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IVIG infusions

Maria Cristina Maggio, Giovanni Corsello, Eugenia Prinzi, Rolando Cimaz
BACKGROUND: Kawasaki disease (KD) is an acute systemic vasculitis of small and middle size arteries; 15-25 % of untreated patients and 5 % of patients treated with intravenous immunoglobulin (IVIG) develop coronary artery lesions (CAL). Many studies tried to find the most effective treatment in the management of resistant KD and to select the risk factors for CAL. Our data are assessed on children from west Sicily, characterized by a genetic heterogeneity. METHODS: We studied the clinical data of 70 KD Sicilian children (36 males: 51 %; 34 females: 49 %), analysed retrospectively, including: demographic and laboratory parameters; echocardiographic findings at diagnosis, at 2, 6 and 8 weeks, and at 1 year after the onset of the illness...
November 2, 2016: Italian Journal of Pediatrics
Wolfgang Frenzel, Stefan Wietek, Tor-Einar Svae, Anette Debes, Daniel Svorc
OBJECTIVE: To evaluate the tolerability and safety of Octagam<sup>®</sup> 5% and 10% across all indications, ages, and treatment regimens, using data from four non-interventional post-authorization safety studies (PASS); this analysis was performed following changes in the preparation of raw material used to manufacture Octagam. METHODS: All four studies included in- and out-patients prescribed Octagam for treatment of their medical condition. Physicians used case report forms to document baseline demographics, Octagam treatment details, and data on the efficacy of Octagam, and recorded all adverse drug reactions (ADRs) and other safety data...
November 2016: International Journal of Clinical Pharmacology and Therapeutics
Motomi Arai
This report concerns a case of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) with frequent treatment-dependent relapses. A 75-year-old male presented with a 2-month history of progressive weakness of the limbs with no sensory symptoms. Neurological examination revealed normal cranial nerves, MRC grade 4 power in the proximal and distal muscles of the limbs, and generalized areflexia. However, the sensory examination results, serum immunoelectrophoresis, anti-HIV antibody, and vitamins B1 and B12 levels were normal...
October 28, 2016: Rinshō Shinkeigaku, Clinical Neurology
Kyle A Williams, Susan E Swedo, Cristan A Farmer, Heidi Grantz, Paul J Grant, Precilla D'Souza, Rebecca Hommer, Liliya Katsovich, Robert A King, James F Leckman
OBJECTIVE: Pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections (PANDAS) are hypothesized to occur as a result of cross-reactive antibodies produced in response to group A streptococcal infections. Previous research suggests that immunomodulatory therapies, such as intravenous immunoglobulin (IVIG), may lead to rapid and sustained symptom improvement in patients with PANDAS. METHOD: A total of 35 children meeting criteria for PANDAS and moderate to severe obsessive-compulsive disorder (OCD) were enrolled in a randomized-entry, double-blind, placebo-controlled, 6-week trial of IVIG (1 g/kg/day on 2 consecutive days), followed by optional open-label treatment for nonresponders, with follow-up at 12 and 24 weeks...
October 2016: Journal of the American Academy of Child and Adolescent Psychiatry
Bernhard Steger, Savitha Madhusudan, Stephen B Kaye, Amira Stylianides, Vito Romano, Sundas E Maqsood, Janice Harper, Sajjad Ahmad
PURPOSE: Despite the availability of systemic immunosuppressants, cicatricial conjunctivitis (CC) remains a potentially blinding ocular surface disease. We aim to describe the combined use of rituximab (RTX) and intravenous immunoglobulin (IVIg) for severe recalcitrant autoimmune CC. METHODS: In this single-center retrospective interventional case series with follow-up between 32 and 65 months, 3 cases with mucous membrane pemphigoid (patients 1-3) and 1 case with linear IgA disease (patient 4) were included...
September 21, 2016: Cornea
Mallory L Downie, Cedric Manlhiot, Giuseppe A Latino, Tanveer H Collins, Nita Chahal, Rae S M Yeung, Brian W McCrindle
OBJECTIVES: To characterize the pattern of temperature response to intravenous immunoglobulin (IVIG) infusion in patients with Kawasaki disease (KD). STUDY DESIGN: Patients nonresponsive to IVIG (axillary temperature ≥37.5°C >24 hours after end of IVIG) were identified. Each patient with IVIG-nonresponsive KD was matched to a control patient with IVIG-responsive KD of the same age, sex, and duration of fever before IVIG. Hourly temperature profiles were obtained from immediately before the start of IVIG infusion until complete defervescence...
