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macrophagic activation syndrome

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https://www.readbyqxmd.com/read/29243243/shikonin-inhibits-myeloid-differentiation-protein-2-to-prevent-lipopolysaccharide-induced-acute-lung-injury
#1
Yali Zhang, Tingting Xu, Zheer Pan, Xiangting Ge, Chuchu Sun, Chun Lu, Hongjin Chen, Zhongxiang Xiao, Bing Zhang, Yuanrong Dai, Guang Liang
BACKGROUND AND PURPOSE: Acute lung injury (ALI) is a challenging clinical syndrome which manifests as an acute inflammatory response. Myeloid differentiation protein 2 (MD2) has an important role in mediating lipopolysaccharide (LPS)-induced inflammation. Currently, there are no effective molecular-based therapies for ALI or viable biomarkers for predicting the severity of disease. Recent preclinical studies have shown that shikonin (SHI), a natural naphthoquinone, prevents LPS-inflammation...
December 15, 2017: British Journal of Pharmacology
https://www.readbyqxmd.com/read/29241877/adult-onset-still-s-disease
#2
REVIEW
Javier Narváez
Adult onset Still's disease is a rare systemic condition at the crossroads between auto-inflammatory syndromes and autoimmune diseases, with considerable heterogeneity in terms of clinical presentation, evolution and severity. This article reviews the main advances and lesser known aspects of this entity related to its clinical spectrum (atypical cutaneous lesions, unusual manifestations, macrophage activation syndrome, disease phenotypes), the emerging controversy around its association with delayed malignancy, the search for new biomarkers for its diagnosis, evaluation of prognosis (clinical factors, prognostic indexes and biomarkers to identify patients at risk of severe organ failure or life-threatening complications), and the determinants in the choice of biological treatment...
December 11, 2017: Medicina Clínica
https://www.readbyqxmd.com/read/29233022/reduced-inflammatory-response-by-transcatheter-as-compared-to-surgical-aortic-valve-replacement
#3
Karoline K H Fiane, Gry Dahle, Bjørn Bendz, Per Steinar Halvorsen, Michael Abdelnoor, Tom Eirik Mollnes, Erik Fosse
OBJECTIVES: The inflammatory response to on-pump cardiac surgery is well known. Systemic inflammatory response syndrome after transcatheter valve implantation (TAVI) has been reported. The objective of this study was to study the inflammatory response during TAVI, and compare with the response during surgical aortic valve replacement. METHODS: Eighteen patients undergoing transcatheter implantation, either by a transfemoral (n = 9) or transaortal (n = 9) approach were compared with eighteen patients admitted for surgical replacement...
December 12, 2017: Scandinavian Cardiovascular Journal: SCJ
https://www.readbyqxmd.com/read/29229270/intratracheal-instillation-of-alveolar-type-ii-cells-enhances-recovery-from-acute-lung-injury-in-rats
#4
Raquel Guillamat-Prats, Ferranda Puig, Marta Camprubí-Rimblas, Raquel Herrero, Anna Serrano-Mollar, Maria Nieves Gómez, Jessica Tijero, Michael A Matthay, Lluís Blanch, Antonio Artigas
BACKGROUND: Acute lung injury (ALI) and acute respiratory distress syndrome (ARDS) are characterized by excess production of inflammatory factors. Alveolar type II (ATII) cells help repair damaged lung tissue, rapidly proliferating and differentiating into alveolar type I cells after epithelial cell injury. In ALI, the lack of viable ATII favors progression to more severe lung injury. ATII cells regulate the immune response by synthesizing surfactant and other anti-inflammatory proteins and lipids...
November 8, 2017: Journal of Heart and Lung Transplantation
https://www.readbyqxmd.com/read/29228063/what-matters-in-chronic-burkholderia-cenocepacia-infection-in-cystic-fibrosis-insights-from-comparative-genomics
#5
Jaroslav Nunvar, Vaclav Capek, Karel Fiser, Libor Fila, Pavel Drevinek
Burkholderia cenocepacia causes severe pulmonary infections in cystic fibrosis (CF) patients. Since the bacterium is virtually untreatable by antibiotics, chronic infections persist for years and might develop into fatal septic pneumonia (cepacia syndrome, CS). To devise new strategies to combat chronic B. cenocepacia infections, it is essential to obtain comprehensive knowledge about their pathogenesis. We conducted a comparative genomic analysis of 32 Czech isolates of epidemic clone B. cenocepacia ST32 isolated from various stages of chronic infection in 8 CF patients...
