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https://www.readbyqxmd.com/read/29330741/coronary-artery-aneurysm-regression-after-kawasaki-disease-and-associated-risk-factors-a-3-year-follow-up-study-in-east-china
#1
Yunjia Tang, Wenhua Yan, Ling Sun, Qiuqin Xu, Yueyue Ding, Haitao Lv
Kawasaki disease (KD) is the leading cause of acquired heart disease due to its complicated coronary artery lesions. Up to now, few studies were focused on the status of persistent coronary artery aneurysms (CAA) in KD patients. The present study was designed to identify the coronary artery outcomes and seek the risk factors associated with the regression of CAA in KD patients. One hundred and twenty KD patients with CAA hospitalized in Children's Hospital of Soochow University from Jan 2008 to Dec 2013 were prospectively studied by a 3-year follow-up...
January 12, 2018: Clinical Rheumatology
https://www.readbyqxmd.com/read/29326369/successful-treatment-of-postural-orthostatic-tachycardia-and-mast-cell-activation-syndromes-using-naltrexone-immunoglobulin-and-antibiotic-treatment
#2
Leonard B Weinstock, Jill B Brook, Trisha L Myers, Brent Goodman
A patient with severe postural orthostatic tachycardia syndrome (POTS) and mast cell activation syndrome (MCAS) received immunotherapy with low-dose naltrexone (LDN) and intravenous immunoglobulin (IVIg) and antibiotic therapy for small intestinal bacterial overgrowth (SIBO). A dramatic and sustained response was documented. The utility of IVIg in autoimmune neuromuscular diseases has been published, but clinical experience with POTS is relatively unknown and has not been reported in MCAS. As a short-acting mu-opioid antagonist, LDN paradoxically increases endorphins which then bind to regulatory T cells which regulate T-lymphocyte and B-lymphocyte production and this reduces cytokine and antibody production...
January 11, 2018: BMJ Case Reports
https://www.readbyqxmd.com/read/29322700/factors-predicting-resistance-to-intravenous-immunoglobulin-and-coronary-complications-in-kawasaki-disease-ivig-resistance-in-kawasaki-disease
#3
EDITORIAL
Ji Whan Han
No abstract text is available yet for this article.
January 2018: Korean Circulation Journal
https://www.readbyqxmd.com/read/29315141/the-treatment-of-antibody-mediated-rejection-in-kidney-transplantation-an-updated-systematic-review-and-meta-analysis
#4
Susan S Wan, Tracey D Ying, Kate Wyburn, Darren M Roberts, Melanie Wyld, Steven J Chadban
BACKGROUND: Current treatments for antibody-mediated rejection (AMR) in kidney transplantation are based on low-quality data from a small number of controlled trials. Novel agents targeting B-cells, plasma-cells and the complement system have featured in recent studies of AMR. METHODS: We conducted a systematic review and meta-analysis of controlled trials in kidney transplant recipients using Medline, EMBASE and CENTRAL from inception to February 2017. RESULTS: Of 14,380 citations we identified 21 studies, including 10 randomized controlled trials, involving 751 participants...
January 8, 2018: Transplantation
https://www.readbyqxmd.com/read/29312777/anti-n-methyl-d-aspartate-receptor-encephalitis-associated-with-mediastinal-teratoma-a-rare-case-report-and-literature-review
#5
Chuan Li, Chengwu Liu, Feng Lin, Lunxu Liu
Anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis is a recently identified neuroautoimmune disorder characterized by antibodies against the heteromeric NR1-NR2 receptor complex. To our best knowledge, anti-NMDAR encephalitis combined with mediastinal teratoma is extremely rare with only three cases been reported in literature worldwide. In this article, we present an 18 years old female diagnosed as anti-NMDAR encephalitis caused by a large mature mediastinal teratoma whose initial manifestations were acute headache and seizures...
December 2017: Journal of Thoracic Disease
https://www.readbyqxmd.com/read/29308782/septic-miscarriage-with-toxic-shock-syndrome-and-disseminated-intravascular-coagulation-dic-the-role-of-surgery-recombinant-activated-factor-vii-and-intravenous-immunoglobulin-ivig
#6
L N Tan, G Mariappa, H Y Voon, H Suharjono
Severe sepsis with multi-organ failure is associated with a high mortality rate. This case report highlights the challenges and modalities available in the management of a lady with refractory shock and disseminated intravascular coagulation (DIC) due to toxic shock syndrome (TSS) from genital tract sepsis. Early surgical intervention to remove the source of infection, the use of recombinant activated factor VII to treat intractable disseminated intravascular coagulation and intravenous immunoglobulin to neutralise the circulating exotoxins, have been employed and shown to drastically improve outcomes...