December 2016: Journal of Pediatrics
Madhavi Lakkaraja, Jenny C Jin, Karen C Manotas, Cheryl A Vinograd, Polina Ferd, Julia Gabor, Megan Wissert, Richard L Berkowitz, Janice G McFarland, James B Bussel
BACKGROUND: Incompatibility between parental platelet (PLT) antigens may lead to sensitization of mother and development of fetal and neonatal alloimmune thrombocytopenia (FNAIT) resulting in fetal thrombocytopenia. Intravenous immunoglobulin (IVIG) with or without prednisone is the most effective, evidence-based antenatal treatment for subsequent FNAIT-affected pregnancies. IVIG infusion causes hemolysis in other settings, the degree depending upon patient blood groups (BGs). STUDY DESIGN AND METHODS: In ClinicalTrials...
October 2016: Transfusion
Julio César Cambray-Gutiérrez, Ulises Noel García-Ramírez, Leonel Gerardo Del Rivero-Hernández, Patricia López-Pérez, Aurora Chávez-García
BACKGROUND: Common variable immunodeficiency is the most commonly-diagnosed primary immunodeficiency in adults; it is characterized by recurrent sinopulmonary and gastrointestinal infections, and increased incidence of malignancy and autoimmune processes. Many patients begin to have clinical manifestations during reproductive age. CASE REPORT: A 34-year-old woman with 12 weeks of gestation who was diagnosed with common variable immunodeficiency after recurrent episodes of rhinosinusitis, pharyngoamygdalitis, and pneumonia...
July 2016: Revista Alergia Mexico: Organo Oficial de la Sociedad Mexicana de Alergia e Inmunología, A.C
Caroline Galeotti, Srini V Kaveri, Rolando Cimaz, Isabelle Koné-Paut, Jagadeesh Bayry
Kawasaki disease (KD) is an acute febrile childhood inflammatory disease, associated with coronary artery abnormalities. The disease is believed to result from an aberrant inflammatory response to an infectious trigger in a genetically predisposed individual. KD is associated with an endothelial cell injury as a consequence of T cell activation and cytotoxic effects of various proinflammatory cytokines. Intravenous immunoglobulin (IVIG) infusion and aspirin are the standard treatment of acute KD. However, 10-20% of patients show resistance to IVIG therapy and present higher risk of coronary vasculitis...
August 6, 2016: Drug Discovery Today
Miguel Chuquilin, Raghav Govindarajan, Dawn Peck, Esperanza Font-Montgomery
Leigh syndrome is a mitochondrial disease caused by mutations in different genes, including ATP6A for which no known therapy is available. We report a case of adult-onset Leigh syndrome with response to immunotherapy. A twenty year-old woman with baseline learning difficulties was admitted with progressive behavioral changes, diplopia, headaches, bladder incontinence, and incoordination. Brain MRI and PET scan showed T2 hyperintensity and increased uptake in bilateral basal ganglia, respectively. Autoimmune encephalitis was suspected and she received plasmapheresis with clinical improvement...
September 2016: Molecular Genetics and Metabolism Reports
Lars Høj Markvardsen, Ingelise Christiansen, Johannes Jakobsen
BACKGROUND: Intravenous immunoglobulin (IVIG) is recommended treatment for chronic inflammatory demyelinating polyneuropathy (CIDP) and multifocal motor neuropathy (MMN). Recent studies have demonstrated that subcutaneous immunoglobulin (SCIG) is feasible, safe, and effective in both disorders. IVIG leads to transient hemolysis and, consequently, we hypothesized that frequent small doses of SCIG exerts less hemolytic activity than a few larger doses of IVIG. STUDY DESIGN AND METHODS: In an open-label study, 23 three patients treated with IVIG for CIDP or MMN were switched to SCIG at an equal dosage...
July 12, 2016: Transfusion
Dustin Anderson, Cecile Phan, Wendy S Johnston, Zaeem A Siddiqi
We examined the clinical effectiveness of rituximab in fourteen patients with refractory myasthenia gravis (MG). Manual muscle testing (MMT) score was recorded at baseline and followed during the course of the study. Steroid dose, frequency of intravenous immunoglobulin (IVIG) infusions, and plasma exchange (PLEX) were also monitored throughout the duration of the study. All patients responded dramatically to rituximab, as measured by a change in MMT score, prednisone dose, or the frequency of IVIG infusions or PLEX...
July 2016: Annals of Clinical and Translational Neurology
Karen I Gutiérrez-Aguirre, Michael W Stewart, Jason C Sluzevich
BACKGROUND/PURPOSE: To describe the findings and clinical course of a patient with scleromyxedema complicated by retinal vasculitis and macular edema. METHODS: Interventional case report. RESULTS: A 64-year old Caucasian woman with recently diagnosed, biopsy proven scleromyxedema presented with decreased visual acuity (20/50 OD; 20/100 OS) due to retinal vasculitis and macular edema. She received intravitreal bevacizumab and subtenon's triamcinolone acetonide for the macular edema, and bilateral pars plana vitrectomies were required for vitreous hemorrhages...
