Common Immunodeficiency Research Articles

Defective Candida Opsonin and Procidin Function in Patients with Recurrent Pyogenic Infection: A Common Immunodeficiency

Polymorphonuclear cell function tests were performed in 340 patients with recurrent unexplained infections. Serum-dependent defects in phagocytosis and intracellular killing of Candida albicans (Candida opsonin and procidin defects) were specifically associated with susceptibility to recurrent pyogenic infections, Candida opsonin defects occurring in 12.7% and Candida procidin defects in 20.8% of patients compared with 3.7% of healthy controls. Saccharomyces cerevisiae opsonin deficiencies were also found frequently in patients with recurrent infections, being present in 18.2% compared with 7.4% controls, although not specifically in those with pyogenic infection.

Different amino acids at position 57 of the HLA-DQβ chain associated with susceptibility and resistance to IgA deficiency

The human leukocyte antigens (HLA) are implicated in the genetic susceptibility to a large number of diseases. Some of the diseases associated with HLA class II are related to specific amino acids or epitopes of the domain of the HLA class II molecule that is distal to the membrane. In man, selective immunoglobulin A deficiency is the most common immunodeficiency, frequently resulting in recurrent sino-pulmonary infections and gastro-intestinal disorders. Associations have been described with HLA class I, and to a lesser extent with different class II alleles, which might indicate that they share some common feature. Here we study 95 IgA-D patients and find positive associations with three DR-DQ haplotypes and a strong negative association with a fourth haplotype. Comparison of the sequences of the polymorphic amino-terminal domain of the DQ beta chain showed that the three 'susceptibility' haplotypes all had a neutral alanine or valine at position 57. The 'protective' allele had the negatively charged aspartic acid at this position (Asp57). Codon 57 of the HLA-DQ beta chain has been implicated in the susceptibility to insulin-dependent diabetes mellitus. Our data suggest that the same amino acid position could possibly also influence susceptibility and resistance to selective immunoglobulin A deficiency.

Serum IgG subclass levels in patients with primary immunodeficiency syndromes or abnormal susceptibility to infections

Serum IgG subclass levels were measured using an indirect competitive immunoenzymatic assay with monoclonal antibodies in 221 patients affected with definite immunodeficiency (ID) syndromes and 229 patients presenting with infection patterns suggestive of ID, but with normal immunoglobulin class levels and no clear evidence of ID. In common variable ID and IgG-IgA deficiency with normal or high IgM, subclass imbalance (mostly IgG1-IgG3 or IgG2-IgG4 deficiency) was the rule, with a higher incidence of severe infections in IgG2-IgG4 defects. One-fifth of patients with IgA deficiency, especially those with autoimmune cytopenia, had subclass deficiencies with no significant correlation with the occurrence of infections. Subclass (mostly IgG2-IgG4) deficiencies were also observed in severe combined ID, defective expression of HLA class II antigens, chronic mucocutaneous candidiasis, and IgM deficiency. Subclass levels were normal in all but one (who was IgG3 deficient) patient with the Wiskott-Aldrich syndrome and in the Buckley's syndrome, except for an unusual patient who presented with low IgG and IgA levels. Subclass (mainly IgG2) deficiency occurred in 24% of infected patients without known ID.

Selective IgA Deficiency and the Gastrointestinal Tract

IgA deficiency is a common immunodeficiency disorder in which the absence of a single immunoglobulin can be related to a number of disease states. Since secretory IgA has a major role in preventing antigens from mucosal invasion and systemic absorption, it is not surprising that diseases of the mucosal surfaces are the hallmarks of IgA deficiency. Although respiratory tract infections are common, this article focuses on GI diseases and immunologic perturbations in this area. There is also a prevalence of autoimmune diseases in IgA deficiency; the connection between these illnesses and a lack of IgA is not understood, but it has been tempting to relate the development of autoantibodies to the excess mucosal permeability that is a cardinal feature of this deficiency. Many individuals with IgA deficiency are healthy; this appears to be attributable to a range of potential immunologic mechanisms that can partially or wholly compensate for the deficiency.

A common congenital immunodeficiency predisposing to infection and atopy in infancy.

Twenty six infants with a congenital immunodeficiency, characterised by failure of their sera to opsonise heat killed bakers' yeast for phagocytosis by normal polymorphonuclear leucocytes, were studied during infancy to determine the frequency of infection and development of atopy. They were compared with controls, matched prospectively for birth date, sex, parental smoking, and atopy and in whom feeding patterns were similar. In 18 of 26 infants the serum defect persisted at age one year. The incidence of infection and atopy, was appreciably greater in the study group than in controls. The 8 children in whom the defect was transient had a similar incidence of infection but a higher incidence of atopy than controls. Eight of 26 mothers and four of 9 fathers tested also had the serum defect, suggesting a strong genetic component. We support the hypothesis that immunodeficiency predisposes to infection and atopy, and that transient immunodeficiency predisposes to atopy.

Zinc deficiency: A common immunodeficiency state

Zinc deficiency: A common immunodeficiency state

Allergy and Immunology: An Annotated Bibliography of Recent Literature

Allergy and Immunology: An Annotated Bibliography of Recent Literature