23,512 results match your criteria Intravenous Immunoglobulin


Human T-cell lymphotropic virus 1/2 and human immunodeficiency virus antibodies identification among transactional sex workers and drug users in the Dominican Republic.

Trans R Soc Trop Med Hyg 2019 Mar 20. Epub 2019 Mar 20.

McGill University Health Centre: Glenn Site, Research Institute, Block E Suite EM 3-3232, Mezzanine 3M, 1001 Boulevard Décaire, Montreal, Quebec, Canada.

Background: Human immunodeficiency virus (HIV) increases the risk of acquiring human T-cell lymphotropic virus (HTLV) and subsequently HTLV's progression to tropical spastic paraparesis/HTLV-1-associated myelopathy (TSP/HAM). Existing data have exclusively reported generalized rates of HIV and HTLV-1 chronic viral infections in the Dominican Republic. To our knowledge, no published studies have focused on the rates of HTLV-1/2 in transactional sex workers and drug users, both higher risk groups, in the Dominican Republic. Read More

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http://dx.doi.org/10.1093/trstmh/trz012DOI Listing

Successful pregnancy in a recipient of an ABO-incompatible renal allograft.

Obstet Med 2019 Mar 7;12(1):42-44. Epub 2018 Mar 7.

Central and Northern Adelaide Renal and Transplantation Service, Royal Adelaide Hospital, Adelaide, SA, Australia.

Kidney transplantation restores fertility in patients with end-stage renal disease, with many successful pregnancies after kidney transplantation being reported. , there are little data regarding pregnancy in women transplanted under modern-era desensitisation protocols that utilise rituximab, plasma exchange and intravenous immunoglobulin, including ABO-incompatible transplants. Pregnancies in ABO-incompatible recipients can pose new challenges from an immunological perspective. Read More

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http://dx.doi.org/10.1177/1753495X17745390DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6416689PMC

Features of Childhood Arterial Ischemic Stroke in China.

Fetal Pediatr Pathol 2019 Mar 19:1-9. Epub 2019 Mar 19.

a Shengjing Hospital of China Medical University , Shenyang , China.

Objective: The aim of this study was to identify the features and risk factors for arterial ischemic stroke (AIS) in children.

Methods: We retrospectively analyzed the initial symptoms, clinical manifestations, risk factors, neuroradiological findings, and treatment data of 75 Chinese children aged between 1 month and 14 years (median 5.7 years) who were diagnosed with AIS in our hospital between 2013 and 2018. Read More

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https://www.tandfonline.com/doi/full/10.1080/15513815.2019.1
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http://dx.doi.org/10.1080/15513815.2019.1588438DOI Listing
March 2019
1 Read

How We Treat Autoimmune Small Fiber Polyneuropathy with Immunoglobulin Therapy.

Eur Neurol 2019 Mar 19;80(5-6):304-310. Epub 2019 Mar 19.

Eastern Virginia Medical School, Norfolk, Virginia, USA.

Intravenous immunoglobulin therapy is FDA approved for the immune-mediated peripheral nerve disorders Guillain-Barre syndrome, chronic inflammatory demyelinating polyneuropathy, and multifocal motor neuropathy. Immunoglobulin therapy has been used increasingly with significant efficacy in the treatment of patients with disabling autoimmune forms of dysautonomia, which are most often small fiber (autonomic and/or sensory) polyneuropathies. It is recognized by most who treat these disorders, however, that patients with autonomic dysfunction treated with intravenous immunoglobulin therapy develop aseptic meningitis or severe lingering headache more frequently than other patient populations when this therapy is dosed in the traditional fashion. Read More

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http://dx.doi.org/10.1159/000498858DOI Listing

[Therapy of pemphigus].

Hautarzt 2019 Mar 18. Epub 2019 Mar 18.

Klinik für Dermatologie, Allergologie und Venerologie, Universitätsklinikum Schleswig-Holstein, Campus Lübeck, Ratzeburger Allee 160, 23538, Lübeck, Deutschland.

Background: Pemphigus diseases are a heterogeneous group of potentially life-threatening autoimmune bullous disorders. Therefore, rapidly acting and effective therapeutic approaches are essential.

Objectives: In this review, current therapeutic options in line with available guidelines are presented and new therapeutic approaches are discussed. Read More

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http://dx.doi.org/10.1007/s00105-019-4385-9DOI Listing

[Epidermolysis bullosa acquisita].

Hautarzt 2019 Mar 18. Epub 2019 Mar 18.

Universitäts-Hautklinik Kiel, Klinik für Dermatologie, Venerologie und Allergologie, UKSH Kiel, Kiel, Deutschland.

