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https://www.readbyqxmd.com/read/29790674/potential-pathogenicity-of-inquilinus-limosus-in-a-pediatric-patient-with-cystic-fibrosis
#1
T Spencer Poore, Isabel Virella-Lowell, Jennifer S Guimbellot
PRESENTATION: Patient is a 6-year-old male with CF, MRSA colonization, and pancreatic insufficiency that presented with worsening ppFEV1 and systemic symptoms despite multiple interventions. BAL grew NTM, Stenotrophomonas maltophilia, and Inquilinus limosus, a rare organism found in patients with CF. COURSE: I. limosus treatment was deferred. Despite treatment of other pathogens, symptoms worsened. I. limosus was targeted with meropenem, amikacin, and ciprofloxacin along with clindamycin for MRSA colonization...
May 23, 2018: Pediatric Pulmonology
https://www.readbyqxmd.com/read/29789057/case-study-cystic-fibrosis-in-the-newborn
#2
Paulina A Saravia, Cheryl Riley
Cystic fibrosis (CF) is considered one of the most commonly occurring fatal genetic disorders. This disorder is associated with pancreatic insufficiency and pulmonary complications. However, at birth the initial complications are associated with bowel obstruction. Cystic fibrosis management warrants an interdisciplinary team because this disorder affects various organ systems. Effective management of the newborn with CF assists in improving the child's overall prognosis. Family support is critical throughout the prenatal and postnatal periods...
May 1, 2018: Neonatal Network: NN
https://www.readbyqxmd.com/read/29787423/autoimmune-granulomatous-inflammation-of-lacrimal-glands-and-axonal-neuritis-following-treatment-with-ipilimumab-and-radiation-therapy
#3
Ecaterina Ileana Dumbrava, Veronica Smith, Rasha Alfattal, Adel K El-Naggar, Marta Penas-Prado, Apostolia M Tsimberidou
Immune checkpoint inhibitors such as anti-CTLA-4 (cytotoxic T-lymphocyte-associated protein 4), anti PD-1 (programmed cell death protein 1) and PD-L1 (programmed cell death protein-ligand 1) monoclonal antibodies are emerging as standard oncology treatments in various tumor types. The indications will expand as immunotherapies are being investigated in various tumors with promising results. Currently, there is inadequate identification of predictive biomarkers of response or toxicity. Unique response patterns include pseudoprogression and delayed response...
May 21, 2018: Journal of Immunotherapy
https://www.readbyqxmd.com/read/29779043/glis1-3-transcription-factors-critical-roles-in-the-regulation-of-multiple-physiological-processes-and-diseases
#4
REVIEW
Anton M Jetten
Krüppel-like zinc finger proteins form one of the largest families of transcription factors. They function as key regulators of embryonic development and a wide range of other physiological processes, and are implicated in a variety of pathologies. GLI-similar 1-3 (GLIS1-3) constitute a subfamily of Krüppel-like zinc finger proteins that act either as activators or repressors of gene transcription. GLIS3 plays a critical role in the regulation of multiple biological processes and is a key regulator of pancreatic β cell generation and maturation, insulin gene expression, thyroid hormone biosynthesis, spermatogenesis, and the maintenance of normal kidney functions...
May 19, 2018: Cellular and Molecular Life Sciences: CMLS
https://www.readbyqxmd.com/read/29778250/surgical-management-of-intraductal-papillary-mucinous-neoplasm-with-main-duct-involvement-an-international-expert-survey-and-case-vignette-study
#5
Lianne Scholten, Nadine C M van Huijgevoort, Marco J Bruno, Carlos Fernandez-Del Castillo, Sohei Satoi, Alain Sauvanet, Christopher Wolfgang, Paul Fockens, Suresh T Chari, Marco Del Chiaro, Jeanin E van Hooft, Marc G Besselink
BACKGROUND: The risk of invasive cancer in resected intraductal papillary mucinous neoplasm with main pancreatic duct involvement is 33%-60%. Most guidelines, therefore, advise resection of main duct intraductal papillary mucinous neoplasm and mixed type intraductal papillary mucinous neoplasm in surgically fit patients, although advice on the surgical strategy (partial or total pancreatectomy) differs. We performed a survey amongst international experts to guide the design of future studies and help to prepare for a single international set of guidelines...
