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https://www.readbyqxmd.com/read/28228103/disease-manifestations-and-burden-of-illness-in-patients-with-acid-sphingomyelinase-deficiency-asmd
#1
REVIEW
Margaret M McGovern, Ruzan Avetisyan, Bernd-Jan Sanson, Olivier Lidove
Acid sphingomyelinase deficiency (ASMD), a rare lysosomal storage disease, is an autosomal recessive genetic disorder caused by different SMPD1 mutations. Historically, ASMD has been classified as Niemann-Pick disease (NPD) types A (NPD A) and B (NPD B). NPD A is associated with a uniformly devastating disease course, with rapidly progressing psychomotor degeneration, leading to death typically by the age of 3 years, most often from respiratory failure. In contrast, the clinical phenotype and life expectancy of patients with NPD B may vary widely...
February 23, 2017: Orphanet Journal of Rare Diseases
https://www.readbyqxmd.com/read/28223802/fe3o4-based-plga-nanoparticles-as-mr-contrast-agents-for-the-detection-of-thrombosis
#2
Jia Liu, Jie Xu, Jun Zhou, Yu Zhang, Dajing Guo, Zhigang Wang
Thrombotic disease is a great threat to human health, and early detection is particularly important. Magnetic resonance (MR) molecular imaging provides noninvasive imaging with the potential for early disease diagnosis. In this study, we developed Fe3O4-based poly(lactic-co-glycolic acid) (PLGA) nanoparticles (NPs) surface-modified with a cyclic Arg-Gly-Asp (cRGD) peptide as an MR contrast agent for the detection of thrombosis. The physical and chemical characteristics, biological toxicity, ability to target thrombi, and biodistribution of the NPs were studied...
2017: International Journal of Nanomedicine
https://www.readbyqxmd.com/read/28222323/a-case-report-of-the-clear-cell-variant-of-gallbladder-carcinoma
#3
Ravi Maharaj, Christo Cave, Kevin Sarran, Nigel Bascombe, Dilip Dan, Wesley Greaves, Wayne A Warner
INTRODUCTION: Clear cell gallbladder carcinoma accounts for less than 1% of all gallbladder malignancies and demonstrates its unique histopathological characteristics in patients with no prior medical illness or familial predisposition. PRESENTATION OF CASE: Here we present a case of a 56-year-old female, with no prior medical conditions presented with a 2-month history of upper abdominal pain. Routine hematological and biochemical tests were unremarkable. An abdominal ultrasound revealed the presence of a gallbladder calculi, and a fundic mass while magnetic resonance cholangiopancreatography revealed a 8...
January 12, 2017: International Journal of Surgery Case Reports
https://www.readbyqxmd.com/read/28218709/pet-imaging-for-initial-staging-and-therapy-assessment-in-multiple-myeloma-patients
#4
REVIEW
Clément Bailly, Rodolphe Leforestier, Bastien Jamet, Thomas Carlier, Mickael Bourgeois, François Guérard, Cyrille Touzeau, Philippe Moreau, Michel Chérel, Françoise Kraeber-Bodéré, Caroline Bodet-Milin
Multiple myeloma (MM) is a hematological neoplasm characterized by the clonal proliferation of malignant plasma cells in the bone marrow. MM results in diffuse or focal bone infiltration and extramedullary lesions. Over the past two decades, advances have been made with regard to the diagnosis, staging, treatment, and imaging of MM. Computed tomography (CT) and magnetic resonance imaging (MRI) are currently recommended as the most effective imaging modalities at diagnostic. Yet, recent data from the literature suggest that positron emission tomography combined with computed tomography (PET/CT) using (18)F-deoxyglucose (FDG) is a promising technique for initial staging and therapeutic monitoring in this pathology...
February 18, 2017: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/28213738/cytomegalovirus-infection-in-patients-with-lupus-nephritis-clinical-and-laboratory-features-and-therapeutic-considerations
#5
Lei Zhang, Jianling Tao, Yubing Wen, Li Li, Xueyi Wu, Xuewang Li, Xuemei Li
To better clarify the clinical features and therapeutic strategy of CMV infection in lupus nephritis patients, we retrospectively surveyed a total of 40 lupus nephritis patients, who had been hospitalized and underwent renal biopsy and diagnosed as having CMV infection during their hospitalization at our institution within the last 10 years. The percentage of CMV infections in the entire hospitalized lupus nephritis population was 5.3% (40/755). The principal clinical features of the 40 CMV-infected patients were hematological disorders (n = 25), fever (n = 21), liver dysfunction (n = 19), and respiratory symptoms (n = 12)...
