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https://www.readbyqxmd.com/read/29323077/epidermoid-cyst-of-the-renal-pelvis-masquerading-as-malignancy
#1
Dinesh Pradhan, Gabriela Quiroga-Garza, Ronald Hrebinko, Rajiv Dhir, Anil V Parwani
Epidermoid cyst of the renal pelvis is exceptionally rare. The histogenetic mechanism has not been well characterized. Herein, we report a case of intrarenal epidermoid cyst in a 62-year-old woman who had undergone left nephrolithotomy for a staghorn calculus. She was being followed up for bilateral renal cysts when a complex mass was noted arising from the lower pole of the left kidney. Renal ultrasound showed a small left kidney with a solid vascular echogenic mass. A laparoscopic radical nephrectomy was performed...
October 2017: Indian Journal of Pathology & Microbiology
https://www.readbyqxmd.com/read/29306149/a-case-report-of-intrarenal-epidermoid-cysts-in-a-yellow-bellied-slider-trachemys-scripta-scripta
#2
Simon Stokes, Nicola Di Girolamo, Paolo Selleri, Sara Pagliarani, Chiara Palmieri
A 7-year-old yellow-bellied slider exhibited anorexia, decreased activity, generalised wasting of skeletal muscles and oedema. Haematology examination revealed increased phosphorus and decreased calcium levels. During necropsy performed after spontaneous death, a focal nodular lesion containing tan amorphous material was found in the left kidney. Histopathology examination revealed multiple cystic lesions lined by a multilayered squamous, occasionally cuboidal, and containing keratin. Epithelial cells and keratin material were cytokeratin-positive...
December 27, 2017: Research in Veterinary Science
https://www.readbyqxmd.com/read/29290488/crystal-structure-of-bicc1-sam-polymer-and-mapping-of-interactions-between-the-ciliopathy-associated-proteins-bicc1-anks3-and-anks6
#3
Benjamin Rothé, Catherine N Leettola, Lucia Leal-Esteban, Duilio Cascio, Simon Fortier, Manuela Isenschmid, James U Bowie, Daniel B Constam
Head-to-tail polymers of sterile alpha motifs (SAM) can scaffold large macromolecular complexes. Several SAM-domain proteins that bind each other are mutated in patients with cystic kidneys or laterality defects, including the Ankyrin (ANK) and SAM domain-containing proteins ANKS6 and ANKS3, and the RNA-binding protein Bicc1. To address how their interactions are regulated, we first determined a high-resolution crystal structure of a Bicc1-SAM polymer, revealing a canonical SAM polymer with a high degree of flexibility in the subunit interface orientations...
December 26, 2017: Structure
https://www.readbyqxmd.com/read/29285364/bilateral-and-multiple-mixed-epithelial-and-stromal-tumors-of-the-kidney-a-case-report
#4
Ryo Minoda, Toshio Takagi, Naohiro Toda, Hiroko Itagaki, Tsunenori Kondo, Hideki Ishida, Yoji Nagashima, Kazunari Tanabe
Mixed epithelial and stromal tumor (MEST) of the kidney is a benign tumor occurring predominantly in older women. Histologically, MEST comprises tubuloglandular and cystic structures in an ovarian-like stroma. Bilateral and multiple cases are rare. We herein present a case of bilateral and multiple MEST in a 43-year-old woman. The patient presented with gross hematuria and was referred to a primary care physician. A computed tomography scan revealed 5 tumors in the right and 2 in the left kidney. Renal cell carcinoma was suspected, and left partial nephrectomy followed by right radical nephrectomy was planned...
