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Jing Du, Ying Wang, Yan-Chun Li, Tong-Tong Wang, Yong-Lie Zhou, Zhen-Hua Ying
Systemic lupus erythematosus (SLE) is an autoimmune disease that affects many organs, but multisystem dysfunction is rare. Here, we report a case of a 29-year-old woman who was initially diagnosed with SLE complications including lupus nephritis, lupus encephalopathy, renal hypertension, thrombocytopenia, anaemia and hyperkalaemia. She recovered following treatment with high dose methylprednisolone, intravenous immunoglobulin (IVIG) and continuous renal replacement therapy (CRRT). However, a few days after hospital discharge, she developed gastrointestinal bleeding...
January 1, 2018: Journal of International Medical Research
Michael R Pranzatelli, Tyler J Allison, Elizabeth D Tate
INTRODUCTION: Flow cytometric cerebrospinal fluid (CSF) lymphocyte subset analysis has improved the diagnosis of neuroinflammation and identified multiple markers of inflammation in opsoclonus-myoclonus syndrome (OMS). The aim of this exploratory, retrospective study was to analyze the effect of immunotherapy on these markers to determine which agents are disease modifying. METHODS: Cross-sectional immunological observations were made in an IRB-approved case-control study, and patients were treated empirically...
March 5, 2018: European Journal of Paediatric Neurology: EJPN
Giovanni Cirillo, Vincenzo Todisco, Gioacchino Tedeschi
OBJECTIVE: To assess the long-term effects of subcutaneous immunoglobulin (SCIg) on neurophysiological and clinical parameters in patients affected by chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). METHODS: 16 drug-naïve CIDP patients, fulfilling the clinical and neurophysiological criteria for typical CIDP, were treated with one cycle of intravenous immunoglobulin (IVIg) (0.4 g/kg/day for 5 consecutive days) and then shifted to the subcutaneous treatment (SCIg) (0...
February 19, 2018: Clinical Neurophysiology: Official Journal of the International Federation of Clinical Neurophysiology
Karl Migally, Elizabeth A Braunlin, Lei Zhang, Bryce A Binstadt
BACKGROUND: High-dose aspirin (HDA) is used with intravenous immunoglobulin (IVIg) in Kawasaki Disease (KD). Practice regarding HDA varies, and it is unclear whether HDA duration affects long-term course. METHODS: We retrospectively studied KD patients at our hospital over 10 years. Patients were categorized as having received HDA for 0, 1-7, or >7 days. Primary outcome was maximum coronary Z-score at diagnosis and follow-up; secondary outcomes included inflammatory markers...
March 19, 2018: Pediatric Research
Carolien Zwiers, Mirjam Ea Scheffer-Rath, Enrico Lopriore, Masja de Haas, Helen G Liley
BACKGROUND: Exchange transfusion and phototherapy have traditionally been used to treat jaundice and avoid the associated neurological complications. Because of the risks and burdens of exchange transfusion, intravenous immunoglobulin (IVIg) has been suggested as an alternative therapy for alloimmune hemolytic disease of the newborn (HDN) to reduce the need for exchange transfusion. OBJECTIVES: To assess the effect and complications of IVIg in newborn infants with alloimmune HDN on the need for and number of exchange transfusions...
March 18, 2018: Cochrane Database of Systematic Reviews
Sait Karaman, Semiha Bahçeci Erdem, Nesrin Gülez, Ferah Genel
BACKGROUND: Patients with unclassified hypogammaglobulinemia (UCH) constitute a diagnostic and therapeutic dilemma, because information concerning the clinical and immunological characteristics of these patients is insufficient. OBJECTIVE: To evaluate B-cell subsets in cases with UCH and common variable immunodeficiency (CVID) and their association with treatment requirement in UCH patients. METHODS: The study included 41 UCH, 25 CVID, and 36 healthy individuals between the ages of 4-18 years...