February 17, 2017: Clinical and Experimental Medicine
https://www.readbyqxmd.com/read/28187089/clinical-outcome-of-neoadjuvant-radiochemotherapy-in-locally-advanced-cervical-cancer-results-of-an-open-prospective-multicenter-phase-2-study-of-the-north-eastern-german-society-of-gynecological-oncology
#6
Dominique Koensgen, Jalid Sehouli, Antje Belau, Martin Weiss, Matthias B Stope, Vivien Grokopf, Michael Eichbaum, Peter Ledwon, Werner Lichtenegger, Marek Zygmunt, Günter Köhler, Alexander Mustea
OBJECTIVE: The aim of this study was to determine the response rate, toxicity, operability, and surgical complication rate of neoadjuvant concomitant radiochemotherapy (cRCH) (ifosfamide + carboplatin) followed by radical hysterectomy plus external-beam radiotherapy with curative intention in locally advanced primary inoperable stages IIB and IIIB squamous cell cervical cancer. METHODS: Patients with cervical cancer from 8 departments were enrolled. Patients received 3 cycles of ifosfamide 1...
March 2017: International Journal of Gynecological Cancer
https://www.readbyqxmd.com/read/28182647/performance-analysis-of-a-machine-learning-flagging-system-used-to-identify-a-group-of-individuals-at-a-high-risk-for-colorectal-cancer
#7
Yaron Kinar, Pinchas Akiva, Eran Choman, Revital Kariv, Varda Shalev, Bernard Levin, Steven A Narod, Ran Goshen
Individuals with colorectal cancer (CRC) have a tendency to intestinal bleeding which may result in mild to severe iron deficiency anemia, but for many colon cancer patients hematological abnormalities are subtle. The fecal occult blood test (FOBT) is used as a pre-screening test whereby those with a positive FOBT are referred to colonscopy. We sought to determine if information contained in the complete blood count (CBC) report coud be processed automatically and used to predict the presence of occult colorectal cancer (CRC) in the setting of a large health services plan...
2017: PloS One
https://www.readbyqxmd.com/read/28181678/safety-and-immunological-efficacy-of-a-dna-vaccine-encoding-the-androgen-receptor-ligand-binding-domain-ar-lbd
#8
Brian M Olson, Eric S Bradley, Thomas Sawicki, Weixiong Zhong, Erik A Ranheim, Jordan E Bloom, Viswa T Colluru, Laura E Johnson, Brian T Rekoske, Jens C Eickhoff, Douglas G McNeel
BACKGROUND: The androgen receptor (AR) is a key oncogenic driver of prostate cancer, and has been the primary focus of prostate cancer treatment for several decades. We have previously demonstrated that the AR is also an immunological target antigen, recognized in patients with prostate cancer, and targetable by means of vaccines in rodent models with delays in prostate tumor growth. The current study was performed to determine the safety and immunological efficacy of a GMP-grade plasmid DNA vaccine encoding the ligand-binding domain (LBD) of the AR, pTVG-AR...
February 9, 2017: Prostate
https://www.readbyqxmd.com/read/28173871/characterization-and-pathogenesis-of-aerosolized-eastern-equine-encephalitis-in-the-common-marmoset-callithrix-jacchus
#9
Aimee I Porter, Rebecca A Erwin-Cohen, Nancy Twenhafel, Taylor Chance, Steven B Yee, Steven J Kern, David Norwood, Laurie J Hartman, Michael D Parker, Pamela J Glass, Luis DaSilva
BACKGROUND: Licensed antiviral therapeutics and vaccines to protect against eastern equine encephalitis virus (EEEV) in humans currently do not exist. Animal models that faithfully recapitulate the clinical characteristics of human EEEV encephalitic disease, including fever, drowsiness, anorexia, and neurological signs such as seizures, are needed to satisfy requirements of the Food and Drug Administration (FDA) for clinical product licensing under the Animal Rule. METHODS: In an effort to meet this requirement, we estimated the median lethal dose and described the pathogenesis of aerosolized EEEV in the common marmoset (Callithrix jacchus)...
February 7, 2017: Virology Journal
https://www.readbyqxmd.com/read/28156165/preclinical-safety-assessment-of-furostanol-glycoside-based-standardized-fenugreek-seed-extract-in-laboratory-rats
#10
Pallavi Deshpande, Vishwaraman Mohan, Dilip Ingavale, Jyoti Mane, Mukul Pore, Prasad Thakurdesai PhD
The present work is aimed at studying acute oral toxicity (AOT), subchronic oral toxicity, mutagenicity, and genotoxicity of furostanol glycosides-based standardized fenugreek seed extract (Fenu-FG) using the Organization for Economic Co-operation and Development (OECD) guidelines. The AOT and subchronic (90-day repeated dose) toxicity studies were performed on Wistar rats as per OECD 423 and OECD 408 guidelines, respectively. The mutagenicity (reverse mutation assay, Ames test) and genotoxicity (mammalian chromosome aberration test) were assessed in vitro using OECD 471 and OECD 473 guidelines, respectively...
