title: IgA glomerulonephritis in Wiskott-Aldrich syndrome.
abstract: Renal morphology was evaluated in 2 siblings with Wiskott-Aldrich syndrome (WAS) aged 12 and 4 years. They gave a typical history of recurrent episodes of respiratory infection and presented with microhematuria of glomerular origin and proteinuria. The study disclosed a membranoproliferative glomerulonephritis with IgA mesangial deposition in the elder child, while immunofluorescence was negative in the younger. The data indicate that (1) a specific nephropathy does not exist in WAS and (2) the IgA nephropathy is the result of recurrent infections and of related formation of IgA immune complexes scarcely removed by a deficient reticuloendothelial system. This view is consistent with presenting features in WAS (microhematuria, episodes of macrohematuria, proteinuria, Henoch-Schönlein syndrome) and with the fact that it takes years to develop as indicated by the negativity of immunofluorescence in the younger patient.
authors.affiliation: Department of Pediatrics, University of Naples, Italy.
authors.forename: N G
authors.initials: NG
authors.lastname: DeSanto
authors.forename: A
authors.initials: A
authors.lastname: Sessa
authors.forename: G
authors.initials: G
authors.lastname: Capodicasa
authors.forename: M
authors.initials: M
authors.lastname: Meroni
authors.forename: G
authors.initials: G
authors.lastname: Capasso
authors.forename: L
authors.initials: L
authors.lastname: Esposito
authors.forename: M
authors.initials: M
authors.lastname: Ferrara
authors.forename: L
authors.initials: L
authors.lastname: Torri Tarelli
authors.forename: S
authors.initials: S
authors.lastname: Annunziata
authors.forename: C
authors.initials: C
authors.lastname: Giordano
mesh_terms: D002648:Child; D002675:Child, Preschool; D005922:Glomerulonephritis, IGA / Q000150:complications* / Q000473:pathology; D006801:Humans; D008297:Male; D014923:Wiskott-Aldrich Syndrome / Q000150:complications* / Q000235:genetics / Q000473:pathology