March 2018: Iranian Journal of Immunology: IJI
Keiichi Koizumi, Minako Hoshiai, Nobuyuki Katsumata, Takako Toda, Hiroaki Kise, Yohei Hasebe, Yosuke Kono, Yuto Sunaga, Masashi Yoshizawa, Atsushi Watanabe, Keiko Kagami, Masako Abe, Kanji Sugita
BACKGROUND: The effect of infliximab (IFX) on immune cells has not been fully reported in Kawasaki disease (KD). To investigate the mechanism of IFX in KD, we examined changes in the abundance of CD14+ CD16+ activated monocytes, regulatory T cells (Treg ), and T-helper cell type 17 (Th17) cells following treatment with IFX. METHODS: We collected peripheral blood from patients with intravenous immunoglobulin (IVIG)-resistant KD and analyzed the absolute counts of CD14+ CD16+ monocytes, Treg cells (CD4+ CD25+ FOXP3+ ) and Th17 cells (CD4+ IL-17A+ ) by flow cytometry...
March 15, 2018: Pediatrics International: Official Journal of the Japan Pediatric Society
Jeffrey A Allen, Kenneth C Gorson, Deborah Gelinas
Introduction: We explored adherence to the European Federation of Neurological Societies/Peripheral Nerve Society (EFNS/PNS) guidelines for the diagnosis and treatment of chronic inflammatory demyelinating polyneuropathy (CIDP) by reviewing data from a specialty pharmacy database. Materials and Methods: Clinical and electrophysiologic data were reviewed for 65 consecutive patients treated with intravenous immunoglobulin (IVIG) for CIDP. Three neuromuscular neurologists independently classified cases according to EFNS/PNS criteria as (1) fulfilling CIDP criteria; (2) non-CIDP (neither clinical nor electrophysiologic criteria met); or (3) unknown (insufficient information)...
March 2018: Brain and Behavior
Sandesh Parajuli, Didier A Mandelbrot, Brenda Muth, Maha Mohamed, Neetika Garg, Fahad Aziz, Robert R Redfield, Weixiong Zhong, Brad C Astor, Arjang Djamali
Background: There is limited information on treatment strategies and monitoring strategies for late antibody-mediated rejection (ABMR) after kidney transplantation. Methods: In this observational and nonrandomized study, we compared 78 patients diagnosed with late ABMR (>3 months after transplant) who were treated with standard of care steroids/IVIG (n = 38) ± rituximab (n = 40) at our center between March 1, 2013 and December 31, 2016. All patients had follow-up biopsy and donor-specific antibodies (DSA) monitoring within 3 to 12 weeks...
December 2017: Transplantation Direct
Eun Song Song, Somy Yoon, Joo Hyun Cho, Eun Mi Yang, Hwa Jin Cho, Young Youn Choi, Jae Sook Ma, Gwang Hyeon Eom, Young Kuk Cho
BACKGROUND: Kawasaki disease (KD) is known as systemic vasculitis, and more than half of the patients with KD have myocarditis, which can induce ventricular dysfunction. In this study, we evaluate left ventricular (LV) dysfunction in patients with KD based on the myocardial performance index (MPI) using pulse Doppler (PD) and tissue Doppler imaging (TDI), from the acute to convalescent phases. METHODS: We retrospectively studied 89 children diagnosed with KD from January 2010 to August 2012...
March 12, 2018: World Journal of Pediatrics: WJP
Rolf Spirig, Ian K Campbell, Sandra Koernig, Chao-Guang Chen, Bonnie J B Lewis, Rebecca Butcher, Ineke Muir, Shirley Taylor, Jenny Chia, David Leong, Jason Simmonds, Pierre Scotney, Peter Schmidt, Louis Fabri, Andreas Hofmann, Monika Jordi, Martin O Spycher, Susann Cattepoel, Jennifer Brasseit, Con Panousis, Tony Rowe, Donald R Branch, Adriana Baz Morelli, Fabian Käsermann, Adrian W Zuercher
Activation of Fc receptors and complement by immune complexes is a common important pathogenic trigger in many autoimmune diseases and so blockade of these innate immune pathways may be an attractive target for treatment of immune complex-mediated pathomechanisms. High-dose IVIG is used to treat autoimmune and inflammatory diseases, and several studies demonstrate that the therapeutic effects of IVIG can be recapitulated with the Fc portion. Further, recent data indicate that recombinant multimerized Fc molecules exhibit potent anti-inflammatory properties...
