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https://www.readbyqxmd.com/read/29166742/-allogeneic-hematopoietic-stem-cell-transplantation-for-the-treatment-of-acute-myeloid-leukemia-with-primary-thrombocytosis-three-cases-report-and-literatures-review
#1
X P Zong, L Tang, J N Cen, S N Chen, A N Sun, D P Wu
Objective: To investigate the characteristics of the essential thrombocythemia (ET) cases transformed to the acute myeloid leukemia (AML) and the role of allogeneic hematopoietic stem cell transplantation (allo-HSCT) in the treatment of this disease. Methods: The clinical and laboratory characteristics of 3 ET cases before and after transformation and after allo-HSCT were retrospectively analyzed, meanwhile the related literatures were reviewed and discussed. Results: Case 1 was a male patient of 44 years old, whose PLT was 500×10(9)/L when firstly diagnosed ET...
October 14, 2017: Zhonghua Xue Ye Xue za Zhi, Zhonghua Xueyexue Zazhi
https://www.readbyqxmd.com/read/29165768/recent-perspectives-of-pediatric-leukemia-an-update
#2
Q An, C-H Fan, S-M Xu
Leukemia is defined as an aberrant hyper-proliferation of immature blood cells that do not form solid tumor masses (i.e., liquid cancer). Usually, leukemia could be either of the myeloid or lymphoid lineages, and is classified as acute or chronic in nature. Chronic leukemias tend to have more mature cells and are rare in pediatric patients. Acute leukemias, on the other hand, are typically less mature and commonly occur in patients of all ages and are potentially rapidly fatal if not readily treated. The acute lymphoblastic leukemia (ALL) is the most common childhood malignancy...
October 2017: European Review for Medical and Pharmacological Sciences
https://www.readbyqxmd.com/read/29165246/regulatory-coiled-coil-domains-promote-head-to-head-assemblies-of-aaa-chaperones-essential-for-tunable-activity-control
#3
Marta Carroni, Kamila B Franke, Michael Maurer, Jasmin Jäger, Ingo Hantke, Felix Gloge, Daniela Linder, Sebastian Gremer, Kürşad Turgay, Bernd Bukau, Axel Mogk
Ring-forming AAA+ chaperones exert ATP-fueled substrate unfolding by threading through a central pore. This activity is potentially harmful requiring mechanisms for tight repression and substrate-specific activation. The AAA+ chaperone ClpC with the peptidase ClpP forms a bacterial protease essential to virulence and stress resistance. The adaptor MecA activates ClpC by targeting substrates and stimulating ClpC ATPase activity. We show how ClpC is repressed in its ground state by determining ClpC cryo-EM structures with and without MecA...
November 22, 2017: ELife
https://www.readbyqxmd.com/read/29164009/next-generation-gene-sequencing-differentiates-hypoplastic-myelodysplastic-syndrome-from-aplastic-anemia
#4
Jeffrey L Lew, Joshua L Fenderson, Mark G Carmichael
Hypoplastic Myelodysplastic Syndrome (h-MDS) comprises 15% of all MDS and has traditionally been difficult to distinguish from aplastic anemia (AA) by current testing. Accurate differentiation is important because treatment and prognosis differ. Since the publication of the 2008 World Health Organization classification of MDS, next-generation DNA sequencing has discovered novel mutations strongly associated with AA and MDS. Recent research supports the utility of identifying these mutations in the diagnosis and management of MDS; however, use of next-generation sequencing is not yet recommended in guidelines and the study is not routinely performed...
November 2017: Hawai'i Journal of Medicine & Public Health: a Journal of Asia Pacific Medicine & Public Health
https://www.readbyqxmd.com/read/29160918/impact-of-weight-loss-and-eating-difficulties-on-health-related-quality-of-life-up-to-10%C3%A2-years-after-oesophagectomy-for-cancer
#5
P Anandavadivelan, A Wikman, A Johar, P Lagergren
BACKGROUND: Severe weight loss is experienced by patients with eating difficulties after surgery for oesophageal cancer. The aim of this prospective cohort study was to asssess the influence of eating difficulties and severe weight loss on health-related quality of life (HRQoL) up to 10 years after oesophagectomy. METHODS: Data on bodyweight and HRQoL were collected at 6 months, 3, 5 and 10 years in patients who underwent surgery for oesophageal cancer in Sweden between 2001 and 2005...
