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https://www.readbyqxmd.com/read/29659973/the-diagnostic-challenge-of-acquired-thrombotic-thrombocytopenic-purpura-in-children-case-report-and-review-of-the-literature
#1
Elise Schapkaitz, Tanya Marié Schickerling
Acquired thrombotic thrombocytopenic purpura (TTP) is a rare disease in children. Despite advances in diagnosis and treatment, acquired TTP remains a challenging disease due to the lack of a simple diagnostic test and the variable response to plasma exchange. Herein, we describe a case of a 5-year-old of black ethnicity boy who presented with a sudden onset of fatigue and body aches. Laboratory investigations revealed a thrombotic microangiopathic manifestation. TTP was diagnosed, and plasma exchange and corticosteroids were initiated, with an excellent response...
April 12, 2018: Laboratory Medicine
https://www.readbyqxmd.com/read/29657197/adamts-13-level-in-children-with-severe-diarrhea-associated-hemolytic-uremic-syndrome-unmasking-new-association
#2
Naglaa A Khalifa, Heba H Gawish, Noora A Khalifa, Doaa M Tawfeek, Saed M Morsy
Severe deficiency of ADAMTS-13 leads to thrombotic thrombocytopenic purpura. Few studies have reported reduced activity of ADAMTS-13 in patients with atypical and typical hemolytic uremic syndrome (HUS). We hypothesized that ADAMTS-13 deficiency might play a role in the pathogenesis of severe HUS. This study aimed to evaluate the ADAMTS-13 level in severe typical HUS. This prospective case-control study was carried out in the Pediatric Nephrology Unit and Clinical Pathology Department, Faculty of Medicine, Zagazig University from February 2013 to February 2014...
March 2018: Saudi Journal of Kidney Diseases and Transplantation
https://www.readbyqxmd.com/read/29649084/vertebral-augmentation-can-induce-early-signs-of-degeneration-in-the-adjacent-intervertebral-disc-evidence-from-a-rabbit-model
#3
Zhiyun Feng, Lunhao Chen, Xiaojian Hu, Ge Yang, Yue Wang, Zhong Chen
STUDY DESIGN: An experimental study. OBJECTIVE: The aim of this study was to determine the effect of polymethylmethacrylate (PMMA) augmentation on the adjacent disc. SUMMARY OF BACKGROUND DATA: Vertebral augmentation with PMMA reportedly may predispose the adjacent vertebra to fracture. The influence of PMMA augmentation on the adjacent disc, however, remains unclear. METHODS: Using a retroperitoneal approach, PMMA augmentation was performed for 23 rabbits...
April 11, 2018: Spine
https://www.readbyqxmd.com/read/29618156/adamts-13-activity-predicts-outcome-in-acute-ischaemic-stroke-patients-undergoing-endovascular-treatment
#4
Ramona Schuppner, Meike Dirks, Gerrit M Grosse, Matthias Böckmann, Friedrich Goetz, Thomas Pasedag, Stefanie M Bode-Böger, Jens Martens-Lobenhoffer, Ulrich Budde, Heinrich Lanfermann, Ralf Lichtinghagen, Karin Weissenborn, Hans Worthmann
BACKGROUND:  Endovascular treatment improves outcome in patients with acute ischaemic stroke due to large vessel occlusion in general. But outcome in some of these patients is jeopardized by recanalization failure or bleeding. OBJECTIVES:  This study aimed to determine a possible association of mediators of inflammation and haemostasis (C-reactive protein, interleukin-6, matrix metalloproteinase-9, monocyte chemoattractant protein-1, asymmetric dimethylarginine [ADMA], symmetric dimethylarginine, von Willebrand factor and a disintegrin and metalloproteinase with a thrombospondin type 1 motif 13 [ADAMTS-13]) with the post-intervention grade of reperfusion, complications and clinical outcome in patients who underwent endovascular treatment of ischaemic stroke...
April 2018: Thrombosis and Haemostasis
https://www.readbyqxmd.com/read/29594148/c3-glomerulopathy-and-atypical-hemolytic-uremic-syndrome-two-important-manifestations-of-complement-system-dysfunction
#5
Ravneet Bajwa, John A DePalma, Taimoor Khan, Anmol Cheema, Sheila A Kalathil, Mohammad A Hossain, Attiya Haroon, Anne Madhurima, Min Zheng, Ali Nayer, Arif Asif
The advances in our understanding of the alternative pathway have emphasized that uncontrolled hyperactivity of this pathway causes 2 distinct disorders that adversely impact the kidney. In the so-called atypical hemolytic uremic syndrome (aHUS), renal dysfunction occurs along with thrombocytopenia, anemia, and target organ injury to multiple organs, most commonly the kidney. On the other hand, in the so-termed C3 glomerulopathy, kidney involvement is not associated with thrombocytopenia, anemia, or other system involvement...
