Access to circulation by permanent arteriovenous fistula in regular dialysis treatment.

Abstract

This isolated and severe yM deficiency can be classified as a dysgammaglobulinaemia. It is distinct from the Giedion-Scheidegger variety (type 1), where yA is also very low and where there is a continual history of all kinds of bacterial infec- tions from birth. Case 4 initially showed a low yA and yM, but this may have been due to delay in immunological matura- tion, for both are now developing normally. Cases I and 2 are also distinct from the Wiskott-Aldrich syndrome (type VA), a sex-linked recessive condition usually fatal to affected males before 10 years of age, where though -yM deficiency appears it is usually associated with a marked increase of yA. Clinically there is a long history of eczema, thrombocytopenia, and recur- rent infections from soon after birth. Cases 1 and 2 have therefore been classified as type VB by Hobbs et al. (1967a). Furthermore, it is realized that isolated deficiency of yM can be found in apparently healthy subjects (type VO) and can be associated with defects of other classes of immunoglobulins in both sexes of given families (Burtin et al., 1964). Thus the finding of yA deficiency in four other relatives of Cases 1 and 2 adds support to a genetic basis for their fatal abnormality.