Wiscott-Aldrich syndrome

The initial Taj RIA that employs rabbit hetero mtiserum has been used to evaluate serum levels in children with primary immunodeficiency disorders (D. Wara et al., 1982). With this assay, 11 of 13 children with combined immunodeficiency, 5 to 7 children with ataxia-telangiectasia and all 3 patients with Wiscott-Aldrich syndrome exhibited serum Taj levels below the mean of the normal age-matched donors. Such findings were similar to those reported using the Bach-Dardenne bioassay (Iwata et al., 1981). A major inconsistency, however, was in children with DiGeorge s syndrome. Whereas all such children manifested low serum thymic hormone bioactivity, only 3 of 6 exhibited levels below the mean of normals using the Taj RIA and serum levels were actually quite high in 2. 

Thymus transplantation has also been employed experimentally in a variety of conditions including chronic mucocutaneous candidiasis, ataxia-telangiectsia, and Wiscott-Aldrich syndrome, but in these cases results have generally been disappointing (reviewed in Pahwa et al., 1979 Skotnicki et al., 1984). Thus, the only immunodeficiency state that has clearly demonstrated a lasting improvement of T cell immunity following thymus transplantation is DiGeorge s syndrome. 

WISCOTT-ALDRICH SYNDROME Inherited syndrome characterized by chronic skin disease and immune system defects. 

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