Epidermolysis bullosa acquisita (EBA) is a rare acquired subepidermal bullous autoimmune dermatosis, associated with autoantibodies against collagen type VII, the most important component of dermal anchoring fibrils. Blister induction occurs after binding of autoantibodies to collagen type VII, leading to complement activation, recruitment of neutrophils and secretion of proteases. Clinically, the disease is mostly characterized by tense blisters on trauma-exposed body areas which heal with scarring (mechanobullous form of EBA). Read More

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http://dx.doi.org/10.1007/s00105-019-4387-7DOI Listing

Cost-utility analysis comparing hospital-based intravenous immunoglobulin with home-based subcutaneous immunoglobulin in patients with secondary immunodeficiency.

Vox Sang 2019 Mar 18. Epub 2019 Mar 18.

Menzies Health Institute Queensland, Griffith University, Nathan, QLD, Australia.

Background And Objective: Immunoglobulin replacement therapy (IRT) is often used to support patients with primary immunodeficiency disease (PID) and secondary immunodeficiency disease (SID). Home-based subcutaneous immunoglobulin (SCIg) is reported to be a cheaper and more efficient option compared to hospital-based intravenous immunoglobulin (IVIg) for PID. In contrast, there is little information on the cost-effectiveness of IRT in SID. Read More

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http://dx.doi.org/10.1111/vox.12760DOI Listing

Augmented ILT3/LILRB4 Expression of Peripheral Blood Antibody Secreting Cells in the Acute Phase of Kawasaki Disease.

Pediatr Infect Dis J 2019 Apr;38(4):431-438

Department of Pediatrics, Sendai Medical Center, Sendai, Japan.

Background: Kawasaki disease (KD) is an acute, systemic vasculitis syndrome that occurs in children. The clinical symptoms and epidemiologic features of KD strongly suggest that KD is triggered by unidentified infectious agents in genetically predisposed patients. In addition, a number of studies have described the role of B cells in the development of KD. Read More

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http://dx.doi.org/10.1097/INF.0000000000002259DOI Listing

Hirudin ameliorates immunoglobulin A nephropathy by inhibition of fibrosis and inflammatory response.

Ren Fail 2019 Nov;41(1):104-112

c Department of Traditional Chinese Medicine , Sichuan Academy of Medical Science & Sichuan Provincial People's Hospital , Chengdu , PR China.

Immunoglobulin A nephropathy (IgAN) is characterized by mesangial IgA and IgG co-deposition. As the clinical course of IgAN is highly variable, a lot of patients will eventually develop to end-stage renal disease (ESRD) within years. Hirudin, a potent and specific thrombin inhibitor, has been reported to treat IgAN with hematuria, but the mechanism is unclear. Read More

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http://dx.doi.org/10.1080/0886022X.2019.1583113DOI Listing
November 2019

[Use of intravenous and subcutaneous human immunoglobulins].

Soins 2019 Mar;64(833):13-18

Institut IE3M, groupe hospitalier Pitié-Salpêtrière, 47-83, boulevard de l'Hôpital, 75013 Paris, France. Electronic address:

Immunoglobulin preparations are medicines derived from blood used as a replacement therapy for immunodeficiencies or as an immunomodulator. While they are generally well-tolerated, side effects, rarely severe, can nevertheless occur when administered intravenously. They are usually related to an excessive perfusion rate. Read More

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http://dx.doi.org/10.1016/j.soin.2019.01.016DOI Listing

ABO-Incompatible Kidney Transplantation: Low Rates of Infectious Complications and Excellent Patient Survival.

Transplant Proc 2019 Mar 4;51(2):512-516. Epub 2019 Jan 4.

King Faisal Specialist Hospital & Research Center, Department of Kidney and Pancreas Transplant, Riyadh, Saudi Arabia. Electronic address:

Background: A significant gap exists between demand and supply of organs for patients with end-stage renal disease. To increase the donor pool, kidney transplantation is performed across ABO- and HLA-incompatible barriers. ABO-incompatible kidney transplant (ABOi-KT) recipients are at increased risk of antibody-mediated rejection, infection, and mortality. Read More

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http://dx.doi.org/10.1016/j.transproceed.2019.01.002DOI Listing

Seronegative neuromyelitis optica spectrum disorder: severe polysymptomatic presentation with successful treatment response.

BMJ Case Rep 2019 Mar 16;12(3). Epub 2019 Mar 16.

Intermediate Care Unit, Department of Internal Medicine, Centro Hospitalar Universitário de São João, Porto, Portugal.

We report the case of a 50-year-old caucasian man presenting with lumbar pain, bilateral ataxia, central facial palsy, ophthalmoparesis and urinary retention. Cerebral MRI hinted a hyperintensity of the medulla oblongata and cervical medulla suggestive of myelitis. Cerebrospinal fluid displayed lymphocytic pleocytosis and elevated protein concentration. Read More

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http://casereports.bmj.com/lookup/doi/10.1136/bcr-2018-22855
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http://dx.doi.org/10.1136/bcr-2018-228553DOI Listing
March 2019
1 Read

Unexpected worsening of pemphigus vulgaris after rituximab: A report of three cases.

Int Immunopharmacol 2019 Mar 12;71:40-42. Epub 2019 Mar 12.

