FDA OKs Immunoglobulin Xembify for Primary Immunodeficiencies FDA OKs Immunoglobulin Xembify for Primary Immunodeficiencies

Xembify is a 20% immune globulin solution for subcutaneous injection indicated to treat primary immunodeficiencies in patients aged 2 years and older.FDA Approvals
Source: Medscape Allergy Headlines - Category: Allergy & Immunology Tags: Allergy & Clinical Immunology News Alert Source Type: news

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Publication date: Available online 8 October 2020Source: The Journal of Allergy and Clinical Immunology: In PracticeAuthor(s): Hsi-en Ho, Sheryl Mathew, Michael J. Peluso, Charlotte Cunningham-Rundles
Source: The Journal of Allergy and Clinical Immunology: In Practice - Category: Allergy & Immunology Source Type: research
Authors: Zaremehrjardi F, Baniadam L, Seif F, Arshi S, Bemanian MH, Shokri S, Rezaeifar A, Fallahpour M, Nabavi M Abstract Increased susceptibility to autoimmunity, malignancy, and allergy in addition to recurrent infections are the main characteristics suggesting for the primary immunodeficiency diseases (PID). CTLA-4 is predominantly expressed on activated and regulatory T-cells, which can bind to CD80/CD86 molecules on antigen-presenting cells as a negative regulator. Here, we describe a 24-year-old male born from consanguineous parents with heterozygous CTLA-4 mutation who presented with multiple autoimmune dis...
Source: Iranian Journal of Immunology - Category: Allergy & Immunology Tags: Iran J Immunol Source Type: research
In 2015, heterozygous mutations in NFKB1, encoding the central component of the canonical NF- κB pathway, were identified in families with an autosomal-dominant form of common variable immunodeficiency (CVID), the most common primary immunodeficiency. Subsequently, NFKB1 defects were recognized to be associated with highly variable phenotypes, ranging from severe immune dysregulation involv ing almost any organ to nearly clinically unapparent disease. In this issue of the Journal, Lorenzini et al (p 901) describe the spectrum of this rare monogenetic condition in an international cohort comprising 231 individual...
Source: Journal of Allergy and Clinical Immunology - Category: Allergy & Immunology Authors: Tags: The Editors ’ Choice Source Type: research
Autoinflammatory diseases are monogenic and polygenic disorders due to dysregulation of the innate immune system. The inherited conditions have been clustered with primary immunodeficiencies in the latest practice parameters, however, these diseases have unique clinical presentations, genetics and available therapies. Given the presentation of fevers, rashes and mucosal symptoms observed in many of these syndromes, patients are likely to present to an allergist/immunologist. While there has been attention in the literature to diagnosis and treatment of rare, genetically-defined autoinflammatory disorders, physicians are ch...
Source: Journal of Allergy and Clinical Immunology - Category: Allergy & Immunology Authors: Source Type: research
Primary immunodeficiencies (PID) constitute a heterogeneous group of more than 350 monogenetic diseases. PID patients with antibody impairment require lifelong administration of immunoglobulin G replacement th...
Source: Allergy, Asthma and Clinical Immunology - Category: Allergy & Immunology Authors: Tags: Short report Source Type: research
Next-generation sequencing has become a first-line tool for the diagnosis of primary immunodeficiency. However, patient access remains limited because of restricted insurance coverage and a lack of guidelines addressing the use of targeted panels versus whole-exome sequencing (WES).
Source: Journal of Allergy and Clinical Immunology - Category: Allergy & Immunology Authors: Tags: Brief report Source Type: research
NGS has become a first-line tool for diagnosis of PID. However, patient access remains limited due to restricted insurance coverage and a lack of guidelines addressing use of targeted panels vs WES.
Source: Journal of Allergy and Clinical Immunology - Category: Allergy & Immunology Authors: Source Type: research
Without an established transition program, a significant number of patients affected with Inborn Errors of Immunity (IEIs) may be lost to follow-up during transfer with consequently increased morbidity and mortality rate.
Source: Journal of Allergy and Clinical Immunology - Category: Allergy & Immunology Authors: Source Type: research
We present a case of a patient with a history of CVID and severely low IgG levels due to a lapse in immune globulin replacement, who died of complications related to COVID-19, despite receiving convalescent plasma and high-dose intravenous immune globulin (IVIG).
Source: Annals of Allergy, Asthma and Immunology - Category: Allergy & Immunology Authors: Tags: Letters Source Type: research
Common variable immunodeficiency (CVID) is a heterogeneous group of primary immunodeficiency syndromes characterized by hypogammaglobulinemia and impaired vaccine responses. Although immunodeficiency is described as a risk factor for coronavirus disease 2019 (COVID-19), limited data are available regarding CVID. Of note, 3 recent reports describe mostly positive outcomes in patients with CVID who were diagnosed as having COVID-19,1-3 with only 1 fatality.3 All these patients had adequate immunoglobulin G (IgG) levels at the time of COVID-19 diagnosis.
Source: Annals of Allergy, Asthma and Immunology - Category: Allergy & Immunology Authors: Tags: Letters Source Type: research
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