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https://www.readbyqxmd.com/read/28944850/differentially-expressed-long-non%C3%A2-coding-rnas-and-mrnas-in-patients-with-iga-nephropathy
#1
Nan Zuo, Yun Li, Nan Liu, Lining Wang
Long non‑coding RNAs (lncRNAs) have been reported to serve a crucial role in renal diseases; however, their role in immunoglobulin A nephropathy (IgAN) remains unclear. In the present study, peripheral blood mononuclear cells (PBMCs) were collected from both patients with IgAN and healthy controls. A microarray analysis was then performed to identify differentially expressed lncRNAs and mRNAs in PBMCs, which were confirmed by quantitative polymerase chain reaction. In addition, Gene Ontology (GO), Kyoto Encyclopedia of Genes and Genomes (KEGG) pathway and lncRNA‑mRNA co‑expression network analyses were conducted...
September 20, 2017: Molecular Medicine Reports
https://www.readbyqxmd.com/read/28944163/weathering-the-storm-improving-therapeutic-interventions-for-cytokine-storm-syndromes-by-targeting-disease-pathogenesis
#2
Lehn K Weaver, Edward M Behrens
Cytokine storm syndromes require rapid diagnosis and treatment to limit the morbidity and mortality caused by the hyperinflammatory state that characterizes these devastating conditions. Herein, we discuss the current knowledge that guides our therapeutic decision-making and personalization of treatment for patients with cytokine storm syndromes. Firstly, ICU-level supportive care is often required to stabilize patients with fulminant disease while additional diagnostic evaluations proceed to determine the underlying cause of cytokine storm...
March 2017: Current Treatment Options in Rheumatology
https://www.readbyqxmd.com/read/28944135/management-of-syndromic-diarrhea-tricho-hepato-enteric-syndrome-a-review-of-the-literature
#3
REVIEW
Alexandre Fabre, Patrice Bourgeois, Marie-Edith Coste, Céline Roman, Vincent Barlogis, Catherine Badens
Syndromic diarrhea/tricho-hepato-enteric syndrome (SD/THE) is a rare disease linked to the loss of function of either TTC37 or SKIV2L, two components of the SKI complex. It is characterized by a combination of 9 signs (intractable diarrhea, hair abnormalities, facial dysmorphism, immune abnormalities, IUGR/SGA, liver abnormalities, skin abnormalities, congenital heart defect and platelet abnormalities). We present a comprehensive review of the management of SD/THE and tested therapeutic regimens. A review of the literature was conducted in May 2017: 29 articles and 2 abstracts were included describing a total of 80 patients, of which 40 presented with mutations of TTC37, 14 of SKIV2L...
August 2017: Intractable & Rare Diseases Research
https://www.readbyqxmd.com/read/28943635/ng2-antigen-is-involved-in-leukemia-invasiveness-and-central-nervous-system-infiltration-in-mll-rearranged-infant-b-all
#4
C Prieto, B López-Millán, H Roca-Ho, R W Stam, D Romero-Moya, F J Rodríguez-Baena, A Sanjuan-Pla, V Ayllón, M Ramírez, M Bardini, P De Lorenzo, M G Valsecchi, M Stanulla, M Iglesias, P Ballerini, Á M Carcaboso, J Mora, F Locatelli, A Bertaina, L Padilla, J C Rodríguez-Manzaneque, C Bueno, P Menéndez
MLL-rearranged (MLLr) infant B-cell acute lymphoblastic leukemia (iMLLr-B-ALL) has a dismal prognosis and is associated with a pro-B/mixed phenotype, therapy refractoriness and frequent central nervous system (CNS) disease/relapse. Neuron-glial antigen 2 (NG2) is specifically expressed in MLLr leukemias and is used in leukemia immunophenotyping because of its predictive value for MLLr acute leukemias. NG2 is involved in melanoma metastasis and brain development; however, its role in MLL-mediated leukemogenesis remains elusive...
September 25, 2017: Leukemia: Official Journal of the Leukemia Society of America, Leukemia Research Fund, U.K
https://www.readbyqxmd.com/read/28943295/the-slippery-slope-of-hematopoietic-stem-cell-aging
#5
Martin Wahlestedt, David Bryder
The late stages of life are in most species, including humans, associated with a decline in overall organism maintenance/health. This applies also to the hematopoietic system, where aging associates not only to an increased predisposition for hematological malignancies, but also as a strong comorbidity factor for other diseases. Research during the last two decades have proposed that alterations at the level of hematopoietic stem cells (HSCs) might be a root cause for the hematological changes observed with age...
