Stiehm's Immune Deficiencies

Primary Immune Deficiencies – Principles of Care

Primary immune deficiencies (PIDs) are a growing group of over 230 different disorders caused by ineffective, absent or an increasing number of gain of function mutations in immune components, mainly cells and proteins. Once recognized, these rare disorders are treatable and in some cases curable. Otherwise untreated PIDs are often chronic, serious, or even fatal. The diagnosis of PIDs can be d...

COVID-19: The potential role of nutritional deficiencies, global climatic changes, and immune system dysfunction

Cord blood stem cell transplantation in primary immune deficiencies.

PURPOSE OF REVIEW Umbilical cord haematopoietic stem cell transplantation for primary immunodeficiencies is examined with other developments in treatment. Cord blood biology is reviewed, and advantages and disadvantages of umbilical cord blood stem cell transplantation for primary immunodeficiencies discussed. Clinical outcome data and future developments are reviewed. RECENT FINDINGS Cord bl...

B cell-specific deficiencies in mTOR limit humoral immune responses.

Generation of high-affinity Abs in response to Ags/infectious agents is essential for developing long-lasting immune responses. B cell maturation and Ab responses to Ag stimulation require Ig somatic hypermutation (SHM) and class-switch recombination (CSR) for high-affinity responses. Upon immunization with either the model Ag 4-hydroxy-3-nitrophenylacetyl hapten (NP) conjugated to chicken γ gl...

Advances in neonatal screening for primary immune deficiencies

The congenital disorders of immune competence are known as primary immunodeficiencies (PID) and are mainly characterized by a pathological susceptibility to infection. These infections are mostly of time repetitive and drug resistant in nature. The number of infected infants has reached over 200 and is on the increase. Additionally, clinical severity of the disease has been confirmed to be exte...