December 2017: Medical Journal of Malaysia
https://www.readbyqxmd.com/read/29307884/streptococcus-pneumoniae-related-hemophagocytic-lymphohistiocytosis-treated-with-ivig-and-steroids
#7
Carissa Y Dumancas, Hans Alexi Garay Reyes, Juan Cosico, Amrut Savadkar, Soowhan Lah
BACKGROUND Hemophagocytic lymphohistiocytosis (HLH) is a rare life-threatening condition that has a poor prognosis due to the ensuing cytokine storm leading to severe organ damage. Current treatment guidelines suggest using a combination of steroid- and etoposide-based chemotherapy. CASE REPORT The authors present a case of a 41-year-old African-American female who presented with symptoms of foodborne illness and who developed multi-organ dysfunction. HLH was suspected because of poor response to broad-spectrum antibiotics with a constellation of findings, including cytopenia, hypofibrinogenemia, hypertriglyceridemia, and hyperferritinemia...
January 8, 2018: American Journal of Case Reports
https://www.readbyqxmd.com/read/29307273/kikuchi-fujimoto-disease-triggered-by-salmonella-enteritidis-in-a-child-with-concurrent-auto-immune-thyroiditis-and-papilloedema
#8
Esma Altinel Açoğlu, Eyup Sari, Gürses Şahin, Melahat Melek Oğuz, Meltem Akçaboy, Pelin Zorlu, Saliha Senel
Kikuchi-Fujimoto disease (KFD) is a histiocytic necrotising lymphadenitis characterised by painful cervical lymphadenopathy, fever, malaise and weight loss. Infections, auto-immune pathogenesis and a genetic association have been implicated. A 12-year-old boy presented with a 1-month history of fever, abdominal pain, constipation and weight loss, and a painful lymph node was detected in the right axilla. Chest CT demonstrated multiple lymph nodes, especially in the left mediastinum. Salmonella enteritidis group D was detected in a blood culture and he was treated with ceftriaxone, followed by meropenem...
January 8, 2018: Paediatrics and International Child Health
https://www.readbyqxmd.com/read/29306643/drug-induced-thrombocytopenia-after-anticoagulation-with-rivaroxaban
#9
Marianne K Pop, Farhad Farokhi, Lela Iduna
A 66-year-old man presented to the emergency department with complaints of dark-colored stool and rash developing over the last couple of days. The patient was started on rivaroxaban and flecainide for months prior for atrial fibrillation. Upon arrival, he was awake, alert, and oriented with a blood pressure of 111/63mmHg, heart rate of 68 beats per minute, and oxygen saturation of 96% on room air. A review of systems was unremarkable with the exception of skin rash and light-headedness. The patient's initial laboratory results were significant for red blood cell (RBC) count of 4...
December 24, 2017: American Journal of Emergency Medicine
https://www.readbyqxmd.com/read/29305554/abo-zygosity-but-not-secretor-or-fc-receptor-status-is-a-significant-risk-factor-for-ivig-associated-hemolysis
#10
Donald R Branch, Åsa Hellberg, Christine W Bruggeman, Jill R Storry, Darinka Sakac, Megan Blacquiere, Tik Nga Tong, Emeralda Burke-Murphy, Beth Binnington, Nagina Parmar, Lorna Sampson Riden, Kezia Willie, Chantal Armali, Jiwajee Aziz, Lani Lieberman, Vincent Laroche, Jeannie Callum, Yulia Lin, Nadine Shehata, Katerina Pavenski, Wendy Lau, Barbara Hannach, Taco W Kuijpers, Martin L Olsson, Christine Cserti-Gazdewich, Jacob Pendergrast
No abstract text is available yet for this article.
January 5, 2018: Blood
https://www.readbyqxmd.com/read/29305138/musk-myasthenia-gravis-and-pregnancy
#11
Ernestina Santos, Antonio Braga, Denis Gabriel, Sara Duarte, Ana Martins da Silva, Ilda Matos, Marta Freijo, Joao Martins, Fernando Silveira, Goreti Nadais, Filipa Sousa, Carla Fraga, Rosa Santos Silva, Carlos Lopes, Guilherme Gonçalves, Clara Pinto, Jorge Sousa Braga, Maria Isabel Leite
Muscle specific kinase (MuSK) myasthenia gravis (MG, MuSK-MG) is a rare subgroup of MG affecting mainly women during childbearing years. We investigated the influence of pregnancy in the course of MuSK-MG and pregnancy outcomes in females with MuSK-MG. A multicentre cohort of 17 women with MuSK-MG was studied retrospectively; 13 of them with ≥1 pregnancy. MuSK-MG onset age was 35,4 years; 23,0% had other autoimmune disorder; 46,2% were treatment refractory. Thirteen women experienced 27 pregnancies, either after MG onset (group I) (n = 4; maternal age at conception = 29...
