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https://www.readbyqxmd.com/read/29449131/the-treatment-of-anti-phospholipid-syndrome-a-comprehensive-clinical-approach
#1
REVIEW
Cecilia Beatrice Chighizola, Laura Andreoli, Maria Gerosa, Angela Tincani, Amelia Ruffatti, Pier Luigi Meroni
Anti-phospholipid syndrome (APS) is an acquired pro-thrombotic autoimmune disease that predisposes to thrombotic events and/or obstetric complications, in the persistent presence of anti-phospholipid antibodies (aPL). Life long moderate-intensity anticoagulation is the option of choice for aPL-positive patients with a previous thrombosis; critical issues concern the management of those with a history of arterial event due to the high rate of recurrence. Alternatives comprise anti-platelet agents and high-intensity anticoagulation...
February 12, 2018: Journal of Autoimmunity
https://www.readbyqxmd.com/read/29447659/paraneoplastic-pemphigus-in-a-34-year-old
#2
Laura E Melnick, Jenna M Beasley, Randi Kim, Nooshin Brinster, Kristen Lo Sicco
Paraneoplastic Pemphigus (PNP) is a rare and often fatal autoimmune mucocutaneous blistering disease associated with an underlying malignancy. It is thought to be caused by antibodies to tumor antigenscross-reacting with epithelial antigens, specifically desmosomal and hemidesmosomal antigens. There are at least five clinical morphologic variants of PNP, with the earliest and most consistent finding beingsevere stomatitis. Diagnosis of PNP requires direct immunofluorescence of perilesional skin and indirect immunofluorescence...
December 15, 2017: Dermatology Online Journal
https://www.readbyqxmd.com/read/29447653/rituximab-and-intravenous-immunoglobulin-as-alternatives-to-long-term-systemic-corticosteroids-in-the-treatment-of-pemphigus-a-single-center-case-series-of-63-patients
#3
Ashley E Brown, Kiran Motaparthi, Sylvia Hsu
Rituximab and intravenous immunoglobulin [IVIg] have recently emerged as effective treatments for pemphigus refractory to corticosteroids [CS]. This case series sought to compare the clinical, serologic,and adverse effects of CS, IVIg, and rituximab in patients with pemphigus. A retrospective review of 63 patients with pemphigus vulgaris (PV), pemphigus foliaceus (PF), or paraneoplastic pemphigus (PNP)was performed. Clinical remission (CR), serologic remission (SR), and adverse effects were evaluated. Three study groups were compared: patients treated with systemic CS, refractory patients treated withIVIg, and refractory patients treated with rituximab...
January 19, 2018: Dermatology Online Journal
https://www.readbyqxmd.com/read/29445957/treating-immune-related-epilepsy
#4
REVIEW
Sonal Bhatia, Sarah E Schmitt
PURPOSE OF REVIEW: This review explores different treatment modalities for immune-mediated epilepsy, including epilepsy caused by autoantibodies as well as epilepsy in the context of systemic autoimmune disease. RECENT FINDINGS: Autoimmune epilepsy is an increasingly recognized entity. Conventional treatments for epilepsy, such as antiseizure medications and epilepsy surgery, are less successful in treating epilepsy caused by autoimmune disease. Immunomodulatory therapies such as corticosteroids, intravenous immunoglobulin, and plasma exchange are generally more successful in treating immune-mediated epilepsy than conventional epilepsy therapies...
February 14, 2018: Current Neurology and Neuroscience Reports
https://www.readbyqxmd.com/read/29443442/paediatric-adem-followed-by-optic-neuritis-disease-course-treatment-response-and-outcome
#5
Y Y M Wong, Y Hacohen, T Armangue, E Wassmer, H Verhelst, C Hemingway, E D van Pelt, C E Catsman-Berrevoets, R Q Hintzen, K Deiva, M J Lim, K Rostásy, R F Neuteboom
BACKGROUND: Acute disseminated encephalomyelitis, followed by optic neuritis (ADEM-ON) is a rare demyelinating syndrome different than MS and neuromyelitis optica spectrum disorder. We aim to describe the disease course, treatment response and outcome of these children. METHODS: children <18 years were identified from 6 countries of the EU Paediatric Demyelinating Disease Consortium. Patients fulfilled the diagnostic criteria for ADEM, followed by at least one ON...
