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https://www.readbyqxmd.com/read/28648664/high-dose-ivig-successfully-reduces-high-rhgaa-igg-antibody-titers-in-a-crim-negative-infantile-pompe-disease-patient
#1
Mugdha Rairikar, Zoheb B Kazi, Ankit Desai, Crista Walters, Amy Rosenberg, Priya S Kishnani
Alglucosidase alfa (rhGAA) has altered the course of an otherwise fatal outcome in classic infantile Pompe disease (IPD), which presents with cardiomyopathy and severe musculoskeletal involvement. However, the response to therapy is determined by several factors including the development of high and sustained antibody titers (HSAT) to rhGAA. Cross-reactive immunologic material (CRIM) negative patients are at the highest risk for development of HSAT. Immune tolerance induction (ITI) with methotrexate, rituximab, and intravenous immunoglobulin (IVIG) has been largely successful in preventing the immune response and in achieving tolerance when done in conjunction with enzyme replacement therapy (ERT) initiation...
May 18, 2017: Molecular Genetics and Metabolism
https://www.readbyqxmd.com/read/28648573/personalized-treatment-with-immunoadsorption-and-intravenous-immunoglobulin-in-a-case-of-severe-rh-alloimmunization-during-pregnancy-unresponsive-to-plasma-exchange
#2
Anna Colpo, Tiziana Tison, Maria Teresa Gervasi, Cinzia Vio, Maria Vicarioto, Giustina De Silvestro, Piero Marson
INTRODUCTION: Despite prophylaxis, a small proportion of RhD-negative women may develop anti-D antibodies after a sensitizing event occurring during pregnancy or delivery of a D-positive baby. Intrauterine transfusion (IUT) is the treatment of choice in case of fetal anemia, but it cannot be performed early during pregnancy. Combined treatment with therapeutic plasma-exchange (TPE) and intravenous immunoglobulin (IVIG) can avoid or delay IUT. Immunoadsorption (IA) could represent a more effective treatment in selected cases...
June 6, 2017: Transfusion and Apheresis Science
https://www.readbyqxmd.com/read/28644735/fetal-and-neonatal-alloimmune-thrombocytopenia-evidence-based-antenatal-and-postnatal-management-strategies
#3
Dian Winkelhorst, Dick Oepkes, Enrico Lopriore
Fetal and neonatal alloimmune thrombocytopenia (FNAIT) is a relatively rare but potentially lethal disease, leading to severe bleeding complications in 1 in 11.000 newborns. It is the leading cause of thrombocytopenia in healthy term-born neonates. Areas covered: This review summarizes the antenatal as well as postnatal treatment, thus creating a complete overview of all possible management strategies for FNAIT. Expert commentary: The optimal antenatal therapy in order to prevent bleeding complications in pregnancies complicated by FNAIT is non-invasive treatment with weekly intravenous immunoglobulin (IVIG)...
June 23, 2017: Expert Review of Hematology
https://www.readbyqxmd.com/read/28640458/long-term-graft-survival-in-patients-with-chronic-antibody-mediated-rejection-with-persistent-peritubular-capillaritis-treated-with-intravenous-immunoglobulin-and-rituximab
#4
William R Mulley, Louis L Huang, Sharmila Ramessur Chandran, Anthony Longano, Liv A R Amos, Kevan R Polkinghorne, David J Nikolic-Paterson, John Kanellis
Chronic antibody mediated rejection (cAMR) is the major cause of premature renal allograft loss and is resistant to therapy with 12-month graft failure of up to 50% reported. We examined the duration of graft survival and associates of graft failure in patients with donor specific antibody positive cAMR and treatment-resistant peritubular capillaritis between June 2007 and October 2010. Those with advanced interstitial fibrosis (n=5) were excluded. Included patients (n=24) received treatment with high-dose intravenous immunoglobulin and fixed-dose rituximab (500mg)...
