Human immunoglobulin G (IgG) can be divided into four subclasses that are selectively expressed. For instance, carbohydrate antigens preferentially elicit IgG2 antibodies, whereas protein antigens usually elicit IgG1 and IgG3. Elucidating the biological basis of the selective expression of these IgG subclasses is important to our understanding immunodeficiencies and B lymphocyte development. To...

Infection by unusual microorganisms can be one of the clinical manifestations of primary immunodeficiency (PID). We report on a four-month-old child with pneumonia caused by the fungus Acremonium kiliense as the first clinical manifestation of chronic granulomatous disease. We emphasize the importance of an active search for unusual organisms in immunodeficient patients, and a precise diagnosis...

OBJECTIVE The objective of this study is to evaluate the economic benefits of immunoglobulin replacement therapy achieved subcutaneously (subcutaneous immunoglobulin, SCIG) by the rapid push method compared to intravenous infusion therapy (intravenous immunoglobulin, IVIG) in primary immune deficiency (PID) patients from the healthcare system perspective in the context of the adult SCIG home in...

Paediatricians have long been deeply concerned with problems of infection. The sharp decrease in childhood mortality in recent decades in highly developed countries is mainly due to progress in preventing and treating infection: for example, in Switzerland in 1973 there were about 88 000 births and only 16 deaths due to infection in children aged between 2 and 4 years in contrast to 76 killed b...

PURPOSE OF REVIEW The purpose of this review is to summarize pathogenic mechanisms and clinical implications of the most illustrative genetic entities of congenital neutropenia syndromes. RECENT FINDINGS Congenital neutropenia comprise monogenetic entities with or without additional immunologic and extrahaematopoietic syndromatic features. Continuous careful explorations of known entities suc...

Immune Deficiency Foundation, the national nonprofit organization devoted to research and education for the primary immune deficiency diseases, publishes other materials for physicians and healthcare professionals (eg. Physician’s Primer and Guide for Nurses) and for patients (eg.Patient and Family Handbook, Our Immune System, and Newsletter). For Information about our programs, patient groups ...

Among the primary antibody deficiency syndromes, severe selective IgM deficiency (also previously known as type V dysgammaglobulinaemia) is rare, and the majority of previous reports have indicated a fatal outcome. Three adult patients who were found to have a persistently low serum IgM are described. This deficiency was not obviously related to their presenting illness; in two of the patients,...

Primary immunodeficiency results in recurrent infections, organ dysfunction, and autoimmunity. We studied 237 patients referred for suspicion of immunodeficiency, using a scoring system based on clinical information. The 113 patients with immunodeficiency had higher scores and more episodes of chronic illnesses and were more likely to have neutropenia, lymphopenia, or splenomegaly.

A case of fatal cryptosporidiosis in a child with primary immunoglobulin deficiency is described. This is always a serious complication in immunodeficient patients because there is no known effective therapy.

A 57-year-old woman with common variable immune deficiency and liver failure of unknown etiology presented with recurrent fevers over a 5-month period. She was found to have Helicobacter canis bacteremia. Immunocompromised hosts with exposure to cats or dogs may be at risk for infection with this organism, which may be challenging to diagnose.