February 14, 2018: European Journal of Neurology: the Official Journal of the European Federation of Neurological Societies
https://www.readbyqxmd.com/read/29443427/intravenous-immunoglobulins-for-rituximab-resistant-mucous-membrane-pemphigoid
#6
Mareike Witte, Detlef Zillikens, Iakov Shimanovich
Mucous membrane pemphigoid (MMP) is an autoimmune blistering disease that is notoriously difficult to treat. Rituximab (anti-CD20) and high-dose intravenous immunoglobulins (IVIG) have both been reported to be effective in refractory MMP.1,2Furthermore, treatment regimens based on the concomitant use of rituximab and IVIG in ocular MMP have been published.3,4In the current report, we present 5 patients with treatment-resistant MMP who failed to respond to rituximab but rapidly achieved remission on IVIG. This article is protected by copyright...
February 14, 2018: Journal of the European Academy of Dermatology and Venereology: JEADV
https://www.readbyqxmd.com/read/29441299/livedo-racemosa-reticulated-ulcerations-panniculitis-and-violaceous-plaques-in-a-46-year-old-woman
#7
Alfredo Agulló, Brian Hinds, Mónica Larrea, Ignacio Yanguas
Clinically amyopathic dermatomyositis (CADM) is a subset of dermatomyositis (DM) that has conventional cutaneous manifestations of DM, but paradoxically, little or no muscle involvement. In 2005, a novel antibody was described in association with CADM - anti-melanoma differentiation-associated gene 5 (anti-MDA5). Patients with this serologic marker have a characteristic mucocutaneous phenotype consisting of skin ulceration among other signs. We describe the case of a 46-year-old woman with CADM, elevated anti-MDA5 autoantibodies, and unusual clinical features (livedo racemosa, florid acral edema) among the classical phenotype of MDA5 DM (arthralgias, ulcerations, panniculitis) and classical DM lesions (Gottron papules, heliotrope rash)...
January 2018: Indian Dermatology Online Journal
https://www.readbyqxmd.com/read/29439921/impact-of-organizational-interventions-on-reducing-inappropriate-intravenous-immunoglobulin-ivig-usage-a-systematic-review-and-meta-analysis
#8
Calvin Diep, Andrew W Shih, Erin Jamula, Nancy M Heddle, Michael Parvizian, Christopher M Hillis
BACKGROUND: With increasing global use of intravenous immunoglobulin (IVIG), there is interest in its appropriate usage. Efforts to regulate IVIG usage have primarily taken the form of organizational interventions implemented in hospitals to monitor and improve physician prescribing. Similar interventions have proven effective in reducing the inappropriate and total hospital usage of other blood products, but their efficacy on IVIG use is less understood. Thus, we performed a systematic review of studies reporting the change in inappropriate IVIG use following such interventions in hospitals or regions...
January 31, 2018: Transfusion and Apheresis Science
https://www.readbyqxmd.com/read/29437773/autoimmune-fasciitis-triggered-by-the-anti-programmed-cell-death-1-monoclonal-antibody-nivolumab
#9
Matthew Js Parker, Mark E Roberts, Paul C Lorigan, Daniel G du Plessis, Hector Chinoy
A 43-year-old woman with a history of recently diagnosed metastatic melanoma was commenced on systemic therapy with nivolumab, an anti-programmed cell death-1 monoclonal antibody and one of an increasing group of the so-called 'immune checkpoint inhibitors'. She experienced a dramatic complete response within 6 months of initiation. However, in addition to developing incident autoimmune hypothyroidism, she also developed progressive fatigue, proximal weakness, myalgia and dysphagia. Initial investigations with blood tests, electrophysiology and a muscle biopsy were non-specific or normal...
