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https://www.readbyqxmd.com/read/28220405/altered-cellular-homeostasis-in-murine-mps-i-fibroblasts-evidence-of-cell-specific-physiopathology
#1
Gustavo Monteiro Viana, Cinthia Castro do Nascimento, Edgar Julian Paredes-Gamero, Vânia D'Almeida
Mucopolysaccharidosis type I (MPS I), a rare autosomal recessive disease, is caused by a deficiency of the lysosomal enzyme alfa-L-iduronidase. Impaired enzyme activity promotes glycosaminoglycans accumulation in several tissues and organs, leading to complex multisystemic complications. Several studies using animal models indicated different intracellular pathways involving MPS I physiopathology; however, the exact mechanisms underlying this syndrome are still not understood. Previous results from our group showed alterations in ionic homeostasis and cell viability of splenocytes and macrophages in Idua-/- mice...
February 21, 2017: JIMD Reports
https://www.readbyqxmd.com/read/28215617/genetic-engineering-alveolar-macrophages-for-host-resistance-to-prrsv
#2
REVIEW
Randall S Prather, Kristin M Whitworth, Susan K Schommer, Kevin D Wells
Standard strategies for control of porcine reproductive and respiratory syndrome virus (PRRSV) have not been effective, as vaccines have not reduced the prevalence of disease and many producers depopulate after an outbreak. Another method of control would be to prevent the virus from infecting the pig. The virus was thought to infect alveolar macrophages by interaction with a variety of cell surface molecules. One popular model had PRRSV first interacting with heparin sulfate followed by binding to sialoadhesin and then being internalized into an endosome...
February 10, 2017: Veterinary Microbiology
https://www.readbyqxmd.com/read/28215151/iron-chelators-dictate-immune-cells-inflammatory-ability-potential-adjuvant-therapy-for-ibd
#3
Marcello Chieppa, Vanessa Galleggiante, Grazia Serino, Monica Massaro, Angelo Santino
BACKGROUND: The importance of hemoproteins for life lies largely in their iron-mediated chemical properties. In the human body, there are about 4 g of iron, a precious resource preserved by sophisticated recycling mechanisms. Iron is also important for pathogen growth, so it is not surprising that immune cells developed mechanisms to reduce iron availability in cases of inflammation. In healthy conditions, macrophages degrade hemoproteins and export iron, while if inflammation develops, they retain cytoplasmic iron to reduce extracellular iron concentrations...
February 15, 2017: Current Pharmaceutical Design
https://www.readbyqxmd.com/read/28207870/impaired-hdl2-mediated-cholesterol-efflux-is-associated-with-metabolic-syndrome-in-families-with-early-onset-coronary-heart-disease-and-low-hdl-cholesterol-level
#4
Timo Paavola, Sanna Kuusisto, Matti Jauhiainen, Sakari Kakko, Tiia Kangas-Kontio, Jari Metso, Pasi Soininen, Mika Ala-Korpela, Risto Bloigu, Minna L Hannuksela, Markku J Savolainen, Tuire Salonurmi
OBJECTIVE: The potential of high-density lipoproteins (HDL) to facilitate cholesterol removal from arterial foam cells is a key function of HDL. We studied whether cholesterol efflux to serum and HDL subfractions is impaired in subjects with early coronary heart disease (CHD) or metabolic syndrome (MetS) in families where a low HDL-cholesterol level (HDL-C) predisposes to early CHD. METHODS: HDL subfractions were isolated from plasma by sequential ultracentrifugation...
2017: PloS One
https://www.readbyqxmd.com/read/28205524/interleukin-33-produced-by-m2-macrophages-and-other-immune-cells-contributes-to-th2-immune-reaction-of-igg4-related-disease
#5
Sachiko Furukawa, Masafumi Moriyama, Kensuke Miyake, Hitoshi Nakashima, Akihiko Tanaka, Takashi Maehara, Mana Iizuka-Koga, Hiroto Tsuboi, Jun-Nosuke Hayashida, Noriko Ishiguro, Masaki Yamauchi, Takayuki Sumida, Seiji Nakamura
IgG4-related disease (IgG4-RD) is characterized by elevated serum IgG4 and marked infiltration of IgG4-positive cells in multiple organs. Interleukin-33 (IL-33) is a recently described cytokine that is secreted by damaged epithelial cells, macrophages, and dendritic cells, and potently activates helper T type 2 (Th2) immune responses, which have been suggested to play a major role in IgG4 production of IgG4-RD. Here, we assessed the expression of IL-33 and related molecules in the salivary glands (SGs) of patients with IgG4-RD versus that in patients with Sjögren's syndrome (SS) and controls...
