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https://www.readbyqxmd.com/read/29676020/chronic-neutrophilic-leukemia-an-extremely-rare-cause-of-neutrophilia-in-childhood-cure-with-hematopoietic-stem-cell-transplantation
#1
Vedat Uygun, Hayriye Daloğlu, Seda Öztürkmen, Gülsün Karasu, Zekai Avcı, Akif Yeşilipek
CNL is a rare myeloproliferative disorder frequently seen in older adults. A significant proportion of patients show progression to AML. Here, we report the case of a patient with FA who was monitored for leukopenia but who developed leukocytosis during the follow-up and was diagnosed with CNL probably after an acquired CSF3R mutation. Because the patient had FA, which could accelerate the progression to AML, an HSCT was performed, which resulted in cure. This patient (aged 12 years) is one of the youngest patients reported to develop CNL as well as the first FA patient with a diagnosis of CNL...
April 19, 2018: Pediatric Transplantation
https://www.readbyqxmd.com/read/29672525/comparative-genomic-and-transcriptomic-analysis-of-selected-fatty-acid-biosynthesis-genes-and-cnl-disease-resistance-genes-in-oil-palm
#2
Rozana Rosli, Nadzirah Amiruddin, Mohd Amin Ab Halim, Pek-Lan Chan, Kuang-Lim Chan, Norazah Azizi, Priscilla E Morris, Eng-Ti Leslie Low, Meilina Ong-Abdullah, Ravigadevi Sambanthamurthi, Rajinder Singh, Denis J Murphy
Comparative genomics and transcriptomic analyses were performed on two agronomically important groups of genes from oil palm versus other major crop species and the model organism, Arabidopsis thaliana. The first analysis was of two gene families with key roles in regulation of oil quality and in particular the accumulation of oleic acid, namely stearoyl ACP desaturases (SAD) and acyl-acyl carrier protein (ACP) thioesterases (FAT). In both cases, these were found to be large gene families with complex expression profiles across a wide range of tissue types and developmental stages...
2018: PloS One
https://www.readbyqxmd.com/read/29587671/coexisting-of-bone-marrow-fibrosis-dysplasia-and-an-x-chromosomal-abnormality-in-chronic-neutrophilic-leukemia-with-csf3r-mutation-a-case-report-and-literature-review
#3
Xue Bin Wu, Wei Wei Wu, Yue Zhou, Xuan Wang, Jia Li, Yang Yu
BACKGROUND: Chronic neutrophilic leukemia (CNL) is a rare myeloproliferative neoplasm (MPN) with less than 40 cases of patients being reported or clinically suspected meeting with 2008 World Health Organization ("WHO") diagnostic criteria. The current diagnosis of CNL remains to exclude other diseases. Recently, a new biomarker of CSF3R mutations that is almost invariably present in CNL has been identified. There is no effective treatment for CNL, therefore prognosis of the disease is poor, but it may be attributed to the presence of both SETBP1 and CSF3R gene mutations...
March 27, 2018: BMC Cancer
https://www.readbyqxmd.com/read/29587254/evaluation-of-the-freestyle%C3%A2-libre-flash-glucose-monitoring-system-in-children-and-adolescents-with-type-1-diabetes
#4
Guy G Massa, Inge Gys, Anniek Op 't Eyndt, Esmiralda Bevilacqua, Anne Wijnands, Peter Declercq, Renate Zeevaert
BACKGROUND/AIMS: The FreeStyle® Libre Flash Glucose Monitoring System (FGM, Abbott) measures glucose concentrations in the interstitial fluid for up to 14 days. It has been approved for use in children aged > 4 years in January 2016. Experience in children is still limited. We evaluated the accuracy and usability of the FGM in children with type 1 diabetes mellitus (DM). METHODS: 67 children with type 1 DM (35 girls), aged 4-18 years, were included. Subjects wore a sensor on the back of their upper arm...