Autoimmune Bullous Diseases Research Center, Tehran University of Medical Sciences, Tehran, Iran. Electronic address:

During the recent decade, several studies have confirmed the high efficacy of targeting the CD20 molecules using rituximab (RTX). Recently, RTX has been suggested as the first-line treatment of pemphigus vulgaris (PV). In this study, the records of all the PV patients, who had received RTX in Autoimmune Bullous Diseases Research Center, Tehran University of Medical Sciences between the 2009 and 2017, have been reviewed for any sign of disease worsening within three months after treatment. Read More

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http://dx.doi.org/10.1016/j.intimp.2019.02.037DOI Listing
March 2019
1 Read

Immune-Checkpoint-Inhibitor-Induced Severe Autoimmune Encephalitis Treated by Steroid and Intravenous Immunoglobulin.

J Clin Neurol 2019 Mar 11. Epub 2019 Mar 11.

Department of Neurology, Seoul National University, College of Medicine, Seoul, Korea.

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Outcomes and treatment strategies for autoimmunity and hyperinflammation in patients with RAG deficiency.

J Allergy Clin Immunol Pract 2019 Mar 12. Epub 2019 Mar 12.

Division of Allergy and Immunology, Massachusetts General Hospital for Children, Boston, MA, USA. Electronic address:

Background: While autoimmunity and hyperinflammation secondary to recombinase activating gene (RAG) deficiency have been associated with delayed diagnosis and even death, our current understanding is limited primarily to small case series.

Objective: Understand the frequency, severity, and treatment responsiveness of autoimmunity and hyperinflammation in RAG deficiency.

Methods: In reviewing the literature and our own database, we identified 85 patients with RAG deficiency, reported between 2001 and 2016, and compiled the largest case series to date of 63 patients with prominent autoimmune and/or hyperinflammatory pathology. Read More

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http://dx.doi.org/10.1016/j.jaip.2019.02.038DOI Listing

[Management of bullous pemphigoid].

Authors:
M Göbel R Eming

Hautarzt 2019 Mar 12. Epub 2019 Mar 12.

Klinik für Dermatologie und Allergologie, Universitätsklinikum Gießen und Marburg GmbH, Standort Marburg, Baldingerstr., 35043, Marburg, Deutschland.

Background: Bullous pemphigoid, the most common autoimmune blistering disease of the skin in adults, is caused by autoantibodies against hemidesmosomal adhesion proteins (BP180/type XVII collagen and BP230), leading into subepidermal blistering. Therefore patients, mostly older than 70 years, show tight bullae and erosions of the skin and rarely at mucous membranes.

Problem: Usually the disease shows a chronically relapsing course. Read More

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http://dx.doi.org/10.1007/s00105-019-4390-zDOI Listing
March 2019
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First Report of the Italian Registry on Immune-Mediated Congenital Heart Block (Lu.Ne Registry).

Front Cardiovasc Med 2019 28;6:11. Epub 2019 Feb 28.

Struttura Complessa Medicina Interna, ASST Papa Giovanni XXIII, Bergamo, Italy.

Neonatal Lupus (NL) is a rare syndrome caused by placental transfer of maternal anti-SSA/Ro and anti-La/SSB autoantibodies to the fetus. The rarity of this condition requires the establishment of multidisciplinary registries in order to improve our knowledge. Inclusion criteria in this retrospective study were the maternal confirmed positivity for anti-SSA/Ro and/or anti-SSB/La antibodies, and the presence of II or III degree congenital heart block (CHB) or neonatal period (up to 27 days after birth). Read More

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https://www.frontiersin.org/article/10.3389/fcvm.2019.00011/
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http://dx.doi.org/10.3389/fcvm.2019.00011DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6404544PMC
February 2019
2 Reads

Severe Fetal Hemolysis and Cholestasis Due to High-Titer Maternal IgG Anti-A Antibodies.

Pediatrics 2019 Mar 14. Epub 2019 Mar 14.

Department of Experimental Immunohematology, Sanquin, Amsterdam, Netherlands.

ABO blood group incompatibility between mother and fetus can lead to hemolytic disease of the fetus and newborn (HDFN). We present the first case of severe O/A HDFN associated with extremely high-titer (1:32 000) immunoglobulin G anti-A antibodies in a Cameroon mother. Cord blood analysis revealed severe fetal hemolytic anemia and conjugated hyperbilirubinemia. Read More

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http://pediatrics.aappublications.org/lookup/doi/10.1542/ped
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http://dx.doi.org/10.1542/peds.2018-2859DOI Listing
March 2019
3 Reads

Hepatitis A infection related haemophagocytic syndrome: a case report and systematic review.

Trop Doct 2019 Mar 14:49475519834804. Epub 2019 Mar 14.

