Background Inborn errors of immunity (IEI) are rare diseases, which makes diagnosis a
challenge. A better description of the initial presenting manifestations should improve …

Complement not only plays a key role in host microbial defense but also modulates the
adaptive immune response through modification of T-and B-cell reactivity. Moreover, a …

Background Inborn errors of immunity (IEIs) are a heterogeneous group of genetic defects of
immunity, which cause high rates of morbidity and mortality mainly among children due to …

We recommend that clinicians tailor their approach to the individual based on their
underlying immune deficit and maintain a high index of suspicion and low threshold for …

A global gold standard framework for primary immunodeficiency (PID) care, structured
around six principles, was published in 2014. To measure the implementation status of …

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 …

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

Objective: This minireview gathers the scientific foundations of the literature on genetic
errors in the development of the humoral immune system to help pediatricians suspect these …

Primary immunodeficiencies or inborn errors of immunity (IEIs), are a group of more than
400 monogenic diseases that present with recurrent infections, immune dysregulation …

Background The inborn errors of immunity (IEIs) are a group of heterogeneous disorders
mainly characterized by severe and recurrent infections besides other complications …