May 16, 2018: Surgery
https://www.readbyqxmd.com/read/29777233/visceral-adiposity-and-high-adiponectin-levels-are-associated-with-the-prevalence-of-pancreatic-cystic-lesions
#6
Suguru Mizuno, Yousuke Nakai, Hiroyuki Isayama, Takeharu Yoshikawa, Kei Saito, Naminatsu Takahara, Hirofumi Kogure, Minoru Tada, Naoto Hayashi, Kazuhiko Koike
OBJECTIVES: Obesity is increasing in developed countries and is a risk factor for pancreatic cancer (PaC). We previously reported that obesity was associated with pancreatic cystic lesions (PCLs), which are both precursors of, and risk factors for, PaC. In the present study, we further investigated the relationship between visceral adiposity and adiponectin levels and the extent of PCLs. METHODS: Individuals who underwent comprehensive health screening at our institution between January 2008 and March 2013 were analyzed...
May 17, 2018: International Journal of Obesity: Journal of the International Association for the Study of Obesity
https://www.readbyqxmd.com/read/29773689/risk-factors-for-lung-disease-progression-in-children-with-cystic-fibrosis
#7
Marieke van Horck, Kim van de Kant, Bjorn Winkens, Geertjan Wesseling, Vincent Gulmans, Han Hendriks, Chris van der Grinten, Quirijn Jӧbsis, Edward Dompeling
To identify potential risk factors for lung disease progression in children with cystic fibrosis (CF), we studied the longitudinal data of all children with CF (aged ≥5 years) registered in the Dutch CF Registry (2009-2014). Lung disease progression was expressed as a decline in lung function (FEV1 %pred) and the pulmonary exacerbation rate (PEx). Potential risk factors at baseline included: sex, age, best FEV1%pred, best forced vital capacity (FVC)%pred, genotype, BMI z-score, pancreatic insufficiency, medication use (proton pump inhibitors [PPI], prophylactic antibiotics, and inhaled corticosteroids), CF-related diabetes, allergic bronchopulmonary aspergillosis, and colonisation with Pseudomonas aeruginosa The data of 545 children were analysed...
May 17, 2018: European Respiratory Journal: Official Journal of the European Society for Clinical Respiratory Physiology
https://www.readbyqxmd.com/read/29771769/systemic-chemotherapy-for-advanced-rare-pancreatic-histotype-tumors-a-retrospective-multicenter-analysis
#8
Oronzo Brunetti, Giuseppe Aprile, Paolo Marchetti, Enrico Vasile, Andrea Casadei Gardini, Mario Scartozzi, Sandro Barni, Sara Delfanti, Fernando De Vita, Francesco Di Costanzo, Michele Milella, Chiara Alessandra Cella, Rossana Berardi, Ivana Cataldo, Aldo Scarpa, Debora Basile, Federica Mazzuca, Giusi Graziano, Antonella Argentiero, Daniele Santini, Michele Reni, Stefano Cascinu, Nicola Silvestris
OBJECTIVES: Two issues were put forth by clinicians in the management of the advanced stages of rare variants of pancreatic ductal adenocarcinoma and other exocrine histotypes with peculiar clinical and pathological features: Do chemotherapy regimens recommended in pancreatic ductal adenocarcinoma patients have a clinical activity in rare pancreatic tumors? Or should other chemotherapy combinations be considered in this subset of patients? METHODS: We conducted a multicenter retrospective study that collected data from 2005 to 2016 at 14 Italian cancer centers with the aim to evaluate tumor response and time to progression for first- and second-line and overall survival...
May 15, 2018: Pancreas
https://www.readbyqxmd.com/read/29768684/practical-approach-to-the-gastrointestinal-manifestations-of-cystic-fibrosis
#9
REVIEW
Rishi Bolia, Chee Y Ooi, Peter Lewindon, Jonathan Bishop, Sarath Ranganathan, Jo Harrison, Kristyn Ford, Natalie van der Haak, Mark R Oliver
Cystic fibrosis (CF) is the most common, life-shortening, genetic illness affecting children in Australia and New Zealand. The genetic abnormality results in abnormal anion transport across the apical membrane of epithelial cells in a number of organs, including the lungs, gastrointestinal tract, liver and genito-urinary tract. Thus, CF is a multi-system disorder that requires a multi-disciplinary approach. Respiratory disease is the predominant cause of both morbidity and mortality in patients with CF. However, there are significant and clinically relevant gastrointestinal, liver, pancreatic and nutritional manifestations that must be detected and managed in a timely and structured manner...