January 2018: Case Reports in Nephrology and Dialysis
https://www.readbyqxmd.com/read/29567368/efficacy-of-eculizumab-in-severe-adamts13-deficient-thrombotic-thrombocytopenic-purpura-ttp-refractory-to-standard-therapies
#6
Ernesto Vigna, Annamaria Petrungaro, Anna Perri, Dario Terzi, Anna Grazia Recchia, Francesco Mendicino, Antonella La Russa, Sabrina Bossio, Laura De Stefano, Francesco Zinno, Renzo Bonofiglio, Fortunato Morabito, Massimo Gentile
Thrombotic thrombocytopenic purpura (TTP) is a rare microangiopathic hemolytic anemia (MAHA) defined by mechanical hemolytic anemia, severe thrombocytopenia, and systemic visceral ischemia due to systemic platelet-rich microthrombi. Forty percent of patients with autoimmune TTP experience one or multiple relapses. Patients with refractory TTP are currently managed by corticosteroids, twice-daily PEX, and the anti-CD20 monoclonal antibody rituximab. Herein, we report two cases of severe TTP, refractory to those standard agents...
March 15, 2018: Transfusion and Apheresis Science
https://www.readbyqxmd.com/read/29540226/vasoactive-intestinal-peptide-alleviates-osteoarthritis-effectively-via-inhibiting-nf-%C3%AE%C2%BAb-signaling-pathway
#7
Yaozhong Liang, Shu Chen, Yuhao Yang, Chunhai Lan, Guowei Zhang, Zhisheng Ji, Hongsheng Lin
BACKGROUND: To investigate the treatment effect of vasoactive intestinal peptide (VIP) on osteoarthritis (OA) and the relative mechanism. METHOD: The OA model on the SD rat knee was established using the modified Hulth method, and the recombinant pcDNA3.1+/VIP plasmid was constructed. One month after the plasmids VIP were injected intra-articularly into the right knee joint of OA and sham-operated rats, the pathological changes of the OA knee joint were observed by Hematoxylin-eosin (HE) and Safranin O/fast green staining...
March 14, 2018: Journal of Biomedical Science
https://www.readbyqxmd.com/read/29488610/local-intra%C3%A2-articular-injection-of-vascular-endothelial-growth-factor-accelerates-articular-cartilage-degeneration-in-rat-osteoarthritis-model
#8
Shuang Deng, Jian-Lin Zhou, Hao Peng, Hong-Song Fang, Feng Liu, Jin-Qing Weng
In the pathophysiology of osteoarthritis (OA), articular cartilage degeneration exhibits a significant role. Vascular endothelial growth factor (VEGF) is considered to be an effective angiogenic factor and a crucial regulator of articular cartilage degeneration in the development of OA. Therefore, the present study aimed to investigate the underlying influences of exogenous VEGF on articular cartilage degeneration in OA model rat. A total of 24 male Sprague‑Dawley rats were randomly allocated into 3 groups...
February 28, 2018: Molecular Medicine Reports
https://www.readbyqxmd.com/read/29484338/polydatin-inhibits-the-il-1%C3%AE-induced-inflammatory-response-in-human-osteoarthritic-chondrocytes-by-activating-the-nrf2-signaling-pathway-and-ameliorates-murine-osteoarthritis
#9
Shangkun Tang, Qian Tang, Jialei Jin, Gang Zheng, Jianchen Xu, Wu Huang, Xiaobin Li, Ping Shang, Haixiao Liu
Osteoarthritis (OA), which is characterized by progressive degradation of the articular cartilage, is the most prevalent form of human arthritis. Accumulating evidence has shown that polydatin (PD) exerts special biological functions in a variety of diseases. However, whether it protects against OA development has remained unknown. Here, we investigated the anti-inflammatory and chondroprotective effects of PD on interleukin (IL)-1β-induced human osteoarthritic chondrocytes and in the surgical destabilization of medial meniscus mouse (DMM) OA models...
February 27, 2018: Food & Function
https://www.readbyqxmd.com/read/29473075/monascin-inhibits-il-1%C3%AE-induced-catabolism-in-mouse-chondrocytes-and-ameliorates-murine-osteoarthritis
#10
Gang Zheng, Yu Zhan, Qian Tang, Tianhe Chen, Fanghong Zheng, Haoli Wang, Jianle Wang, Dengying Wu, Xiaobing Li, Ying Zhou, Xiaobin Wang, Yaosen Wu, Yifei Zhou, Huazi Xu, Naifeng Tian, Xiaolei Zhang
Osteoarthritis (OA) is an age-related degenerative disease and is the fourth major cause of disability, but there are no effective therapies because of its complex pathology and the side effects of the drugs. Previous research demonstrated that inflammation and ECM degradation play major roles in OA development. Monascin is an azaphilonoid pigment extracted from Monascus-fermented rice with a potential anti-inflammatory effect reported in various preclinical studies. In the present study, we investigated the protectiveness of monascin on interleukin (IL)-1β-induced mouse chondrocytes and surgical destabilization of the medial meniscus mouse (DMM) OA models...
