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
Activated PI3K delta syndrome (APDS) belongs to the heterogeneous group of primary immunodeficiency disorders (PIDs). Progress in next-generation sequencing (NGS) enabled identification of gain-of-function mut...
Serum immunoglobulin G (IgG) concentrations are integral to the workup of immune deficiencies and IgG4-related disease (IgG4-RD). Demographic differences in IgG concentrations are poorly described but could influence test interpretation, contribute to racial disparities in primary immunodeficiency diagnosis, and explain demographic differences in IgG concentrations in IgG4-RD.
Abstract BACKGROUND: Serum immunoglobulin G (IgG) concentrations are integral to the workup of immune deficiencies and IgG4-related disease (IgG4-RD). Demographic differences in IgG concentrations are poorly described but could influence test interpretation, contribute to racial disparities in primary immunodeficiency diagnosis, and explain demographic differences in IgG concentrations in IgG4-RD. OBJECTIVE: To assess differences in IgG and IgG subclass concentrations according to sex and race. METHODS: We identified patients with IgG and IgG subclass concentrations measured in a large healthcare system....
AbstractPurpose of ReviewChronic rhinosinusitis and other sinopulmonary infections can be a hallmark of impaired immunity in children. Rhinosinusitis in the setting of immunodeficiency can often be chronic and refractory, and involve opportunistic pathogens. We review the evaluation and management of pediatric chronic rhinosinusitis in the context of primary immunodeficiency diseases.Recent FindingsImmunodeficiencies found to be associated with chronic rhinosinusitis include common variable immune deficiency, X-linked agammaglobulinemia, antibody deficiencies, hyper-IgM syndrome, hyper-IgE syndrome, Wiskott –Aldrich ...
Conclusion: The awareness of the association between several primary immunodeficiencies and cancer is crucial to provide the best care for these patients.What is Known:•Patients with primary immunodeficiency have an increased risk of malignancy. The type of malignancy is highly dependent on the specific primary immunodeficiency disorder.What is New:•Survival in patients with primary immunodeficiency disorders has been improving, and conversely also their lifetime risk of malignancy.•International collaboration and multinational registries are needed to improve our knowledge and therapeutic strategies.
Pediatric Allergy, Immunology, and Pulmonology, Ahead of Print.
CONCLUSIONS: The initial application of FCM, particularly with known protein targets on immune cells, would facilitate the timely diagnosis of PIDs and thus would support clinical decisions and improve the clinical outcome. PMID: 32009323 [PubMed]
Immune Globulin Subcutaneous (Human), 16.5% solution (IGSC 16.5%) is a new subcutaneous immunoglobulin for the treatment of primary immunodeficiencies (PID). Available from Octapharma as Cutaquig ® since May 2019, we report the first real-world data regarding use of IGSC 16.5%.
Hospital admissions place a financial burden onto healthcare and the quality of life for patients and families. We aimed to assess admission rates and diagnoses, as well as length of stay in patients with Primary Immunodeficiency Disease (PIDD).
The Hizentra Label Optimization (HILO) study (NCT03033745) assessed safety and tolerability of increasing infusion rates/volumes of subcutaneous IgPro20 (Hizentra ®) in PID patients.