December 2017: Molecular and Clinical Oncology
https://www.readbyqxmd.com/read/29280706/renal-endometriosis-mimicking-complicated-cysts-of-kidney-report-of-two-cases
#5
D Giambelluca, D Albano, E Giambelluca, A Bruno, F Panzuto, A Agrusa, G Di Buono, F Cannizzaro, Cesare Gagliardo, M Midiri, R Lagalla, G Salvaggio
Endometriosis is a common gynecologic disorder characterized by ectopic endometrial tissue growth outside the uterine cavity. Although usually occurring in pelvic organs, endometrial lesions may involve urinary tract. Renal endometriosis is extremely rare and it has only occasionally been reported in the past. We report two cases of patients with renal cystic lesions, incidentally found at imaging techniques during oncologic follow-up for gastric sarcoma and melanoma, initially misinterpreted as complicated haemorrhagic cysts and then histologically characterized as renal localizations of extragenital endometriosis...
September 2017: Il Giornale di Chirurgia
https://www.readbyqxmd.com/read/29230706/differential-diagnosis-of-the-small-renal-masses-role-of-the-apparent-diffusion-coefficient-of-the-diffusion-weighted-mri
#6
Yulian Mytsyk, Ihor Dutka, Borys Yuriy, Iryna Maksymovych, Martin Caprnda, Katarina Gazdikova, Luis Rodrigo, Peter Kruzliak, Polina Illjuk, Ammad Ahmad Farooqi
INTRODUCTION: Renal cell carcinoma (RCC) accounts for approximately 3% of adult malignancies and more than 90% of neoplasms arising from the kidney. Uninformative percutaneous kidney biopsies vary from 10 to 23%. As a result, 7.5-33.6% of partial nephrectomies in patients with small renal masses (SRM) are performed on benign renal tumors. The aim of this study was to assess the feasibility of the apparent diffusion coefficient (ADC) of the diffusion-weighted imaging (DWI) of MRI, as RCC imaging biomarker for differentiation of SRM...
December 11, 2017: International Urology and Nephrology
https://www.readbyqxmd.com/read/29229467/renal-involvement-in-pmm2-cdg-a-mini-review
#7
REVIEW
Ruqaiah Altassan, Peter Witters, Zubaida Saifudeen, Dulce Quelhas, Jaak Jaeken, Elena Levtchenko, David Cassiman, Eva Morava
Phosphomannomutase 2 deficiency (PMM2-CDG) is the most common N-linked glycosylation disorder. The majority of patients present with a multisystem phenotype, including central nervous system involvement, hepatopathy, gastrointestinal and cardiac symptoms, endocrine dysfunction and abnormal coagulation. Renal abnormalities including congenital malformations and altered renal function are part of the multisystem manifestations of congenital disorders of glycosylation. We reviewed the literature on 933 patients with molecularly and/or enzymatically confirmed PMM2 deficiency to evaluate the incidence of renal involvement in PMM2-CDG...
November 28, 2017: Molecular Genetics and Metabolism
https://www.readbyqxmd.com/read/29220099/supportive-palliative-care-should-be-integrated-into-routine-care-for-paediatric-patients-with-life-limiting-kidney-disease
#8
REVIEW
Julia Thumfart, Tobias Reindl, Cornelia Rheinlaender, Dominik Müller
Paediatric palliative care is no longer restricted to patients with cancer and has been extended to patients with other chronic conditions, such as cystic fibrosis or neuromuscular disorders. This review focused on the current state of palliative care for children and adolescents with chronic kidney disease (CKD). We assessed the literature on CKD published up to August 2017. All the papers, except one from 1996, were published this century. This review discusses the role that palliative care plays in the process of decision-making and explores the possibilities of implementing palliative care into the routine therapy of affected patients and providing support for their families...
December 8, 2017: Acta Paediatrica
https://www.readbyqxmd.com/read/29218489/contrast-enhanced-ultrasound-of-the-kidney-a-single-institution-experience
#9
Sheng F Oon, Robert W Foley, Deirdre Quinn, David M Quinlan, Robert G Gibney
BACKGROUND: Focal renal masses are typically evaluated by means of triphasic contrast-enhanced CT or MRI scan but use of iodinated contrast or gadolinium is unsuitable for some patients. Contrast-enhanced ultrasound (CEUS) is an imaging alternative in this scenario but has limited availability in Ireland. AIM: The aim of the study was to retrospectively evaluate experience with selective use of CEUS for non-invasive characterization of focal renal masses in a tertiary referral institution in Ireland, with a particular focus on cystic renal lesions and the influence of CEUS on final Bosniak classification and treatment outcomes...