September 21, 2017: Experimental Hematology
https://www.readbyqxmd.com/read/28943250/in%C3%A2-vivo-generation-of-engraftable-murine-hematopoietic-stem-cells-by-gfi1b-c-fos-and-gata2-overexpression-within-teratoma
#6
Masao Tsukada, Yasunori Ota, Adam C Wilkinson, Hans J Becker, Motomi Osato, Hiromitsu Nakauchi, Satoshi Yamazaki
Generation of hematopoietic stem cells (HSCs) from pluripotent stem cells (PSCs) could potentially provide unlimited HSCs for clinical transplantation, a curative treatment for numerous blood diseases. However, to date, bona fide HSC generation has been largely unsuccessful in vitro. We have previously described proof of concept for in vivo HSC generation from PSCs via teratoma formation. However, our first-generation system was complex and the output low. Here, we further optimize this technology and demonstrate the following: (1) simplified HSC generation using transcription factor overexpression; (2) improved HSC output using c-Kit-deficient host mice, and (3) that teratomas can be transplanted and cryopreserved...
September 8, 2017: Stem Cell Reports
https://www.readbyqxmd.com/read/28942016/positron-emission-tomography-based-assessment-of-metabolic-tumor-volume-predicts-survival-after-autologous-hematopoietic-cell-transplantation-for-hodgkin-lymphoma
#7
Vít Procházka, Rakhee S Gawande, Zuzan Cayci, Jerry W Froelich, Qing Cao, Chris Wilke, Kathryn Dusenbery, Daniel J Weisdorf, Veronika Bachanova
Autologous hematopoietic cell transplantation (AHCT) is curative for 60% of patients with relapsed or refractory Hodgkin lymphoma (R/R HL). A more precise assessment of the depth of remission prior to AHCT may help to identify patients likely to benefit from AHCT. We aimed to determine whether PET-based quantitative parameters of total metabolic tumor volume (TMTV), total lesion glycolysis (TLG), and maximal standardized uptake volume (SUVmax) measured prior to AHCT predict progression-free survival (PFS) after transplant...
September 20, 2017: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/28941161/from-clinical-observations-and-molecular-dissection-to-novel-therapeutic-strategies-for-primary-immunodeficiency-disorders
#8
REVIEW
Hans D Ochs, Daniel Petroni
The field of primary immunodeficiency diseases (PID) is rapidly expanding with more than 300 genetically defined disorders that have been clinically described and molecularly analyzed. The molecular dissection of these entities has led to the discovery of new immunologic pathways and to novel and effective disease-specific therapies. This review provides a summary of these primary immune defects categorized by clinical phenotype and molecular similarity as defined by the International Union of Immunologic Societies (IUIS) Expert Committee for PID...
September 21, 2017: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/28940837/a-case-of-the-nephrotic-syndrome-in-bone-marrow-transplantation-recipient-histologically-showing-overlapped-glomerular-lesions-of-thrombotic-microangiopathy-and-membranous-nephropathy
#9
Naoko Masuzawa, Ayako Nishimura, Takashi Kitani, Keiichi Tamagaki, Mio Sugitani, Hisao Nagoshi, Junya Kuroda, Eiichi Konishi
Nephrotic syndrome (NS) rarely occurs in post-hematopoietic stem cell transplantation (HSCT) recipients but represents the renal manifestation of graft-versus-host disease (GVHD). Membranous nephropathy (MN) accounts for almost two thirds of post-HSCT NS and is caused by immune complex deposition. Renal thrombotic microangiopathy (TMA) without fulfillment of clinical criteria for TMA has been underreported because of reduced opportunity for histological examination. However, renal TMA has recently been reported in association with GVHD and humoral immunological reactions...
September 20, 2017: Pathology International
https://www.readbyqxmd.com/read/28939876/thrombopoietic-stimulating-activity-of-rhtyrrs-y341a
#10
Yu Jinchao, Zhang Yanling, Wang Xu, Zhao Bing, Ye Yuhao, Zhou Weiran, Sun Shaoyang, Ma Liyun, Shi Yun, Zhan Ling, Yu Min, Mo Wei
Tumor radiotherapy induces hematopoietic organ damage and reduces thrombocyte counts. Thrombocytopenia is a common disease. Some studies have shown that tRNA synthetase plays not only catalytic tRNA aminoacylation roles, but also functions similarly to cytokines. Recombinant human tyrosyl-tRNA synthetase with a mutated Y341A (rhTyrRS (Y341A)) promotes megakaryocyte migrate from bone marrow to peripheral blood. It would promote megakaryocytes in the lungs adhering to vascular endothelial cells and resulting in the platelet production...
September 22, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28939456/combination-of-high-dose-methylprednisolone-and-defibrotide-for-veno-occlusive-disease-in-pediatric-hematopoietic-stem-cell-transplant-recipients
#11
Nicholas J Gloude, Sonata Jodele, Ashley Teusink-Cross, Michael Grimley, Stella M Davies, Adam Lane, Kasiani C Myers
Veno-occlusive disease (VOD) is a serious complication of hematopoietic stem cell transplant (HSCT), with high mortality in severe cases, and until recently very limited therapeutic options, consisting largely of supportive care. Defibrotide was recently approved in the United States for the treatment of severe VOD in patients with renal or pulmonary dysfunction following HSCT. Our group previously published on the use of high-dose methylprednisolone (500 mg/m(2)/dose every 12 hours for 6 doses) in patients with VOD showing good success...
