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https://www.readbyqxmd.com/read/28534442/genotype-and-haplotype-analysis-of-abcb1-at-1236-2677-and-3435-among-systemic-sclerosis-patients
#1
Małgorzata Barańska, Mariola Rychlik-Sych, Jadwiga Skrętkowicz, Michał Dudarewicz, Bożena Dziankowska-Bartkowiak, Jacek Owczarek, Elżbieta Waszczykowska
Systemic sclerosis (SSc) belongs to the group of systemic diseases of the connective tissue, which are characterized by a chronic autoimmune inflammatory process. P-glycoprotein, initially associated with the drug resistance in patients with cancer, becomes more and more often a subject of considerations in terms of its significance in the development of illnesses, including autoimmune diseases. The aim of the study was an attempt to answer the question whether there was a relationship between ABCB1 polymorphisms and morbidity of systemic sclerosis in a Polish population...
May 23, 2017: Autoimmunity
https://www.readbyqxmd.com/read/28532772/echocardiographic-predictors-for-worsening-of-six-minute-walk-distances-in-patients-with-systemic-sclerosis-scleroderma
#2
Kenya Kusunose, Hirotsugu Yamada, Susumu Nishio, Yukina Hirata, Hiromitsu Seno, Yoshihito Saijo, Takayuki Ise, Takeshi Tobiume, Koji Yamaguchi, Shusuke Yagi, Takeshi Soeki, Tetsuzo Wakatsuki, Masataka Sata
Change in 6-minute walk distance (6MWD) has been used as a clinical marker in pulmonary hypertension. Determinants and worsening of 6MWD remain a matter of debate because nonpulmonary factors have an impact on the 6MWD. We hypothesized that future reduction of 6MWD in patients with systemic sclerosis (SSc) was more closely associated with cardiac dysfunction. We prospectively performed standard clinical and echocardiographic evaluations in SSc patients with the 6-minute walk test at enrollment. Features associated with the 6MWD were sought in a multiple linear regression analysis and compared using standardized β...
April 26, 2017: American Journal of Cardiology
https://www.readbyqxmd.com/read/28532580/sirt1-ameliorates-systemic-sclerosis-by-targeting-the-mtor-pathway
#3
Xiaoxia Zhu, Haiyan Chu, Shuai Jiang, Qingmei Liu, Lei Liu, Yu Xue, Shucong Zheng, Weiguo Wan, Jianhua Qiu, Jiucun Wang, Hejian Zou
BACKGROUND: Systemic sclerosis (SSc) is a chronic autoimmune disease characterized by inflammation and fibrosis. Our previous research has indicated that Sirtuin1 (Sirt1) plays a role in the regulation of TNF-α-induced inflammation; however, whether Sirt1 may inhibit the progress of SSc by blocking inflammation remains unknown. OBJECTIVE: We aimed to investigate the function of Sirt1 in SSc. METHODS: The function and its mechanism of Sirt1 were evaluated in fibroblasts or scleroderma mice...
May 3, 2017: Journal of Dermatological Science
https://www.readbyqxmd.com/read/28531375/mouse-minipuberty-coincides-with-gonocyte-transformation-into-spermatogonial-stem-cells-a-model-for-human-minipuberty
#4
Ruili Li, Amanda Vannitamby, Sarah S K Yue, David Handelsman, John Hutson
As the transient postnatal hormone surge in humans, known as 'minipuberty', occurs simultaneously with key steps in germ-cell development, we investigated whether similar changes occur in the hypothalamic-pituitary-testicular axis of neonatal mice at a time that would coincide with gonocyte transformation into spermatogonial stem cells (SSC). Serum and testes were collected from C57Bl/6 mice at embryonic Day 17 (E17), birth (postnatal Day 0; P0) and daily until P10. Serum FSH and testosterone levels in both serum and testes were analysed and gene expression of FSH receptor (Fshr), luteinising hormone receptor (Lhr), anti-Müllerian hormone (Amh), octamer-binding transcription factor 4 (Oct-4), membrane type 1 metalloprotease (Mt1-mmp), proto-oncogene C-kit and promyelocytic leukaemia zinc finger (Plzf ) was quantified by real-time polymerase chain reaction...
