federal Drug Administration)-approved therapies ameliorate ADPKD progression...
November 21, 2016: Human Genomics
https://www.readbyqxmd.com/read/27862070/are-ultrasound-renal-aspects-associated-with-urinary-biochemistry-in-fetuses-with-lower-urinary-tract-obstruction
#10
Ahmed A Nassr, Chester Koh Koh, Alireza A Shamshirsaz, Jimmy Espinoza, Haleh Sangi-Haghpeykar, Dina Sharhan, Stephen Welty, Joseph Angelo, David Roth, Michael A Belfort, Michael Braun, Rodrigo Ruano
OBJECTIVE: To evaluate the association between ultrasonographic renal parameters and urine biochemistry in fetuses with lower urinary tract obstruction (LUTO). METHODS: Data were collected prospectively from 31 consecutive fetuses with LUTO that underwent vesicocentesis for fetal urinary biochemistry between April 2013 and September 2015. The following renal ultrasound markers were assessed immediately before the vesicocentesis: renal echogenicity, presence of cortical cysts, presence of findings suggestive of 'renal dysplasia' (hyperechogenic cystic kidneys with no cortical-medullary differentiation) and severe oligohydramnios (amniotic fluid < 5th percentile)...
November 12, 2016: Prenatal Diagnosis
https://www.readbyqxmd.com/read/27853915/renal-cell-carcinoma-applicability-of-the-apparent-coefficient-of-the-diffusion-weighted-estimated-by-mri-for-improving-their-differential-diagnosis-histologic-subtyping-and-differentiation-grade
#11
Yulian Mytsyk, Ihor Dutka, Yuriy Borys, Iryna Komnatska, Iryna Shatynska-Mytsyk, Ammad Ahmad Farooqi, Katarina Gazdikova, Martin Caprnda, Luis Rodrigo, Peter Kruzliak
BACKGROUND: Renal cell carcinoma (RCC) represents the most common malignant epithelial neoplasm of the kidney. Accurate assessment of the renal masses, defining the histologic subtype and the grade of differentiation of the tumor, is vital to ensure an adequate case management as well as for staging and prognosis. Recently, diffusion-weighted imaging (DWI) magnetic resonance imaging (MRI) tends to be increasingly appealing for the clinicians as an imaging procedure of choice for the diagnosis and staging of the RCC, which is predetermined by several advantages over CT...
November 16, 2016: International Urology and Nephrology
https://www.readbyqxmd.com/read/27853247/six2crefrs2%C3%AE-knockout-mice-are-a-novel-model-of-renal-cystogenesis
#12
Pawan Puri, Daniel Bushnell, Caitlin M Schaefer, Carlton M Bates
Six2cre-mediated deletion of Frs2α (Six2creFrs2αKO), a major fibroblast growth factor receptor (Fgfr) docking protein in mouse nephron progenitors results in perinatal renal hypoplasia; however, postnatal Six2creFrs2αKO kidneys develop cysts. We sought to determine the pathogenesis of Six2creFrs2αKO cyst formation. We performed histological assays, Western blots, and quantitative PCR (qPCR). While embryonic day (E) 18.5 Six2Frs2αKO kidneys were hypoplastic and not cystic, postnatal day (P) 7 mutants had proximal tubular-derived cysts that nearly replaced the renal parenchyma by P21...
November 17, 2016: Scientific Reports
https://www.readbyqxmd.com/read/27831959/cystic-nephroma-in-adults-a-clinicopathologic-study-of-46-cases
#13
Anna Caliò, John N Eble, David J Grignon, Brett Delahunt
Cystic nephroma in adults is an uncommon multicystic benign tumor of kidney and, over the last century, only a few small series or case reports have studied it. We analyzed 46 tumors from adults (41 female, median age: 55 y), collecting data on gross, microscopic, and immunohistochemical findings. All of the tumors were composed entirely of cysts separated by septa; the majority were surrounded by a pseudocapsule. Hypocellular fibrous and hypercellular spindle cell stroma were the most common components of the septa...
December 2016: American Journal of Surgical Pathology
https://www.readbyqxmd.com/read/27812213/hif-stabilization-weakens-primary-cilia
#14
Andrew Resnick
Although solitary or sensory cilia are present in most cells of the body and their existence has been known since the sixties, very little is known about their functions. One suspected function is fluid flow sensing- physical bending of cilia produces an influx of Ca++, which can then result in a variety of activated signaling pathways. Defective cilia and ciliary-associated proteins have been shown to result in cystic diseases. Autosomal Dominant Polycystic Kidney Disease (ADPKD) is a progressive disease, typically appearing in the 5th decade of life and is one of the most common monogenetic inherited human diseases, affecting approximately 600,000 people in the United States...
