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https://www.readbyqxmd.com/read/28922662/treatment-of-autoimmune-glial-fibrillary-acidic-protein-astrocytopathy-follow-up-in-7-cases
#1
Xinguang Yang, Junyan Liang, Qingmei Huang, Huiming Xu, Cong Gao, Youming Long, Xiaoyu Xiao
OBJECTIVE: The aim of this work was to report an autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy with long-term follow-up in 7 patients. METHODS: Antibodies were detected by indirect immunofluorescence assay and patient data were analyzed retrospectively. RESULTS: Seven patients (4 female, 3 male) with ≥1 year follow-up were included. All patients had positive GFAP antibodies in their cerebral spinal fluid (CSF). Their median age at disease onset was 56 years (range 27-69) and the median disease duration was 1 year (range 1-4)...
September 19, 2017: Neuroimmunomodulation
https://www.readbyqxmd.com/read/28916232/circulating-normal-igg-as-stimulator-of-regulatory-t-cells-lessons-from-intravenous-immunoglobulin
#2
Mohan S Maddur, Srini V Kaveri, Jagadeesh Bayry
Intravenous immunoglobulin (IVIG), a pooled normal IgG formulation prepared from thousands of healthy donors' plasma, is extensively used for the immunotherapy of autoimmune and inflammatory disorders. Recent reports demonstrate that IVIG exerts anti-inflammatory actions by stimulating the activation and expansion of regulatory T (Treg) cells by multiple mechanisms via antigen-presenting cells (APCs).
September 12, 2017: Trends in Immunology
https://www.readbyqxmd.com/read/28913992/effects-of-immunoglobulin-replacement-on-asthma-exacerbation-in-adult-asthmatics-with-igg-subclass-deficiency
#3
Joo Hee Kim, Young Min Ye, Ga Young Ban, Yoo Seob Shin, Hyun Young Lee, Young Hee Nam, Soo Keol Lee, You Sook Cho, Seung Hun Jang, Ki Suck Jung, Hae Sim Park
PURPOSE: Recurrent respiratory tract infection is a common manifestation of primary immunodeficiency disease, and respiratory viruses or bacteria are important triggers of asthma exacerbations. Asthma often coexists with humoral immunodeficiency in adults, and some asthmatics with immunoglobulin (Ig) G subclass deficiency (IgGSCD) suffer from recurrent exacerbations. Although some studies suggest a benefit from Ig replacement, others have failed to support its use. This study aimed to assess the effect of Ig replacement on asthma exacerbation caused by respiratory infection as well as the asthma control status of adult asthmatics with IgGSCD...
November 2017: Allergy, Asthma & Immunology Research
https://www.readbyqxmd.com/read/28903347/retrospective-analysis-of-risk-factors-associated-with-kawasaki-disease-in-china
#4
Lihua Bai, Tienan Feng, Lifang Yang, Yi Zhang, Xuejuan Jiang, Jiayao Liao, Lihua Chen, Xiaoyan Feng, Yanming Rong, Yuehua Li, Zhiqiang Qin, Jing Qiao
In order to provide early intervention for coronary artery lesion (CAL) caused by Kawasaki Disease (KD), we analyzed clinical characteristics of typical and incomplete KD cases from 1998 to 2008 in Northwest and Central China. A total of 383 patients included 298 cases of typical KD and 85 cases of incomplete KD. The morbidity of incomplete KD was 28.5%, a percentage significantly lower than that of typical KD. The occurrence of bulbar conjunctiva congestion, erythra, crissum red, film-like decrustation, lip red, rhagades, raspberry tongue, bilateral toe-end decrustation, limb sclerosis, cervical lymph nodes enlargement, agitation and irritability in incomplete KD group was lower than that in the group of typical KD (p < 0...
August 15, 2017: Oncotarget
https://www.readbyqxmd.com/read/28900133/fcn1-m-ficolin-which-directly-associates-with-immunoglobulin-g1-is-a-molecular-target-of-intravenous-immunoglobulin-therapy-for-kawasaki-disease
#5
Daisuke Okuzaki, Kaori Ota, Shin-Ichi Takatsuki, Yukari Akiyoshi, Kazuyuki Naoi, Norikazu Yabuta, Tsutomu Saji, Hiroshi Nojima
Kawasaki disease (KD), an acute systemic vasculitis of early childhood, is of unknown etiology. High-dose intravenous immunoglobulin (IVIG) is an effective treatment, but its molecular target remains elusive. DNA microarray analysis of peripheral blood mononuclear cells (PBMCs) revealed that at least 21 genes are drastically down-regulated after IVIG treatment in most KD patients. qRT-PCR analysis confirmed that the mRNA levels of five of these genes were considerably reduced in almost all KD patients after IVIG treatment...