June 22, 2017: Clinical Transplantation
https://www.readbyqxmd.com/read/28639937/a-multicentre-randomised-controlled-trial-of-intravenous-immunoglobulin-compared-with-standard-therapy-for-the-treatment-of-transverse-myelitis-in-adults-and-children-strive
#5
Michael Absoud, Peter Brex, Olga Ciccarelli, Onyinye Diribe, Gavin Giovannoni, Jennifer Hellier, Rosemary Howe, Rachel Holland, Joanna Kelly, Paul McCrone, Caroline Murphy, Jackie Palace, Andrew Pickles, Michael Pike, Neil Robertson, Anu Jacob, Ming Lim
BACKGROUND: Transverse myelitis (TM) is an immune-mediated disorder of the spinal cord that affects adults and children and that causes motor, sensory and autonomic dysfunction. There is a prolonged recovery phase, which may continue for many years. Neuromyelitis optica (NMO) is an uncommon relapsing inflammatory central nervous system condition in which TM can be the first presenting symptom. As TM and NMO affect many patients in the prime of their working life, the disorder can impose a significant demand on health resources...
May 2017: Health Technology Assessment: HTA
https://www.readbyqxmd.com/read/28634589/hla-class-ia-and-ib-polyreactive-anti-hla-e-igg2a-monoclonal-antibodies-tfl-006-and-tfl-007-suppress-anti-hla-igg-production-by-cd19-b-cells-and-proliferation-of-cd4-t-cells-while-upregulating-tregs
#6
REVIEW
Mepur H Ravindranath
The anti-HLA-E IgG2a mAbs, TFL-006 and TFL-007, reacted with all HLA-I antigens, similar to the therapeutic preparations of IVIg. Indeed, IVIg lost its HLA reactivity, when its HLA-E reactivity was adsorbed out. US-FDA approved IVIg to reduce antibodies in autoimmune diseases. But the mechanism underlying IVIg-mediated antibody reduction could not be ascertained due to the presence of other polyclonal antibodies. In spite of it, the cost prohibitive high or low IVIg is administered to patients waiting for donor organ and for allograft recipients for lowering antiallograft antibodies...
2017: Journal of Immunology Research
https://www.readbyqxmd.com/read/28631531/interleukin-1-receptor-antagonist-anakinra-intravenous-immunoglobulin-and-corticosteroids-in-the-management-of-critically-ill-adult-patients-with-hemophagocytic-lymphohistiocytosis
#7
Philipp Wohlfarth, Hermine Agis, Guido A Gualdoni, Johannes Weber, Thomas Staudinger, Peter Schellongowski, Oliver Robak
BACKGROUND: Hemophagocytic lymphohistiocytosis (HLH) causes multiple organ dysfunction frequently leading to intensive care unit (ICU) referral and/or death. We report on a series of critically ill adult patients treated with a non-etoposide-based regimen including interleukin 1 antagonist anakinra, intravenous immunoglobulin (IVIG), and/or corticosteroids (CS) for HLH. METHODS: Eight adult (≥18 years) ICU patients having received treatment with anakinra ± IVIG ± CS for HLH between March 2014 and March 2016 at a large tertiary care university hospital (Medical University of Vienna, Vienna, Austria) were retrospectively analyzed...
January 1, 2017: Journal of Intensive Care Medicine
https://www.readbyqxmd.com/read/28630121/a-novel-recombinant-human-thrombopoietin-therapy-for-the-management-of-immune-thrombocytopenia-in-pregnancy
#8
Zhangyuan Kong, Ping Qin, Shan Xiao, Hai Zhou, Hong Li, Renchi Yang, Xiaofan Liu, Jianmin Luo, Zhichun Li, Guochao Ji, Zhongguang Cui, Yusheng Bai, Yuxia Wu, Linlin Shao, Jun Peng, Jun Ma, Ming Hou
The aim of this study was to determine the safety and efficacy of recombinant human thrombopoietin (rhTPO) for the management of immune thrombocytopenia (ITP) during pregnancy. Pregnant ITP patients were enrolled in the study if they had a platelet count of < 30 × 10(9)/L, were experiencing bleeding manifestations, had failed to respond to corticosteroids and/or intravenous immunoglobulin (IVIG), and had developed refractoriness to platelet transfusion. Thirty-one patients received rhTPO at an initial dose of 300U/kg once daily for 14 days...