November 21, 2017: British Journal of Surgery
https://www.readbyqxmd.com/read/29158926/acquired-elliptocytosis-as-a-manifestation-of-myelodysplastic-syndrome-with-ring-sideroblasts-and-multilineage-dysplasia
#6
Jacob D Kjelland, Denis M Dwyre, Brian A Jonas
Acquired elliptocytosis is a known but rarely described abnormality in the myelodysplastic syndromes (MDS). Here we report the case of an elderly male who was admitted to the hospital with chest pain, dyspnea, and fatigue and was found to be anemic with an elliptocytosis that had only recently been noted on peripheral smears of his blood. After bone marrow biopsy he was diagnosed with MDS with ring sideroblasts and multilineage dysplasia and acquired elliptocytosis. Here we report a rare case of acquired elliptocytosis cooccurring with MDS with ring sideroblasts and multilineage dysplasia...
2017: Case Reports in Hematology
https://www.readbyqxmd.com/read/29156198/the-evolving-role-of-genomic-testing-in-assessing-prognosis-of-patients-with-myelodysplastic-syndromes
#7
REVIEW
David P Steensma
The introduction into routine hematology-oncology clinical practice of molecular genetic testing assays based on next-generation sequencing platforms is prompting reassessment of the importance of molecular assay results in comparison to existing disease-specific risk stratification tools based on clinical assessment and light microscopy. For patients with myelodysplastic syndromes (MDS), the most commonly used tools for prognostication currently include the International Prognostic Scoring System (IPSS) and the Revised IPSS (IPSS-R), which are based on marrow blast proportion, number and degree of cytopenias, and the metaphase karyotype...
December 2017: Best Practice & Research. Clinical Haematology
https://www.readbyqxmd.com/read/29156197/mutational-complexity-in-myelodysplasia
#8
REVIEW
R Coleman Lindsley
Myelodysplastic syndromes are characterized by genetic and clinical heterogeneity. Some mutations are able to drive clonal hematopoiesis without causing clinical consequences, while other mutations may have significant impact, including the transformation to leukemia. This review aims to describe the pathogenesis of myelodysplastic syndromes (MDS) by focusing on 3 aspects: combinatorial genetic events, environmental factors, and inherited genetic conditions.
December 2017: Best Practice & Research. Clinical Haematology
https://www.readbyqxmd.com/read/29156196/familial-myelodysplastic-syndrome-acute-myeloid-leukemia
#9
REVIEW
Jane E Churpek
A growing number of inherited genetic loci that contribute to myelodysplastic syndrome/acute myeloid leukemia (MDS/AML) development in both children as well as adults are rapidly being identified. In recognition of the clinical impact of this emerging field, the World Health Organization, National Comprehensive Cancer Network, and European LeukemiaNet have all added consideration of inherited predisposition to MDS/AML classification and management. Study of these disorders is providing unique insight into the biology of both sporadic and familial MDS/AML...
December 2017: Best Practice & Research. Clinical Haematology
https://www.readbyqxmd.com/read/29155988/opioid-prescribing-patterns-and-patient-outcomes-by-prescriber-type-in-the-oregon-prescription-drug-monitoring-program
#10
Patrick B Fink, Richard A Deyo, Sara E Hallvik, Christi Hildebran
Objective: Prescription drug monitoring programs (PDMPs) were created to facilitate responsible use of controlled substances. In Oregon, physicians, physician's assistants (MDs/DOs/PAs), dentists, nurse practitioners (NPs), and naturopathic physicians (NDs) may prescribe opioids, but differences in prescribing practices, patient mix, and patient outcomes among prescriber types have not been characterized. Methods: De-identified Oregon PDMP data from October 2011 through October 2014 were linked with vital records and a statewide hospital discharge registry...
November 16, 2017: Pain Medicine: the Official Journal of the American Academy of Pain Medicine
https://www.readbyqxmd.com/read/29154147/selecting-the-minimum-data-set-and-quantitative-soil-quality-indexing-of-alkaline-soils-under-different-land-uses-in-northeastern-china
#11
Pujia Yu, Shiwei Liu, Liang Zhang, Qiang Li, Daowei Zhou
Understanding the influences of land use conversions on soil quality (SQ) and function are essential to adopt proper agricultural management practices for a specific region. The primary objective of this study was to develop soil quality indices (SQIs) to assess the short-term influences of different land uses on SQ in semiarid alkaline grassland in northeastern China. Land use treatments were corn cropland (Corn), alfalfa perennial forage (Alfalfa), monoculture Lyemus chinensis grassland (MG) and successional regrowth grassland (SRG), which were applied for five years...