February 8, 2018: BMJ Case Reports
https://www.readbyqxmd.com/read/29434001/validation-and-implementation-of-planova%C3%A2-bioex-virus-filters-in-the-manufacture-of-a-new-liquid-intravenous-immunoglobulin-in-china
#10
Shan Ma, Guang Li Pang, Yu Juan Shao, Tomoko Hongo-Hirasaki, Meng Xian Shang, Marcus Inouye, Chang Yong Jian, Meng Zhao Zhu, Hu Hu Yang, Jian Feng Gao, Zhi Ying Xi, Dian Wei Song
There is a continuous need to improve the viral safety of plasma products, and we here report the development and optimization of a manufacturing-scale virus removal nanofiltration step for intravenous immunoglobulin (IVIG) using the recently introduced Planova™ BioEX filter. IVIG throughput was examined for various operating parameters: transmembrane pressure, temperature, protein concentration, and prefiltration methods. The developed procedure was based on filtering undiluted process solution (50.0 g/l IVIG) under constant transmembrane pressure filtration at 294 kPa and 25 °C following prefiltration with a 0...
February 9, 2018: Biologicals: Journal of the International Association of Biological Standardization
https://www.readbyqxmd.com/read/29433947/antiglutamic-acid-decarboxylase-65-gad65-antibody-associated-epilepsy
#11
REVIEW
Ahmad Daif, Rimas V Lukas, Naoum P Issa, Adil Javed, Stephen VanHaerents, Anthony T Reder, James X Tao, Peter Warnke, Sandra Rose, Vernon L Towle, Shasha Wu
Glutamic acid decarboxylase (GAD) antibody-associated encephalitis causes both acute seizures and chronic epilepsy with predominantly temporal lobe onset. This condition is challenging in diagnosis and management, and the incidence of GAD antibody (Ab)-related epilepsy could be much higher than commonly believed. Imaging and CSF evidence of inflammation along with typical clinical presentations, such as adult onset temporal lobe epilepsy (TLE) with unexplained etiology, should prompt testing for the diagnostic antibodies...
February 9, 2018: Epilepsy & Behavior: E&B
https://www.readbyqxmd.com/read/29433111/-acute-flaccid-myelitis
#12
Ryutaro Kira
Acute flaccid myelitis (AFM) is a newly defined, rare, but clinically distinct syndrome of acute flaccid paralysis with spinal motor neuron involvement of unknown etiology. Clusters of AFM coincided with a nationwide outbreak of enterovirus D68 (EV-D68) in the United States during autumn to winter of 2014 and in Japan during the autumn of 2015. Although EV-D68 was detected in only 20% of the AFM patients, mainly from respiratory specimens without apparent viral detection from blood and cerebrospinal fluid (CSF) samples, strong temporal associations were noted...
February 2018: Brain and Nerve, Shinkei Kenkyū No Shinpo
https://www.readbyqxmd.com/read/29429551/dermatology-and-immunoglobulin-therapy-who-to-treat-and-how-to-administer-immunoglobulins
#13
F J Navarro-Triviño, I Pérez-López, R Ruíz-Villaverde
Intravenous immunoglobulin (IVIG) replacement therapy has been used in immune deficiency diseases for more than 50 years. The indications for this treatment have evolved, however, and IVIG therapy is now used in various diseases in which the immune system plays a prominent role. IVIG therapy has carved out a niche in dermatology for the treatment of such conditions as dermatomyositis, autoimmune bullous diseases, and toxic epidermal necrolysis. Special attention has been paid to this therapy in recent years...