2 Professor, Department of Gastroenterology, PGIMER, Chandigarh, India.

Haemophagocytic lymphohistiocytosis (HLH) is a clinical syndrome of excessive inflammation and tissue destruction owing to abnormal immune activation. We report an unusual case of haemophagocytosis associated with hepatitis A virus (HAV) infection in a 21-year-old man. This was further complicated by haemolysis secondary to G-6-PD deficiency and fungal sepsis. Read More

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http://dx.doi.org/10.1177/0049475519834804DOI Listing
March 2019
2 Reads

Systemic Epstein-Barr Virus-positive T-Cell Lymphoma of Childhood Presentation With Hemophagocytosis.

J Pediatr Hematol Oncol 2019 Mar 12. Epub 2019 Mar 12.

Department of Anatomy and Cell Biology, School of Medicine, College of Medicine, Taipei Medical University, Taipei, Taiwan.

A 2-year-old Asian girl presented to our facility for the evaluation of thrombocytopenia. She was treated with intravenous immunoglobulin under the impression of immune thrombocytopenia. However, her body temperature spiked and progressive pancytopenia, hepatosplenomegaly, abnormal liver function, coagulopathy, and pulmonary infiltration developed. Read More

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http://dx.doi.org/10.1097/MPH.0000000000001452DOI Listing
March 2019
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Anti-Neurofascin-155 IgG4 antibodies prevent paranodal complex formation in vivo.

J Clin Invest 2019 Mar 14;130. Epub 2019 Mar 14.

Neurofascin-155 (Nfasc155) is an essential glial cell adhesion molecule expressed in paranodal septate-like junctions of peripheral and central myelinated axons. The genetic deletion of Nfasc155 results in the loss of septate-like junctions and in conduction slowing. In humans, IgG4 antibodies against Nfasc155 are implicated in the pathogenesis of chronic inflammatory demyelinating polyneuropathy (CIDP). Read More

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http://dx.doi.org/10.1172/JCI124694DOI Listing
March 2019
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Interpretation and management of positive anti-hepatitis B core antibody tests in immunocompromised pediatric patients.

Transpl Infect Dis 2019 Mar 13:e13074. Epub 2019 Mar 13.

Division of Infectious Diseases, Department of Pediatrics, The Children's Hospital of Philadelphia, Philadelphia, PA.

Intravenous immunoglobulin (IVIg) therapy is increasingly used in the pediatric population, in particular among children with immune-compromising conditions. Pooled immunoglobulin products are routinely tested for hepatitis B surface antigen (HBsAg) and nucleic acid, however screening for hepatitis B core antibody (anti-HBc) is not commonly performed. Thus, the administration of IVIg containing anti-HBc to children with immune-compromising conditions may complicate the interpretation of hepatitis B serologic testing in that a positive anti-HBc test may represent passive transfer of antibody from IVIg or may indicate resolved or chronic hepatitis B infection. Read More

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http://dx.doi.org/10.1111/tid.13074DOI Listing
March 2019
2 Reads

Efficacy of Therapeutic Plasma Exchange in Patients with Severe Refractory Anti-NMDA Receptor Encephalitis.

Neurotherapeutics 2019 Mar 13. Epub 2019 Mar 13.

Department of Neurology, Xuanwu Hospital, Capital Medical University, Beijing, 100053, China.

The objective of the present study was to assess the efficacy of therapeutic plasma exchange (TPE) in patients with severe refractory anti-N-methyl-D-aspartate (anti-NMDA) receptor encephalitis. Patients with severe anti-NMDA receptor encephalitis who showed no improvement after steroids and/or intravenous immunoglobulin treatment for at least 10 days were enrolled. All patients received immunotherapy and were divided into a TPE group and a non-TPE group according to treatment received. Read More

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http://link.springer.com/10.1007/s13311-019-00725-4
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http://dx.doi.org/10.1007/s13311-019-00725-4DOI Listing
March 2019
6 Reads

Electrosurgical debulking of pretibial myxedema of the foot.

Dermatol Online J 2019 Feb 15;25(2). Epub 2019 Feb 15.

Department of Dermatology, Mount Sinai Hospital, New York City, New York.

Pretibial myxedema or thyroid dermopathy constitutes dermal deposition of mucin, primarily hyaluronic acid and chondroitin sulfate. It is a manifestation of autoimmune thyroiditis, seen more in Graves disease than in Hashimoto thyroiditis. The time delay from treatment of hyperthyroidism to appearance of localized myxedema varies from one month to 16 years (mean 5. Read More

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February 2019
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Dry Beriberi Due to Thiamine Deficiency Associated with Peripheral Neuropathy and Wernicke's Encephalopathy Mimicking Guillain-Barré syndrome: A Case Report and Review of the Literature.

Am J Case Rep 2019 Mar 13;20:330-334. Epub 2019 Mar 13.

Department of Clinical Pharmacy, University of Colorado Skaggs School of Pharmacy and Pharmaceutical Sciences, Aurora, CO, USA.

