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https://www.readbyqxmd.com/read/28817601/bortezomib-initiates-endoplasmic-reticulum-stress-elicits-autophagy-and-death-in-echinococcus-granulosus-larval-stage
#1
María Celeste Nicolao, Julia A Loos, Christian Rodriguez Rodrigues, Viviana Beas, Andrea C Cumino
Cystic echinococcosis (CE) is a worldwide distributed helminthic zoonosis caused by Echinococcus granulosus. Benzimidazole derivatives are currently the only drugs for chemotherapeutic treatment of CE. However, their low efficacy and the adverse effects encourage the search for new therapeutic targets. We evaluated the in vitro efficacy of Bortezomib (Bz), a proteasome inhibitor, in the larval stage of the parasite. After 96 h, Bz showed potent deleterious effects at a concentration of 5 μM and 0.5 μM in protoscoleces and metacestodes, respectively (P < 0...
2017: PloS One
https://www.readbyqxmd.com/read/28817404/primary-renal-sarcomas-with-bcor-ccnb3-gene-fusion-a-report-of-2-cases-showing-histologic-overlap-with-clear-cell-sarcoma-of-kidney-suggesting-further-link-between-bcor-related-sarcomas-of-the-kidney-and-soft-tissues
#2
Pedram Argani, Yu-Chien Kao, Lei Zhang, Carlos Bacchi, Andres Matoso, Rita Alaggio, Jonathan I Epstein, Cristina R Antonescu
We report 2 primary renal sarcomas demonstrating BCOR-CCNB3 gene fusions that have recently been identified in undifferentiated round cell sarcomas of bone and soft tissue. These neoplasms occurred in male children aged 11 and 12 years, and both were cystic as a result of entrapment and dilatation of native renal tubules. Both cases were composed of variably cellular bland spindle cells with fine chromatin set in myxoid stroma and separated by a branching capillary vasculature. Both neoplasms demonstrated immunoreactivity for BCOR, cyclin D1, TLE1, and SATB2 in the spindle neoplastic cells and negativity in the prominent capillary vasculature...
August 16, 2017: American Journal of Surgical Pathology
https://www.readbyqxmd.com/read/28817128/epithelial-inclusions-following-a-bilaminar-root-coverage-procedure-with-a-subepithelial-connective-tissue-graft-a-histologic-and-clinical-study
#3
Federica Romano, Stefano Perotto, Luca Cricenti, Stefano Gotti, Mario Aimetti
The aim of this study was to histologically examine any epithelial cell inclusions in submerged subepithelial connective tissue graft (SCTG) after clinical healing was achieved. A total of 16 patients with Miller Class I or II gingival recessions were consecutively treated with a bilaminar procedure. At 2 months after surgery, a gingival tissue specimen was harvested from all SCTG-treated sites and stained with hematoxylin-eosin. The histologic evaluation revealed connective tissue in active reorganization without epithelial inclusions in 14 of the 16 tissue specimens...
September 2017: International Journal of Periodontics & Restorative Dentistry
https://www.readbyqxmd.com/read/28816699/ceruminous-adenoid-cystic-carcinoma-of-external-auditory-canal
#4
Andrew E Ebelhar, Dava S West, Rony K Aouad
An adenoid cystic carcinoma (AdCC) of the ceruminous glands is very rare; its diagnosis is most often challenging, and simple biopsies may be misleading. Our paper describes a case of a circumferential mass of the left ear canal that was initially reported as a basal cell carcinoma on biopsies in the clinic and on frozen sections intraoperatively. The final pathology was an AdCC of the ceruminous glands of the external auditory canal. Our case reflects the difficulty in the diagnosis of an AdCC of the ceruminous gland and the importance of keeping broad differential diagnoses in mind when counseling patients with masses in the ear canals until final pathology is obtained...
August 2017: Journal of International Advanced Otology
https://www.readbyqxmd.com/read/28816553/a-rare-case-of-giant-multicystic-solitary-fibrous-tumor-of-the-orbit
#5
Shahid Alam, Varsha Backiavathy, Bipasha Mukherjee, Krishnakumar Subramanian
Solitary fibrous tumor (SFT) is a rare spindle cell tumor of the orbit of mesenchymal origin. Though these tumors are mostly solid, partial or complete cystic changes can rarely occur. Only six such previous cases of cystic fibrous tumor of the orbit have been mentioned in the literature. We report a case of an elderly male who presented with a huge left sided medial orbital mass. Magnetic resonance imaging showed a predominant cystic orbital mass separated by septae and suggested a diagnosis of Hydatid cyst...