May 23, 2017: Reproduction, Fertility, and Development
https://www.readbyqxmd.com/read/28530671/cardiopulmonary-assessment-of-patients-with-systemic-sclerosis-for-hematopoietic-stem-cell-transplantation-recommendations-from-the-european-society-for-blood-and-marrow-transplantation-autoimmune-diseases-working-party-and-collaborating-partners
#5
REVIEW
D Farge, R K Burt, M-C Oliveira, E Mousseaux, M Rovira, Z Marjanovic, J de Vries-Bouwstra, N Del Papa, R Saccardi, S J Shah, D C Lee, C Denton, T Alexander, D G Kiely, J A Snowden
Systemic sclerosis (SSc) is a rare disabling autoimmune disease with a similar mortality to many cancers. Two randomized controlled trials of autologous hematopoietic stem cell transplantation (AHSCT) for SSc have shown significant improvement in organ function, quality of life and long-term survival compared to standard therapy. However, transplant-related mortality (TRM) ranged from 3-10% in patients undergoing HSCT. In SSc, the main cause of non-transplant and TRM is cardiac related. We therefore updated the previously published guidelines for cardiac evaluation, which should be performed in dedicated centers with expertize in HSCT for SSc...
May 22, 2017: Bone Marrow Transplantation
https://www.readbyqxmd.com/read/28528914/systemic-sclerosis-dermal-fibroblasts-suppress-th1-cytokine-production-via-galectin-9-overproduction-due-to-fli1-deficiency
#6
Ryosuke Saigusa, Yoshihide Asano, Kouki Nakamura, Megumi Hirabayashi, Shunsuke Miura, Takashi Yamashita, Takashi Taniguchi, Yohei Ichimura, Takehiro Takahashi, Ayumi Yoshizaki, Tomomitsu Miyagaki, Makoto Sugaya, Shinichi Sato
Dermal fibroblasts promote skin-localized transdifferentiation of Tregs to Th2-like cells in systemic sclerosis (SSc). However, the entire effect of SSc dermal fibroblasts on immune cells still remains unknown. Since galectin-9 induces Th2 cytokine-predominant immune imbalance by negatively regulating Th1/Th17 cells in inflammatory diseases, we investigated the contribution of galectin-9 to Th immune balance in SSc lesional skin. We utilized human clinical samples and Fli1(+/-) mice because Fli1 deficiency induces SSc-like phenotypes in various cell types...
May 18, 2017: Journal of Investigative Dermatology
https://www.readbyqxmd.com/read/28528622/introductions-during-time-outs-do-surgical-team-members-know-one-another-s-names
#7
David J Birnbach, Lisa F Rosen, Maureen Fitzpatrick, John T Paige, Kristopher L Arheart
BACKGROUND: Introductions are the first item of the time-out in the World Health Organization Surgical Safety Checklist (SSC). It has yet to be established that surgical teams use colleagues' names or consider the use of names important. A study was conducted to determine if using the SSC has a measurable impact on name retention and to assess if operating room (OR) personnel believe it is important to know the names of their colleagues or for their colleagues to know theirs. METHODS: All OR personnel were individually interviewed at the end of 25 surgical cases in which the SSC was used...
June 2017: Joint Commission Journal on Quality and Patient Safety
https://www.readbyqxmd.com/read/28528521/myeloid-populations-in-systemic-autoimmune-diseases
#8
REVIEW
María Morell, Nieves Varela, Concepción Marañón
Systemic autoimmune diseases (SADs) encompass a wide spectrum of clinical signs as a reflection of their complex physiopathology. A variety of mechanisms related with the innate immune system are in the origin of the loss of self-tolerance in these diseases, and for most of them, the myeloid leukocytes are key actors. Monocytes, macrophages, dendritic cells, and neutrophils are first-line immune effectors located in the interface between innate and adaptive immunity. They are crucial in the organization of the local and systemic responses to damage-associated molecular patterns (DAMPs) and determine the intensity, orientation, and duration of the local immune response through the expression of chemokines, costimulatory or protolerogenic factors...
