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https://www.readbyqxmd.com/read/29215110/quercetin-inhibits-renal-cyst-growth-in-vitro-and-via-parenteral-injection-in-a-polycystic-kidney-disease-mouse-model
#1
Yangyang Zhu, Tian Teng, Hu Wang, Hao Guo, Lei Du, Baoxue Yang, Xiaoxing Yin, Ying Sun
Autosomal dominant polycystic kidney disease (ADPKD) is a common monogenic disease characterized by massive enlargement of fluid-filled cysts in the kidney. There is an urgent need to develop effective ADPKD therapies. We used an in vitro Madin-Darby canine kidney (MDCK) cyst model and a murine embryonic kidney cyst model to evaluate whether quercetin inhibits cyst development. We then used a polycystic kidney disease (PKD) mouse model to further determine the in vivo effects of quercetin (100 mg per kg body weight twice per day) on PKD mice via subcutaneous injections...
December 7, 2017: Food & Function
https://www.readbyqxmd.com/read/29208204/durable-renal-response-after-combination-of-bortezomib-corticosteroids-rituximab-and-plasmapheresis-for-late-antibody-mediated-renal-transplant-rejection%C3%A2
#2
Yanli Ding, Jean Francis, Amitabh Gautam, Linda Pelletier, Vaishali Sanchorawala, Karen Quillen
Late occurrence of antibody-mediated rejection (AMR), defined as occurring 6 months after transplantation, is associated with poor renal allograft survival, compared to early acute AMR and acute cellular rejection. The proteasome inhibitor bortezomib has multiple immunomodulatory effects on plasma cells, the source of donor-specific HLA antibodies which mediate AMR. MATERIALS AND METHODS: Consecutive patients who presented with biopsy-proven AMR and donor-specific anti-HLA antibodies (DSA) at a single institution between July 2011 and February 2015 were included...
December 6, 2017: Clinical Nephrology
https://www.readbyqxmd.com/read/29204517/proteomic-analysis-of-aqp11-null-kidney-proximal-tubular-type-polycystic-kidney-disease
#3
Tatsuya Saito, Yasuko Tanaka, Yoshiyuki Morishita, Kenichi Ishibashi
Autosomal Dominant Polycystic Kidney Disease (ADPKD) is caused by the mutation of polycystins (PC-1 or PC-2), in which cysts start from the collecting duct to extend to all nephron segments with eventual end stage renal failure. The cyst development is attenuated by a vasopressin V2 receptor antagonist tolvaptan which, however, will not affect proximal tubule cysts devoid of V2 receptor. Aquaporin-11 (AQP11) is expressed selectively in the proximal tubule of the kidney and AQP11-null kidneys have a disruptive PC-1 trafficking to the plasma membrane to develop polycystic kidneys...
March 2018: Biochemistry and Biophysics Reports
https://www.readbyqxmd.com/read/29203870/ift-proteins-spatially-control-the-geometry-of-cleavage-furrow-ingression-and-lumen-positioning
#4
Nicolas Taulet, Benjamin Vitre, Christelle Anguille, Audrey Douanier, Murielle Rocancourt, Michael Taschner, Esben Lorentzen, Arnaud Echard, Benedicte Delaval
Cytokinesis mediates the physical separation of dividing cells and, in 3D epithelia, provides a spatial landmark for lumen formation. Here, we unravel an unexpected role in cytokinesis for proteins of the intraflagellar transport (IFT) machinery, initially characterized for their ciliary role and their link to polycystic kidney disease. Using 2D and 3D cultures of renal cells, we show that IFT proteins are required to correctly shape the central spindle, to control symmetric cleavage furrow ingression and to ensure central lumen positioning...
December 4, 2017: Nature Communications
https://www.readbyqxmd.com/read/29184052/notch-transactivates-rheb-to-maintain-the-multipotency-of-tsc-null-cells
#5
Jun-Hung Cho, Bhaumik Patel, Santosh Bonala, Sasikanth Manne, Yan Zhou, Surya K Vadrevu, Jalpa Patel, Marco Peronaci, Shanawaz Ghouse, Elizabeth P Henske, Fabrice Roegiers, Krinio Giannikou, David J Kwiatkowski, Hossein Mansouri, Maciej M Markiewski, Brandon White, Magdalena Karbowniczek
Differentiation abnormalities are a hallmark of tuberous sclerosis complex (TSC) manifestations; however, the genesis of these abnormalities remains unclear. Here we report on mechanisms controlling the multi-lineage, early neuronal progenitor and neural stem-like cell characteristics of lymphangioleiomyomatosis (LAM) and angiomyolipoma cells. These mechanisms include the activation of a previously unreported Rheb-Notch-Rheb regulatory loop, in which the cyclic binding of Notch1 to the Notch-responsive elements (NREs) on the Rheb promoter is a key event...