December 7, 2017: Irish Journal of Medical Science
https://www.readbyqxmd.com/read/29217307/autosomal-dominant-tubulointerstitial-kidney-disease-due-to-muc1-mutation
#10
Samuel Mon-Wei Yu, Anthony J Bleyer, Kisra Anis, Leal Herlitz, Martina Živná, Helena Hůlková, Glen S Markowitz, Belinda Jim
Mucin 1 kidney disease, previously referred to as medullary cystic kidney disease type 1, is a rare hereditary kidney disease. It is one of several diseases now termed autosomal dominant tubulointerstitial kidney disease, as proposed by a KDIGO (Kidney Disease: Improving Global Outcomes) consensus report in 2014. Autosomal dominant tubulointerstitial kidney diseases share common clinical findings, such as autosomal dominant inheritance, bland urinary sediment, absent to mild proteinuria, and progressive loss of kidney function...
December 4, 2017: American Journal of Kidney Diseases: the Official Journal of the National Kidney Foundation
https://www.readbyqxmd.com/read/29215110/quercetin-inhibits-renal-cyst-growth-in-vitro-and-via-parenteral-injection-in-a-polycystic-kidney-disease-mouse-model
#11
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/29206744/detectable-concentrations-of-inhaled-tobramycin-in-critically-ill-children-without-cystic-fibrosis-should-routine-monitoring-be-recommended
#12
Jamie L Miller, Trisha M Lepa, Courtney Ranallo, Hala Chaaban, Grant H Skrepnek, Peter N Johnson
OBJECTIVES: To determine the percentage of detectable tobramycin troughs and acute kidney injury in critically ill children without cystic fibrosis on inhaled therapy. DESIGN: Historic cohort. SETTING: Academic hospital. PATIENTS: Forty children less than 18 years receiving inhaled tobramycin across 6.5 years. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: The primary objective was to determine the percentage of detectable tobramycin troughs greater than or equal to 0...
December 2017: Pediatric Critical Care Medicine
https://www.readbyqxmd.com/read/29198187/unusual-presentation-of-prune-belly-syndrome-a-case-report
#13
Abayneh Girma Demisse, Ashenafi Berhanu, Temesgen Tadesse
BACKGROUND: Prune belly syndrome is a rare congenital malformation of unknown etiology, with the following triad of findings: abdominal muscle wall weakness, undescended testes, and urinary tract abnormalities. In most cases, detection of prune belly syndrome occurs during neonatal or infancy period. In this case report, we describe a 12-year-old boy from Ethiopia with the triad of findings of prune belly syndrome along with skeletal malformations. We are unaware of any previous report of prune belly syndrome in Ethiopia...
December 4, 2017: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/29187303/prevalence-and-characteristics-of-chronic-kidney-disease-among-danish-adults-with-cystic-fibrosis
#14
Kristina H Berg, Lene Ryom, Daniel Faurholt-Jepsen, Tania Pressler, Terese L Katzenstein
BACKGROUND: With improved prognosis of CF, comorbidities including chronic kidney disease (CKD) are becoming increasingly important. Identification of those at highest CKD risk is hence a priority. METHODS: In this cross-sectional study, adults with CF attending the Copenhagen CF Centre at Rigshospitalet with ≥2 measurements of serum creatinine from 2013 to 2015 were included. Data was obtained from an electronic CF database, which contains anonymised clinical and laboratory data on all individuals attending the clinic...