May 20, 2017: Clinical Reviews in Allergy & Immunology
https://www.readbyqxmd.com/read/28527380/measurement-differences-between-turbidity-instruments-and-their-implications-for-suspended-sediment-concentration-and-load-calculations-a-sensor-inter-comparison-study
#9
A Rymszewicz, J J O'Sullivan, M Bruen, J N Turner, D M Lawler, E Conroy, M Kelly-Quinn
The use of turbidity for indicating environmentally detrimental levels of suspended and colloidal matter in freshwater systems, and for defining acceptable water quality standards in national and European drinking water regulations, is well established. Turbidity is therefore frequently adopted as a surrogate for suspended sediment concentrations (SSC), or as a relative and objective measure of water clarity in monitoring programmes. Through systematic, controlled experimentation, we tested the response of 12 commercially available turbidity sensors, of various designs, to gauge their measurement consistency when benchmarked against pre-prepared sediment suspensions of known SSC...
May 17, 2017: Journal of Environmental Management
https://www.readbyqxmd.com/read/28526340/unfolding-the-pathogenesis-of-scleroderma-through-genomics-and-epigenomics
#10
REVIEW
Pei-Suen Tsou, Amr H Sawalha
With unknown etiology, scleroderma (SSc) is a multifaceted disease characterized by immune activation, vascular complications, and excessive fibrosis in internal organs. Genetic studies, including candidate gene association studies, genome-wide association studies, and whole-exome sequencing have supported the notion that while genetic susceptibility to SSc appears to be modest, SSc patients are genetically predisposed to this disease. The strongest genetic association for SSc lies within the MHC region, with loci in HLA-DRB1, HLA-DQB1, HLA-DPB1, and HLA-DOA1 being the most replicated...
May 16, 2017: Journal of Autoimmunity
https://www.readbyqxmd.com/read/28522286/increased-proportions-of-functionally-impaired-regulatory-t-cell-subsets-in-systemic-sclerosis
#11
Emese Ugor, Diána Simon, Giovanni Almanzar, Ramóna Pap, József Najbauer, Péter Németh, Péter Balogh, Martina Prelog, László Czirják, Tímea Berki
Treg abnormalities have been implicated in the pathogenesis of systemic sclerosis (SSc). Treg subpopulations and their cytokines, IL-10 and TGF-β in the peripheral blood of early stage SSc patients were investigated. We hypothesized that epigenetically regulated methylation of the FOXP3 promoter and enhancer regions are altered in Tregs of SSc patients, which might be involved in the T cell imbalance. CD4+CD25+Foxp3+CD127- Treg cells were significantly elevated in patients with diffuse cutaneous SSc and in patients with anti-Scl-70/RNA-Pol-III autoantibody positivity and with lung fibrosis...
May 15, 2017: Clinical Immunology: the Official Journal of the Clinical Immunology Society
https://www.readbyqxmd.com/read/28522072/cardiovascular-magnetic-resonance-in-systemic-sclerosis-pearls-and-pitfalls
#12
REVIEW
Sophie I Mavrogeni, Juerg Schwitter, Luna Gargani, Alessia Pepe, Lorenzo Monti, Yannick Allanore, Marco Matucci-Cerinic
Systemic sclerosis (SSc) is an autoimmune disease characterized by vascular dysfunction and excessive fibrosis, involving internal organs including the heart. The estimated prevalence of cardiac involvement in SSc is high and remains subclinical until the late stages. It is either primary, related to myocardial inflammation and fibrosis, or secondary, due to pulmonary arterial hypertension (SSc-PAH) or systemic hypertension, in those patients with renal involvement. Cardiovascular magnetic resonance (CMR) is a useful tool for the early assessment of cardiac involvement in SSc...