June 19, 2017: Blood
https://www.readbyqxmd.com/read/28626510/multispecialty-rating-of-evidence-based-conditions-for-intravenous-immunoglobulin-therapy-using-a-3-axis-prioritization-algorithm
#9
Jordan S Orange, Matt Johnson, Barb Lennert, Katarzyna Shields, Michael Eaddy
BACKGROUND: A 3-axis prioritization algorithm was proposed and was evaluated in a US multispecialist pilot study to obtain uniform consensus regarding effective practices for the use of intravenous immunoglobulin (IVIG) therapy. OBJECTIVE: The primary objective was to use consensus-building methodologies to rate disease states for IVIG utilization while considering disease severity and the efficacy of alternative therapeutic options to IVIG from the perspective of US multispecialists...
May 2017: American Health & Drug Benefits
https://www.readbyqxmd.com/read/28623282/a-nationwide-study-of-severe-and-protracted-diarrhoea-in-patients-with-primary-immunodeficiency-diseases
#10
Wen-I Lee, Chien-Chang Chen, Tang-Her Jaing, Liang-Shiou Ou, Chuen Hsueh, Jing-Long Huang
Diarrhoea lasting longer than 14 days and failing to respond to conventional management is defined as severe and protracted diarrhoea (SD). In this study, we investigated the prevalence, pathogens and prognosis of SD in primary immunodeficiency diseases (PIDs). Among 246 patients with predominantly paediatric-onset PIDs from 2003-2015, 21 [Btk (six), IL2RG (four), WASP, CD40L, gp91 (three each), gp47, RAG2 (one each)] and five [CVID (four), SCID (one)] without identified mutations had SD before prophylactic treatment...
June 16, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28622710/effect-of-intravenous-immunoglobulin-on-th1-and-th2-lymphocytes-and-improvement-of-pregnancy-outcome-in-recurrent-pregnancy-loss-rpl
#11
Majid Ahmadi, Samaneh Abdolmohammadi-Vahid, Mahnaz Ghaebi, Leili Aghebati-Maleki, Amir Afkham, Shahla Danaii, Sedigheh Abdollahi-Fard, Lida Heidari, Farhad Jadidi-Niaragh, Vahid Younesi, Mohammad Nouri, Mehdi Yousefi
BACKGROUND: Women with elevated natural killer (NK) cell frequency and function during pregnancy, suffer from recurrent pregnancy loss (RPL). In the present study, the possible effect of intravenous immunoglobulin (IVIG) administration on Th1 and Th2 cell frequency, cytokine secretion, and expression of transcription factors is compared between RPL patients and control group. MATERIALS AND METHODS: Totally, 44 women with a history of RPL (32 women as treated group and 12 as control group) were enrolled in the study...
August 2017: Biomedicine & Pharmacotherapy, Biomédecine & Pharmacothérapie
https://www.readbyqxmd.com/read/28620559/ivig-associated-aseptic-meningitis-in-a-renal-transplant-patient
#12
Tamara Wanigasekera, Rachel J Grainger, Donal J Sexton, Colm Magee
The management of antibody-mediated rejection in renal transplant recipients involves plasmapheresis with IVIG. Aseptic meningitis is a rare adverse effect of IVIG therapy and is a diagnosis of exclusion. We report a case of a renal transplant patient who developed IVIG associated aseptic meningitis in the context of management of antibody-mediated rejection, four years after transplantation.