February 3, 2017: Journal of Dietary Supplements
https://www.readbyqxmd.com/read/28149266/national-external-quality-assurance-program-pakistan-neqapp-a-milestone-in-proficiency-testing-in-pakistan
#11
Muhammad Usman Munir, Aamir Ijaz
OBJECTIVE: The objective of this study was to highlight current status and importance of National External Quality Assurance Program Pakistan (NEQAPP). Study Design: Cross sectional study. PLACE AND DURATION OF STUDY: Department of Chemical Pathology and Endocrinology, Armed Forces Institute of Pathology (AFIP) from August to October 2015. METHODS: The study data was extracted from electronic NEQAPP database. Results from 2014-2015 were evaluated for clinical chemistry, hematology, microbiology, and immunoassay programs...
December 2016: EJIFCC
https://www.readbyqxmd.com/read/28137827/s100a9-induces-differentiation-of-acute-myeloid-leukemia-cells-through-tlr4
#12
Malika Laouedj, Mélanie R Tardif, Laurine Gil, Marie-Astrid Raquil, Asmaa Lachaab, Martin Pelletier, Philippe A Tessier, Frédéric Barabé
S100A8 and S100A9 are calcium-binding proteins predominantly expressed by neutrophils and monocytes, and play key roles in both normal and pathological inflammation. Recently, both proteins were found to promote tumor progression through the establishment of pre-metastatic niches and to inhibit antitumor immune responses. Although S100A8 and S100A9 have been studied in solid cancers, their functions in hematological malignancies remain poorly understood. However, S100A8 and S100A9 are highly expressed in acute myeloid leukemia (AML), and S100A8 expression has been linked to a poor prognosis in AML...
January 30, 2017: Blood
https://www.readbyqxmd.com/read/28133251/-a-giant-appendiceal-mucinous-neoplasm-treated-with-laparoscopic-surgery
#13
Yoshito Nako, Shigeki Yamazaki, Takashi Kitade, Tomoki Hariu, Motomi Wakasa, Masayoshi Nakanishi, Teruhisa Sonoyama
We encountered a case of giant appendiceal mucinous neoplasm that was treated with laparoscopic surgery. The patient was a 77-year-old man with constipation. Lower endoscopy demonstrated a giant SMT-like tumor in the cecum, and abdominal computed tomography revealed a giant appendiceal mucinous neoplasm and nearby lymph nodes swelling. Hematological examination showed an elevated serum CEA level. We performed laparoscopic ileocecal resection for a definite diagnosis and treatment. The postoperative course was uneventful...
November 2016: Gan to Kagaku Ryoho. Cancer & Chemotherapy
https://www.readbyqxmd.com/read/28133012/-preoperative-chemoradiotherapy-for-stage-ii-or-iii-esophageal-squamous-cell-carcinoma
#14
Takaharu Kawai, Mitsugu Kochi, Masashi Fujii, Keio Song, Ken Hagiwara, Hidenori Tamegai, Megumu Watanabe, Yoritaka Matsuno, Yuriko Takayama, Hiroshi Suda, Tadatoshi Takayama
BACKGROUND: The goal of this retrospective study was to investigate the efficacy and safety of preoperative chemoradiotherapy( NACR)in patients with Stage II or Stage III esophageal squamous cell carcinoma(SCC). METHODS: Between 2004 and 2014, a total of 86 patients underwent surgical resection in conjunction with NACR for esophageal SCC at our institute. Thirty-one patients(36.0%)had Stage II disease and 55 patients(64.0%)had Stage III disease. RESULTS: The median age was 64(43-81)years...
November 2016: Gan to Kagaku Ryoho. Cancer & Chemotherapy
https://www.readbyqxmd.com/read/28127781/preliminary-evaluation-of-virtual-touch-tissue-imaging-quantification-for-differential-diagnosis-of-metastatic-and-nonmetastatic-cervical-lymph-nodes
#15
Yingyan Zhao, Jiaying Xi, Baozhen Zhao, Wenfeng Xiong, Dong Jiang, Li Yang, Zhuhong Cai, Tianjia Liu, Haibo Jiang, Shu Rong, Xiucai Jin
OBJECTIVES: Virtual Touch tissue imaging quantification (VTIQ; Siemens Medical Solutions, Mountain View, CA) is useful for assessing tissue hardness. This study aimed to investigate the value of VTIQ in differential diagnosis of cervical lymph nodes. METHODS: We retrospectively analyzed conventional sonograms and VTIQ images of 85 pathologically confirmed patients with superficial lymph node lesions. Conventional sonography was first performed, with 2-dimensional images described...