March 12, 2018: Journal of Immunology: Official Journal of the American Association of Immunologists
Philip Crispin, Maria Burgess, Amy Beath
Although manufacturers recommend varying infusion rates for differing intravenous immunoglobulin products (IVIg), there may be improved efficiency and reduced potential for error with the application of a single infusion policy for all IVIg products. During the transition from a 6% to a 10% IVIg, we prospectively evaluated patient reported adverse reactions to IVIg with the 10% product (Intragam 10) given at a rate faster than recommended by the manufacturer. While there was a significant increase in the rate of immediate infusion reactions when compared with the previous IVIg preparation (Intragam P), there was no increase in the rate of reactions post infusion...
February 23, 2018: Transfusion and Apheresis Science
Weiguo Qian, Yunjia Tang, Wenhua Yan, Ling Sun, Haitao Lv
BACKGROUND: Kawasaki disease (KD) is the most common pediatric vasculitis. Several models have been established to predict intravenous immunoglobulin (IVIG) resistance. The present study was aimed to evaluate the efficacy of prediction models using the medical data of KD patients. METHODS: We collected the medical records of patients hospitalized in the Department of Cardiology in Children's Hospital of Soochow University with a diagnosis of KD from Jan 2015 to Dec 2016...
March 9, 2018: Italian Journal of Pediatrics
Isaac R Melamed, Michael Borte, Laurenz Trawnicek, Ai-Lan Kobayashi, Roger H Kobayashi, Alan Knutsen, Sudhir Gupta, William Smits, Anna Pituch-Noworolska, Magdalena Strach, Grazyna Pulka, Hans D Ochs, James N Moy
Intravenous immunoglobulin (IVIG) therapy is commonly used to treat patients with primary antibody deficiency. This prospective, open-label, non-randomised, multicentre, phase III trial investigated the pharmacokinetics of a new 10% liquid IVIG product (panzyga®; Octapharma) in 51 patients aged 2-75 years with common variable immunodeficiency (n = 43) or X-linked agammaglobulinaemia (n = 8). Patients were treated with IVIG 10% every 3 (n = 21) or 4 weeks (n = 30) at a dose of 200-800 mg/kg for 12 months...
March 6, 2018: European Journal of Pharmaceutical Sciences
Lina Ma, Caiyun You, Mikhail Hernandez, Arash Maleki, Andres Lasave, Alexander Schmidt, Andrew Stephenson, Thongzen Zhao, Stephen Anesi, C Stephen Foster
PURPOSE: To assess the long-term efficacy and safety of IVIg monotherapy in patients with recalcitrant ocular cicatricial pemphigoid (OCP). METHODS: A chart review of all OCP patients seen at the Massachusetts Eye Research and Surgery Institution (MERSI) between 2005 and 2015 was completed. Stage was graded by using the Foster grading system. IVIg infusion was 2g/kg/cycle administered in 3 consecutive days monthly. RESULTS: Of 512 OCP patients, 17 patients (34 eyes) treated with IVIg monotherapy were identified...
March 8, 2018: Ocular Immunology and Inflammation
Marzieh Tavakol, Seyed Alireza Mahdaviani, Mir Reza Ghaemi, Mohammad Vaezi, Atosa Dorudinia, Hamidreza Jamaati, Ali Akbar Velayati
Good's syndrome, the adult onset hypogammaglobulinemia associated with thymoma has been explained about six decades ago. It generally presents with recurrent infections and several paraneoplastic syndromes including myasthenia gravis, pure red cell aplasia, connective tissue disorders, superior vena cava, Horner's syndrome, lichen planus and inflammatory bowel disease. Lack of B cell, dysfunction of T cell, CD4+ T cell lymphopenia, reversed CD4/CD8+ T cell ratio, autoantibodies against Th17 related cytokines have been respected as the pathogenesis of the immune dysregulation this syndrome...