May 16, 2018: Journal of Paediatrics and Child Health
https://www.readbyqxmd.com/read/29764411/contrast-enhanced-ultrasonography-of-the-pancreas-shows-impaired-perfusion-in-pancreas-insufficient-cystic-fibrosis-patients
#10
Trond Engjom, Kim Nylund, Friedemann Erchinger, Marcus Stangeland, Birger Norderud Lærum, Martin Mézl, Radovan Jiřík, Odd Helge Gilja, Georg Dimcevski
BACKGROUND: Perfusion assessment of the pancreas is challenging and poorly evaluated. Pancreatic affection is a prevalent feature of cystic fibrosis (CF). Little is known about pancreatic perfusion in CF. We aimed to assess pancreatic perfusion by contrast-enhanced ultrasound (CEUS) analysed in the bolus-and-burst model and software. METHODS: We performed contrast enhanced ultrasound of the pancreas in 25 CF patients and 20 healthy controls. Perfusion data was analysed using a dedicated perfusion model providing the mean capillary transit-time (MTT), blood flow (BF) and blood-volume (BV)...
May 15, 2018: BMC Medical Imaging
https://www.readbyqxmd.com/read/29761141/unexpected-height-loss-in-an-adolescent-with-cystic-fibrosis
#11
Anne Schendel, Jillian Mai, Rachel Espinosa, Carlye T Tomcyzk, Theresa A Laguna
This case report describes an adolescent with pancreatic insufficient cystic fibrosis, malnutrition, and cystic fibrosis-related diabetes found to have a loss in height at a routine outpatient clinic visit. He was subsequently admitted to the hospital for treatment of a pulmonary exacerbation and was found to have multiple, nontraumatic vertebral compression fractures. This report emphasizes the importance of routine review of growth charts and aggressive treatment of vitamin deficiencies and malnutrition in people with cystic fibrosis...
2018: Global Pediatric Health
https://www.readbyqxmd.com/read/29760585/mir-93-5p-enhance-lacrimal-gland-adenoid-cystic-carcinoma-cell-tumorigenesis-by-targeting-brms1l
#12
Jie Hao, Xin Jin, Yan Shi, Hong Zhang
Background: Lacrimal adenoid cystic carcinoma (LACC) is one of the most common malignancies that affects lacrimal gland. MicroRNAs are known to play a crucial role as oncogenes or tumor suppressors. Specifically, miR-93 has been reported to play a crucial role in colorectal, breast, pancreatic, lung cancer and hepatocellular carcinoma. However, the role of miR-93 in LACC and the potential molecular mechanisms involved remain unknown. Therefore, we took the challenge to determine the involvement of miR-93 in the LACC by targeting BRMS1L...
2018: Cancer Cell International
https://www.readbyqxmd.com/read/29753278/adult-pancreatoblastoma-a-case-report-and-clinicopathological-review-of-the-literature
#13
Menglin Chen, Haijie Zhang, Yixin Hu, Kai Liu, Yanjia Deng, Yuanmeng Yu, Yulan Wu, Anqi Qi, Yingjia Li, Ge Wen
PURPOSE: Our purpose was to report a case of adult pancreatoblastoma, and review the literature in order to assist clinicians in the management of the disease. MATERIALS AND METHODS: The demographic, clinical, and imaging findings of 41 patients with pathologically proven pancreatoblastoma from 1986 to 2017 identified in PubMed were reviewed. The key words used for searching PubMed were: "pancreatoblastoma", "pancreatic tumor", and "adult pancreatoblastoma...
May 4, 2018: Clinical Imaging
https://www.readbyqxmd.com/read/29751790/obliterative-cholangiopathy-in-acquired-cystic-biliary-atresia-type-iii-after-cyst-perforation-a-case-report
#14
Tsugumichi Koshinaga, Kensuke Ohashi, Kakou Ono, Hide Kaneda, Takeshi Furuya
BACKGROUND: In biliary atresia, the disease process of obliterative cholangiopathy may begin in the perinatal period; however, no chronological evidence exists on how the cholangiopathy progresses to biliary obliteration. This is the first acquired case with the final diagnosis of type III cystic biliary atresia with an extrahepatic biliary cyst which showed the progression of obliterative cholangiopathy in chronological order after birth. CASE PRESENTATION: An 81-day-old girl presented with acute abdominal distress due to bilious peritonitis caused by biliary cyst perforation, for which she underwent emergency biliary drainage...
May 11, 2018: BMC Pediatrics
https://www.readbyqxmd.com/read/29751773/an-unusual-finding-in-a-desmoid-type-fibromatosis-of-the-pancreas-a-case-report-and-review-of-the-literature
#15
Joseph Clarence Torres, Chen Xin
BACKGROUND: Desmoid-type fibromatoses are rare benign and fibrous tumors that account for approximately 0.03% of total neoplasms. Within this category of neoplasms, pancreatic desmoid-type fibromatosis is an extremely rare subgroup, accounting for approximately 5% of desmoid-type fibromatoses. Although the etiology is unknown, some risk factors include trauma, surgery, family history of desmoid tumor, pregnancy, use of contraceptives, genetic mutation, and familial adenomatous polyposis or Gardner syndrome...