January 27, 2017: Journal of Ultrasound in Medicine: Official Journal of the American Institute of Ultrasound in Medicine
https://www.readbyqxmd.com/read/28112703/learning-more-and-spending-less-with-neglected-laboratory-parameters-the-paradigmatic-case-of-red-blood-cell-distribution-width
#16
Giuseppe Lippi, Camilla Mattiuzzi, Gianfranco Cervellin
Due to increasing shortage of public healthcare resources in many countries around the globe, the use of simple, rapid and inexpensive laboratory parameters may be seen as a valuable aid for preliminary and cost-effective risk stratification of patients. Anisocytosis, conventionally measured by hematologic analyzers as the red blood cell distribution width (RDW), is an index of the heterogeneity of erythrocytes volumes. Several lines of evidence now attest that increased RDW values are commonplace in patients with many disorders, especially in those with the most prevalent conditions such as cardiovascular disease, diabetes, cancer and infections...
January 16, 2017: Acta Bio-medica: Atenei Parmensis
https://www.readbyqxmd.com/read/28104371/in-vitro-and-in-vivo-evaluation-of-anti-arthritic-antioxidant-efficacy-of-fucoidan-from-undaria-pinnatifida-harvey-suringar
#17
Abdul-Rehman Phull, Muhammad Majid, Ihsan-Ul Haq, Muhammad Rashid Khan, Song Ja Kim
Seaweed and their constituents have been traditionally employed for the management of various human pathologic conditions such as edema, urinary disorders and inflammatory anomalies. The current study was performed to investigate the antioxidant and anti-arthritic effects of fucoidan from Undaria pinnatifida. A noteworthy in vitro antioxidant potential at 500μg/ml in 2, 2-diphenyl-1-picrylhydrazyl scavenging assay (80% inhibition), nitrogen oxide inhibition assay (71.83%), hydroxyl scavenging assay (71.92%), iron chelating assay (73...
April 2017: International Journal of Biological Macromolecules
https://www.readbyqxmd.com/read/28103204/transthoracic-ultrasonography-for-the-immunocompromised-patient-a-pilot-project-that-introduces-transthoracic-ultrasonography-for-the-follow-up-of-hematological-patients-in-romania
#18
Ioana Frinc, Petru Ilies, Florin Zaharie, Delia Dima, Alina Tanase, Ljubomir Petrov, Alexandru Irimie, Cristian Berce, Cosmin Lisencu, Ioana Berindan-Neagoe, Ciprian Tomuleasa, Anca Bojan
In the last decade, there is significant progress in clinical hematology with the discovery of targeted molecules and thus the achievement of both hematologic and molecular responses. Nevertheless, chemotherapy remains the treatment of choice for many types of hematological malignancies. Aggressive chemotherapy leads to immunosuppression, accompanied by a high rate of infections and an increased rate of treatment-related mortality. Invasive fungal infections, as well as more common bacterial and viral infections are frequent in immunocompromised patients as they are difficult to diagnose and treat...
January 18, 2017: Romanian Journal of Internal Medicine, Revue Roumaine de Médecine Interne
https://www.readbyqxmd.com/read/28103116/acute-leukemia-and-concurrent-mediastinal-germ-cell-tumor-case-report-and-literature-review
#19
Luke Maese, K David Li, Xinjie Xu, Zeinab Afify, Christian N Paxton, Angelica Putnam
There is a known association of primary nonseminomatous mediastinal germ cell tumors (NSMGCT) and hematologic malignancy in younger males not linked to treatment. When combined these two rare entities convey a very poor prognosis. Here we report a 16-year-old male with an anterior mediastinal mass diagnosed as a malignant germ cell tumor based on elevation of serologic markers. He was found to have acute leukemia with megakaryocytic differentiation several days later. We focus our report on the pathologic findings, including a review of the literature, and a novel molecular analysis of the germ cell tumor...
January 19, 2017: Fetal and Pediatric Pathology
https://www.readbyqxmd.com/read/28099910/multiple-biological-functions-of-twist1-in-various-cancers
#20
Zhixiang Zhao, Mohammad Aminur Rahman, Zhuo G Chen, Dong M Shin
Twist1 is a well-known regulator of transcription during embryonic organogenesis in many species. In humans, Twist1 malfunction was first linked to Saethre-Chotzen syndrome and later identified to play an essential role in tumor initiation, stemness, angiogenesis, invasion, metastasis, and chemo-resistance in a variety of carcinomas, sarcomas, and hematological malignances. In this review, we will first focus on systematically elaborating the diverse pathological functions of Twist1 in various cancers, then delineating the intricate underlying network of molecular mechanisms, based on which we will summarize current therapeutic strategies in cancer treatment that target and modulate Twist1-involved signaling pathways...
January 12, 2017: Oncotarget
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