February 2018: Iranian Journal of Allergy, Asthma, and Immunology
Masaru Miura, Tohru Kobayashi, Tetsuji Kaneko, Mamoru Ayusawa, Ryuji Fukazawa, Naoya Fukushima, Shigeto Fuse, Kenji Hamaoka, Keiichi Hirono, Taichi Kato, Yoshihide Mitani, Seiichi Sato, Shinya Shimoyama, Junko Shiono, Kenji Suda, Hiroshi Suzuki, Jun Maeda, Kenji Waki, Hitoshi Kato, Tsutomu Saji, Hiroyuki Yamagishi, Aya Ozeki, Masako Tomotsune, Makiko Yoshida, Yohei Akazawa, Kentaro Aso, Shouzaburoh Doi, Yoshi Fukasawa, Kenji Furuno, Yasunobu Hayabuchi, Miyuki Hayashi, Takafumi Honda, Norihisa Horita, Kazuyuki Ikeda, Masahiro Ishii, Satoru Iwashima, Masahiro Kamada, Masahide Kaneko, Hiroshi Katyama, Yoichi Kawamura, Atushi Kitagawa, Akiko Komori, Kenji Kuraishi, Hiroshi Masuda, Shinichi Matsuda, Satoshi Matsuzaki, Sayaka Mii, Tomoyuki Miyamoto, Yuji Moritou, Noriko Motoki, Kiyoshi Nagumo, Tsuneyuki Nakamura, Eiki Nishihara, Yuichi Nomura, Shohei Ogata, Hiroyuki Ohashi, Kenichi Okumura, Daisuke Omori, Tetsuya Sano, Eisuke Suganuma, Tsutomu Takahashi, Shinichi Takatsuki, Atsuhito Takeda, Masaru Terai, Manatomo Toyono, Kenichi Watanabe, Makoto Watanabe, Masaki Yamamoto, Kenichiro Yamamura
Importance: Few studies with sufficient statistical power have shown the association of the z score of the coronary arterial internal diameter with coronary events (CE) in patients with Kawasaki disease (KD) with coronary artery aneurysms (CAA). Objective: To clarify the association of the z score with time-dependent CE occurrence in patients with KD with CAA. Design, Setting, and Participants: This multicenter, collaborative retrospective cohort study of 44 participating institutions included 1006 patients with KD younger than 19 years who received a coronary angiography between 1992 and 2011...
March 5, 2018: JAMA Pediatrics
Cristina João, Vir Singh Negi, Michel D Kazatchkine, Jagadeesh Bayry, Srini V Kaveri
The immunoregulatory and anti-infective properties of normal circulating polyclonal Abs have been exploited for the therapeutic purposes in the form of IVIG as well as several hyperimmune globulins. Current knowledge on the therapeutic use of normal Igs is based on the discoveries made by several pioneers of the field. In this paper, we review the evolution of IVIG over the years. More importantly, the process started as an s.c. replacement in γ globulin-deficient patients, underwent metamorphosis into i.m...
March 15, 2018: Journal of Immunology: Official Journal of the American Association of Immunologists
Anas Alrohimi, Rajive Jassal
Headache is an uncommon symptom in Guillain-Barré syndrome (GBS). We review four clinical settings related to GBS in which headache may be present. We focus on pathophysiological explanations, alerting the clinician to further potential investigations and treatment. Most reports of headache in GBS occur in the context of the posterior reversible encephalopathy syndrome, an increasingly recognized dysautonomia-related GBS complication. Less frequent is headache in the setting of increased intracranial pressure and papilledema (secondary intracranial hypertension), Miller Fisher syndrome, and cerebral venous sinus thrombosis...
March 2018: Canadian Journal of Neurological Sciences. le Journal Canadien des Sciences Neurologiques
Ying-Hsien Huang, Hsing-Chun Kuo, Sung-Chou Li, Xin-Yuan Cai, Shih-Feng Liu, Ho-Chang Kuo
Kawasaki disease (KD) is the most common coronary vasculitis to appear in children with anemia and has been associated with elevated plasma hepcidin levels. We recruited a total of 241 cases, including 18 KD patients, who were tested both prior to receiving intravenous immunoglobulin (IVIG) and at least 3 weeks after IVIG treatment, and 18 febrile controls, who were observed in the Illumina HumanMethylation450 BeadChip study for their CpG markers. The remaining cases consisted of another 92 KD patients and 113 controls that were used for validation by pyrosequencing...
February 28, 2018: Journal of Molecular and Cellular Cardiology
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