February 13, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28204893/gastrointestinal-system-manifestations-in-juvenile-systemic-lupus-erythematosus
#6
Hafize Emine Sönmez, Asuman Nur Karhan, Ezgi Deniz Batu, Yelda Bilginer, Ersin Gümüş, Hülya Demir, Aysel Yüce, Seza Özen
Systemic lupus erythematosus (SLE) is an autoimmune disease which may involve gastrointestinal system (GIS). The aim of this study was to present GIS manifestations of pediatric SLE patients. The medical files of 69 children with SLE followed between January 2011 and January 2016 were reviewed. All fulfilled the Systemic Lupus International Collaborating Clinics criteria. All patients (≤18 years of age) with GIS manifestations were included. GIS manifestations were observed in 19 (27.5%) out of 69 SLE patients and present at the time of SLE diagnosis in 13 (68...
February 16, 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/28204878/unusual-onset-of-adult-still-s-disease-due-to-a%C3%A2-systemic-reaction-to-artificial-breast-implants
#7
A Wehr, G Grieb, C Trautwein, K Streetz
In this manuscript, we report an exceptional observation study of a young woman suffering from an autoimmune syndrome induced by adjuvants (ASIA). Until today only seven cases of adult-onset Still's disease (AOSD) induced by breast implants have been published. This may be due to the fact that this illness itself is very rare; however, the reason might also be that the community is not sensitized to the case-specific symptoms. Within this article, we show for the first time highly detailed diagnostic test procedures such as PET-CT scans and specific histological staining of the breast tissue, displaying proinflammatory macrophages that are a well-known activator and booster of autoimmune diseases...
February 15, 2017: Zeitschrift Für Rheumatologie
https://www.readbyqxmd.com/read/28202546/identification-of-a-novel-series-of-anti-inflammatory-and-anti-oxidative-phospholipid-oxidation-products-containing-cyclopentenone-moiety-in-vitro-and-in-vivo-implication-in-atherosclerosis
#8
Jianhong Lu, Shuyuan Guo, Xinli Xue, Qun Chen, Jing Ge, Yujuan Zhuo, Huiqin Zhong, Buxing Chen, Mingming Zhao, Wei Han, Takashi Suzuki, Mingjiang Zhu, Lin Xia, Claus Schneider, Timothy S Blackwell, Ned A Porter, Lemin Zheng, Sotirios Tsimikas, Huiyong Yin
Oxidative stress and inflammation are two major contributing factors to atherosclerosis, a leading cause for cardiovascular diseases (CVDs). Oxidation of phospholipids on the surface of low density lipoprotein (LDL) particles generated under oxidative stress has been associated with the progression of atherosclerosis but the underlying molecular mechanisms remain poorly defined. We identified a novel series of oxidation products containing cyclopentenone moiety, termed deoxy-A2/J2-isoprostanes-phosphocholine (deoxy-A2/J2-IsoP-PC), from 1-palmitoyl-2-arachidonoyl-sn-glycero-3-phosphocholine (PAPC) in vivo using mass spectrometry and by comparison to a chemically synthesized standard...
February 15, 2017: Journal of Biological Chemistry
https://www.readbyqxmd.com/read/28202118/-clinical-and-laboratory-features-of-macrophage-activation-syndrome
#9
Li Guo, Mei-Ping Lu, Gui-Juan Dong, Li-Ping Teng, Yi-Ping Xu, Li-Xia Zou, Qi Zheng
OBJECTIVE: To study the clinical and laboratory features of macrophage activation syndrome (MAS) at the early stage of diagnosis, and to explore a method for early identification of MAS. METHODS: A retrospective analysis was performed for the demographic data, clinical and laboratory features, and treatment outcomes of 21 MAS patients. RESULTS: Of the 21 MAS patients, 14 had systemic juvenile idiopathic arthritis, 5 had Kawasaki disease (KD), and 2 had connective tissue disease (CTD) as primary diseases...
February 2017: Zhongguo Dang Dai Er Ke za Zhi, Chinese Journal of Contemporary Pediatrics
https://www.readbyqxmd.com/read/28196537/characterization-of-a-novel-disease-causing-mutation-in-exon-1-of-sh2d1a-gene-through-amplicon-sequencing-a-case-report-on-hlh
#10
Shiyuan Zhou, Hongyu Ma, Bo Gao, Guangming Fang, Yi Zeng, Qing Zhang, GaoFu Qi
BACKGROUND: Hemophagocytic lymphohistocytosis (HLH) is a rare but fatal hyperinflammatory syndrome caused by uncontrolled proliferation of activated macrophages and T lymphocytes secreting high amounts of inflammatory cytokines. Genetic defect is a common cause of HLH. HLH is complicated to be diagnosed as there are many common symptoms with other disorders. CASE PRESENTATION: Here we report on an HLH case caused by 1 bp deletion in gene SH2D1A. Patient was a 3-years-old boy and had fever for more than 8 days...