May 12, 2018: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/29742687/a-case-report-of-autoimmune-pancreatitis-associated-with-a-pancreatic-pseudocyst
#16
Kai Zhang, Xianying Liu, Lei Yi, Jiannan Li, Jian Shi, Tongjun Liu
RATIONALE: Autoimmune pancreatitis (AIP) is a special type of chronic pancreatitis, which is rarely associated with pseudocyst. PATIENT CONCERNS: A 48-year-old man complained of a recurrent upper abdominal pain in our hospital. DIAGNOSES: A cystic mass of size 4 × 3 cm in his pancreatic tail was found by computed tomography. The concentrations of serum carbohydrate antigen19-9 (81 U/mL) and serum immunoglobulin G4 (181 mg/dL) were elevated...
May 2018: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29742360/disparities-in-mortality-of-hispanic-cystic-fibrosis-patients-in-the-united-states-a-national-and-regional-cohort-study
#17
Jason Rho, Chul Ahn, Ang Gao, Gregory S Sawicki, Ashley Keller, Raksha Jain
RATIONALE: Cystic Fibrosis (CF) patients of Hispanic origin are the largest growing minority, representing 8.5% of CF patients in the United States. No national survival analysis of this group has ever been undertaken. OBJECTIVE: We aimed to determine whether Hispanic ethnicity within the CF population is associated with worse outcomes and whether any geographic differences exist. METHODS: Using U.S. CF Foundation Patient Registry data from 2010-2014, we performed a retrospective cohort analysis comparing survival rates between Hispanics and non-Hispanics using Kaplan-Meier and Cox regression analysis...
May 9, 2018: American Journal of Respiratory and Critical Care Medicine
https://www.readbyqxmd.com/read/29737317/first-robotic-pylorus-preserving-pancreaticoduodenectomy-for-frantz-s-tumour-in-an-adolescent-girl
#18
Palanivelu Chinnusamy, Sumanta Dey, Bhushan Chittawadagi, Srivatsan Gurumurthy, Sandeep Sabnis, Senthilnathan Palanisamy
Solid pseudopapillary tumour (SPT) is one of the uncommon benign cystic neoplasms of pancreas occurring predominantly in young females. Being benign in nature, surgical resection is the treatment of choice with excellent 5-year survival. A 14-year-old girl presented with pain abdomen for 1 week. On evaluation, she was found to have a large SPT involving head and uncinate process of Pancreas She underwent robotic pylorus preserving pancreaticoduodenectomy (R-PPPD) with da Vinci® Si Robotic System (Intuitive Surgical, Sunnyvale, CA, USA)...
May 4, 2018: Journal of Minimal Access Surgery
https://www.readbyqxmd.com/read/29736662/serous-pancreatic-cystadenoma-with-compression-of-wirsung-s-duct
#19
Enio Campos Amico, José Roberto Alves, Adriano de Araújo Lima Liguori, Rogério Lacerda Sousa
Serous cystadenoma of the pancreas is a common cystic neoplasm typically of benign evolution that rarely communicates with the pancreatic ductal system. We present several images originating from two cases of serous cystadenoma of the pancreas which led to compression and dilatation of Wirsung's duct. These cases suggest that when the diagnosis of pancreatic microcystic lesion is detected, associated, or not associated with a central fibrous scar and a low carcinoembryonic antigen level in the aspirated fluid, the presence of dilatation of Wirsung's duct does not exclude the diagnosis of serous pancreatic cystadenoma...
May 7, 2018: Journal of Gastrointestinal Surgery: Official Journal of the Society for Surgery of the Alimentary Tract
https://www.readbyqxmd.com/read/29732356/clinical-significance-of-pancreatic-intraepithelial-neoplasia-in-resectable-pancreatic-cancer-on-survivals
#20
Da-Young Yu, Young-Dong Yu, Wan-Bae Kim, Hyung-Joon Han, Sae-Byul Choi, Dong-Sik Kim, Sang-Yong Choi, Joo-Young Kim, Hyeyoon Chang, Baek-Hui Kim
Purpose: Noninvasive precursor lesions for pancreatic adenocarcinoma include pancreatic intraepithelial neoplasia (PanIN), intraductal papillary mucinous neoplasm, and mucinous cystic neoplasm. PanIN is often found synchronously adjacent to resected pancreatic ductal adenocarcinoma (PDAC) tumors. However, its prognostic significance on outcome after PDAC resection is unknown. The purpose of the current study was to determine if the presence of PanIN has a prognostic or predictive effect on survival after resection for PDAC with curative intent...
May 2018: Annals of Surgical Treatment and Research
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