February 8, 2018: Actas Dermo-sifiliográficas
https://www.readbyqxmd.com/read/29428821/recombinant-igg1-fc-hexamers-block-cytotoxicity-and-pathological-changes-in-experimental-in-vitro-and-rat-models-of-neuromyelitis-optica
#14
Lukmanee Tradtrantip, Christian M Felix, Rolf Spirig, Adriana Baz Morelli, A S Verkman
Intravenous human immunoglobulin G (IVIG) may have therapeutic benefit in neuromyelitis optica spectrum disorders (herein called NMO), in part because of the anti-inflammatory properties of the IgG Fc region. Here, we evaluated recombinant Fc hexamers consisting of the IgM μ-tailpiece fused with the Fc region of human IgG1. In vitro, the Fc hexamers prevented cytotoxicity in aquaporin-4 (AQP4) expressing cells and in rat spinal cord slice cultures exposed to NMO anti-AQP4 autoantibody (AQP4-IgG) and complement, with >500-fold greater potency than IVIG or monomeric Fc fragments...
February 8, 2018: Neuropharmacology
https://www.readbyqxmd.com/read/29428273/high-sustained-antibody-titers-in-patients-with-classic-infantile-pompe-disease-following-immunomodulation-at-start-of-enzyme-replacement-therapy
#15
Esther Poelman, Marianne Hoogeveen-Westerveld, Marian A Kroos-de Haan, Johanna M P van den Hout, Kees J Bronsema, Nico C van de Merbel, Ans T van der Ploeg, W W M Pim Pijnappel
OBJECTIVE: To evaluate whether immunomodulation at start of enzyme replacement therapy induces immune tolerance to recombinant human acid alpha-glucosidase (rhGAA) in patients with classic infantile Pompe disease. STUDY DESIGN: Three patients (1 cross reactive immunologic material negative, 2 cross reactive immunologic material positive) were treated with 4 weekly doses of rituximab, weekly methotrexate, and monthly intravenous immunoglobulin and enzyme replacement therapy at 40 mg/kg/week...
February 7, 2018: Journal of Pediatrics
https://www.readbyqxmd.com/read/29427532/a-pilot-study-of-high-dose-intravenous-immunoglobulin-5-for-autism-impact-on-autism-spectrum-and-markers-of-neuroinflammation
#16
Isaac R Melamed, Melinda Heffron, Alessandro Testori, Kellie Lipe
Research has shown that a subset of the autism spectrum disorder (ASD) population presents with immune dysregulation. To explore this topic further, we investigated the efficacy and tolerability of intravenous immunoglobulin (IVIG) infusion in children with ASD. In this study, participants were recruited based on a diagnosis of autistic disorder, Asperger's disorder, or pervasive developmental disorder not otherwise specified. Participants also showed evidence of immune dysfunction based on abnormal levels of specific biomarkers, including CD40 ligand (CD154), lymphocyte stimulation, and T or B cell dysfunction...
February 10, 2018: Autism Research: Official Journal of the International Society for Autism Research
https://www.readbyqxmd.com/read/29423199/iga-nephropathy-in-greece-data-from-the-registry-of-the-hellenic-society-of-nephrology
#17
Maria Stangou, Marios Papasotiriou, Dimitrios Xydakis, Theodora Oikonomaki, Smaragdi Marinaki, Synodi Zerbala, Constantinos Stylianou, Pantelitsa Kalliakmani, Aimilios Andrikos, Antonia Papadaki, Olga Balafa, Spyridon Golfinopoulos, Georgios Visvardis, Georgios Moustakas, Evangelos Papachristou, Theodora Kouloukourgiotou, Eleni Kapsia, Angeliki Panagiotou, Constantinos Koulousios, Christos Kavlakoudis, Maria Georgopoulou, Stylianos Panagoutsos, Demetrios V Vlahakos, Theophanis Apostolou, Ioannis Stefanidis, Kostas Siamopoulos, Ioannis Tzanakis, Apostolos Papadogiannakis, Eugene Daphnis, Christos Iatrou, John N Boletis, Aikaterini Papagianni, Dimitrios S Goumenos
Background: Natural history, predisposing factors to an unfavourable outcome and the effect of various therapeutic regimens were evaluated in a cohort of 457 patients with immunoglobulin A nephropathy (IgAN) and follow-up of at least 12 months. Methods: Patients with normal renal function and proteinuria <1 g/24 h as well as those with serum creatinine (SCr) >2.5 mg/dL and/or severe glomerulosclerosis received no treatment. Patients with normal or impaired renal function and proteinuria >1 g/24 h for >6 months received daily oral prednisolone or a 3-day course of intravenous (IV) methylprednisolone followed by oral prednisolone per os every other day or a combination of prednisolone and azathioprine...