November 27, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/29183364/unusual-presentations-of-functional-parathyroid-cysts-a-case-series-and-review-of-the-literature
#15
Youssef El-Housseini, Martin Hübner, Ariane Boubaker, Jan Bruegger, Maurice Matter, Olivier Bonny
BACKGROUND: Cysts of parathyroid origin are sometimes encountered and can easily be mistaken as thyroidal cysts. Functional parathyroid cysts, with symptoms and signs of hyperparathyroidism, are rare and may be a diagnostic challenge to clinicians. We report here on three cases of functional parathyroid cysts that illustrate diagnosis difficulties related to unusual clinical presentations in three Caucasian women, including negative parathyroid scintigraphy. CASE PRESENTATIONS: Patient 1, an 87-year-old Caucasian woman presented with confusion and dysphagia...
November 29, 2017: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/29181500/perinatal-diagnosis-management-and-follow-up-of-cystic-renal-diseases-a-clinical-practice-recommendation-with-systematic-literature-reviews
#16
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/29178879/residual-renal-function-in-chronic-dialysis-is-not-associated-with-reduced-erythropoietin-stimulating-agent-dose-requirements-a-cross-sectional-study
#17
Elizabeth Helene Louw, Mogamat-Yazied Chothia
BACKGROUND: Anaemia is a very common problem in patients with end-stage kidney disease (ESKD) and the use of erythropoietin-stimulating agents (ESA) has revolutionised its treatment. Residual renal function (RRF) is associated with a reduction in ESA resistance and mortality in chronic dialysis. The primary aim was to establish whether RRF has an association with ESA dose requirements in ESKD patients receiving chronic dialysis. METHODS: A single center, cross-sectional study involving 100 chronic dialysis patients was conducted from December 2015 to May 2016...
November 25, 2017: BMC Nephrology
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
#18
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/29162218/renal-histology-and-mri-in-a-25-year-old-japanese-man-with-nephronophthisis-4%C3%A2
#19
Daisuke Takada, Akinari Sekine, Junko Yabuuchi, Yuta Kogure, Toshiharu Ueno, Masayuki Yamanouchi, Keiichi Sumida, Tatsuya Suwabe, Noriko Hayami, Junichi Hoshino, Kenmei Takaichi, Keiichi Kinowaki, Takeshi Fujii, Kenichi Ohashi, Takayasu Mori, Eisei Sohara, Shinichi Uchida, Yoshifumi Ubara
We investigated a 25-year-old Japanese man who had polycystic kidneys and end-stage renal failure without a positive family history. Ultrasonography revealed enlarged kidneys with increased echogenicity and multiple cystic lesions. MRI showed replacement of both kidneys by cystic lesions without definite walls. Renal biopsy demonstrated interstitial fibrosis, especially at the corticomedullary junction. The residual tubular system showed starfish-like disruption. Tubules with cystic dilation were mainly the distal loop of Henle and the distal tubules since immunohistochemical staining was positive for cytokeratin 7 (the distal loop of Henle and the distal tubule) and Tamm-Horsfall protein (the distal loop of Henle), while being negative for aquaporin 3 (the collecting duct) and CD10 (proximal tubule)...
November 22, 2017: Clinical Nephrology
https://www.readbyqxmd.com/read/29159032/use-of-ceftolozane-tazobactam-in-a-cystic-fibrosis-patient-with-multidrug-resistant-pseudomonas-infection-and-renal-insufficiency
#20
Katie Stokem, Jonathan B Zuckerman, David P Nicolau, Minkey Wungwattana, Edmund H Sears
We report the successful use of ceftolozane/tazobactam (C/T) to treat a pulmonary exacerbation in a 35 year old female, post lung transplant, with cystic fibrosis (CF), malnutrition, chronic kidney disease, and multi-drug resistant Pseudomonas aeruginosa infection (MDR PSA). Given the complexity of the clinical profile, we measured drug levels of C/T during treatment of her current exacerbation to determine pharmacokinetics. The patient achieved an estimated ceftolozane peak of 174.1 μg/mL and trough of 9...
2018: Respiratory Medicine Case Reports
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