Inborn errors of immunity (IEI), formerly known as primary immunodeficiency disorders
(PIDs), are inherited disorders caused by damaging germline variants in single genes …

Inborn errors of immunity (IEI) comprise a group of about 490 genetic disorders that lead to
aberrant functioning or the development of distinct immune system components. So far, a …

Primary immunodeficiency (PID) is a genetic disorder with a defect of one of the important
components of our immune system. Classical PID has been recognized as a disorder with …

Human inborn errors of immunity (IEI) comprise a group of diseases resulting from molecular
variants that compromise innate and adaptive immunity. Clinical features of IEI patients are …

Background Immune dysregulation is as important as susceptibility to infection in defining
primary immunodeficiencies (PIDs). Because of the variability and nonspecificity of the …

We report the updated classification of Inborn Errors of Immunity/Primary
Immunodeficiencies, compiled by the International Union of Immunological Societies Expert …

Abstract The International Union of Immunological Societies (IUIS) expert committee (EC) on
Inborn Errors of Immunity (IEI) reports here the 2022 updated phenotypic classification …

Primary immunodeficiencies and immune dysregulatory disorders (PIDDs; now referred to
as inborn errors in immunity) are rare disorders with a prevalence of 41. 4 or 50.5 per …

Patients with inborn errors of immunity (IEI) present with a heterogeneous clinical and
immunological phenotype, therefore a correct molecular diagnosis is crucial for the …

Primary immunodeficiencies (PIDs) are inherited disorders classically characterized by
increased susceptibility to infections. Nevertheless, in the last two decades, genomic …