The Latin American Group for Primary Immunodeficiencies, formed in 1993, presently includes 12 countries. One goal was to study the frequency of primary immunodeficiencies in various regions of the American continent and to enhance knowledge about these diseases among primary-care physicians, as well as allergist-immunologists. Important for this purpose was the development of a registry of pri...

As of March 31, 1987, the U.S. Centers for Disease Control had reported 33,350 cases of acquired immune deficiency syndrome. Yet by that date, physicians had actually diagnosed 42,670 cases. The difference arises from significant delays in the reporting of AIDS cases to public health authorities. An estimated 70% of cases are reported two or more months after diagnosis; about 23% are reported s...

Primary immune deficiency disorders (PIDs) are a group of diseases associated with a genetic predisposition to recurrent infections, malignancy, autoimmunity and allergy. The molecular basis of many of these disorders has been identified in the last two decades. Most are inherited as single gene defects. Identifying the underlying genetic defect plays a critical role in patient management inclu...

Question: I understand that anti-IgA antibodies are quite rare in patients with IgA deficiency, but, when starting intravenous immunoglobulin (IVIG) in patients with common variable immune deficiency and with very low serum IgA levels, is the product with the lowest IgA concentration, the only choice or could one choose a product with a low IgA concentration (<25 mg/mL)? I know that subcutaneou...

Malnutrition, anaemia, and gut parasites are commonly interrelated. Using the Nippostrongylus brasiliensis-rat model, the effect of iron and protein deficiency on the efficacy of benzimidazole anthelmintics was studied. It was demonstrated that the anthelmintics mebendazole and fenbendazole were significantly less effective in eradicating parasites when animals were deficient in iron and protei...

Approximately 20 years ago, gene therapy was first introduced when Michael Blaese and colleagues applied ex vivo modified autologous T cells to children suffering from adenosine deaminase deficiency (ADA-SCID), a Mendelian genetic error that causes severe combined immunodeficiency syndrome (Blaese et al, 1995). The investigators used retroviral vectors; the most advanced gene delivery system at...

Methods SCIG and IVIG options for immunoglobulin replacement therapy in adult PID patients were compared in a costminimization model over three years of treatment. The model focused on direct medical costs for infusion supplies and personnel. A three-year budget impact model assessed the economic impact on the healthcare system of switching from IVIG to SCIG for PID patients of the BC Central T...

The clinico-epidemiologic characteristics of 54 children with HD in 0-6 years of age group were retrospectively analyzed. This group represented 27% of 200 HD cases observed in our center and was named as early type-I pattern HD. The association of EBV with HD was also shown by serologic and immunohistochemical methods (LMP1) in these very young Turkish patients. T-cell immune deficiency, cytok...

The nature of the association of adenosine deaminase deficiency and severe combined immunodeficiency disease is reviewed . The basis for the molecular heterogeneity exhibited by adenosine deaminase in human tissue and the mechanisms whereby a deficiency of this activity results in the extreme perturbation of the immune system as observed in severe combined immunodeficiency are critically discus...

BACKGROUND IgG replacement therapy (IgRT) in primary immunodeficiencies (PID) is a lifelong treatment which may be administered intravenously (IVIg) or subcutaneously (SCIg), at hospital or at home. The objective of the VISAGE study was to investigate if route and/or place for IgRT impact patients' satisfaction regarding IgRT and quality of life (QoL) in real-life conditions. METHODS The stud...