December 11, 2017: PLoS Pathogens
https://www.readbyqxmd.com/read/29227812/overexpression-of-arginase-1-is-linked-to-dnmt3a-and-tet2-mutations-in-lower-grade-myelodysplastic-syndromes-and-chronic-myelomonocytic-leukemia
#6
A H Cull, D Mahendru, B Snetsinger, D Good, K Tyryshkin, A Chesney, Z Ghorab, M Reis, R Buckstein, R A Wells, M J Rauh
Immune dysregulation is a common feature of myelodysplastic syndromes (MDS) and chronic myelomonocytic leukemia (CMML), particularly in early stages. However, the genetic basis remains poorly understood. We recently reported that macrophages from mice deficient in tet methylcytosine dioxygenase 2 (Tet2), a model of MDS/CMML, are hyperinflammatory and have increased expression of arginase 1 (Arg1). In macrophages and myeloid derived suppressor cells (MDSCs) expression of Arg1 contributes to T-cell suppression and immune evasion by L-arginine depletion, in the setting of chronic inflammation and cancer...
December 6, 2017: Leukemia Research
https://www.readbyqxmd.com/read/29209549/macrophage-activation-syndrome-a-report-of-two-cases-and-a-literature-review
#7
Asaad Alkoht, Ibrahem Hanafi, Basheer Khalil
Macrophage activation syndrome (MAS) is a severe, potentially fatal condition that may complicate autoimmune diseases, and it belongs to hemophagocytic lymphohistiocytosis (HLH) disorders. MAS occurs in adults and children. However, it is rare in juvenile systemic lupus erythematosus (jSLE), and it is extremely rare to be the initial presentation of jSLE. Here, we report two patients with juvenile SLE who initially presented with MAS. One of the two patients is 4 years old. This is the youngest reported patient to our knowledge...
2017: Case Reports in Rheumatology
https://www.readbyqxmd.com/read/29207675/thio-barbiturate-derived-compounds-are-novel-antioxidants-to-prevent-lps-induced-inflammation-in-the-liver
#8
Kyoung Mi Moon, Bonggi Lee, Ji Won Jeong, Dae Hyun Kim, Yun Jung Park, Hye Rim Kim, Ji Young Park, Min Jo Kim, Hye Jin An, Eun Kyeong Lee, Young Mi Ha, Eunok Im, Pusoon Chun, Jin Yeul Ma, Won-Kyung Cho, Hyung Ryong Moon, Hae Young Chung
Liver inflammation is closely associated with metabolic syndrome. Oxidative stress plays a synergistic role in inflammation by activating nuclear factor kappa B (NF-κB) signaling in the liver. Therefore, substantial efforts have been made to develop compounds that inhibit the generation of oxidative stress and activation of NF-κB. We synthesized twenty-six novel 5-(substituted benzyl)-2-oxo- and 5-(substituted benzyl)-2-thioxo-dihydropyrimidine-4,6(1H,5H)-dione derivatives for the development of potential antioxidants and examined their biological activities in vitro and in vivo...
October 31, 2017: Oncotarget
https://www.readbyqxmd.com/read/29203639/a-knock-in-tristetraprolin-ttp-zinc-finger-point-mutation-in-mice-comparison-with-complete-ttp-deficiency
#9
Wi S Lai, Deborah J Stumpo, Lianqun Qiu, Roberta Faccio, Perry J Blackshear
Tristetraprolin (TTP) is a tandem CCCH zinc finger protein that can bind to AU-rich element-containing mRNAs and promote their decay. TTP knockout mice develop a severe inflammatory syndrome, largely due to excess tumor necrosis factor (TNF), whose mRNA is a direct target of TTP binding and destabilization. TTP's RNA binding activity, and its ability to promote mRNA decay, are lost when one of the zinc-coordinating residues of either zinc finger is mutated. To address several long-standing questions about TTP activity in intact animals, we developed a knock-in mouse with a cysteine to arginine mutation within the first zinc finger...
December 4, 2017: Molecular and Cellular Biology
https://www.readbyqxmd.com/read/29197110/macrophage-activation-syndrome-and-post-transplant-microangiopathy-following-haploidentical-bone-marrow-transplantation-for-sickle-cell-anemia
#10
James O J Davies, Alice C Hart, Josu De La Fuente, Barbara J Bain
No abstract text is available yet for this article.