February 23, 2018: Food & Function
https://www.readbyqxmd.com/read/29448253/lycorine-suppresses-endplate-chondrocyte-degeneration-and-prevents-intervertebral-disc-degeneration-by-inhibiting-nf-%C3%AE%C2%BAb-signalling-pathway
#11
Gangliang Wang, Kangmao Huang, Yangxin Dong, Shuai Chen, Jianfeng Zhang, Jiying Wang, Ziang Xie, Xianfeng Lin, Xiangqiang Fang, Shunwu Fan
BACKGROUND/AIMS: Cartilaginous endplate (CEP) degeneration is an important cause for intervertebral disc (IVD) degeneration that leads to low-back pain. The identification of compounds that may prevent CEP degeneration is of interest for the prevention of IVD degeneration. METHODS: Catabolic protease expression in the CEP of disc degeneration patients was first assessed. The toxicity, function and underlying mechanism of lycorine (LY) on CEP-derived chondrocytes degeneration were assessed in vitro by flow cytometry analysis and western blotting...
February 9, 2018: Cellular Physiology and Biochemistry
https://www.readbyqxmd.com/read/29443370/beneficial-effects-of-high-density-lipoproteins-on-acquired-von-willebrand-syndrome-in-aortic-valve-stenosis
#12
C Gebhard, F Maafi, B E Stähli, A Bonnefoy, C E Gebhard, W Nachar, A Benjamim de Oliveira Moraes, M Mecteau, T Mihalache-Avram, V Lavoie, A E Kernaleguen, Y Shi, D Busseuil, M Chabot-Blanchet, L P Perrault, D Rhainds, E Rhéaume, J C Tardif
BACKGROUND: Infusions of apolipoprotein A-I (apoA-I), the major protein component of high-density lipoproteins (HDL), result in aortic valve stenosis (AVS) regression in experimental models. Severe AVS can be complicated by acquired von Willebrand syndrome, a haemorrhagic disorder associated with loss of high-molecular-weight von Willebrand factor (vWF) multimers (HMWM), the latter being a consequence of increased shear stress and enhanced vWF-cleaving protease (ADAMTS-13) activity. Although antithrombotic actions of HDL have been described, its effects on ADAMTS-13 and vWF in AVS are unknown...
February 2018: Thrombosis and Haemostasis
https://www.readbyqxmd.com/read/29435466/opana-er-oxymorphone-induced-thrombotic-microangiopathy-an-atypical-presentation-in-a-patient-with-hepatitis-c
#13
Hassan Mehmood, Muzammil Khan, Asghar Marwat, Medha Joshi, Varun Malhotra
Oxymorphone is a semisynthetic extended release opiate used to treat moderate to severe chronic pain. The Food and Drug Administration approved the oral form of oxymorphone available as Opana and Opana ER (extended release) since 2006. The Food and Drug Administration and the Centers for Disease Control and Prevention issued warning against intravenous use of Opana ER. We are presenting a case report of a 37-year-old female with a history of active intravenous drug abuse who presented to our hospital with acute kidney injury...
January 2018: Journal of Investigative Medicine High Impact Case Reports
https://www.readbyqxmd.com/read/29344548/recombinant-adamts-13-in-thrombotic-thrombocytopenic-purpura
#14
EDITORIAL
Marie Scully, Christopher Hibbard, Bruce Ewenstein
No abstract text is available yet for this article.
November 2017: Oncoscience
https://www.readbyqxmd.com/read/29337416/the-role-of-adamts-13-in-the-coagulopathy-of-sepsis
#15
REVIEW
M Levi, M Scully, M Singer
The interaction between platelets and the vessel wall is mediated by various receptors and adhesive proteins, of which von Willebrand factor (VWF) is the most prominent. The multimeric size of VWF is an important determinant of a more intense platelet-vessel wall interaction, and is regulated by the VWF-cleaving protease ADAMTS-13. A deficiency in ADAMTS-13 leads to higher concentrations of ultralarge VWF multimers and pathological platelet-vessel wall interactions, in its most typical and extreme form leading to thrombocytopenic thrombotic purpura, a thrombotic microangiopathy characterized by thrombocytopenia, non-immune hemolysis, and organ dysfunction...
