HENOCH-SCHONLEIN PURPURA WITH IMMUNOGLOBULIN-A NEPHROPATHY AND ABNORMALITIES OF IMMUNOGLOBULIN-A IN A WISKOTT-ALDRICH SYNDROME CARRIER

Abnormalities of immunoglobulin A1 (IgA1) glycosylation have been desc ribed in patients with IgA nephropathy (IgAN), whether primitive or se condary to Henoch-Schonlein purpura. The Wiskott-Aldrich syndrome, an X-linked recessive disorder, is associated with abnormalities of IgA, Renal involvement with mesangial IgA deposition identical to that foun d in IgAN has been reported during this affection, We report the case of a female carrier of the Wiskott-Aldrich syndrome presenting with He noch-Schonlein purpura and abnormalities of IgA glycosylation, as prev iously reported in patients with ISAN. The galactosylation abnormaliti es of IgA could be linked to the patient's status as carrier of the Wi skott-Aldrich syndrome and could contribute to the pathogenesis of IgA N. (C) 1997 by the National Kidney Foundation, Inc.