November 15, 2017: Science of the Total Environment
https://www.readbyqxmd.com/read/29151133/treatment-of-relapsed-aml-and-mds-after-allogeneic-stem-cell-transplantation-with-decitabine-and-dli-a-retrospective-multicenter-analysis-on-behalf-of-the-german-cooperative-transplant-study-group
#12
Thomas Schroeder, Christina Rautenberg, William Krüger, Uwe Platzbecker, Gesine Bug, Juliane Steinmann, Stefan Klein, Olaf Hopfer, Kathrin Nachtkamp, Mustafa Kondakci, Stefanie Geyh, Rainer Haas, Ulrich Germing, Martin Bornhäuser, Guido Kobbe
In contrast to the evidence regarding azacitidine (Aza), there is limited knowledge about the combination of decitabine (DAC) and donor lymphocyte infusions as salvage therapy for relapse after allogeneic stem cell transplantation (allo-SCT) so far. We retrospectively analyzed data of 36 patients with hematological (n = 35) or molecular relapse (n = 1) of acute myeloid leukemia (AML, n = 29) or myelodysplastic syndrome (MDS, n = 7) collected from 6 German transplant centers. Patients were treated with a median of 2 cycles DAC (range, 1 to 11)...
November 18, 2017: Annals of Hematology
https://www.readbyqxmd.com/read/29150717/genetic-diversity-and-phylogenetic-study-of-the-chinese-gelao-ethnic-minority-via-23-y-str-loci
#13
Pengyu Chen, Yanyan Han, Guanglin He, Haibo Luo, Tianzhen Gao, Feng Song, Dengfu Wan, Jian Yu, Yiping Hou
Haplotype diversity for 23 Y chromosomal short tandem repeat (Y-STR) loci included in the PowerPlex(®) Y23 System was analyzed in the Chinese Gelao minority group of 234 unrelated males living in Daozhen Gelao-Miao Autonomous County in Guizhou Province, southwest China. A total of 216 different haplotypes were detected, of which 199 haplotypes were unique. The overall haplotype diversity (HD) and discrimination capacity (DC) were 0.9996 and 0.9231, respectively. The gene diversity (GD) ranged from 0.4159 (DYS438) to 0...
November 17, 2017: International Journal of Legal Medicine
https://www.readbyqxmd.com/read/29150478/early-versus-late-application-of-subthalamic-deep-brain-stimulation-to-parkinson-s-disease-patients-with-motor-complications-elass-protocol-of-a-multicentre-prospective-and-observational-study
#14
Lulu Jiang, Wai Sang Poon, Elena Moro, Wenbiao Xian, Chao Yang, Xian Lun Zhu, Jing Gu, Xiaodong Cai, Jinlong Liu, Vincent Mok, Yanmei Liu, Shaohua Xu, Qiyu Guo, Wanru Chen, Ling Chen
INTRODUCTION: Deep brain stimulation (DBS) of the subthalamic nucleus (STN) is a well-established surgical treatment for Parkinson's disease (PD). However, there is currently no consensus on the best timing for this surgery. The aim of our study is to compare the therapeutic efficacy of bilateral STN DBS in patients with PD with early and late motor complications. METHODS AND ANALYSIS: 200 patients with PD will be enrolled in this multicentre, prospective, observational study, and will be followed up for 4 years...
November 16, 2017: BMJ Open
https://www.readbyqxmd.com/read/29150183/genetic-analysis-of-29-y-str-loci-in-the-chinese-han-population-from-shanghai
#15
Yuxiang Zhou, Chengchen Shao, Liming Li, Yaqi Zhang, Baonian Liu, Qinrui Yang, Qiqun Tang, Shilin Li, Jianhui Xie
The analysis of Y chromosomal short tandem repeats (Y-STRs) provides important information that can be used to forensic investigation and population studies. In this study, typing of 29 Y-STRs included in the PowerPlex(®) Y23 system (PPY23) and Yfiler™ Plus system (Yfiler plus) was performed on 843 unrelated male samples from Han population in Shanghai. Besides null, duplicate, and intermediate alleles reported in previous studies, an allele of 10 at DYS643 with a 2-base deletion in the flanking region was initially observed...
November 11, 2017: Forensic Science International. Genetics
https://www.readbyqxmd.com/read/29149650/iron-chelation-therapy-in-lower-ipss-risk-myelodysplastic-syndromes-which-subtypes-benefit
#16
Shannon A Wong, Heather A Leitch
BACKGROUND: Analyses suggest MDS patients with higher serum ferritin levels (SF) have inferior overall survival (OS), in one study across MDS subtypes. Multiple analyses suggest those with high SF receiving iron chelation therapy (ICT) have superior OS, but which MDS subtypes benefit from ICT remains undefined. METHODS: We performed survival analyses of MDS subtypes by receipt of ICT. RESULTS: 182 MDS were lower IPSS risk and received red blood cell (RBC) transfusions; 63 received ICT...
