An increasing number of critically ill patients are immunocompromised. Acute hypoxemic
respiratory failure (ARF), chiefly due to pulmonary infection, is the leading reason for ICU …

Introduction During the last 4 decades, registration of patients with primary
immunodeficiencies (PID) has played an essential role in different aspects of these diseases …

CT scanning can also aid in identifying an aetiology of bronchiectasis eg Allergic
bronchopulmonary aspergillosis (ABPA), Non-tuberculous mycobacteria (NTM), primary …

The International Collaboration in Asthma, Allergy and Immunology initiated an international
coalition among the American Academy of Allergy, Asthma & Immunology; the European …

Background Activated phosphoinositide 3-kinase δ syndrome (APDS) is a recently
described combined immunodeficiency resulting from gain-of-function mutations in PIK3CD …

The protein cytotoxic T lymphocyte antigen-4 (CTLA-4) is an essential negative regulator of
immune responses, and its loss causes fatal autoimmunity in mice. We studied a large family …

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

Background Primary immunodeficiencies (PIDs) are inherited diseases associated with a
considerable increase in susceptibility to infections. It is known that PIDs can also …

Non-infectious complications in common variable immunodeficiency (CVID) have emerged
as a major clinical challenge. Detailed clinical spectrum, organ-specific pathologies and …

Background Primary immunodeficiency diseases (PIDDs) are clinically and genetically
heterogeneous disorders thus far associated with mutations in more than 300 genes. The …