March 27, 2018: Hormone Research in Pædiatrics
https://www.readbyqxmd.com/read/29512199/chronic-neutrophilic-leukemia-2018-update-on-diagnosis-molecular-genetics-and-management
#5
Michelle A Elliott, Ayalew Tefferi
DISEASE OVERVIEW AND DIAGNOSIS: Chronic neutrophilic leukemia (CNL) is a potentially aggressive myeloproliferative neoplasm, for which current WHO diagnostic criteria include leukocytosis of ≥ 25 x 109 /L of which ≥ 80% are neutrophils, with < 10% circulating neutrophil precursors with blasts rarely observed. In addition, there is no dysplasia, nor clinical or molecular criteria for other myeloproliferative neoplasms. UPDATE ON DIAGNOSIS: Previously the diagnosis of CNL was often as one of exclusion based on no identifiable cause for physiologic neutrophilia in patients fulfilling the aforementioned criteria...
August 2018: American Journal of Hematology
https://www.readbyqxmd.com/read/29440636/chronic-neutrophilic-leukemia-new-science-and-new-diagnostic-criteria
#6
REVIEW
Natasha Szuber, Ayalew Tefferi
Chronic neutrophilic leukemia (CNL) is a distinct myeloproliferative neoplasm defined by persistent, predominantly mature neutrophil proliferation, marrow granulocyte hyperplasia, and frequent splenomegaly. The seminal discovery of oncogenic driver mutations in CSF3R in the majority of patients with CNL in 2013 generated a new scientific framework for this disease as it deepened our understanding of its molecular pathogenesis, provided a biomarker for diagnosis, and rationalized management using novel targeted therapies...
February 13, 2018: Blood Cancer Journal
https://www.readbyqxmd.com/read/29406142/a-clinical-nurse-leader-cnl-practice-development-model-to-support-integration-of-the-cnl-role-into-microsystem-care-delivery
#7
Lorraine Kaack, Miriam Bender, Michael Finch, Linda Borns, Katherine Grasham, Alice Avolio, Shawna Clausen, Nadine A Terese, Diane Johnstone, Marjory Williams
The Veterans Health Administration (VHA) Office of Nursing Services (ONS) was an early adopter of Clinical Nurse Leader (CNL) practice, generating some of the earliest pilot data of CNL practice effectiveness. In 2011 the VHA ONS CNL Implementation & Evaluation Service (CNL I&E) piloted a curriculum to facilitate CNL transition to effective practice at local VHA settings. In 2015, the CNL I&E and local VHA setting stakeholders collaborated to refine the program, based on lessons learned at the national and local level...
January 2018: Journal of Professional Nursing: Official Journal of the American Association of Colleges of Nursing
https://www.readbyqxmd.com/read/29377168/technical-note-feasibility-study-of-titanium-markers-in-choroidal-melanoma-localization-for-proton-beam-radiation-therapy
#8
Inder K Daftari, Jeanne M Quivey, Jennifer S Chang, Kavita K Mishra
PURPOSE: The purpose of this study is to explore the feasibility of the use of titanium fiducial markers to minimize the metallic artifact seen with tantalum markers which causes significant distortion on postoperative orbital CT scans. METHOD: We designed and constructed the titanium markers in the shop of Crocker Nuclear Laboratory, UC Davis, CA. The markers were placed on an eyeball phantom. The eyeball was inserted into the Rando phantom in the orbital space...
January 29, 2018: Medical Physics
https://www.readbyqxmd.com/read/29346188/evaluation-of-role-acquisition-and-preparation-for-the-cnl-certification-examination-through-an-online-immersion-course
#9
Janie T Best, Annette Hines
No abstract text is available yet for this article.
January 16, 2018: Journal of Nursing Care Quality
https://www.readbyqxmd.com/read/29245892/mek-erk-addiction-in-cnl-acml
#10
EDITORIAL
Meenu Kesarwani, Zachary Kincaid, Mohammad Azam
No abstract text is available yet for this article.
November 21, 2017: Oncotarget
https://www.readbyqxmd.com/read/29244743/hematological-and-hemodynamic-responses-to-acute-and-short-term-creatine-nitrate-supplementation
#11
Ryan L Dalton, Ryan J Sowinski, Tyler J Grubic, Patrick B Collins, Adriana M Coletta, Aimee G Reyes, Brittany Sanchez, Majid Koozehchian, Yanghoon P Jung, Christopher Rasmussen, Mike Greenwood, Peter S Murano, Conrad P Earnest, Richard B Kreider
In a double-blind, crossover, randomized and placebo-controlled trial; 28 men and women ingested a placebo (PLA), 3 g of creatine nitrate (CNL), and 6 g of creatine nitrate (CNH) for 6 days. Participants repeated the experiment with the alternate supplements after a 7-day washout. Hemodynamic responses to a postural challenge, fasting blood samples, and bench press, leg press, and cycling time trial performance and recovery were assessed. Data were analyzed by univariate, multivariate, and repeated measures general linear models (GLM)...
