Performance status and deaths among children registered in Kuwait National Primary ImmunoDeficiency Disorders Registry.

Children with primary immunodeficiency disorders (PIDD) have an increased risk of suffering from physical, social, and psychological problems. The aim of this study was to evaluate the performance status and mortality of children with PIDD in Kuwait and to determine the variables and co-morbidities that may affect their performance and risk of death. The data for the children were obtained from Kuwait National Primary Immunodeficiency Disorders Registry describes the patients' characteristics, comorbidities and their treatment regimens.

Expansion of immunoglobulin-secreting cells and defects in B cell tolerance in Rag-dependent immunodeficiency.

The contribution of B cells to the pathology of Omenn syndrome and leaky severe combined immunodeficiency (SCID) has not been previously investigated. We have studied a mut/mut mouse model of leaky SCID with a homozygous Rag1 S723C mutation that impairs, but does not abrogate, V(D)J recombination activity. In spite of a severe block at the pro-B cell stage and profound B cell lymphopenia, significant serum levels of immunoglobulin (Ig) G, IgM, IgA, and IgE and a high proportion of Ig-secreting cells were detected in mut/mut mice.