2017: Case Reports in Transplantation
https://www.readbyqxmd.com/read/28620050/engineering-the-fragment-crystallizable-fc-region-of-human-igg1-multimers-and-monomers-to-fine-tune-interactions-with-sialic-acid-dependent-receptors
#13
Patricia A Blundell, Ngoc Phuong Lan Le, Joel Allen, Yasunori Watanabe, Richard J Pleass
Multimeric fragment crystallizable (Fc) regions and Fc-fusion proteins are actively being explored as biomimetic replacements for intravenous immunoglobulin (IVIG) therapy, which is deployed to manage many diseases and conditions but is expensive and not always efficient. The Fc region of human IgG1 (IgG1-Fc) can be engineered into multimeric structures (hexa-Fcs) that bind their cognate receptors with high avidity. The critical influence of the unique N-linked glycan attached at Asn-297 on the structure and function of IgG1-Fc is well documented; however, whether the N-linked glycan has a similarly critical role in multimeric, avidly binding Fcs is unknown...
June 15, 2017: Journal of Biological Chemistry
https://www.readbyqxmd.com/read/28619520/cardiac-complications-earlier-treatment-and-initial-disease-severity-in-kawasaki-disease
#14
Joseph Y Abrams, Ermias D Belay, Ritei Uehara, Ryan A Maddox, Lawrence B Schonberger, Yosikazu Nakamura
OBJECTIVES: To assess if observed higher observed risks of cardiac complications for patients with Kawasaki disease (KD) treated earlier may reflect bias due to confounding from initial disease severity, as opposed to any negative effect of earlier treatment. STUDY DESIGN: We used data from Japanese nationwide KD surveys from 1997 to 2004. Receipt of additional intravenous immunoglobulin (IVIG) (data available all years) or any additional treatment (available for 2003-2004) were assessed as proxies for initial disease severity...
June 12, 2017: Journal of Pediatrics
https://www.readbyqxmd.com/read/28614191/anti-cd20-blocker-rituximab-in-kidney-transplantation
#15
Puneet Sood, Sundaram Hariharan
Rituximab is a chimeric anti-CD20 monoclonal protein used in various clinical scenarios in kidney transplant recipients. However, its evidence-based use there remains limited due to lack of controlled studies, limited sample size, short follow-up and poorly defined endpoints. Rituximab is indicated for CD20+ PTLD. It may be beneficial for treating recurrent MN and recurrent allograft ANCA vasculitis and possibly for recurrent FSGS. Rituximab, in combination with IVIG/PP, appears to decrease antibody level and increase the odds of transplantation in sensitized recipients...
June 13, 2017: Transplantation
https://www.readbyqxmd.com/read/28610606/biologic-therapies-for-refractory-juvenile-dermatomyositis-five-years-of-experience-of-the-childhood-arthritis-and-rheumatology-research-alliance-in-north-america
#16
C H Spencer, K Rouster-Stevens, H Gewanter, G Syverson, R Modica, K Schmidt, H Emery, C Wallace, S Grevich, K Nanda, Y D Zhao, S Shenoi, S Tarvin, S Hong, C Lindsley, J E Weiss, M Passo, K Ede, A Brown, K Ardalan, W Bernal, M L Stoll, B Lang, R Carrasco, C Agaiar, L Feller, H Bukulmez, R Vehe, H Kim, H Schmeling, D Gerstbacher, M Hoeltzel, B Eberhard, R Sundel, S Kim, A M Huber, A Patwardhan
BACKGROUND: The prognosis of children with juvenile dermatomyositis (JDM) has improved remarkably since the 1960's with the use of corticosteroid and immunosuppressive therapy. Yet there remain a minority of children who have refractory disease. Since 2003 the sporadic use of biologics (genetically-engineered proteins that usually are derived from human genes) for inflammatory myositis has been reported. In 2011-2016 we investigated our collective experience of biologics in JDM through the Childhood Arthritis and Rheumatology Research Alliance (CARRA)...