February 14, 2017: BMC Medical Genetics
https://www.readbyqxmd.com/read/28194148/animal-models-of-emerging-tick-borne-phleboviruses-determining-target-cells-in-a-lethal-model-of-sftsv-infection
#11
Keita Matsuno, Yasuko Orba, Kimberly Maede-White, Dana Scott, Friederike Feldmann, Mifang Liang, Hideki Ebihara
The pathogenesis of clinical manifestations caused by newly emerging tick-borne phleboviruses [i.e., Severe fever with thrombocytopenia syndrome virus (SFTSV) and Heartland virus (HRTV)], such as severe thrombocytopenia and lymphocytopenia, are not yet fully understood. In the present study, to establish an animal model mimicking the profile of fatal human cases, we examined the susceptibilities of adult mice from 12 strains, aged mice from two strains, and cynomolgus macaques to SFTSV and/or HRTV infections...
2017: Frontiers in Microbiology
https://www.readbyqxmd.com/read/28194019/il-25-stimulates-m2-macrophage-polarization-and-thereby-promotes-mitochondrial-respiratory-capacity-and-lipolysis-in-adipose-tissues-against-obesity
#12
Juan Feng, Lingyi Li, Zhiying Ou, Qiao Li, Baoyong Gong, Zhenxian Zhao, Weiwei Qi, Ti Zhou, Jun Zhong, Weibin Cai, Xia Yang, Aiping Zhao, Guoquan Gao, Zhonghan Yang
Obesity and associated metabolic diseases are characterized by a chronic low-grade inflammatory state with the infiltration of many inflammatory cells, especially macrophages. Immune molecules, including some cytokines, have a close relationship with metabolism. Interleukin (IL)-25 is a member of the IL-17 cytokine family that can regulate macrophages and alleviate some metabolic dysfunction; however, its role and mechanisms in lipid metabolism remain to be extensively clarified. Human serum and liver biopsy specimens, high-fat diet-induced obesity mice and DB/DB (Lepr-/-) animal models were used to examine IL-25 expression in obesity and nonalcoholic fatty liver diseases (NAFLD)...
February 13, 2017: Cellular & Molecular Immunology
https://www.readbyqxmd.com/read/28192889/effects-of-aristolochic-acid-i-and-or-hypokalemia-on-tubular-damage-in-c57bl-6-rat-with-aristolochic-acid-nephropathy
#13
Joo-Hark Yi, Sang-Woong Han, Wan-Young Kim, Jin Kim, Moon-Hyang Park
Background/Aims: This study was designed to investigate the roles of aristolochic acid I (AA-I) and hypokalemia in acute aristolochic acid nephropathy (AAN). Methods: After an adaptation period (1 week), a total of 40 C57BL/6 mice (male, 8 weeks old) were divided into four groups: I (control group), II (low potassium [K] diet), III (normal K diet with administration of AA-I [10 mg/kg weight]), and IV (low K diet with AA-I). After collecting 24 hours of urine at 2 weeks, the mice were sacrificed, and their blood and kidneys were obtained to perform immunochemical staining and/or Western blot analysis...
February 15, 2017: Korean Journal of Internal Medicine
https://www.readbyqxmd.com/read/28187313/il-20-contributes-to-low-grade-inflammation-and-weight-gain-in-the-psammomys-obesus
#14
Helena Cucak, Lise Høj Thomsen, Alexander Rosendahl
The metabolic syndrome has been demonstrated in gene deficient animals, e.g. db/db mice, to include a systemic inflammation leading to insulin resistance, obesity and type 2 diabetes (T2D). To determine the importance of inflammation in obesity and diabetes, in a normal non-genetically modified species, an intervention study with neutralizing anti-IL-20 antibodies was conducted in the spontaneous T2D model Psammomys obesus. All IL-20 receptor chains were expressed on protein level in the Psammomys obesus. Neutralization of IL-20 did not modulate blood glucose, HbA1c, insulin levels or lymphocyte numbers after five weeks treatment although a trend to reduced weight gain rate was observed upon anti-IL-20 treatment...
February 7, 2017: International Immunopharmacology
https://www.readbyqxmd.com/read/28182362/upregulation-of-foxo-1-signaling-mediates-the-proinflammatory-cytokine-upregulation-in-the-macrophage-from-polycystic-ovary-syndrome-patients
#15
Ning Li, Xiaoyan Wang, Xiaojie Wang, Hongna Yu, Li Lin, Chengming Sun, Peng Liu, Yongli Chu, Jianqing Hou
BACKGROUND: Chronic activation of macrophage-mediated inflammatory signals in insulin-sensitive metabolic tissues is thought to be one of the causes of insulin resistance-one of the hallmarks of the metabolic syndrome. Insulin resistance is a feature of polycystic ovary syndrome (PCOS) and is related to mitochondrial and endothelial function. METHODS: In the present study, we investigated the phosphorylation level of FoxO 1, which is suppressed by the action of AKT, triggers the TLR4 inflammatory signaling pathway in the macrophages, from polycystic ovary syndrome patients or normal subjects...