December 2, 2017: American Journal of Hematology
https://www.readbyqxmd.com/read/29195002/validation-of-classification-criteria-of-macrophage-activation-syndrome-in-japanese-patients-with-systemic-juvenile-idiopathic-arthritis
#11
Masaki Shimizu, Mao Mizuta, Takahiro Yasumi, Naomi Iwata, Yuka Okura, Noriko Kinjo, Hiroaki Umebayashi, Tomohiro Kubota, Yasuo Nakagishi, Kenichi Nishimura, Masato Yashiro, Junko Yasumura, Kazuko Yamazaki, Hiroyuki Wakiguchi, Nami Okamoto, Masaaki Mori
OBJECTIVE: To validate whether the 2016 ACR/EULAR classification criteria of macrophage activation syndrome (MAS) complicating systemic juvenile idiopathic arthritis (s-JIA) is practical in the real world. METHODS: A combination of expert consensus and analysis of real patient data was conducted by a panel of 15 paediatric rheumatologists. Sixty five profiles comprised 18 patients with s-JIA-associated MAS and 47 patients with active s-JIA without evidence of MAS...
December 1, 2017: Arthritis Care & Research
https://www.readbyqxmd.com/read/29184458/update-on-the-management-of-systemic-juvenile-idiopathic-arthritis-and-role-of-il-1-and-il-6-inhibition
#12
REVIEW
Sriharsha Grevich, Susan Shenoi
Systemic juvenile idiopathic arthritis (SJIA) is a disease marked with arthritis and several features of systemic inflammation including fevers, rashes, hepatosplenomegaly, lymphadenopathy, and serositis. The presentation can be variable and arthritis can be a later feature. Macrophage activation syndrome can be a life-threatening complication of this illness and requires early recognition and prompt therapy. Advancements in understanding the biology of SJIA have led to the development of cytokine-targeted therapies, mainly interleukin-1 (IL-1) and IL-6 inhibitors that have significantly improved outcomes...
2017: Adolescent Health, Medicine and Therapeutics
https://www.readbyqxmd.com/read/29179879/p2x4-a-fast-and-sensitive-purinergic-receptor
#13
REVIEW
Jaanus Suurväli, Pierre Boudinot, Jean Kanellopoulos, Sirje Rüütel Boudinot
Extracellular nucleotides have been recognized as important mediators of activation, triggering multiple responses via plasma membrane receptors known as P2 receptors. P2 receptors comprise P2X ionotropic receptors and G protein-coupled P2Y receptors. P2X receptors are expressed in many tissues, where they are involved in a number of functions including synaptic transmission, muscle contraction, platelet aggregation, inflammation, macrophage activation, differentiation and proliferation, neuropathic and inflammatory pain...
October 2017: Biomedical Journal
https://www.readbyqxmd.com/read/29178573/macrophages-common-culprit-in-obesity-and-asthma
#14
REVIEW
Nikunj Sharma, Mustafa Akkoyunlu, Ronald L Rabin
Macrophages are essential innate immune cells that also regulate local metabolism. Endogenous or exogenous stimuli may polarize macrophages towards phenotypes that serve distinct innate immunological metabolic functions. IFNγ or lipopolysaccharide (LPS) polarizes macrophages towards the M1, or "classically activated" phenotype that participates in defense against intracellular pathogens. IL-4, IL-13, or chitin polarizes macrophages towards the M2, or "alternatively activated" phenotype, which defends against multicellular nematodes and fungi...
November 27, 2017: Allergy
https://www.readbyqxmd.com/read/29177911/mechanisms-of-sustained-neutrophilia-in-patient-whim-09-cured-of-whim-syndrome-by-chromothripsis
#15
Qian Liu, Zhanzhuo Li, Alexander Y Yang, Ji-Liang Gao, Daniel S Velez, Elena J Cho, David H McDermott, Philip M Murphy
WHIM-09 is the first patient described with WHIM syndrome, an autosomal dominant form of neutropenia related to bone marrow retention of neutrophils. Originally diagnosed incorrectly with autoimmune neutropenia, the patient underwent splenectomy at age 9, but the absolute neutrophil count (ANC) did not rise. Subsequently, she was spontaneously cured by chromothripsis (chromosome shattering), which deleted the disease allele CXCR4 R334X , and 163 other genes, on chromosome 2 in a single hematopoietic stem cell (HSC)...