June 24, 2016: Retinal Cases & Brief Reports
Richard L Wasserman, William Lumry, James Harris, Robyn Levy, Mark Stein, Lisa Forbes, Charlotte Cunningham-Rundles, Isaac Melamed, Ai Lan Kobayashi, Wei Du, Roger Kobayashi
PURPOSE: Immune globulins for IgG supplementation have been produced for over 35 years with essentially no differentiating features regarding their specific antibody composition. Furthermore, the compositions of plasma donor pools used for IG manufacturing are not standardized. While all immune globulin products meet the specifications set by the US FDA for antibodies to pathogens like measles and polio, they have variable levels of antibodies to other important viruses and infectious pathogens, particularly respiratory syncytial virus (RSV)...
August 2016: Journal of Clinical Immunology
Mark Ballow, Paul J Pinciaro, Timothy Craig, Gary Kleiner, James Moy, Hans D Ochs, John Sleasman, William Smits
PURPOSE: The previous studies with Flebogamma(®) 5 % DIF intravenous immunoglobulin (IVIG) contained insufficient numbers of pediatric subjects to fully warrant a pediatric indication by the FDA. The objective of this study was to evaluate the efficacy, safety, and pharmacokinetics of Flebogamma® 5 % DIF for replacement therapy in children (age 2-16) with primary immunodeficiency diseases (PIDD). METHODS: IVIG was administered at eight clinical sites to 24 subjects with well-defined PIDD at a dose of 300-800 mg/kg every 21-28 days for 12 months...
August 2016: Journal of Clinical Immunology
Alessandro Prezzo, Filomena Monica Cavaliere, Caterina Bilotta, Metello Iacobini, Isabella Quinti
The study of the expression of CD16, CD11b and Siglec 9 receptors and the oxidative burst provides insights on polymorphonuclear leukocytes (PMN) functionality in common variable immunodeficiency (CVID) and on the possible effects of intravenous immunoglobulin (IVIg) infusion. We evaluated in vivo before and soon after IVIg administration the CD16, CD11b and Siglec 9 expression on unstimulated and Escherichia coli-stimulated PMN and the oxidative burst induced by Escherichia coli and PMA. The E. coli stimulation up-regulated CD16 and Siglec 9 expression and it induced a strong CD11b up-regulation at baseline and soon after IVIg...
August 2016: Cellular Immunology
Chia-Pei Chou, I-Chun Lin, Kuang-Che Kuo
BACKGROUND: Kawasaki disease is an acute, febrile, self-limiting, inflammatory systemic vasculitis seen in early childhood, most commonly in those below 5 years of age. In Kawasaki disease, the coronary arteries are most commonly affected, which may lead to asymptomatic coronary artery ectasia or formation of an aneurysm. Paroxysmal supraventricular tachycardia(PSVT) is a severe and rare cardiovascular complication of Kawasaki disease. A case of Kawasaki disease presenting with unusual findings, including subdural effusion and PSVT is reported...
2016: BMC Pediatrics
Naushin S Sholapur, Korinne Hamilton, Lianna Butler, Nancy M Heddle, Donald M Arnold
BACKGROUND: A global assessment of patient satisfaction that considers therapeutic effect, toxicities, and convenience is needed to evaluate the acceptability of intravenous immunoglobulin (IVIG) as a treatment for patients with immune thrombocytopenia (ITP). STUDY DESIGN AND METHODS: We performed a cross-sectional pilot study to assess the feasibility and usefulness of a treatment satisfaction questionnaire for adult patients with ITP receiving IVIG in an academic hematology clinic...
July 2016: Transfusion
Clemence Perraudin, Aline Bourdin, Francois Spertini, Jérôme Berger, Olivier Bugnon
PURPOSE: Home-based subcutaneous immunoglobulin (SCIg) therapy is an alternative to hospital-based intravenous infusions (IVIg). However, SCIg requires patient training and long-term support to ensure proper adherence, optimal efficacy and safety. We evaluated if switching patients to home-based SCIg including an interprofessional drug therapy management program (physician, community pharmacist and nurse) would be cost-effective within the Swiss healthcare system. METHODS: A 3-year cost-minimization analysis was performed from a societal perspective comparing monthly IVIg in an outpatient clinic and home-based weekly SCIg including an interprofessional program...
July 2016: Journal of Clinical Immunology
B Cherqaoui, A Chausset, J-L Stephan, E Merlin
OBJECTIVE: Severe gastrointestinal involvement of Henoch-Schönlein purpura (HSP) is rare but potentially life-threatening. Management of severe gastrointestinal involvement in HSP is not codified. Symptomatic care and steroids are a first-line therapy. Nonsteroidal immunomodulatory therapies have been anecdotally used to treat steroid-refractory forms. The aim of this study was to describe the outcome of patients with severe gastrointestinal involvement of HSP who required nonsteroidal immunomodulatory therapy...
June 2016: Archives de Pédiatrie: Organe Officiel de la Sociéte Française de Pédiatrie
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