Inborn errors of immunity (IEIs) are a group of conditions whereby parts of the immune
system are missing or dysfunctional. Once thought to primarily be a pediatric disorder, it is …

Most primary immunodeficiency diseases, and select secondary immunodeficiency
diseases, are treated with immunoglobulin (IG) therapy, administered intravenously or …

Inborn errors of immunity (IEIs) or primary immunodeficiency diseases, are disorders caused
by genetic defects affecting immune function. Clinically, IEI presents mainly as recurrent or …

Abstract Human Inborn Errors of Immunity (IEIs) encompass a clinically and genetically
heterogeneous group of disorders, ranging from mild cases to severe, life-threatening types …

Transcription factors are an extremely important group of proteins that are responsible for
the process of selective activation or deactivation of other cellular proteins, usually at the last …

In 2019, the novel coronavirus, SARS-CoV-2, caused a worldwide pandemic, affecting more
than 630 million individuals and causing 6.5 million deaths. In the general population …

Background and Objectives Reports on skin manifestations in inborn errors of immunity (IEI)
are based on retrospective analysis, small series, or isolated case reports. The present …

Introduction Primary immunodeficiency diseases (PIDs) are chronic diseases that affect the
various aspects of a patient's life. However, the impact of living with PIDs is poorly described …

Background Patients with antibody deficiency may experience exceptionally long diagnostic
delays, increasing the risk of life-threatening infections, end-organ damage, mortality, and …

We conducted a nationwide survey of inborn errors of immunity (IEI) in Japan for the second
time in 10 years, focusing on protective measures for IEI patients against infectious …