BACKGROUND Beriberi due to thiamine (vitamin B1) deficiency has two clinical presentations. Patients with dry beriberi present with neuropathy, and patients with wet beriberi present with heart failure, with or without neuropathy. Dry beriberi can mimic the most common form of Guillain-Barre syndrome (GBS), an acute inflammatory demyelinating polyradiculoneuropathy (AIDP). Read More

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http://dx.doi.org/10.12659/AJCR.914051DOI Listing
March 2019
4 Reads

Prevention of hemolytic transfusion reactions with intravenous immunoglobulin prophylaxis in U- patients with anti-U.

Transfusion 2019 Mar 12. Epub 2019 Mar 12.

Red Cell Immunohaematology, NHS Blood and Transplant (Filton Centre), Bristol, UK.

Background: The U- phenotype is extremely rare and is found predominantly in black African populations at a frequency of between 0.2 and 1.7%. Read More

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http://dx.doi.org/10.1111/trf.15230DOI Listing
March 2019
1 Read

Adenovirus in Solid Organ Transplant Recipients: Guidelines from the American Society of Transplantation Infectious Diseases Community of Practice.

Clin Transplant 2019 Mar 12:e13527. Epub 2019 Mar 12.

David Geffen School of Medicine, Division of Infectious Diseases, Los Angeles, United States.

These updated guidelines from the Infectious Diseases Community of Practice of the American Society of Transplantation review the diagnosis, prevention, and management of adenovirus infections after solid organ transplantation. Adenovirus is an important cause of infectious complications in both stem cell transplant and SOT patients, causing a range of clinical syndromes including pneumonitis, colitis, and disseminated disease. The current update of the guidelines highlights that adenovirus surveillance testing should not be performed in asymptomatic recipients. Read More

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http://dx.doi.org/10.1111/ctr.13527DOI Listing
March 2019
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BK Polyomavirus in Solid Organ Transplantation - Guidelines from the American Society of Transplantation Infectious Diseases Community of Practice.

Clin Transplant 2019 Mar 12:e13528. Epub 2019 Mar 12.

Division of Transplantation Pathology, Department of Pathology, University of Pittsburgh.

The present AST-IDCOP guidelines update information on BK polyomavirus (BKPyV) infection, replication and disease, which impact kidney transplantation (KT), but rarely non-kidney solid organ transplantation (SOT). As pre-transplant risk factors in KT-donors and -recipients presently do not translate into clinically validated measures regarding organ allocation, antiviral prophylaxis, or screening, all KT-recipients should be screened for BKPyV-DNAemia monthly until month 9, and then every 3 months until 2 years posttransplant. Extended screening after 2 years may be considered in pediatric KT. Read More

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http://dx.doi.org/10.1111/ctr.13528DOI Listing
March 2019
1 Read

Plasma exchange for Alzheimer's disease Management by Albumin Replacement (AMBAR) trial: Study design and progress.

Alzheimers Dement (N Y) 2019 26;5:61-69. Epub 2019 Feb 26.

Bioscience Research Group. Grifols S.A., Barcelona, Spain.

Introduction: Preliminary studies have shown that treatment with plasma exchange (PE) plus therapeutic albumin replacement in patients with Alzheimer's disease (AD) induced mobilization of plasma and cerebrospinal fluid amyloid β protein, associated with an improvement in memory and language functions, as well as the stabilization of brain perfusion, which persisted after treatment discontinuation.

Methods: lzheimer's anagement y lbumin eplacement (AMBAR) is a multicenter, randomized, blinded and placebo-controlled, parallel-group, phase IIb/III trial enrolling patients with mild to moderate AD. The study evaluates PE with different replacement volumes of therapeutic albumin (5% and 20% Albutein, Grifols), with or without intravenous immunoglobulin (Flebogamma 5% DIF, Grifols). Read More

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http://dx.doi.org/10.1016/j.trci.2019.01.001DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6395854PMC
February 2019
2 Reads

Clinical Experience with Rituximab and Intravenous Immunoglobulin for Pretibial myxedema: A Case Series.

Thyroid 2019 Mar 11. Epub 2019 Mar 11.

Mayo Clinic Division of Endocrinology Diabetes Metabolism and Nutrition, 314193, Rochester, Minnesota, United States ;

Background: Severe pretibial myxedema (PTM) can be difficult to manage, highlighting the need to investigate newer therapies. Rituximab and intravenous immunoglobulin (IVIg) have been tried in Graves' orbitopathy. Since PTM and orbitopathy share a similar underlying pathophysiology, we aimed to explore these therapies for progressive PTM. Read More

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http://dx.doi.org/10.1089/thy.2018.0706DOI Listing
March 2019
1 Read

Understanding the burden of refractory myasthenia gravis.

Ther Adv Neurol Disord 2019 1;12:1756286419832242. Epub 2019 Mar 1.

University of Münster, Münster, Germany.