November 11, 2017: Leukemia Research
https://www.readbyqxmd.com/read/29149140/optimization-of-postoperative-intravenous-patient-controlled-analgesia-with-opioid-dexmedetomidine-combinations-an-updated-meta-analysis-with-trial-sequential-analysis-of-randomized-controlled-trials
#17
Ke Peng, Juan Zhang, Xiao-Wen Meng, Hua-Yue Liu, Fu-Hai Ji
BACKGROUND: It is still a challenge to optimize postoperative pain management. The effects of adding dexmedetomidine (DEX) to opioid-based postoperative intravenous patient-controlled analgesia (PCA) are not fully understood. OBJECTIVES: The aim of this study is to assess the efficacy and safety of opioid-DEX combinations for postoperative PCA, and a trial sequential analysis (TSA) is utilized to evaluate the robustness of the current evidence. STUDY DESIGN: A systematic review and meta-analysis...
November 2017: Pain Physician
https://www.readbyqxmd.com/read/29147080/effect-of-the-ginger-derivative-6-shogaol-on-ferritin-levels-in-patients-with-low-to-intermediate-1-risk-myelodysplastic-syndrome-a-small-investigative-study
#18
Terry Golombick, Terrence H Diamond, Arumugam Manoharan, Rajeev Ramakrishna, Vladimir Badmaev
Background: Myelodysplastic syndrome (MDS) is a heterogeneous group of clonal stem cell disorders characterized by dysplastic and ineffective hematopoiesis and peripheral cytopenias. Elevated serum ferritin (SF) is often observed in nontransfused, lower risk MDS. It has been reported that ineffective erythropoiesis enhances iron absorption in MDS through downregulation of hepcidin and its prohormones such that SF rises. Aim: To determine the effect of 6-shogaol, a dehydration derivative of ginger, known to have hepatoprotective and chemotherapeutic activity, on 6 early-stage, transfusion-independent patients with MDS, 3 of whom had raised levels of SF...
2017: Clinical Medicine Insights. Blood Disorders
https://www.readbyqxmd.com/read/29146900/the-genomic-landscape-of-pediatric-myelodysplastic-syndromes
#19
Jason R Schwartz, Jing Ma, Tamara Lamprecht, Michael Walsh, Shuoguo Wang, Victoria Bryant, Guangchun Song, Gang Wu, John Easton, Chimene Kesserwan, Kim E Nichols, Charles G Mullighan, Raul C Ribeiro, Jeffery M Klco
Myelodysplastic syndromes (MDS) are uncommon in children and have a poor prognosis. In contrast to adult MDS, little is known about the genomic landscape of pediatric MDS. Here, we describe the somatic and germline changes of pediatric MDS using whole exome sequencing, targeted amplicon sequencing, and/or RNA-sequencing of 46 pediatric primary MDS patients. Our data show that, in contrast to adult MDS, Ras/MAPK pathway mutations are common in pediatric MDS (45% of primary cohort), while mutations in RNA splicing genes are rare (2% of primary cohort)...
November 16, 2017: Nature Communications
https://www.readbyqxmd.com/read/29146882/physiological-srsf2-p95h-expression-causes-impaired-hematopoietic-stem-cell-functions-and-aberrant-rna-splicing-in-mice
#20
Ayana Kon, Satoshi Yamazaki, Yasuhito Nannya, Keisuke Kataoka, Yasunori Ota, Masahiro Marshall Nakagawa, Kenichi Yoshida, Yusuke Shiozawa, Maiko Morita, Tetsuichi Yoshizato, Masashi Sanada, Manabu Nakayama, Haruhiko Koseki, Hiromitsu Nakauchi, Seishi Ogawa
Splicing factor (SF) mutations are characteristic of myelodysplastic syndromes (MDS) and related myeloid neoplasms and implicated in their pathogenesis, but their roles in the development of MDS have not fully been elucidated. Here, we investigated the consequence of mutant Srsf2 expression using newly generated Vav1-Cre-mediated conditional knock-in mice. Mice carrying a heterozygous Srsf2 P95H mutation showed significantly reduced numbers of hematopoietic stem and progenitor cells (HSPCs) and differentiation defects both in the steady-state condition and transplant settings...
November 16, 2017: Blood
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