August 17, 2017: Orbit
https://www.readbyqxmd.com/read/28816423/infants-with-cystic-fibrosis-still-lag-on-some-growth-measures-babies-weight-on-par-with-healthy-peers-but-growth-is-still-stunted
#6
(no author information available yet)
No abstract text is available yet for this article.
September 2017: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/28816297/-perirenal-cystic-lymphangioma-in-an-adult-a-case-report-and-literature-review
#7
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/28816191/histologic-variants-of-calcifying-odontogenic-cyst-a-study-of-52-cases
#8
Soussan Irani, Forough Foroughi
AIM: This study aimed at evaluating histological features of 52 cases of calcifying odontogenic cyst (COC), which is an uncommon benign odontogenic lesion. The World Health Organization (WHO) classified COC as a neoplasm and used the term calcifying cystic odontogenic tumor (CCOT) for benign cystic type and the dentinogenic ghost cell tumor (DGCT) for the benign solid-type lesions. There is no agreement regarding COC classification. MATERIALS AND METHODS: A total of 52 cases of COC were selected and reviewed from the archive of the Pathology Department of Taleghani Educational Hospital, Tehran, Iran...
August 1, 2017: Journal of Contemporary Dental Practice
https://www.readbyqxmd.com/read/28816015/liver-transplantation-in-adult-cystic-fibrosis-clinical-imaging-and-pathological-evidence-of-obliterative-portal-venopathy
#9
Sophie Hillaire, Dominique Cazals-Hatem, Onorina Bruno, Sandra De Miranda, Dominique Grenet, Nicolas Poté, Olivier Soubrane, Serge Erlinger, Florence Lacaille, Francois Mellot, Valerie Vilgrain, Valerie Paradis
No abstract text is available yet for this article.
August 16, 2017: Liver Transplantation
https://www.readbyqxmd.com/read/28815999/incident-stenotrophomonas-maltophilia-infection-and-lung-function-decline-in-cystic-fibrosis
#10
Emily E Barsky, Kathryn A Williams, Gregory P Priebe, Gregory S Sawicki
OBJECTIVES: To determine whether incident detection of Stenotrophomonas maltophilia (SM) in patients with cystic fibrosis (CF) is associated with accelerated lung function decline and increased hospitalizations and to determine whether this effect is more pronounced in individuals with subsequent chronic infection. METHODS: We performed a longitudinal, retrospective single-center, pre-post study of 88 patients with CF, ages 6-51 years, with first positive respiratory culture for SM between 2008 and 2014...
August 16, 2017: Pediatric Pulmonology
https://www.readbyqxmd.com/read/28815937/the-airway-microbiota-in-early-cystic-fibrosis-lung-disease
#11
Katherine B Frayman, David S Armstrong, Keith Grimwood, Sarath C Ranganathan
Infection plays a critical role in the pathogenesis of cystic fibrosis (CF) lung disease. Over the past two decades, the application of molecular and extended culture-based techniques to microbial analysis has changed our understanding of the lungs in both health and disease. CF lung disease is a polymicrobial disorder, with obligate and facultative anaerobes recovered alongside traditional pathogens in varying proportions, with some differences observed to correlate with disease stage. While healthy lungs are not sterile, differences between the lower airway microbiota of individuals with CF and disease-controls are already apparent in childhood...
August 16, 2017: Pediatric Pulmonology
https://www.readbyqxmd.com/read/28815891/cystic-kidneys-in-fetal-walker-warburg-syndrome-with-pomt2-mutation-intrafamilial-phenotypic-variability-in-four-siblings-and-review-of-literature
#12
Marwa M Nabhan, Nour ElKhateeb, Daniela A Braun, Sungho Eun, Sahar N Saleem, Heon YungGee, Friedhelm Hildebrandt, Neveen A Soliman
Walker-Warburg syndrome (WWS) is a severe form of congenital muscular dystrophy secondary to α-dystroglycanopathy with muscle, brain, and eye abnormalities often leading to death in the first weeks of life. It is transmitted in an autosomal recessive pattern, and has been linked to at least 15 different genes; including protein O-mannosyltransferase 1 (POMT1), protein O-mannosyltransferase 2 (POMT2), protein O-mannose beta-1,2-N acetylglucosaminyltransferase (POMGNT1), fukutin (FKTN), isoprenoid synthase domain-containing protein (ISPD), and other genes...
August 17, 2017: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/28815810/proteome-characterization-of-human-pancreatic-cyst-fluid-from-intraductal-papillary-mucinous-neoplasm-by-lc-ms-ms
#13
Joonho Park, Dohyun Han, Misol Do, Jongmin Woo, Joseph I Wang, Youngmin Han, Wooil Kwon, Sun-Whe Kim, Jin-Young Jang, Youngsoo Kim
RATIONALE: In recent years, the molecular components of pancreatic cyst fluid has been used for diagnosis and prognosis. Because the protein markers that are currently used in clinical tests are unreliable, proteomic studies to find new protein markers are being conducted. However, such researches have been limited due to the complexity of pancreatic cyst fluid and the immaturity of proteomic techniques. METHODS: To overcome these limitations and provide a pancreatic cyst proteome dataset, we examined cyst fluid proteome with tandem mass spectrometry...