2016: PloS One
https://www.readbyqxmd.com/read/27796021/-renal-transplantation-in-autosomal-dominant-polycystic-kidney-disease-adpkd
#15
Norberto Perico, Monica Cortinovis, Giuseppe Remuzzi
Transplantation is the optimal choice for renal replacement therapy in patients with autosomal dominant polycystic kidney disease (ADPKD). However, some specific issues should be addressed before transplantation, including nephrectomy of native kidneys, cystic liver involvement, screening for intracranial aneurysms and living related-donor transplantation. After kidney transplantation, patient and graft survival rates are excellent and the size of native kidneys typically declines. Nevertheless, a number of renal and extrarenal complications have been documented in kidney transplant recipients with ADPKD...
September 2016: Giornale Italiano di Nefrologia: Organo Ufficiale Della Società Italiana di Nefrologia
https://www.readbyqxmd.com/read/27796020/-treatment-of-autosomal-dominant-polycystic-kidney-disease-adpkd-somatostatin-analogues-and-mtor-inhibitors
#16
Norberto Perico, Monica Cortinovis, Giuseppe Remuzzi
Recent advances in the understanding of the molecular mechanisms underlying autosomal dominant polycystic kidney disease (ADPKD) set the stage for the development of various treatments aimed to arrest or delay disease progression. In particular, clinical trials showed that the use of somatostatin analogues in patients with ADPKD is able to slow down the increase in total kidney volume and the progressive decline in renal function over the long-term. Treatment with these agents is generally well tolerated and it also enables to control cyst growth in the liver in patients with this extra-renal manifestation of the disease...
September 2016: Giornale Italiano di Nefrologia: Organo Ufficiale Della Società Italiana di Nefrologia
https://www.readbyqxmd.com/read/27793968/ciliopathies
#17
Daniela A Braun, Friedhelm Hildebrandt
Nephronophthisis-related ciliopathies (NPHP-RC) are a group of inherited diseases that affect genes encoding proteins that localize to primary cilia or centrosomes. With few exceptions, ciliopathies are inherited in an autosomal recessive manner, and affected individuals manifest early during childhood or adolescence. NPHP-RC are genetically very heterogeneous, and, currently, mutations in more than 90 genes have been described as single-gene causes. The phenotypes of NPHP-RC are very diverse, and include cystic-fibrotic kidney disease, brain developmental defects, retinal degeneration, skeletal deformities, facial dimorphism, and, in some cases, laterality defects, and congenital heart disease...
October 28, 2016: Cold Spring Harbor Perspectives in Biology
https://www.readbyqxmd.com/read/27790546/bilateral-renal-lymphangiectasia
#18
Vaidehi K Pandya, Maulin K Shah, Shruti P Gandhi, Himanshu V Patel
Renal Lymphangiectasia (RLM) is very rare benign lymphatic malformation. It can be misdiagnosed for other cystic renal masses, most commonly polycystic kidneys. Though incidentally found in most cases, it may be the cause for hypertension and renal failure in undiagnosed patients. Here, we report a case of an adult asymptomatic male with bilateral RLM which was detected as an incidental finding on ultrasound. Confirmation by CT-scan and laboratory diagnosis of aspirated fluid was done, and patient was managed conservatively...
September 2016: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/27779799/diagnosis-and-long-term-outcome-of-renal-cysts-after-laparoscopic-partial-nephrectomy-in-children
#19
Ciro Esposito, Maria Escolino, Bernardita Troncoso Solar, Roberta Iacona, Rosanna Esposito, Alessandro Settimi, Imran Mushtaq
OBJECTIVE: To document the imaging follow-up of laparoscopic partial nephrectomy (LPN) in children and to investigate the natural history of cystic lesions following LPN. PATIENTS AND METHODS: We reviewed the ultrasonography (US) imaging reports performed during the follow-up of 125 children (77 girls, 48 boys; mean age 3.2 years) who underwent LPN in two centres of paediatric surgery in the period 2005-2015. RESULTS: A transperitoneal approach was adopted in 83 children and a retroperitoneal approach in 42...
October 25, 2016: BJU International
https://www.readbyqxmd.com/read/27776379/-unknown-title
#20
M Kadihasanoglu, M Kilciler, O Atahan
A 44-year-old woman, who had had left flank pain for the previous 3 months, was treated successfully for renal hydatid cyst disease by using an endoscopic technique with percutaneous access through an all seeing needle. Abdominal ultrasonography showed a Gharbi type III cyst in the lower pole of the left kidney. Computerised tomography of the abdomen revealed a bulky solid-cystic mass with calcified and well-defined wall and daughter cyst without contrast enhancement. The patient, who refused any renal operation, underwent a percutaneous intervention with access through an all seeing needle access...
October 24, 2016: Aktuelle Urologie
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