December 15, 2017: Nutrients
https://www.readbyqxmd.com/read/29240571/developing-the-clinical-nurse-leader-survey-instrument-a-modified-delphi-study
#12
Miriam Bender, Alice Avolio, Patricia Baker, James L Harris, Nancy Hilton, Lisle Hites, Linda Roussel, Bobbi Shirley, Patricia L Thomas, Marjory Williams
Clinical nurse leader (CNL)-integrated care delivery is an emerging nursing model, with growing adoption in diverse health systems. To generate a robust evidence base for this promising nursing model, it is necessary to measure CNL practice to explicitly link it to observed quality and safety outcome improvements. This study used a modified Delphi approach with an expert CNL panel to develop and test the face, content, and construct validity of the CNL Practice Survey instrument.
December 13, 2017: Journal of Nursing Care Quality
https://www.readbyqxmd.com/read/29079707/ceramide-nanoliposomes-as-a-mlkl-dependent-necroptosis-inducing-chemotherapeutic-reagent-in-ovarian-cancer
#13
Xuewei Zhang, Kazuyuki Kitatani, Masafumi Toyoshima, Masumi Ishibashi, Toshinori Usui, Junko Minato, Mahy Egiz, Shogo Shigeta, Todd Fox, Tye Deering, Mark Kester, Nobuo Yaegashi
Ceramides are bioactive lipids that mediate cell death in cancer cells, and ceramide-based therapy is now being tested in dose-escalating phase I clinical trials as a cancer treatment. Multiple nanoscale delivery systems for ceramide have been proposed to overcome the inherent toxicities, poor pharmacokinetics, and difficult biophysics associated with ceramide. Using the ceramide nanoliposomes (CNL), we now investigate the therapeutic efficacy and signaling mechanisms of this nanoscale delivery platform in refractory ovarian cancer...
January 2018: Molecular Cancer Therapeutics
https://www.readbyqxmd.com/read/29025591/identification-of-a-novel-csf3r-sptan1-fusion-gene-in-an-atypical-chronic-myeloid-leukemia-patient-with-t-1-9-p34-q34-by-rna-seq
#14
Guangying Sheng, Jian Zhang, Zhao Zeng, Jinlan Pan, Qinrong Wang, Lijun Wen, Yang Xu, Depei Wu, Suning Chen
Membrane-proximal and truncated mutations of colony-stimulating factor 3 receptor (CSF3R) are frequently found in chronic neutrophilic leukemia (CNL) and atypical chronic myeloid leukemia (aCML). However, rearrangement involving CSF3R in hematological neoplasms has not been reported. Here, we report a case of a 21-year-old female diagnosed as aCML with t(1;9)(p34;q34) who presented a CSF3R rearrangement. First, RNA sequencing identified a novel fusion transcript involving exon 17 of CSF3R and exon 50 of non-erythrocytic-1-spectrin-alpha (SPTAN1)...
October 2017: Cancer Genetics
https://www.readbyqxmd.com/read/28983816/recent-progress-in-chronic-neutrophilic-leukemia-and-atypical-chronic-myeloid-leukemia
#15
REVIEW
Kim-Hien T Dao, Jeffrey W Tyner, Jason Gotlib
PURPOSE OF REVIEW: We reviewed recent diagnostic and therapeutic progress in chronic neutrophilic leukemia (CNL) and atypical chronic myeloid leukemia (aCML). We summarized recent genetic data that may guide future efforts towards implementing risk-adapted therapy based on mutational profile and improving disease control and survival of affected patients. RECENT FINDINGS: Recent genetic data in CNL and aCML prompted modifications to the World Health Organization (WHO) diagnostic criteria, which have improved our understanding of how CNL and aCML are different diseases despite sharing common findings of peripheral granulocytosis and marrow myeloid hyperplasia...