August 15, 2017: Rapid Communications in Mass Spectrometry: RCM
https://www.readbyqxmd.com/read/28815737/retroperitoneal-gastric-duplication-mimicking-a-prenatal-adrenal-cyst
#14
Aurora Lucía Castillo-Fernández, Fernando Vázquez-Rueda, Mª Dolores Cañete, Javier Caballero-Villarraso
Duplications of the gastric tract are made up of a wide variety of masses throughout the gastrointestinal tract. They may have tubular or cystic morphology. Some duplications are associated with malformations, especially those located in the thorax, which can be associated with intestinal atresia, and malformations of the urinary tract or vetebras (Lund. 2012).
August 16, 2017: Congenital Anomalies
https://www.readbyqxmd.com/read/28815197/autosomal-recessive-inheritance-cystic-fibrosis
#15
D Yitzchak Goldstein, Michael Prystowsky
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/28815024/stem-cell-derived-organoids-to-model-gastrointestinal-facets-of-cystic-fibrosis
#16
REVIEW
Meike Hohwieler, Lukas Perkhofer, Stefan Liebau, Thomas Seufferlein, Martin Müller, Anett Illing, Alexander Kleger
Cystic fibrosis (CF) is one of the most frequently occurring inherited human diseases caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) which lead to ample defects in anion transport and epithelial fluid secretion. Existing models lack both access to early stages of CF development and a coeval focus on the gastrointestinal CF phenotypes, which become increasingly important due increased life span of the affected individuals. Here, we provide a comprehensive overview of gastrointestinal facets of CF and the opportunity to model these in various systems in an attempt to understand and treat CF...
August 2017: United European Gastroenterology Journal
https://www.readbyqxmd.com/read/28814498/both-ways-at-once-keeping-small-airways-clean
#17
REVIEW
Paul M Quinton
The small airways of the lungs are under constant assault from the pathogens and debris in the air that they must conduct to alveoli. Although hygiene is of paramount importance for respiratory health, the underlying principles of airway clearance have not been well integrated or established. Newly emerging concepts of simultaneous absorption and secretion of airway surface liquid (ASL) and the role of [Formula: see text] in the maturation of mucins have advanced from experimental evidence as well as observations from the congenital disease cystic fibrosis (CF) to present a novel model that integrates microanatomy with organ physiology to meet the constant challenge of cleaning small airways...
September 2017: Physiology
https://www.readbyqxmd.com/read/28814334/abernethy-malformation-associated-with-caroli-s-syndrome-in-a-patient-with-a-pkhd1-mutation-a-case-report
#18
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/28814218/diagnosis-and-management-of-pancreatic-exocrine-insufficiency
#19
Mehrdad Nikfarjam, Jeremy S Wilson, Ross C Smith
In 2015, the Australasian Pancreatic Club (APC) published the Australasian guidelines for the management of pancreatic exocrine insufficiency (http://pancreas.org.au/2016/01/pancreatic-exocrine-insufficiency-guidelines). Pancreatic exocrine insufficiency (PEI) occurs when normal digestion cannot be sustained due to insufficient pancreatic digestive enzyme activity. This may be related to a breakdown, at any point, in the pancreatic digestive chain: pancreatic stimulation; synthesis, release or transportation of pancreatic enzymes; or synchronisation of secretions to mix with ingested food...
August 21, 2017: Medical Journal of Australia
https://www.readbyqxmd.com/read/28813430/reprogramming-human-gallbladder-cells-into-insulin-producing-%C3%AE-like-cells
#20
Feorillo Galivo, Eric Benedetti, Yuhan Wang, Carl Pelz, Jonathan Schug, Klaus H Kaestner, Markus Grompe
The gallbladder and cystic duct (GBCs) are parts of the extrahepatic biliary tree and share a common developmental origin with the ventral pancreas. Here, we report on the very first genetic reprogramming of patient-derived human GBCs to β-like cells for potential autologous cell replacement therapy for type 1 diabetes. We developed a robust method for large-scale expansion of human GBCs ex vivo. GBCs were reprogrammed into insulin-producing pancreatic β-like cells by a combined adenoviral-mediated expression of hallmark pancreatic endocrine transcription factors PDX1, MAFA, NEUROG3, and PAX6 and differentiation culture in vitro...
2017: PloS One
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