February 2018: Clinical Kidney Journal
https://www.readbyqxmd.com/read/29420464/potential-confounding-of-diagnosis-of-rabies-in-patients-with-recent-receipt-of-intravenous-immune-globulin
#18
Neil M Vora, Lillian A Orciari, J Bradford Bertumen, Inger Damon, James A Ellison, Vance G Fowler, Richard Franka, Brett W Petersen, P S Satheshkumar, Stephen M Schexnayder, Todd G Smith, Ryan M Wallace, Susan Weinstein, Carl Williams, Pamela Yager, Michael Niezgoda
Rabies is an acute encephalitis that is nearly always fatal. It is caused by infection with viruses of the genus Lyssavirus, the most common of which is Rabies lyssavirus. The Council of State and Territorial Epidemiologists (CSTE) defines a confirmed human rabies case as an illness compatible with rabies that meets at least one of five different laboratory criteria.* Four of these criteria do not depend on the patient's rabies vaccination status; however, the remaining criterion, "identification of Lyssavirus-specific antibody (i...
February 9, 2018: MMWR. Morbidity and Mortality Weekly Report
https://www.readbyqxmd.com/read/29416316/durvalumab-an-investigational-anti-pd-l1-monoclonal-antibody-for-the-treatment-of-urothelial-carcinoma
#19
REVIEW
Izak Faiena, Amy L Cummings, Anna M Crosetti, Allan J Pantuck, Karim Chamie, Alexandra Drakaki
Our expanding knowledge of immunotherapy for solid tumors has led to an explosion of clinical trials aimed at urothelial carcinoma. The primary strategy is centered on unleashing the immune system by releasing the inhibitory signals propagated by programmed cell death-1 (PD-1) and its ligand programmed cell death ligand-1 (PD-L1). Many antibody constructs have been developed to block these interactions and are used in clinical trials. The Food and Drug Administration has already approved a number of checkpoint inhibitors such as anti-cytotoxic T-lymphocyte-associated protein 4 (CTLA4) monoclonal antibodies including ipilimumab; anti-PD-1 monoclonal antibodies including nivolumab and pembrolizumab; anti-PD-L1 antibodies including atezolizumab, avelumab, and durvalumab...
2018: Drug Design, Development and Therapy
https://www.readbyqxmd.com/read/29415936/-recurrent-invasive-pneumococcal-disease-in-a-patient-with-igg-%C3%AE%C2%BA-smoldering-multiple-myeloma
#20
Masahiro Chiba, Kazuo Oshimi, Toshihiro Matsukawa, Kouhei Okada, Takuto Miyagishima
A 68-year-old female with smoldering multiple myeloma (IgG-κ type) was admitted to the hospital owing to general fatigue, fever, and pain in the right leg. On the day following admission, she developed shock, and a blood culture revealed Streptococcus pneumoniae. She was diagnosed with septic shock and invasive pneumococcal disease (IPD). She received antibiotics and intravenous immunoglobulin and improved after several days. She had a history of recurrent IPD and had received the pneumococcal polysaccharide vaccine 23 (PPSV23) 2 years earlier...
2018: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
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