September 19, 2017: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/28939455/neurocognitive-dysfunction-in-hematopoietic-cell-transplant-recipients-expert-review-from-the-late-effects-and-quality-of-life-working-committee-of-the-cibmtr-and-complications-and-quality-of-life-working-party-of-the-ebmt
#12
REVIEW
Debra Lynch Kelly, David Buchbinder, Rafael F Duarte, Jeffrey J Auletta, Neel Bhatt, Michael Byrne, Melissa Gabriel, Anuj Mahindra, Maxim Norkin, Helene Schoemans, Ami J Shah, Ibrahim Ahmed, Yoshiko Atsuta, Grzegorz W Basak, Sara Beattie, Sita Bhella, Christopher Bredeson, Nancy Bunin, Jignesh Dalal, Andrew Daly, James Gajewski, Robert Peter Gale, John Galvin, Mehdi Hamadani, Robert J Hayashi, Kehinde Adekola, Jason Law, Catherine J Lee, Jane Liesveld, Adriana K Malone, Arnon Nagler, Seema Naik, Taiga Nishihori, Susan K Parsons, Angela Scherwath, Hannah-Lise Schofield, Robert Soiffer, Jeff Szer, Ida Twist, Anne Warwick, Baldeep M Wirk, Jean Yi, Minoo Battiwalla, Mary E Flowers, Bipin Savani, Bronwen E Shaw
Hematopoietic cell transplantation (HCT) is a potentially curative treatment for children and adults with malignant and non-malignant diseases. Despite increasing survival rates, long-term morbidity following HCT is substantial. Neurocognitive dysfunction is a serious cause of morbidity, yet little is known about neurocognitive dysfunction following HCT. To address this gap, collaborative efforts of the Center for International Blood and Marrow Transplant Research and the European Society for Blood and Marrow Transplantation undertook an expert review of neurocognitive dysfunction following HCT...
September 19, 2017: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/28939453/bone-marrow-wt1-levels-in-allogeneic-hematopoietic-stem-cell-transplant-hct-for-acute-myeloid-leukemia-and-myelodysplasia-clinically-relevant-time-points-and-100-copies-threshold-value
#13
Josep F Nomdedéu, Albert Esquirol, Maite Carricondo, Marta Pratcorona, Montserrat Hoyos, Ana Garrido, Miguel Rubio, Elena Bussaglia, Irene García-Cadenas, Camino Estivill, Salut Brunet, Rodrigo Martino, Jorge Sierra
The outcome of allogeneic hematopoietic stem cell transplant (HCT) in patients with myeloid malignancies is better in those without minimal residual disease (MRD) than in those with MRD+ as assessed by multiparametric flow cytometry (MPFC). WT1 quantitation has also been used to assess the probability of relapse in acute myeloid leukemia treated with chemotherapy. We analyzed the clinical value of normalized bone marrow WT1 levels as a measure of the expanded myeloid progenitor compartment in a consecutive series of 193 adult patients with myeloid malignancies who underwent HCT...
September 19, 2017: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/28939416/the-gnas-r201c-mutation-associated-with-clonal-hematopoiesis-supports-transplantable-hematopoietic-stem-cell-activity
#14
Elizabeth L Ostrander, Won Kyun Koh, Cates Mallaney, Ashley C Kramer, W Casey Wilson, Bo Zhang, Grant A Challen
Genome sequencing efforts have identified virtually all of the important mutations in adult myeloid malignancies. More recently, population studies have identified cancer-associated variants in the blood of otherwise healthy individuals as they age, a phenomenon termed clonal hematopoiesis of indeterminate potential (CHIP). This suggests these mutations may occur in HSCs long before any clinical presentation, but are not necessarily harbingers of transformation as only a fraction of individuals with CHIP develop hematopoietic pathologies...