November 28, 2017: Neuromuscular Disorders: NMD
https://www.readbyqxmd.com/read/29302828/analysis-of-biomarker-serum-levels-in-ivig-and-infliximab-refractory-kawasaki-disease-patients
#12
Akira Hachiya, Norimoto Kobayashi, Satoshi Matsuzaki, Yusuke Takeuchi, Yohei Akazawa, Tomonari Shigemura, Noriko Motoki, Junya Masumoto, Kazunaga Agematsu
Infliximab (IFX) is effective for treatment of refractory Kawasaki disease (KD). However, the precise mechanisms and biomarkers for IFX efficacy are unknown. We tried to evaluate the effect and response to IFX therapy by measuring serum cytokine levels. Twenty-nine children with KD who had been resistant to two courses of high-dose intravenous immunoglobulin were enrolled and treated with IFX. Plasma samples were analyzed for cytokines before and after IFX administration. Serum levels of interleukin-6, granulocyte colony-stimulating factor (G-CSF), interferon-gamma-induced monokine, interferon-gamma inducible protein 10 (IP-10), monocyte chemotactic protein 1, and soluble tumor necrosis factor-alpha receptor (sTNFR) 1 and 2 were significantly elevated before IFX treatment, but promptly decreased after the administration...
January 4, 2018: Clinical Rheumatology
https://www.readbyqxmd.com/read/29300958/sweet-signs-igg-glycosylation-leads-the-way-in-ivig-mediated-resolution-of-inflammation
#13
Christin Brückner, Christian Lehmann, Diana Dudziak, Falk Nimmerjahn
A hallmark of many chronic inflammatory and autoimmune diseases is that there is an impaired resolution of inflammation and return to the steady state. The infusion of high doses of pooled serum IgG preparations from thousands of donors [intravenous immunoglobulin (IVIG) therapy] has been shown to induce resolution of inflammation in a variety of chronic inflammatory and autoimmune diseases, suggesting that IgG molecules can instruct the immune system to stop inflammatory processes and initiate the return to the steady state...
December 30, 2017: International Immunology
https://www.readbyqxmd.com/read/29299869/combination-therapy-with-low-dose-ivig-and-a-c1-esterase-inhibitor-ameliorates-brain-damage-and-functional-deficits-in-experimental-ischemic-stroke
#14
Xinzhi Chen, Thiruma V Arumugam, Yi-Lin Cheng, Jong-Hwan Lee, Srinivasulu Chigurupati, Mark P Mattson, Milan Basta
Acute ischemic stroke causes a high rate of deaths and permanent neurological deficits in survivors. Current interventional treatment, in the form of enzymatic thrombolysis, benefits only a small percentage of patients. Brain ischemia triggers mobilization of innate immunity, specifically the complement system and Toll-like receptors (TLRs), ultimately leading to an exaggerated inflammatory response. Here we demonstrate that intravenous immunoglobulin (IVIG), a scavenger of potentially harmful complement fragments, and C1-esterase inhibitor (C1-INH), an inhibitor of complement activation, exert a beneficial effect on the outcome of experimental brain ischemia (I) and reperfusion (R) injury induced by transient occlusion of middle cerebral artery in mice...
January 3, 2018: Neuromolecular Medicine
https://www.readbyqxmd.com/read/29297955/predictive-factors-for-response-to-ivig-in-children-with-itp
#15
Yukiko Higashide, Tsukasa Hori, Yuko Yoto, Hiroyuki Kabutoya, Saho Honjo, Yoshiyuki Sakai, Masanori Nojima, Minami Yoda, Masaki Yamamoto, Hiroyuki Tsutsumi
BACKGROUND: Immune thrombocytopenic purpura (ITP) is commonly treated with intravenous immunoglobulin (IVIG). METHODS: We retrospectively evaluated whether pretreatment clinical and laboratory findings could predict the short- and long-term response to IVIG. RESULTS: Short-term response was estimated by platelet counts 2 weeks after IVIG, and long-term response was assessed by thrombocytopenia-free survival (TFS). TFS was defined as the probability of survival without treatment failure after initial IVIG, such as relapse, requiring additional therapeutic interventions, or progressing to chronic ITP...
