Human inborn errors of immunity (IEI) are a group of 485 distinct genetic disorders affecting
children and adults. Signs and symptoms of IEI are heterogeneous, and accurate diagnosis …

Bronchiectasis has historically been considered to be irreversible dilatation of the airways,
but with modern imaging techniques it has been proposed that 'irreversible'be dropped from …

Background Common variable immunodeficiency disorders (CVID) are a group of rare
innate disorders characterized by specific antibody deficiency and increased rates of …

Background Insight into the pathogenesis of very early onset-inflammatory bowel disease
(VEO-IBD) has expanded through the identification of causative monogenic defects detected …

Objectives: Identify the mechanism of action of intravenous immunoglobulin. Describe the
potential adverse effects of intravenous immunoglobulin. Review the appropriate monitoring …

Objectives Ten warning signs of primary immunodeficiency (PID) were suggested by the
Jeffrey Modell Foundation (JMF), to increase physician awareness of PID. These warning …

Background Recurrent respiratory tract infections (rRTIs) frequently affect young children
and are associated with antibody deficiencies. We investigated the prevalence of and …

Objective Different sets of warning signs can be used if primary immunodeficiency (PID) is
suspected: those of the Jeffrey Modell Foundation (JMF), the German Patients' Organisation …

Background and Objectives: Inborn errors of immunity (IEI) refer to genetically determined
disorders presenting with recurrent infections, autoimmunity, allergies, and malignancies. IEI …

Background Primary immunodeficiency diseases (PIDDs) are rare yet life-threatening
chronic conditions in children. The prevalence and outcomes of PIDDs in the pediatric …