September 19, 2017: Experimental Hematology
https://www.readbyqxmd.com/read/28938875/predictive-implications-of-albumin-and-c-reactive-protein-for-progression-to-pneumonia-and-poor-prognosis-in-stenotrophomonas-maltophilia-bacteremia-following-allogeneic-hematopoietic-stem-cell-transplantation
#15
Kaito Harada, Noritaka Sekiya, Tatsuya Konishi, Akihito Nagata, Yuta Yamada, Toshiaki Takezaki, Satoshi Kaito, Shuhei Kurosawa, Masahiro Sakaguchi, Shunichiro Yasuda, Shugo Sasaki, Kosuke Yoshioka, Kyoko Watakabe-Inamoto, Aiko Igarashi, Yuho Najima, Takeshi Hagino, Hideharu Muto, Takeshi Kobayashi, Noriko Doki, Kazuhiko Kakihana, Hisashi Sakamaki, Kazuteru Ohashi
BACKGROUND: Stenotrophomonas maltophilia (S. maltophilia) bacteremia causes significant morbidity and mortality in immunocompromised hosts. However, incidence and risk factors for mortality in S. maltophilia bacteremia following allogeneic hematopoietic stem cell transplantation (allo-HSCT) remain controversial. The primary aim of this study is to clarify factors associated with poor prognosis of allo-HSCT recipients with S. maltophilia bacteremia. METHODS: From January 2005 to December 2014, patients with hematological diseases and S...
September 22, 2017: BMC Infectious Diseases
https://www.readbyqxmd.com/read/28938702/correction-endothelial-microparticles-delivering-microrna-155-into-t-lymphocytes-are-involved-in-the-initiation-of-acute-graft-versus-host-disease-following-allogeneic-hematopoietic-stem-cell-transplantation
#16
Ran Zhang, Xiaoxiao Wang, Mei Hong, Ting Luo, Miaomiao Zhao, Haorui Shen, Jun Fang, Xiaojie Li, Sibin Zang, Ping Chen, Dimin Nie, Peng Zheng, Qiuling Wu, Linghui Xia
[This corrects the article DOI: 10.18632/oncotarget.15579.].
August 29, 2017: Oncotarget
https://www.readbyqxmd.com/read/28936743/therapeutic-potential-of-hematopoietic-stem-cell-derived-exosomes-in-cardiovascular-disease
#17
Jana Radosinska, Monika Bartekova
As other stem cells, hematopoietic stem cells (HSCs) are able to produce extracellular vesicles (EVs) including exosomes and microvesicles. This chapter summarizes the knowledge about the production of EVs by the HSCs, their role in the intercellular communication, and will discuss the cargo of these EVs as well as protective effects of HSCs-derived exosomes and microvesicles in cardiovascular diseases (CVD). Available data showed that cardioprotective action of injected HSCs could not be explained by direct transdifferentiationof injected cells into the cardiomyocytes, this effect is suggested to be mediated via paracrine communication (by EVs) between donor and recipient cells...
2017: Advances in Experimental Medicine and Biology
https://www.readbyqxmd.com/read/28936210/novel-mutations-of-the-tetratricopeptide-repeat-domain-7a-gene-and-phenotype-genotype-comparison
#18
Reyin Lien, Yung-Feng Lin, Min-Wei Lai, Hui-Ying Weng, Ren-Chin Wu, Tang-Her Jaing, Jing-Long Huang, Shih-Feng Tsai, Wen-I Lee
The gastrointestinal tract contains the largest lymphoid organ to react with pathogenic microorganisms and suppress excess inflammation. Patients with primary immunodeficiency diseases (PIDs) can suffer from refractory diarrhea. In this study, we present two siblings who began to suffer from refractory diarrhea with a poor response to aggressive antibiotic and immunosuppressive treatment after surgical release of neonatal intestinal obstruction. Their lymphocyte proliferation was low, but superoxide production and IL-10 signaling were normal...
2017: Frontiers in Immunology
https://www.readbyqxmd.com/read/28934563/hemophagocytic-lymphohistiocytosis
#19
Hanny Al-Samkari, Nancy Berliner
Hemophagocytic lymphohistiocytosis is a life-threatening disorder characterized by unbridled activation of cytotoxic T lymphocytes, natural killer (NK) cells, and macrophages resulting in hypercytokinemia and immunemediated injury of multiple organ systems. It is seen in both children and adults and is recognized as primary (driven by underlying genetic mutations that abolish critical proteins required for normal function of cytotoxic T cells and NK cells) or secondary (resulting from a malignant, infectious, or autoimmune stimulus without an identifiable underlying genetic trigger)...
September 13, 2017: Annual Review of Pathology
https://www.readbyqxmd.com/read/28934481/automatic-identification-of-informative-regions-with-epigenomic-changes-associated-to-hematopoiesis
#20
Enrique Carrillo-de-Santa-Pau, David Juan, Vera Pancaldi, Felipe Were, Ignacio Martin-Subero, Daniel Rico, Alfonso Valencia
Hematopoiesis is one of the best characterized biological systems but the connection between chromatin changes and lineage differentiation is not yet well understood. We have developed a bioinformatic workflow to generate a chromatin space that allows to classify 42 human healthy blood epigenomes from the BLUEPRINT, NIH ROADMAP and ENCODE consortia by their cell type. This approach let us to distinguish different cells types based on their epigenomic profiles, thus recapitulating important aspects of human hematopoiesis...
September 19, 2017: Nucleic Acids Research
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