April 2018: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/29329518/the-use-of-eculizumab-in-gemcitabine-induced-thrombotic-microangiopathy
#16
Vinod Krishnappa, Mohit Gupta, Haikoo Shah, Abhijit Das, Natthavat Tanphaichitr, Robert Novak, Rupesh Raina
BACKGROUND: Thrombotic microangiopathy (TMA) secondary to gemcitabine therapy (GiTMA) is a very rare pathology that carries a poor prognosis, with nearly half of the cases progressing to end stage renal disease. GiTMA is most commonly associated with adenocarcinomas, most notably pancreatic cancers. The mainstay of management is withdrawal of the offending drug and supportive care. Plasmapheresis has a limited role and hemodialysis may help in the management of fluid overload secondary to renal failure...
January 12, 2018: BMC Nephrology
https://www.readbyqxmd.com/read/29291551/ghrelin-prevents-articular-cartilage-matrix-destruction-in-human-chondrocytes
#17
REVIEW
Jie Liu, Lie Cao, Xueming Gao, Zhixin Chen, Shifang Guo, Zongru He, Yaowen Qian, Yongzhi Yu, Gang Wang
Osteoarthritis (OA) is the most common form of arthritis worldwide. Excessive production of pro-inflammatory cytokines such as interleukin-1β (IL-1β) plays a key role in the pathogenesis of OA. OA is generally characterized by degradation of extracellular matrixes such as type II collagen and aggrecans mediated by matrix metalloproteinases (MMPs) and a disintegrin and metalloproteinase with thrombospondin motifs (ADAMTS). Ghrelin is a secreted peptide hormone regulating appetite and the distribution and rate of use of energy...
February 2018: Biomedicine & Pharmacotherapy, Biomédecine & Pharmacothérapie
https://www.readbyqxmd.com/read/29247327/il-21-enhances-the-degradation-of-cartilage-through-the-jak-stat-signaling-pathway-during-osteonecrosis-of-femoral-head-cartilage
#18
Bin Chen, Yi Liu, Lei Cheng
This study aims to investigate the role of interleukin-21 (IL-21) in the mechanism of osteonecrosis of the femoral head. The serum content of IL-21 in patients with osteonecrosis of the femoral head (ONFH) and with femoral neck fracture (FNF) was examined by an enzyme-linked immunosorbent assay (ELISA). The cartilage specimens were stained with safranin-O. The expression of IL-21, tumor necrosis factor-α (TNF-α), cyclooxygenase (COX-2), a disintegrin and metalloproteinase with thrombospondin motifs 4 (ADAMTS-4), matrix metalloproteinase-13(MMP-13), and aggrecan in the cartilage was detected, by immunohistochemistry and western blot analysis...
December 15, 2017: Inflammation
https://www.readbyqxmd.com/read/29245931/silibinin-protects-against-osteoarthritis-through-inhibiting-the-inflammatory-response-and-cartilage-matrix-degradation-in-vitro-and-in-vivo
#19
Wenhao Zheng, Zhenhua Feng, Yiting Lou, Chunhui Chen, Chuanxu Zhang, Zhenyu Tao, Hang Li, Liang Cheng, Xiaozhou Ying
Osteoarthritis (OA) is a degenerative joint disease characterized by cartilage degradation and inflammation. Silibinin, a polyphenolic flavonoid derived from fruits and seeds of Silybum marianum , has been reported to possess various potent beneficial biological effects, such as antioxidant, anti-cancer, hepatoprotective and anti-inflammatory activities. However, the anti-inflammatory effects of silibinin on OA have not been reported. This study aimed to assess the effects of silibinin on OA both in vitro and in vivo ...
November 21, 2017: Oncotarget
https://www.readbyqxmd.com/read/29236309/leptin-induces-mmp1-13-and-adamts-4-expressions-through-bone-morphogenetic-protein-2-autocrine-effect-in-human-chondrocytes
#20
Yu-Ping Su, Cheng-Nan Chen, Kuo-Chin Huang, Hsin-I Chang, Ko-Chao Lee, Chun-Min Lo, Shun-Fu Chang
The induction of bone morphogenetic protein (BMP)2 in injured and arthritis articular cartilage has been proposed, but the precise mechanism has not been clearly clarified. Our previous study has found that leptin could stimulate the BMP2 autocrine effect to increase the anabolic collagen II expression when it initiates the catabolic response in human chondrocytes. It has been suggested that this BMP2 autocrine effect contributes to a reparative role in leptin-stimulated human chondrocytes. In this study, we further determined whether this BMP2 autocrine effect also affect the expressions of catabolic matrix metalloproteinases (MMPs) and a disintegrin and metalloproteinase with thrombospondin motif (ADAMTS)...
April 2018: Journal of Cellular Biochemistry
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