March 31, 2017: Seminars in Arthritis and Rheumatism
https://www.readbyqxmd.com/read/28516884/patient-participation-in-patient-reported-outcome-instrument-development-in-systemic-sclerosis
#13
REVIEW
John D Pauling, Tracy M Frech, Robyn T Domsic, Marie Hudson
OBJECTIVES: The patient perspective captured using Patient-Reported Outcome (PRO) instruments provide insight into the patient condition not always captured by physician-derived assessment tools. Target patient population involvement is an essential component of PRO instrument development. We have reviewed the level of patient involvement in the development of PRO instruments used in the assessment of systemic sclerosis (SSc). METHODS: A comprehensive literature review was undertaken to identify studies reporting PRO instruments in SSc...
May 2, 2017: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/28516877/validity-of-the-workers-productivity-and-activity-impairment-questionnaire-specific-health-problem-wpai-shp-in-patients-with-systemic-sclerosis
#14
Kathleen Morrisroe, Wendy Stevens, Molla Huq, Joanne Sahhar, Gene-Siew Ngian, Jane Zochling, Janet Roddy, Susanna M Proudman, Mandana Nikpour
OBJECTIVES: To evaluate the construct validity of the Workers Productivity and Impairment Activity Index: Specific Health Problem (WPAI:SHP) in Australian systemic sclerosis (SSc) patients. METHODS: SSc patients, identified through the Australian Scleroderma Cohort Study database, completed the WPAI:SHP and a quality of life instrument (PROMIS-29) cross-sectionally. The construct validity of the WPAI:SHP was assessed by the correlations between the WPAI:SHP and a range of SSc health states...
May 8, 2017: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/28515040/a-systematic-review-of-overlapping-microrna-patterns-in-systemic-sclerosis-and-idiopathic-pulmonary-fibrosis
#15
REVIEW
Gianluca Bagnato, William Neal Roberts, Jesse Roman, Sebastiano Gangemi
Lung fibrosis can be observed in systemic sclerosis and in idiopathic pulmonary fibrosis, two disorders where lung involvement carries a poor prognosis. Although much has been learned about the pathogenesis of these conditions, interventions capable of reversing or, at the very least, halting disease progression are not available. Recent studies point to the potential role of micro messenger RNAs (microRNAs) in cancer and tissue fibrogenesis. MicroRNAs are short non-coding RNA sequences (20-23 nucleotides) that are endogenous, evolutionarily conserved and encoded in the genome...
June 30, 2017: European Respiratory Review: An Official Journal of the European Respiratory Society
https://www.readbyqxmd.com/read/28508950/malignancy-as-a-comorbidity-in-rheumatic-diseases-a-retrospective-hospital-based-study
#16
Hai-Long Wang, Yong-Ming Zhou, Guang-Zhao Zhu, Zhu Yang, Bao-Jin Hua
Patients with Rheumatic diseases (RDs) are at an increased risk of malignancies compared with the general population. The aim of this study was to examine the relative frequency of several cancers in a single homogeneous cohort of patients with different RDs. Patients diagnosed with rheumatoid arthritis (RA), Ankylosing spondylitis (AS), Sjögren's syndrome (SS), systemic lupus erythematosus (SLE), systemic sclerosis (SSc), dermatomyositis (DM), or polymyositis were included. Out of 3982 adult residents admitted to the division of rheumatology, 61 malignancies were observed...
May 16, 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/28507181/fli1-deficiency-induces-cxcl6-expression-in-dermal-fibroblasts-and-endothelial-cells-contributing-to-the-development-of-fibrosis-and-vasculopathy-in-systemic-sclerosis
#17
Takashi Taniguchi, Yoshihide Asano, Kouki Nakamura, Takashi Yamashita, Ryosuke Saigusa, Yohei Ichimura, Takehiro Takahashi, Tetsuo Toyama, Ayumi Yoshizaki, Shinichi Sato
OBJECTIVE: CXCL6, a chemokine with proangiogenic property, is reported to be involved in vasculopathy associated with systemic sclerosis (SSc). We investigated the contribution of CXCL6 to SSc development by focusing on the association of friend leukemia virus integration 1 (Fli1) deficiency, a potential predisposing factor of SSc, with CXCL6 expression and clinical correlation of serum CXCL6 levels. METHODS: mRNA levels of target genes and the binding of Fli1 to the CXCL6 promoter were evaluated by quantitative reverse transcription-PCR and chromatin immunoprecipitation, respectively...