November 29, 2017: Nature Communications
https://www.readbyqxmd.com/read/29181500/perinatal-diagnosis-management-and-follow-up-of-cystic-renal-diseases-a-clinical-practice-recommendation-with-systematic-literature-reviews
#6
Charlotte Gimpel, Fred E Avni, Carsten Bergmann, Metin Cetiner, Sandra Habbig, Dieter Haffner, Jens König, Martin Konrad, Max C Liebau, Lars Pape, Georg Rellensmann, Andrea Titieni, Constantin von Kaisenberg, Stefanie Weber, Paul J D Winyard, Franz Schaefer
Importance: Prenatal and neonatal cystic kidney diseases are a group of rare disorders manifesting as single, multiple unilateral, or bilateral cysts or with increased echogenicity of the renal cortex without macroscopic cysts. They may be accompanied by grossly enlarged kidneys, renal oligohydramnios, pulmonary hypoplasia, extrarenal abnormalities, and neonatal kidney failure. The prognosis is extremely variable from trivial to very severe or even uniformly fatal, which poses significant challenges to prenatal counseling and management...
November 27, 2017: JAMA Pediatrics
https://www.readbyqxmd.com/read/29181208/large-retroperitoneal-haemorrhage-following-cyst-rupture-in-a-patient-with-autosomal-dominant-polycystic-kidney-disease
#7
Holly Mabillard, Shalabh Srivastava, Philip Haslam, Maciej Karasek, John A Sayer
The complications of autosomal dominant polycystic kidney disease (ADPKD) include cyst rupture and haemorrhage leading to loin pain and frank haematuria. Risk factors include large kidney volume, hypertension, and renal impairment. We present a case of a young male who, following trauma to the kidney, had a life threatening bleed from his polycystic kidney. The case was initially treated with fluid resuscitation and blood transfusion but necessitated radiological embolization of bleeding source to control the blood loss...
2017: Case Reports in Nephrology
https://www.readbyqxmd.com/read/29174191/expanding-the-definition-of-a-benign-renal-cyst-on-contrast-enhanced-ct-can-incidental-homogeneous-renal-masses-measuring-21-39%C3%A2-hu-be-safely-ignored
#8
Eric M Hu, James H Ellis, Stuart G Silverman, Richard H Cohan, Elaine M Caoili, Matthew S Davenport
RATIONALE AND OBJECTIVE: We aimed to determine the frequency and clinical significance of homogeneous renal masses measuring 21-39 Hounsfield units on contrast-enhanced computed tomography (CT). METHODS: Subjects 40-69 years old undergoing portal-venous-phase contrast-enhanced abdominal CT from January 1, 2006 to December 31, 2010 with slice thickness ≤5 mm and no prior CT or magnetic resonance imaging were identified (n = 1387) for this institutional review board-approved retrospective cohort study...
November 23, 2017: Academic Radiology
https://www.readbyqxmd.com/read/29169385/standardised-outcomes-in-nephrology-polycystic-kidney-disease-song-pkd-study-protocol-for-establishing-a-core-outcome-set-in-polycystic-kidney-disease
#9
Yeoungjee Cho, Benedicte Sautenet, Gopala Rangan, Jonathan C Craig, Albert C M Ong, Arlene Chapman, Curie Ahn, Dongping Chen, Helen Coolican, Juliana Tze-Wah Kao, Ron Gansevoort, Ronald Perrone, Tess Harris, Vicente Torres, York Pei, Peter G Kerr, Jessica Ryan, Talia Gutman, Martin Howell, Angela Ju, Karine E Manera, Armando Teixeira-Pinto, Lorraine A Hamiwka, Allison Tong
BACKGROUND: Autosomal dominant polycystic kidney disease (ADPKD) is the most common potentially life threatening inherited kidney disease and is responsible for 5-10% of cases of end-stage kidney disease (ESKD). Cystic kidneys may enlarge up to 20 times the weight of a normal kidney due to the growth of renal cysts, and patients with ADPKD have an increased risk of morbidity, premature mortality, and other life-time complications including renal and hepatic cyst and urinary tract infection, intracranial aneurysm, diverticulosis, and kidney pain which impair quality of life...