February 1, 2017: Clinical Laboratory
https://www.readbyqxmd.com/read/28178439/juvenile-idiopathic-arthritis-in-the-era-of-international-cooperation
#16
REVIEW
Yosef Uziel
Juvenile idiopathic arthritis (JIA) is the most common chronic disease of childhood. Improved understanding of its pathogenesis has led to international cooperation in clinical studies. Multicenter, international collaborations and research facilitate rapid enrollment of enough patients to enable a variety of studies, including those of epidemiology, diagnostic and classification criteria, genetic disease predisposition, pathogenesis, outcomes, and treatment protocols. In the last 20 years, the vision of the Pediatric Rheumatology International Trial Organization (PRINTO) has become a reality of worldwide collaboration in pediatric rheumatology research, including North American and European research groups...
January 30, 2017: Rambam Maimonides Medical Journal
https://www.readbyqxmd.com/read/28176791/il-36%C3%AE-is-a-crucial-proximal-component-of-protective-type-1-mediated-lung-mucosal-immunity-in-gram-positive-and-negative-bacterial-pneumonia
#17
M A Kovach, B Singer, G Martinez-Colon, M W Newstead, X Zeng, P Mancuso, T A Moore, S L Kunkel, M Peters-Golden, B B Moore, T J Standiford
Interleukin-36γ (IL-36γ) is a member of novel IL-1-like proinflammatory cytokine family that are highly expressed in epithelial tissues and several myeloid-derived cell types. Little is known about the role of the IL-36 family in mucosal immunity, including lung anti-bacterial responses. We used murine models of IL-36γ deficiency to assess the contribution of IL-36γ in the lung during experimental pneumonia. Induction of IL-36γ was observed in the lung in response to Streptococcus pneumoniae (Sp) infection, and mature IL-36γ protein was secreted primarily in microparticles...
February 8, 2017: Mucosal Immunology
https://www.readbyqxmd.com/read/28171221/the-significance-of-electron-microscopic-examination-of-gingiva-in-cases-of-hunter-syndrome-and-hereditary-gingival-fibromatosis
#18
(no author information available yet)
INTRODUCTION: Electron microscopy has been for decades a basic morphological method still used in diagnostic protocols of some pathological conditions affecting the ultrastructure of cells and extracellular matrix. The aim of this study was an ultrastructural description of gingiva of patients with Hunter syndrome and hereditary gingival fibromatosis. PATIENTS AND METHODS: Gingival biopsies were obtained during surgical periodontal treatment from a 9-year-old boy with Hunter disease (with enzyme replacement therapy with recombinant human idursulphase) and a 15-year-old girl with hereditary gingival fibromatosis...
October 8, 2016: Neuro Endocrinology Letters
https://www.readbyqxmd.com/read/28161232/-correctly-address-the-cause-of-hemophagocytic-lymphohistiocytosis
#19
M Penel-Page, B Ben Said, A Phan, L Hees, C Hartmann-Merlin, S Girard, Y Gillet, A Belot
Hemophagocytic lymphohistiocytosis (HLH) is a rare and severe syndrome usually associated with a cytotoxicity deficiency, which leads to an excess of immune response driven by activated macrophages and cytotoxic T cells. In children, HLH can be genetic, as part of a familial lymphohistiocytosis, or secondary: the most frequent causes are systemic-onset juvenile idiopathic arthritis, hematological malignancies, and severe infections, especially with Ebstein-Barr virus or leishmaniosis. We report on the case of a 3-year-old girl with no past medical history, who presented inaugural Pseudomonas aeruginosa maxillary osteitis, with secondary HLH...
February 1, 2017: Archives de Pédiatrie: Organe Officiel de la Sociéte Française de Pédiatrie
https://www.readbyqxmd.com/read/28161213/new-perspective-of-host-micrornas-in-the-control-of-prrsv-infection
#20
REVIEW
Fang Liu, Yinping Du, Wen-Hai Feng
Porcine reproductive and respiratory syndrome (PRRS) is one of the most important diseases in pigs, leading to significant economic losses in swine industry worldwide. PRRS virus (PRRSV) is an enveloped positive single-stranded RNA virus, which mainly infects cells of monocyte/macrophage lineage. MicroRNAs (miRNAs) are small non-coding RNAs and have emerged as important regulators of virus-host cell interactions. In the past several years, scientists have been trying to understand the interaction between host miRNAs and PRRSV infection...
January 9, 2017: Veterinary Microbiology
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