June 13, 2017: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/28602874/intravenous-immunoglobulins-improve-survival-in-monoclonal-gammopathy-associated-systemic-capillary-leak-syndrome
#17
Marc Pineton de Chambrun, Marie Gousseff, Wladimir Mauhin, Jean-Christophe Lega, Marc Lambert, Sophie Rivière, Antoine Dossier, Marc Ruivard, François Lhote, Gilles Blaison, Laurent Alric, Christian Agard, David Saadoun, Julie Graveleau, Martin Soubrier, Marie-Josée Lucchini-Lecomte, Christine Christides, Annick Bosseray, Hervé Levesque, Jean-François Viallard, Nathalie Tieulie, Pierre-Yves Lovey, Sylvie Le Moal, Béatrice Bibes, Giuseppe Malizia, Pierre Abgueguen, François Lifermann, Jacques Ninet, Pierre-Yves Hatron, Zahir Amoura
BACKGROUND: Monoclonal gammopathy-associated systemic capillary-leak syndrome, also known as Clarkson disease, is a rare condition characterized by recurrent life-threatening episodes of capillary hyper-permeability in the context of a monoclonal gammopathy. This study was conducted to better describe the clinical characteristics, natural history, and long-term outcome of monoclonal gammopathy-associated systemic capillary-leak syndrome. METHODS: We conducted a cohort analysis of all patients included in the European Clarkson disease (EurêClark) registry between January 1997 and March 2016...
June 8, 2017: American Journal of Medicine
https://www.readbyqxmd.com/read/28598267/interaction-of-immunoglobulin-with-cytomegalovirus-infected-cells
#18
Nobuyasu Aiba, Atsuko Shiraki, Misako Yajima, Yukari Oyama, Yoshihiro Yoshida, Ayumu Ohno, Hiroshi Yamada, Masaya Takemoto, Tohru Daikoku, Kimiyasu Shiraki
Intravenous immunoglobulin (IVIG) is used to treat or prevent severe viral infection, especially cytomegalovirus (CMV) infections. IVIG was characterized to understand its interaction with CMV-infected cells. IVIG retarded CMV spread and reduced virus yields depending on the neutralizing (NT) antibody titer. Immediate early protein synthesis was reduced by IVIG in 3 to 15 h, and IVIG specifically reduced the ratio of 66/68k protein synthesis among immediate early proteins in an NT antibody-dependent manner between 4 and 8 h after infection, indicating that antigenic modulation of CMV-infected cells by IVIG reduced viral protein synthesis and virus production...
June 9, 2017: Viral Immunology
https://www.readbyqxmd.com/read/28597710/comparing-treatment-options-for-chronic-inflammatory-neuropathies-and-choosing-the-right-treatment-plan
#19
Eduardo Nobile-Orazio, Francesca Gallia, Fabrizia Terenghi, Mariangela Bianco
Chronic inflammatory neuropathies are disorders caused by an immune response to peripheral nerve. They include chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), multifocal motor neuropathy (MMN) and neuropathy associated with anti-MAG IgM monoclonal gammopathy and other less frequent neuropathies. Several immune therapies have been proven to be effective in these neuropathies even if the best therapeutic option is still unsettled. Areas covered: The authors reviewed the literature to compare the efficacy and safety of currently used immune therapies in these neuropathies...
June 9, 2017: Expert Review of Neurotherapeutics
https://www.readbyqxmd.com/read/28589057/treatment-of-sydenham-s-chorea-a-review-of-the-current-evidence
#20
REVIEW
Shannon L Dean, Harvey S Singer
BACKGROUND: Sydenham's chorea (SC), the neurologic manifestation of rheumatic fever, remains the most prevalent form of chorea in children. Suggested treatments of chorea in SC include prophylactic penicillin, symptomatic (antipsychotic and anticonvulsant) medications, and immunomodulatory therapy (steroids, intravenous immunoglobulin (IVIG), and plasma exchange). In this manuscript, we undertook a systematic review of the published literature to examine the data supporting these therapeutic recommendations...
2017: Tremor and Other Hyperkinetic Movements
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