November 23, 2017: Trials
https://www.readbyqxmd.com/read/29169151/cystic-lung-disease-in-genetic-syndromes-with-deficient-tumor-suppressor-gene-function
#10
Cécile Daccord, Laurent P Nicod, Romain Lazor
Cystic lung diseases constitute a distinct group of rare lung disorders, among which two result from monogenic defects affecting tumor suppressor genes: lymphangioleiomyomatosis, either sporadic or associated with tuberous sclerosis complex, and Birt-Hogg-Dubé syndrome. These disorders have similarities in their clinical expression, including occurrence in young adults, multiple pulmonary cysts, recurrent pneumothorax, skin hamartomas, and renal tumors. However, they markedly differ in their gender distribution, pathogenesis, disease course, and prognosis...
November 23, 2017: Respiration; International Review of Thoracic Diseases
https://www.readbyqxmd.com/read/29167138/recent-advances-in-management-of-autosomal-dominant-polycystic-kidney-disease
#11
REVIEW
Jacob W Potts, Shaker A Mousa
PURPOSE: Promising developments in the search for effective pharmacotherapies for autosomal-dominant polycystic kidney disease (ADPKD) are reviewed. SUMMARY: The formation and development of cysts characteristic of ADPKD result in inexorable renal and extrarenal manifestations that give rise to more rapid disease progression and more widespread complications than are seen with other forms of chronic kidney disease. To date, no agent has gained Food and Drug Administration marketing approval for use in patients with ADPKD, complicating efforts to meet the medical needs of this population...
December 1, 2017: American Journal of Health-system Pharmacy: AJHP
https://www.readbyqxmd.com/read/29159954/unraveling-the-mechanistic-complexity-of-the-glomerulocystic-phenotype-in-dicer-conditional-ko-mice-by-2d-gel-electrophoresis-coupled-mass-spectrometry
#12
Claudia Vincenza Fiumara, Domenica Scumaci, Anna Iervolino, Angela Mena Perri, Antonio Concolino, Laura Tammè, Federica Petrillo, Giovambattista Capasso, Giovanni Cuda
PURPOSE: Dicer, an RNase III type endonuclease, is a key enzyme involved in miRNA biogenesis. It has been shown that this enzyme is essential for several aspects of postnatal kidney functions and homeostasis. In this study, we have examined conditional knockout (cKO) mice for Dicer in Pax8 (Paired-box gene 8) expressing cells to investigate the kidney protein profile. This specific model develops a glomerulocystic phenotype coupled with urinary concentration impairment, proteinuria and severe renal failure...
November 21, 2017: Proteomics. Clinical Applications
https://www.readbyqxmd.com/read/29157599/skin-lesions-of-birt-hogg-dub%C3%A3-syndrome-clinical-and-histopathological-findings-in-31-japanese-patients-who-presented-with-pneumothorax-and-or-multiple-lung-cysts
#13
Chikako Iwabuchi, Hiroki Ebana, Akira Ishiko, Azusa Negishi, Teruaki Mizobuchi, Toshio Kumasaka, Masatoshi Kurihara, Kuniaki Seyama
BACKGROUND: Birt-Hogg-Dubé syndrome (BHDS) (OMIM #135150) is an autosomal dominant disease, characterized by fibrofolliculomas (FFs) of the skin, pulmonary cysts with/without pneumothorax, and renal tumors. The prevalence of skin manifestations reported for Japanese BHDS patients is lower (<30%) compared with that of Western countries (75∼90%), which appear to be underestimated. OBJECTIVE: To precisely examine the prevalence of skin lesions with dermoscopy and histopathology with reference to genetic analyses...
November 2, 2017: Journal of Dermatological Science
https://www.readbyqxmd.com/read/29146893/von-hippel-lindau-disease-with-multi-organ-involvement-a-case-report-and-8-year-clinical-course-with-follow-up
#14
Ali Yaghobi Joybari, Payam Azadeh
BACKGROUND Von Hippel-Lindau (VHL) disease is a rare autosomal dominant syndrome manifested by a spectrum of benign and malignant tumors.  CASE REPORT The patient presented here was a 31-year-old female with unremarkable family history who presented initially complaining of intermittent abdominal pain. Abdominal CT scan revealed an inhomogeneous solid mass (13×9×7 cm) originating from the tail of the pancreas with splenic and gastric invasion as well as several pancreatic cysts. A nucleotide scan showed left adrenal involvement...
November 17, 2017: American Journal of Case Reports
https://www.readbyqxmd.com/read/29145907/-autosomal-dominant-polycystic-kidney-disease-should-patients-young-adult-relatives-be-screened-or-not
#15
B J Kramers, M Storm, R T Gansevoort
Autosomal dominant polycystic kidney disease (ADPKD) is the most common hereditary renal disease, with a global prevalence of 10 per 10,000. It is characterized by the formation of numerous cysts in both kidneys, and leads to renal function loss; the majority of patients will eventually need renal replacement therapy. It is possible to screen patients' presymptomatic family members from a young age, but this has not historically been recommended as until recently there were no treatment options. This year, the vasopressin V2 receptor antagonist tolvaptan was approved for prescription in ADPKD, to slow the rate of renal function decline...