November 24, 2017: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/29149705/toll-like-receptor-4-deficiency-increases-resistance-in-sepsis-induced-immune-dysfunction
#16
Chao Cao, Yanfen Chai, Songtao Shou, Jun Wang, Ying Huang, Tao Ma
Sepsis constitutes a serious life-threatening syndrome associated with complications of deregulated inflammatory response against endotoxin/lipopolysaccharide (LPS)-mediated severe infection. Toll-like receptor 4 (TLR4) plays a critical role in the activation of innate immunity through recognition of LPS. However, the impact of TLR4 signaling on the development of sepsis-induced immune dysfunction remains unclear. The aim of this study was to investigate the effect of TLR4 on regulatory T cells (Tregs) and its potential mechanism...
November 14, 2017: International Immunopharmacology
https://www.readbyqxmd.com/read/29140265/anti-inflammatory-potential-of-1-nitro-2-phenylethylene
#17
Michelle A Sugimoto, Márcia de Jesus Amazonas da Silva, Larissa Froede Brito, Rosivaldo Dos Santos Borges, Flávio Almeida Amaral, Ana Paula de Araujo Boleti, Maritza Echevarria Ordoñez, Jose Carlos Tavares, Lirlandia Pires Sousa, Emerson Silva Lima
Inflammation is a reaction of the host to infectious or sterile stimuli and has the physiological purpose of restoring tissue homeostasis. However, uncontrolled or unresolved inflammation can lead to tissue damage, giving rise to a plethora of chronic inflammatory diseases, including metabolic syndrome and autoimmunity pathologies with eventual loss of organ function. Beta-nitrostyrene and its derivatives are known to have several biological activities, including anti-edema, vasorelaxant, antiplatelet, anti-inflammatory, and anticancer...
November 15, 2017: Molecules: a Journal of Synthetic Chemistry and Natural Product Chemistry
https://www.readbyqxmd.com/read/29132140/mitochondria-targeted-peptide-attenuates-mitochondrial-dysfunction-controls-inflammation-and-protects-against-spinal-cord-injury-induced-lung-injury
#18
Liu-Long Zhu, Mao-Qiang Li, Fan He, Shao-Bo Zhou, Wu Jiang
BACKGROUND/AIMS: Spinal cord injury (SCI) is a common and devastating disease, which results in systemic inflammatory response syndrome and secondary lung injury. Mitochondrial dysfunction and inflammation are closely related to lung injury in diverse disease models. No studies have demonstrated the effects of mitochondrial targeted peptide SS-31 in a mouse model of SCI-induced lung injury. METHODS: Immediately after injury, mice in the treatment groups received a daily, single-dose intraperitoneal injection of SS-31 and for the next 2 days...
November 13, 2017: Cellular Physiology and Biochemistry
https://www.readbyqxmd.com/read/29124251/trail-deletion-prevents-liver-but-not-adipose-tissue-inflammation-during-murine-diet-induced-obesity
#19
Petra Hirsova, Peggy Weng, Warda Salim, Steven F Bronk, Thomas S Griffith, Samar H Ibrahim, Gregory J Gores
Background & Aim: TNF-related apoptosis-inducing ligand (TRAIL) and its cognate receptor(s) are upregulated in human and murine nonalcoholic steatohepatitis (NASH). However, the consequence of this enhanced expression on NASH pathogenesis remains unclear. TRAIL may either accentuate liver injury by promoting hepatic steatosis and inflammation, or it may mitigate the disease process by improving systemic insulin resistance and averting hepatic fibrosis. Herein, we investigated the role of TRAIL in an obesity-induced murine model of NASH...
September 2017: Hepatology Communications
https://www.readbyqxmd.com/read/29106401/irf3-and-type-i-interferons-fuel-a-fatal-response-to-myocardial-infarction
#20
Kevin R King, Aaron D Aguirre, Yu-Xiang Ye, Yuan Sun, Jason D Roh, Richard P Ng, Rainer H Kohler, Sean P Arlauckas, Yoshiko Iwamoto, Andrej Savol, Ruslan I Sadreyev, Mark Kelly, Timothy P Fitzgibbons, Katherine A Fitzgerald, Timothy Mitchison, Peter Libby, Matthias Nahrendorf, Ralph Weissleder
Interferon regulatory factor 3 (IRF3) and type I interferons (IFNs) protect against infections and cancer, but excessive IRF3 activation and type I IFN production cause autoinflammatory conditions such as Aicardi-Goutières syndrome and STING-associated vasculopathy of infancy (SAVI). Myocardial infarction (MI) elicits inflammation, but the dominant molecular drivers of MI-associated inflammation remain unclear. Here we show that ischemic cell death and uptake of cell debris by macrophages in the heart fuel a fatal response to MI by activating IRF3 and type I IFN production...
November 6, 2017: Nature Medicine
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