September 12, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28894082/chronic-inflammatory-demyelinating-polyneuropathy-cidp-an-uncommon-manifestation-of-systemic-lupus-erythematosus-sle
#6
Hrudya Abraham, Jose Kuzhively, Syed W Rizvi
BACKGROUND Chronic inflammatory demyelinating polyneuropathy (CIDP) is an uncommon manifestation of systemic lupus erythematosus (SLE). We report a case of SLE presenting as CIDP and discuss the diagnosis, management, and prognosis of CIDP. CASE REPORT A 40-year-old woman with a past medical history of SLE treated with hydroxychloroquine presented with bilateral, progressive, ascending, sensory and motor neuropathy. Physical examination showed weakness and reduced temperature of all extremities, reduced pinprick and vibration sense of the distal extremities, loss of reflexes, and walking with a wide-based unsteady gait...
September 12, 2017: American Journal of Case Reports
https://www.readbyqxmd.com/read/28892213/evaluation-of-longitudinal-and-radial-left-ventricular-functions-using-two-and-three-dimensional-echocardiography-before-and-after-intravenous-immunoglobulin-administration-in-patients-with-acute-kawasaki-disease
#7
Ikuo Hashimoto, Yu Saitou, Nao Sakata, Ko Shibata
BACKGROUND: The relationship between left ventricular (LV) function and longitudinal or radial contraction has not yet been elucidated in patients with acute Kawasaki disease (KD), especially before and after intravenous immunoglobulin (IVIG) administration. METHODS: We studied 28 KD patients without coronary aneurysms (average 3.2 years). The LV end-diastolic volume (LVEDV), end-systolic volume (LVESV), stroke volume (LVSV), and ejection fraction (LVEF) were assessed using three-dimensional echocardiography before IVIG, after IVIG administration, and in the convalescent phase...
September 11, 2017: Pediatrics International: Official Journal of the Japan Pediatric Society
https://www.readbyqxmd.com/read/28890837/why-it-is-not-always-anxiety-a-tough-diagnosis-of-stiff-person-syndrome
#8
Carmen Elena Cervantes, Hsien Lee Lau, Tina Ataian Binazir, Keith O O'Brien, Jonathan S Cross
Anxiety disorder is a commonly used diagnosis that may mask underlying conditions. Stiff person syndrome (SPS) is a rare neuroimmunological disorder characterized by progressive rigidity and painful muscle spasms affecting axial and lower extremity musculature. These episodes can be triggered by sudden movement, noise, or emotional stress, which may present as a psychiatric condition. We report the case of a 30-year-old female who presented with recurrent panic attacks with multiple prior hospital admissions for anxiety, rigidity, and difficulty in walking...
2017: Case Reports in Neurological Medicine
https://www.readbyqxmd.com/read/28885479/high-dose-subcutaneous-immunoglobulin-in-patients-with-multifocal-motor-neuropathy-a-nursing-perspective
#9
Vilija M Rasutis, Hans D Katzberg, Vera Bril
Multifocal motor neuropathy (MMN), an immune neuromuscular condition causing progressive weakness, usually responds to immune-mediated treatments, including intravenous immunoglobulin (IVIG). Fifteen patients with MMN receiving IVIG were enrolled in an open-label, single-center trial and switched to 20% subcutaneous immunoglobulin (SCIG) using a smooth transition protocol (ie, changing the therapy without interruption or impact on the intended outcome of the therapy). Patients received individualized training and support based on motivation and ability to learn, follow directions, and maintain compliance...
September 2017: Journal of Infusion Nursing: the Official Publication of the Infusion Nurses Society
https://www.readbyqxmd.com/read/28884910/successful-use-of-cyclosporin-a-for-stevens-johnson-syndrome-and-toxic-epidermal-necrolysis-in-three-children
#10
Jessica St John, Vladimir Ratushny, Kristina J Liu, Daniel Q Bach, Omar Badri, Lia E Gracey, Allen W Ho, Adam B Raff, Daniel Y Sugai, Peter Schalock, Daniela Kroshinsky
BACKGROUND/OBJECTIVES: Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are medical emergencies. Mainstays of treatment include removal of the offending agent, supportive care, and wound care. The use of immunosuppressive agents such as corticosteroids and intravenous immunoglobulin (IVIg) is controversial. Some case reports and small studies report the successful use of cyclosporin A (CsA) for SJS/TEN in halting disease progression, fostering reepithelialization, and reducing mortality...