Myasthenia gravis (MG) is an autoantibody-mediated disease that compromises the acetylcholine receptors or associated structures of the postsynaptic membrane of the neuromuscular junction. This leads to impaired neuromuscular transmission and subsequent fluctuating fatigability and weakness of ocular, bulbar, and limb skeletal muscles. Over the past few decades, there have been significant advances in our understanding of the disease pathophysiology and improvements in prognosis due to intensive care medicine and immunomodulation. Read More

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http://dx.doi.org/10.1177/1756286419832242DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6399761PMC
March 2019
1 Read

Phenotype and Genotype of a Cohort of Chinese Children with Early-Onset Protein-Losing Enteropathy.

J Pediatr 2019 Mar 7. Epub 2019 Mar 7.

Department of Gastroenterology, Children's Hospital of Fudan University, Shanghai, China.

Objectives: To examine the phenotypes and perform next-generation sequencing in children with early-onset protein-losing enteropathy.

Study Design: We performed a retrospective review of 27 children with early-onset protein-losing enteropathy. Patients were characterized on clinical, immunologic, and systemic involvements. Read More

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http://dx.doi.org/10.1016/j.jpeds.2018.12.003DOI Listing
March 2019
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Efficacy of primary treatment with immunoglobulin plus ciclosporin for prevention of coronary artery abnormalities in patients with Kawasaki disease predicted to be at increased risk of non-response to intravenous immunoglobulin (KAICA): a randomised controlled, open-label, blinded-endpoints, phase 3 trial.

Lancet 2019 Mar 7. Epub 2019 Mar 7.

Department of Public Health, Chiba University, Chiba, Japan. Electronic address:

Background: Genetic studies have indicated possible involvement of the upregulated calcium-nuclear factor of activated T cells pathway in the pathogenesis of Kawasaki disease. We aimed to assess safety and efficacy of ciclosporin, an immunosuppressant targeting this pathway, for protection of patients with Kawasaki disease against coronary artery abnormalities.

Methods: We did a randomised, open-label, blinded endpoints trial involving 22 hospitals in Japan between May 29, 2014, and Dec 27, 2016. Read More

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http://dx.doi.org/10.1016/S0140-6736(18)32003-8DOI Listing
March 2019
2 Reads

Guillain-Barré syndrome as a complication of hypertensive basal ganglia haemorrhage.

J Clin Neurosci 2019 Mar 6. Epub 2019 Mar 6.

Department of Neurology, Fiona Stanley Hospital, Western Australia, Australia.

We aimed to study the potential relationship of Guillain-Barré syndrome (GBS) and intracranial haemorrhage (ICH). We present a case of a 70-year-old hypertensive woman who developed global weakness, eventually becoming quadriplegic, during an inpatient stay for treatment of a basal ganglia haemorrhagic stroke. Guillain-Barré syndrome was confirmed and treatment initiated. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S09675868183131
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http://dx.doi.org/10.1016/j.jocn.2019.02.018DOI Listing
March 2019
2 Reads

Autoimmune Heparin-Induced Thrombocytopenia: Treatment Obstacles and Challenging Length of Stay.

Am J Case Rep 2019 Mar 9;20:310-313. Epub 2019 Mar 9.

Division of Hematology and Medical Oncology, Brookdale University Hospital, Brooklyn, NY, USA.

BACKGROUND Autoimmune heparin-induced thrombocytopenia (aHIT) refers to a condition, in which antiplatelet factor-4 (PF4) antibodies activate platelets even in the absence of heparin (heparin independent platelet activation). This is a severe hypercoagulable state triggering massive thrombin storm needing additional therapies and aggressive anticoagulation apart from stopping heparin. Thrombocytopenia in these cases seems to be very severe and prolonged compared to classic HIT and poses additional clinical challenges in terms of anticoagulation management. Read More

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http://dx.doi.org/10.12659/AJCR.914575DOI Listing
March 2019
2 Reads

Autoinflammatory constrictive pericarditis and chronic myelomonocytic leukaemia: when one speciality is not enough.

BMJ Case Rep 2019 Mar 7;12(3). Epub 2019 Mar 7.

Cardiovascular Research Unit, Sheffield Teaching Hospitals and University of Sheffield, Sheffield, UK.

We present a case of constrictive pericarditis with concomitant blood and bone marrow appearances of chronic myelomonocytic leukaemia (CMML). Despite surgical treatment with pericardiectomy, the patient deteriorated into multiorgan failure. Pericardial histology disclosed a typical inflammatory picture with no evidence of monocytic or malignant infiltrate. Read More

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http://dx.doi.org/10.1136/bcr-2018-228204DOI Listing
March 2019
1 Read

Necrotizing pneumonia (aetiology, clinical features and management).

Curr Opin Pulm Med 2019 Feb 26. Epub 2019 Feb 26.

Department of Respiratory Medicine, Barts Health NHS Trust.

Purpose Of Review: Necrotizing pneumonia is a severe form of community-acquired pneumonia characterized by rapid progression of consolidation to necrosis and cavitation which may lead to pulmonary gangrene. Morbidity and mortality are high and chronic sequelae are frequent. The lack of guidance supports the review of the latest recommendations in the management of these pneumonias. Read More

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http://dx.doi.org/10.1097/MCP.0000000000000571DOI Listing
February 2019
1 Read

Refractory lupus nephritis: When, why and how to treat.