May 15, 2017: Journal of Rheumatology
https://www.readbyqxmd.com/read/28507177/evidence-for-a-derangement-of-the-microvascular-system-in-patients-with-a-very-early-diagnosis-of-systemic-sclerosis
#18
Inês Chora, Eloisa Romano, Mirko Manetti, Celestina Mazzotta, Raquel Costa, Vera Machado, Alice Cortez, Cosimo Bruni, Gemma Lepri, Serena Guiducci, Amato De Paulis, Raquel Soares, Marco Matucci-Cerinic
OBJECTIVE: To investigate whether patients with a very early diagnosis of systemic sclerosis (VEDOSS) may already present circulating markers and in vitro signs of microvascular dysfunction. METHODS: Serum samples were obtained from 55 patients with systemic sclerosis (SSc), 25 patients with VEDOSS, and 55 matched healthy controls (HC). Serum levels of pan-vascular endothelial growth factor (VEGF) and soluble neuropilin-1 (sNRP-1) were measured by ELISA. Human dermal microvascular endothelial cells (H-MVEC) were cultured and stimulated with SSc, VEDOSS, and HC sera...
May 15, 2017: Journal of Rheumatology
https://www.readbyqxmd.com/read/28506627/rxrb-is-a-mhc-encoded-susceptibility-gene-associated-with-anti-topoisomerase-i-antibody-positive-systemic-sclerosis
#19
Akira Oka, Yoshihide Asano, Minoru Hasegawa, Manabu Fujimoto, Osamu Ishikawa, Masataka Kuwana, Yasushi Kawaguchi, Toshiyuki Yamamoto, Hiroki Takahashi, Daisuke Goto, Hirahito Endo, Masatoshi Jinnin, Shuhei Mano, Kazuyoshi Hosomichi, Tomotaka Mabuchi, Mahoko Takahashi Ueda, So Nakagawa, Stephan Beck, Seiamak Bahram, Kazuhiko Takehara, Shinichi Sato, Hironobu Ihn
Systemic sclerosis (SSc) is a systemic autoimmune and connective tissue disorder associated with the human leukocyte antigen (HLA) locus. However, the functional relationship between HLA gene(s) and disease development remains unknown. To elucidate major histocompatibility complex (MHC)-linked SSc genetics, we performed genotyping of MHC-borne microsatellites and HLA-DPB1 alleles using DNA obtained from 318 anti-topoisomerase I antibody-positive patients and 561 healthy controls, all of Japanese descent. Those results revealed 2 MHC haplotypes associated with SSc...
May 12, 2017: Journal of Investigative Dermatology
https://www.readbyqxmd.com/read/28505891/modelling-sediment-fluxes-in-the-danube-river-basin-with-swat
#20
Olga Vigiak, Anna Malagó, Fayçal Bouraoui, Matthias Vanmaercke, Florin Obreja, Jean Poesen, Helmut Habersack, János Fehér, Samo Grošelj
Sediment management is of prior concern in the Danube Basin for provision of economic and environmental services. This study aimed at assessing current (1995-2009) sediment fluxes of the Danube Basin with SWAT model and identifying sediment budget knowledge gaps. After hydrologic calibration, hillslope gross erosion and sediment yields were broadly calibrated using ancillary data (measurements in plots and small catchments, and national and European erosion maps). Mean annual sediment concentrations (SSC) from 269 gauging stations (2968 station-year entries; median 19mg/L, interquartile range IQR 10-36mg/L) were used for calibrating in-stream sediments...
May 11, 2017: Science of the Total Environment
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