September 2017: Pediatric Dermatology
https://www.readbyqxmd.com/read/28884051/anti-n-methyl-d-aspartate-receptor-nmdar-encephalitis-in-association-with-ovarian-teratoma
#11
Javaad Ahmad, Muhammad Saad Sohail, Amina Khan, Ahmed H Qavi, Pramod Gaudel, Mehr Zahid, Salman Assad
Anti-N-methyl-D-aspartate-Receptor (NMDAR) encephalitis is an autoimmune disorder with a multifaceted presentation that involves memory deficits, psychiatric symptoms, and autonomic instability. This case report describes the classic presentation of Anti-NMDAR encephalitis and highlights its association with ovarian teratomas. We present a 26 -year-old female who came in with new onset seizures and altered mentation who subsequently developed automatism. Electroencephalograms (EEG) showed left frontal spikes and right temporal delta activity...
July 5, 2017: Curēus
https://www.readbyqxmd.com/read/28882075/comparative-treatment-related-adverse-event-cost-burden-in-immune-thrombocytopenic-purpura
#12
Prina Z Donga, Sara P Bilir, Gregg Little, Tim Babinchak, Julie Munakata
AIMS: Real-world evidence on the safety profile and costs associated with immune thrombocytopenic purpura (ITP) treatment in adults is lacking. This study quantifies and compares adverse event (AE) crude rates and costs associated with ITP treatments as found in claims data. MATERIALS AND METHODS: A retrospective claims-based analysis was conducted using IMS Pharmetrics Plus database. Included patients were ≥18 years old, with a diagnosis of ITP (2007-2012); an ITP-related claim for anti-D, intravenous immunoglobulin (IVIG), rituximab, romiplostim, or eltrombopag; and 1-year continuous enrollment (3-years for rituximab) during follow-up...
September 8, 2017: Journal of Medical Economics
https://www.readbyqxmd.com/read/28881695/correlation-of-hamp-gene-polymorphisms-and-expression-with-the-susceptibility-and-length-of-hospital-stays-in-taiwanese-children-with-kawasaki-disease
#13
Ying-Hsien Huang, Kuender D Yang, Yu-Wen Hsu, Hsing-Fang Lu, Henry Sung-Ching Wong, Hong-Ren Yu, Hsing-Chun Kuo, Fu-Chen Huang, Mao-Hung Lo, Kai-Sheng Hsieh, Su-Fen Chen, Wei-Chiao Chang, Ho-Chang Kuo
Kawasaki disease (KD) is a form of systemic vasculitis. Regarding its pathogenesis, HAMP gene encoding hepcidin, which is significant for iron metabolism, has a vital function. In this study, we recruited a total of 381 KD patients for genotyping. Data from 997 subjects (500 subjects from cohort 1; 497 subjects from cohort 2) were used for analysis. Using TaqMan allelic discrimination, we determined five tag SNPs (rs916145, rs10421768, rs3817623, rs7251432, and rs2293689). Treatment outcome data related to such clinical phenotypes as coronary artery lesions (CAL), coronary artery aneurysms (CAA), and intravenous immunoglobulin (IVIG) effects were also collected...
August 1, 2017: Oncotarget
https://www.readbyqxmd.com/read/28881516/-effectiveness-and-safety-of-rituximab-for-children-with-autoimmune-diseases-of-the-nervous-system
#14
Z Fu, X H Bao, Y Wu, J Zhou, Y H Zhang, Y Zhang, T Y Ji, Y Chen
Objective: To assess the effectiveness and safety of rituximab in Chinese children with autoimmune diseases of the nervous system. Method: An ambispective cohort study enrolled patients with refractory and(or) relapse autoimmune diseases of nervous system from June 2010 to June 2016 in Peking University First Hospital.These patients failed to respond to steroids and(or)intravenous immunoglobulin (IVIG) were treated with rituximab and seen for follow-up visits once every 3 months.The effectiveness was assessed by modified Rankin scale (mRs) and the annualized relapse rate...
September 2, 2017: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
https://www.readbyqxmd.com/read/28881389/comparisons-in-fluctuation-of-muscle-strength-and-function-in-patients-with-immune-mediated-neuropathy-treated-with-intravenous-versus-subcutaneous-immunoglobulin
#15
Ingelise Christiansen, Lars H Markvardsen, Johannes Jakobsen
INTRODUCTION: Variation of muscle strength and function have not been studied in patients with chronic inflammatory demyelinating polyneuropathy and multifocal motor neuropathy whose treatment regimen has been changed from intravenous to subcutaneous immunoglobulin (IVIG to SCIG). METHODS: In a prospective open label study, patients were changed from monthly IVIG to weekly SCIG. The primary endpoint was the variation of isokinetic muscle strength (cIKS). Secondary endpoints were variation of Medical Research Council (MRC) score, grip strength (GS), 9-hole-peg test (9-HPT), 40-meter-walk test (40-MWT)...