Autoimmun Rev 2019 Mar 4. Epub 2019 Mar 4.

Vasculitis and Lupus Clinic, Addenbrooke's Hospital, Hills Road, CB2 0QQ, Cambridge, Cambridge University Hospitals, United Kingdom; Department of Medicine, University of Cambridge, CB2 0QQ Cambridge, United Kingdom. Electronic address:

Refractory lupus nephritis indicates an inadequate response to lupus nephritis therapy. It implies persisting or worsening disease activity despite therapy, but the definition is complicated by the parameters of response, proteinuria and renal function, that do not discriminate clearly between activity and irreversible damage. Understanding the causes of refractory disease and developing treatment strategies is important because these patients are more likely to develop poor outcomes, especially end stage renal disease. Read More

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http://dx.doi.org/10.1016/j.autrev.2019.03.004DOI Listing
March 2019
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Aberrant expression of serum circANRIL and hsa_circ_0123996 in children with Kawasaki disease.

J Clin Lab Anal 2019 Mar 6:e22874. Epub 2019 Mar 6.

Diabetes Research Center, School of Medicine, Ningbo University, Ningbo, China.

Background: Kawasaki disease is a childhood systemic vasculitis that causes coronary artery abnormalities. The etiology remains unknown and there are no specific diagnostic tests. Circular non-coding RNAs are a special class of endogenous RNAs that display some characteristics of an ideal biomarker. Read More

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http://dx.doi.org/10.1002/jcla.22874DOI Listing
March 2019
3 Reads

The Kawasaki Disease Comparative Effectiveness (KIDCARE) trial: A phase III, randomized trial of second intravenous immunoglobulin versus infliximab for resistant Kawasaki disease.

Contemp Clin Trials 2019 Mar 3;79:98-103. Epub 2019 Mar 3.

Harbor-UCLA Medical Center, 1124 W. Carson St., Torrance, CA, 90509, United States.

Background: Although intravenous immunoglobulin (IVIG) is effective therapy for Kawasaki disease (KD), the most common cause of acquired heart disease in children, 10-20% of patients are IVIG-resistant and require additional therapy. This group has an increased risk of coronary artery aneurysms (CAA) and there has been no adequately powered, randomized clinical trial in a multi-ethnic population to determine the optimal therapy for IVIG-resistant patients.

Objectives: The primary outcome is duration of fever in IVIG-resistant patients randomized to treatment with either infliximab or a second IVIG infusion. Read More

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http://dx.doi.org/10.1016/j.cct.2019.02.008DOI Listing
March 2019
3 Reads

Correction to: Effects of the timing of administration of IgM- and IgA-enriched intravenous polyclonal immunoglobulins on the outcome of septic shock patients.

Ann Intensive Care 2019 Mar 5;9(1):33. Epub 2019 Mar 5.

Department of Anesthesia, Resuscitation and Pain Therapy, Cattinara Hospital, University of Trieste, Strada di Fiume 447, 34149, Trieste, Italy.

Following publication of the original article [1], we have been notified that the tagging of the author name was done incorrectly in the XML version of the paper. The correct given name is Michele Claudio, and the family name is Vassallo. Read More

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http://dx.doi.org/10.1186/s13613-019-0504-0DOI Listing
March 2019
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Postponing early intrauterine transfusion with intravenous immunoglobulin treatment: the PETIT study on severe hemolytic disease of the fetus and newborn.

Am J Obstet Gynecol 2019 Mar 25;220(3):288. Epub 2018 Oct 25.

Centre National de Référence en Hémobiologie Périnatale Clinique, Service de Médecine Foetale, Hôpital Armand Trousseau, Assistance Publique-Hôpitaux de Paris, Paris, France; Médecine Sorbonne Université, 26 Avenue Arnold Netter, 75012 Paris, France. Electronic address:

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http://dx.doi.org/10.1016/j.ajog.2018.10.025DOI Listing

Pegunigalsidase alfa, a novel PEGylated enzyme replacement therapy for Fabry disease, provides sustained plasma concentrations and favorable pharmacodynamics: a 1-year Phase 1/2 clinical trial.

J Inherit Metab Dis 2019 Mar 4. Epub 2019 Mar 4.

LSDU, Institute of Immunity and Transplantation, Royal Free London NHS Foundation Trust, London, UK.

Background: Pegunigalsidase alfa, a novel PEGylated, covalently crosslinked form of α-galactosidase A developed as enzyme replacement therapy (ERT) for Fabry disease (FD), was designed to increase plasma half-life and reduce immunogenicity, thereby enhancing efficacy compared with available products.