2017: Nederlands Tijdschrift Voor Geneeskunde
https://www.readbyqxmd.com/read/29145282/autosomal-dominant-polycystic-kidney-disease-combined-with-hypertrophic-cardiomyopathy-a-case-report
#16
Yingjing Shen, Chenggang Xu
INTRODUCTION: This report describes the novel sampling of autosomal dominant polycystic kidney disease (ADPKD) combined with hypertrophic cardiomyopathy (HCM). SYMPTOMS AND CLINICAL FINDINGS: A 48-year-old Chinese man presented with anasarca, hypourocrinia, gross hematuria, and weight gain by 10 kg subsequently developed acute kidney injury after struck by acute respiratory distress syndrome, really a threat to his heart. DIAGNOSES: Abdominal ultrasound revealed multiple small cysts in both kidneys, with the right kidney measuring 11...
November 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29132348/extraperitoneal-laparoscopic-resection-for-retroperitoneal-lymphatic-cysts-initial-experience
#17
Yichun Wang, Chen Chen, Chuanjie Zhang, Chao Qin, Ninghong Song
BACKGROUND: To assess the safety and efficacy of laparoscopic retroperitoneal resection for retroperitoneal lymphatic cysts. METHODS: A retrospective analysis was conducted based on clinical data from eight patients with hydronephrosis caused by retroperitoneal lymphatic cysts. All patients underwent laparoscopic retroperitoneal lymphatic cyst resection and received postoperative follow-up. A follow-up ultrasound was performed postoperatively every 6-12 months to evaluate the recovery of the hydronephrosis...
November 13, 2017: BMC Urology
https://www.readbyqxmd.com/read/29131363/automatic-renal-lesion-segmentation-in-ultrasound-images-based-on-saliency-features-improved-lbp-and-an-edge-indicator-under-level-set-framework
#18
Luying Gui, Xiaoping Yang
PURPOSE: Segmentation of lesions in ultrasound images is widely used for preliminary diagnosis. In this paper, we develop an automatic segmentation algorithm for multiple types of lesions in ultrasound images. The proposed method is able to detect and segment lesions automatically as well as generate accurate segmentation results for lesion regions. METHODS: In the detection step, two saliency detection frameworks which adopt global image information are designed to capture the differences between normal and abnormal organs as well as these between lesions and the normal tissues around them...
November 13, 2017: Medical Physics
https://www.readbyqxmd.com/read/29128599/cutaneous-metastasis-of-renal-cell-carcinoma-masquerading-as-an-infected-sebaceous-cyst
#19
R J Boaz, T Vig, A J Tirkey, N T John, R M Kumar, N Kekre
Renal cell carcinoma (RCC) is the third most common genitourinary malignancy with an estimated one third of cases with metastatic disease at the time of diagnosis. Though rare, cutaneous metastasis from RCC is more frequent than any other genitourinary malignancy. Metastasis of RCC to skin carries poor prognosis as coexistent visceral spread is the norm. A 38-year-old man presented one year after radical nephrectomy for clear cell carcinoma of the left kidney with a submental lesion that proved to be metastatic...
November 8, 2017: Journal of Stomatology, Oral and Maxillofacial Surgery
https://www.readbyqxmd.com/read/29123444/imaging-findings-of-the-unusual-presentations-associations-and-clinical-mimics-of-acute-appendicitis
#20
REVIEW
Mustafa Kemal Demir, Yildiray Savas, Yavuz Furuncuoglu, Tarik Cevher, Serdar Demiral, Babek Tabandeh, Melisa Aslan
There are many kinds of unusual presentations or associations and clinical mimics of acute appendicitis, and definitive diagnosis requires knowledge of the imaging findings in some cases. The unusual presentations and associations of acute appendicitis included in this study are perforated appendicitis, acute appendicitis occurring in hernias, acute appendicitis with cystic endosalpingiosis, intussusception of appendix, and acute appendicitis with pregnancy. We also present uncommon gastrointestinal, urinary and gynecologic clinical mimics of acute appendicitis including anomalous congenital band, duplication cysts, giant Meckel's diverticulitis, inflammatory fibroid polyp, renal artery thrombosis, spontaneous urinary extravasation and OHVIRA syndrome...
October 2017: Eurasian Journal of Medicine
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