January 3, 2018: Pediatrics International: Official Journal of the Japan Pediatric Society
https://www.readbyqxmd.com/read/29296968/a-fully-recombinant-human-igg1-fc-multimer-gl-2045-inhibits-complement-mediated-cytotoxicity-and-induces-ic3b
#16
Hua Zhou, Henrik Olsen, Edward So, Emmanuel Mérigeon, Denis Rybin, Jane Owens, Gregory LaRosa, David S Block, Scott E Strome, Xiaoyu Zhang
GL-2045 is a recombinant human immunoglobulin G1 (IgG1)-based Fc multimer designed to recapitulate the anti-inflammatory activities of intravenous immunoglobulin (IVIG) on the innate and adaptive immune responses. We used functional in vitro studies to determine if GL-2045 could mimic the modulatory activity of IVIG on complement activation. GL-2045, at log-order lower concentrations than heat-aggregated IgG (HAGG) and IVIG, protected antibody-opsonized cells from complement-dependent cytotoxicity. These protective effects were completely mediated by the higher order multimer fractions of GL-2045 and were partially dependent upon sequestration of C1q...
March 14, 2017: Blood Advances
https://www.readbyqxmd.com/read/29296090/clinical-and-economic-outcomes-of-a-high-touch-clinical-management-program-for-intravenous-immunoglobulin-therapy
#17
Julia Zhu, Heather S Kirkham, Gretchen Ayer, Chi-Chang Chen, Rolin L Wade, Swapna U Karkare, Chester H Robson, Jordan S Orange
Objective: To compare clinical and economic outcomes of patients who received intravenous immunoglobulin (IVIG) therapies and were managed by a clinical management program vs the outcomes of matched controls using administrative claim data. Methods: This retrospective cohort study used the PharMetrics Plus™ claim database between September 1, 2011 and June 30, 2014. Patients in the intervention group were from a "high-touch" IVIG clinical management program administered by a home infusion specialty pharmacy...
2018: ClinicoEconomics and Outcomes Research: CEOR
https://www.readbyqxmd.com/read/29287309/aspirin-dose-in-kawasaki-disease-the-ongoing-battle
#18
Anita Dhanrajani, Mercedes Chan, Stephanie Pau, Janet Ellsworth, Ross Petty, Jaime Guzman
BACKGROUND: Kawasaki disease (KD) is an acute childhood vasculitis that may result in coronary aneurysms. Treatment of Kawasaki disease (KD) with a single infusion of 2g/kg intravenous immunoglobulin (IVIG) is well established, but acetyl-salicylic acid (ASA) dose remains controversial. METHODS: We reviewed charts of patients with KD from two Canadian centres to assess the impact of ASA dose on IVIG resistance (operationally defined as administration of a second dose of IVIG)...
December 29, 2017: Arthritis Care & Research
https://www.readbyqxmd.com/read/29279594/poems-syndrome
#19
W W T Nyunt, R Remli, F A Abdul Muttlib, C F Leong, N Masir, N R Tumian, S F S Abdul Wahid
POEMS syndrome is the syndrome of Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal protein and typical Skin changes. A 65-year-old lady presented with the 2-day-history of inability to walk, 4-month-history of progressive worsening of muscle weakness of both lower limbs and 1-year-history of progressive worsening of bilateral numbness of lower limbs. Nerve conduction study revealed generalized sensorimotor demyelinating polyneuropathy. She was initially treated as chronic inflammatory demyelinating polyradiculoneuropathy with intravenous immunoglobulin (IVIG) and high-dose prednisolone...
December 2017: Malaysian Journal of Pathology
https://www.readbyqxmd.com/read/29277513/risk-factors-treatment-and-immune-dysregulation-in-autoimmune-cytopenia-following-allogeneic-hematopoietic-stem-cell-transplantation-in-pediatric-patients
#20
Matthijs D Kruizinga, Maarten J D van Tol, Vincent Bekker, Tanja Netelenbos, Frans J Smiers, Dorine Bresters, Anja M Jansen-Hoogendijk, Monique M van Ostaijen-Ten Dam, Wouter J W Kollen, Jaap J Zwaginga, Arjan C Lankester, Robbert G M Bredius
Auto- or alloimmune cytopenia (AIC) is a known rare complication of hematopoietic stem cell transplantation (SCT). AIC after SCT is considered difficult to treat and associated with high morbidity and mortality. In this retrospective study in pediatric patients we evaluated incidence, outcome, potential risk factors and current treatment strategies. A nested matched case-control study was performed to search for biomarkers associated with AIC. Of 531 consecutive SCTs at our center between 2000 and 2016, 26 were complicated by the development of AIC (cumulative incidence 5...
December 19, 2017: Biology of Blood and Marrow Transplantation
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