October 2017: Current Hematologic Malignancy Reports
https://www.readbyqxmd.com/read/28973974/comparative-genomics-of-non-tnl-disease-resistance-genes-from-six-plant-species
#16
Madhav P Nepal, Ethan J Andersen, Surendra Neupane, Benjamin V Benson
Disease resistance genes (R genes), as part of the plant defense system, have coevolved with corresponding pathogen molecules. The main objectives of this project were to identify non-Toll interleukin receptor, nucleotide-binding site, leucine-rich repeat (nTNL) genes and elucidate their evolutionary divergence across six plant genomes. Using reference sequences from Arabidopsis, we investigated nTNL orthologs in the genomes of common bean, Medicago, soybean, poplar, and rice. We used Hidden Markov Models for sequence identification, performed model-based phylogenetic analyses, visualized chromosomal positioning, inferred gene clustering, and assessed gene expression profiles...
September 30, 2017: Genes
https://www.readbyqxmd.com/read/28727829/functional-differentiation-and-spatial-temporal-co-expression-networks-of-the-nbs-encoding-gene-family-in-jilin-ginseng-panax-ginseng-c-a-meyer
#17
Rui Yin, Mingzhu Zhao, Kangyu Wang, Yanping Lin, Yanfang Wang, Chunyu Sun, Yi Wang, Meiping Zhang
Ginseng, Panax ginseng C.A. Meyer, is one of the most important medicinal plants for human health and medicine. It has been documented that over 80% of genes conferring resistance to bacteria, viruses, fungi and nematodes are contributed by the nucleotide binding site (NBS)-encoding gene family. Therefore, identification and characterization of NBS genes expressed in ginseng are paramount to its genetic improvement and breeding. However, little is known about the NBS-encoding genes in ginseng. Here we report genome-wide identification and systems analysis of the NBS genes actively expressed in ginseng (PgNBS genes)...
2017: PloS One
https://www.readbyqxmd.com/read/28676339/characterization-of-cnl-like-protein-fragment-cnl-lpf-from-mature-lageneria-siceraria-seeds
#18
Neha Kumari, Sumit Kumar Chaturvedi, Rehan Khan, Alpana Sharma, Rizwan Hasan Khan, Savita Yadav
Coiled coil domain-nucleotide binding site-leucine rich repeat (CC-NBS-LRR; CNL) proteins are highly conserved family of plant disease resistance proteins, remarkably comprise of coiled-coil domain, which plays significant role in plant innate immunity. The present study reports that moderately elicited oligomerization of plant CNL like protein fragment (CNL-LPF) in presence of ATP/Mg using various biophysical methods Circular dichroism (CD) results depicted a substantial increase in β-sheet structure content of CNL-LPF...
November 2017: International Journal of Biological Macromolecules
https://www.readbyqxmd.com/read/28672205/pep-1-peptide-functionalized-liposome-to-enhance-the-anticancer-efficacy-of-cilengitide-in-glioma-treatment
#19
Zhuomin Jiao, Yang Li, Hengyuan Pang, Yongri Zheng, Yan Zhao
In this study, we have established an effective and novel nanocarrier system for the effective treatment of glioma. We have established the glioma tissue penetrating nanocarrier system by conjugating Pep-1 as a targeting ligand on the liposome surface to enhance the anticancer efficacy of cilengitide (CGL). The particles were nanosized and exhibited a controlled release of drug in both the pH conditions. The cellular uptake assay showed that conjugation of Pep-1 on the liposome surface remarkably increased the cellular uptake...
October 1, 2017: Colloids and Surfaces. B, Biointerfaces
https://www.readbyqxmd.com/read/28649585/data-on-the-genome-wide-identification-of-cnl-r-genes-in-setaria-italica-l-p-beauv
#20
Ethan J Andersen, Madhav P Nepal
We report data associated with the identification of 242 disease resistance genes (R-genes) in the genome of Setaria italica as presented in "Genetic diversity of disease resistance genes in foxtail millet (Setaria italica L.)" (Andersen and Nepal, 2017) [1]. Our data describe the structure and evolution of the Coiled-coil, Nucleotide-binding site, Leucine-rich repeat (CNL) R-genes in foxtail millet. The CNL genes were identified through rigorous extraction and analysis of recently available plant genome sequences using cutting-edge analytical software...
August 2017: Data in Brief
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