Methods: Symptomatic adults with FD participated in this open-label, 3-month dose-ranging study, followed by a 9-month extension. Three cohorts were enrolled in a stepwise manner, each receiving increased doses of pegunigalsidase alfa: 0. Read More

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http://dx.doi.org/10.1002/jimd.12080DOI Listing
March 2019
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Diagnosis and management of typical, newly diagnosed primary immune thrombocytopenia (ITP) of childhood.

Paediatr Child Health 2019 Feb 15;24(1):54-55. Epub 2019 Feb 15.

Canadian Paediatric Society, Acute Care Committee, Ottawa, Ontario.

This practice point applies to children aged 90 days through 17 years who have typical, newly diagnosed primary immune thrombocytopenia (ITP). Current recommendations on management and information from recent studies are summarized with the goal of decreasing variable practice among providers and improving patient-centred care. Options for initially managing young patients with ITP who experience bruising, petechiae, or occasional mild epistaxis not interfering with daily living include observation without pharmacotherapy as a first-line option. Read More

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http://dx.doi.org/10.1093/pch/pxy197DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6376287PMC
February 2019
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Clinical time course of pediatric acute disseminated encephalomyelitis.

Brain Dev 2019 Mar 1. Epub 2019 Mar 1.

Department of Pediatrics, Kobe University Graduate School of Medicine, Kobe, Japan.

The detailed clinical time course in acute disseminated encephalomyelitis (ADEM) from initial symptoms, through exacerbation, to remission has not been widely reported. Hence, this study aimed to investigate the clinical time course of pediatric ADEM. This was a multicenter retrospective study based on registry data from medical chart reviews. Read More

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http://dx.doi.org/10.1016/j.braindev.2019.02.011DOI Listing
March 2019
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1.542 Impact Factor

Mechanism-Based Precision Therapy for the Treatment of Primary Immunodeficiency and Primary Immunodysregulatory Diseases.

J Allergy Clin Immunol Pract 2019 Mar;7(3):761-773

Department of Pediatrics, Immunology Allergy Rheumatology and Retrovirology, Baylor College of Medicine, Texas Children's Hospital Center for Human Immunobiology, Houston, Texas. Electronic address:

Advances in understanding the mechanism, immunobiology, and pathophysiology of primary immunodeficiency diseases have created opportunities for the use of precision medicines for the treatment of disease-related manifestations. Modulation of the immune system to treat autoimmunity began with the use of intravenous immunoglobulin, improved with the development of monoclonal antibodies, and has now become standard in certain diseases with mechanistic-based targets that alter the molecular mechanism of disease. In this article, we review targeted therapies for disorders of hyperinflammation, primary immunodysregulatory diseases, and primary immunodeficiencies. Read More

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http://dx.doi.org/10.1016/j.jaip.2018.12.017DOI Listing
March 2019
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Drug reaction with eosinophilia and systemic symptoms (DRESS) in children.

Acta Biomed 2019 Jan 29;90(3-S):66-79. Epub 2019 Jan 29.

Allergy Unit, Department of Pediatric Medicine, Anna Meyer Children's University Hospital, Florence, Italy.

Drug Reaction with Eosinophilia and Systemic Symptoms (DRESS) is a severe reaction to drugs. Incidence of DRESS in children is not well known and the mortality rate seems to be lower than 10%. Anticonvulsants are the main drugs involved both in adults and in children. Read More

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http://dx.doi.org/10.23750/abm.v90i3-S.8167DOI Listing
January 2019
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Clinical features, outcomes and treatment in children with drug induced Stevens-Johnson syndrome and toxic epidermal necrolysis.

Acta Biomed 2019 Jan 29;90(3-S):52-60. Epub 2019 Jan 29.

Department of Pediatrics, Senigallia Hospital, Senigallia, Italy.

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN), which can be considered a late-onset allergic reaction, can cause serious long-term sequelae. SJS/TEN are considered a spectrum of life-threatening adverse drug reactions. They have the same clinical manifestations and the only difference is in the extent of epidermal detachment. Read More

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http://dx.doi.org/10.23750/abm.v90i3-S.8165DOI Listing
January 2019
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The establishment of in-house neurology driven therapeutic plasma exchange infrastructure in a resource-limited public hospital in Malaysia: Adopting and integrating evidenced-based health care technology through time.

J Clin Apher 2019 Mar 4. Epub 2019 Mar 4.

Department of Neurology, Kuala Lumpur Hospital, Kuala Lumpur, Malaysia.

There has been an increase in the use of therapeutic plasma exchange (TPE) in immune-mediated neurological disorders in recent years. However, accessibility and availability of TPE remains low and costly, especially for a country with limited healthcare funding like Malaysia. With expanding clinical indications in neurological disorders, and increasingly expensive conventional immunomodulatory treatment such as intravenous immunoglobulin and monoclonal antibodies, TPE remains an effective part of first or second-line treatment. Read More

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http://doi.wiley.com/10.1002/jca.21696
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http://dx.doi.org/10.1002/jca.21696DOI Listing
March 2019
5 Reads