September 7, 2017: Muscle & Nerve
https://www.readbyqxmd.com/read/28878085/hcmv-particles-treated-with-specific-antibodies-induce-intrinsic-and-adaptive-but-not-innate-immune-responses
#16
Zeguang Wu, Ruifang Qin, Li Wang, Matteo Bosso, Myriam Scherer, Thomas Stamminger, Dominik Hotter, Thomas Mertens, Giada Frascaroli
Human cytomegalovirus (HCMV) persistently infects 40% to 100% of the human population worldwide. Experimental and clinical evidence indicates that humoral immunity to HCMV plays an important role in restricting virus dissemination and protecting the infected host from disease. Specific immunoglobulin preparations from pooled plasma of adults selected for high titers of HCMV antibodies, have been used for prevention of CMV disease in transplant recipients and pregnant women. Even though incubation of HCMV particles with these preparations leads to neutralization of the viral infectivity, it is still unclear whether the antibody-treated HCMV particles (here defined as HCMV-Ab) enter into the cells and modulate anti-viral immune responses...
September 6, 2017: Journal of Virology
https://www.readbyqxmd.com/read/28874371/commercial-intravenous-immunoglobulin-ivig-preparations-contain-functional-neutralizing-antibodies-against-the-staphylococcus-aureus-leukocidin-lukab-lukgh
#17
James B Wood, Lauren S Jones, Nicole R Soper, Meera Nagarsheth, C Buddy Creech, Isaac P Thomsen
The pathogenesis of Staphylococcus aureus is mediated by an array of important virulence factors, including the two-component leukocidin family of toxins. LukAB (also known as LukGH), the most recently discovered leukocidin, is potently lethal to phagocytes, produced during invasive human disease, and present in all known clinical isolates of S. aureus Intravenous immunoglobulin (IVIg) is often used clinically in severe S. aureus infections. The primary aim of this study was to assess the binding and neutralization potential of IVIg against LukAB...
September 5, 2017: Antimicrobial Agents and Chemotherapy
https://www.readbyqxmd.com/read/28871669/facilitated-subcutaneous-immunoglobulin-fscig-in-autoimmune-cytopenias-associated-with-common-variable-immunodeficiency
#18
Veronica Pedini, Isabella Savore, Giovanna Maria Danieli
BACKGROUND: Common variable immunodeficiency (CVID) is the most common symptomatic primary immune deficiency of adulthood. Besides recurrent infections, autoimmune disorders-mainly cytopenias-affect 30% of patients with CVID. OBJECTIVES: To describe the efficacy and safety of facilitated subcutaneous immunoglobulin (fSCIg), which is a combination of 10% [human] SCIg with recombinant human hyaluronidase for the treatment of CVID-linked cytopenias. METHODS: We describe four women (mean age 54 years) with CVID associated with idiopathic thrombocytopenic purpura (ITP) (n=3) and autoimmune hemolytic anemia (AIHA) (n=1)...
July 2017: Israel Medical Association Journal: IMAJ
https://www.readbyqxmd.com/read/28871523/a-personalized-diagnostic-and-treatment-approach-for-macrophage-activation-syndrome-and-secondary-hemophagocytic-lymphohistiocytosis-in-adults
#19
Bharat Kumar, Sohaib Aleem, Hana Saleh, Jennifer Petts, Zuhair K Ballas
OBJECTIVE: We assessed the clinical features and outcomes based on therapeutic options adopted during hospital stay for adult patients with macrophage activation syndrome and secondary hemophagocytic lymphohistiocytosis (MAS/sHLH). METHODS: We conducted a retrospective chart review of all adult patients (age ≥ 18 years) diagnosed with MAS/sHLH at our center between 2010 and 2015. Inclusion criteria for patients were diagnosis of MAS/sHLH during admission and patients meeting at least 5 out of 8 of Henter's criteria or at least 4 out of 6 of the criteria that were tested...
September 4, 2017: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/28864939/intravenous-immunoglobulins-for-treatment-of-connective-tissue-diseases-in-dermatology
#20
REVIEW
Lyubomir A Dourmishev, Dimitrina V Guleva, Ljubka G Miteva
BACKGROUND: Connective tissue diseases are a heterogeneous group of autoimmune disorders affecting not only skin, but also various organs and systems. First-line treatment of connective tissue diseases is systemic steroids as monotherapy or combined with immunosuppressive drugs. Since intravenous immunoglobulins (IVIGs) have been found to be effective for various autoimmune dermatoses, their indications have expanded tremendously. OBJECTIVE: The aim this review article is to highlight the indications, effectiveness, and side effects of high doses immunoglobulins for treatment of patients with connective tissue diseases...
September 1, 2017: Wiener Medizinische Wochenschrift
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